Vasospasm as a complication of subarachnoid hemorrhage usually occurs early, in the week after the hemorrhage. The progressive deterioration with alteration of the level of consciousness makes us suspect one of the complications that can occur in this condition: hydrocephalus. The diagnostic technique of choice in this case is neuroimaging with cranial CT.

A 55-year-old male who suffers a subarachnoid hemorrhage due to a ruptured middle cerebral artery aneurysm. He is treated surgically. After a good evolution in which he recovers the ability to walk, one month after suffering the hemorrhage, he presents a progressive deterioration, becoming cloudy and with difficulty to obey orders. Mark the correct statement:

She is in the moment of evolution in which vasospasm is more frequent.

This progressive deterioration is probably secondary to aneurysm rebleeding.

Subacute deterioration is most likely due to hydrocephalus.

The diagnostic technique of choice is cerebral angiography.

Intracranial hematomas following aneurysmal rupture: experience with 309 cases.

Three hundred and nine consecutive cases of intracranial hematomas due to aneurysmal rupture--representing 34% of the total number of patients with aneurysms observed in a 12-year period--were evaluated; of these, 211 were submitted to computed tomography scan. Hematomas were present on admission in 71% of patients and occurred at rebleeding in 29%. Ruptured middle cerebral artery aneurysms caused an intracranial hematoma more frequently than aneurysms in other locations. Ventricular hematomas were frequently observed--especially at rebleeding--in cases with anterior communicating artery aneurysms. Basal ganglia hematomas were detected in eight cases with internal carotid bifurcation aneurysms and in three with middle cerebral artery aneurysms. Subdural hematomas were observed in 32 cases, mainly due to ruptured middle-cerebral-artery and internal-carotid-artery aneurysms. As for clinical evolution, a rapid deterioration was observed in 39% of cases and a chronic course in 46%; a subacute deterioration was far less frequent. Delayed deterioration from vasospasm was observed in 8% of cases, and appeared to be related to the amount of subarachnoid bleeding associated with the hematoma. One hundred and forty-two patients were submitted to surgical treatment (evacuation of hematoma together with exclusion of aneurysm); deep coma, poor medical condition, stabilized neurological disability, or combinations of these factors accounted for the high number of patients not operated upon. Regardless of treatment, 24% of patients showed good results and 58% died. Presence of a large hematoma, ventricular hemorrhage, and shift of the ventricles represented significant risk factors, associated with a poor prognosis. A comparison between two groups of patients admitted within 3 days of hemorrhage--47 operated on early, and 149 with delayed treatment--showed that better results were achieved by early operations, especially for cases in Hunt's grades IV and V.

[Consequences of meningeal hemorrhage during the first days after its onset].

Results of subarachnoid hemorrhage (SAH) in the acute phase are represented by the direct threat of vasospasm. The first step still is to recognise SAH, so that all misleading clinical aspects of arterial aneurysm rupture do not misguide, or even fail to do the right diagnosis. If so, rebleeding still remains a real danger. Among biological patterns, hyponatremia is an important factor of vasospasm. Cardiovascular symptoms are represented by a sudden and transient arterial hypertension which can drive to a diagnostic error and electrocardiographic abnormalities, which are directly related with the degree of vasospasm; their evolution is completely regressive. Main intracranial consequences are early hydrocephalus, worsening of consciousness and progressive ventricular distension on CT scan and vasospasm, which occurs between the 4th and the 12th day, may be asymptomatic or symptomatic, responsive for delayed ischemia, followed by deterioration of consciousness and focal neurological signs. The main factors responsible for the vasospasm are a high amount of blood in basal cisterns on CT scan; an increase of substances released by the lysis of hemoglobin in CSF; hyponatremia, hypovolemia, and decrease in cerebral blood flow. Consequences of these disorders have to be well known in the medical treatment before and after operation.

[Management of subarachnoid hemorrhages without detectable aneurysm].

In this study, 21 patients with subarachnoid hemorrhage (SAH) but negative angiography were evaluated. Angiography was performed twice on each patient, that is, on admission and at 2 weeks following admission. All patients had severe headache of sudden onset, a characteristic manifestation of SAH. Clinical grades on admission (Hunt and Kosnik classification) were generally good: 17 patients were in grade I or II and 4 patients were in grade III. SAH was confirmed by the presence of subarachnoid clot on CT in all cases. Based on the distribution of SAH, CT findings were classified into two patterns, i.e., perimesencephalic and non-perimesencephalic patterns. Four patients showed the perimesencephalic pattern and the remaining 17 the non-perimesencephalic. The period of follow-up ranged from 20 days to 11 years 6 months, with a mean of 6 years 10 months. Except for three recent cases, the mean follow-up period in 8 years 9 months. Exploratory craniotomies probing for aneurysms have been performed in four patients, but no aneurysms have been found in any of these cases. Clinical deterioration associated with vasospasm was observed in one patient. A communicating hydrocephalus requiring a shunting procedure was observed in three patients showing the non-perimesencephalic type CT pattern. Rebleeding occurred in one patient who subsequently died of what may be a dissecting aneurysm of the vertebral artery. One patient who was able to return to full activity experienced symptoms attributable to SAH such as frequent headaches and increased fatigability. Complete recovery was observed in the remaining 19 patients. Two of them, however, later died due to myocardial infarction and aging, respectively. Given these generally positive outcomes, it should be possible to inform such patients of the benignity of their condition. Angiography may not demonstrate a ruptured aneurysm on initial examination in all cases of aneurysmal SAH. Serial angiography, however, can provide a definite diagnosis of the dissecting aneurysm. Therefore, repeat angiography, particularly, when possible, digital subtraction angiography, is necessary to rule out aneurysmal SAH. While small aneurysms or microaneurysms are often found through exploratory craniotomy, we do not agree with the opinion that surgery may be appropriate for certain patients with SAH but with negative angiography. The natural history concerning rebleeding in such cases, as well as morbidity and mortality associated with hemorrhage, remains to be defined. Furthermore, there are reservations regarding whether coagulation of these abnormalities with bipolar cautery constitutes definitive treatment.

Impact of coil embolization on overall management and outcome of patients with aneurysmal subarachnoid hemorrhage.

We report on the consequences of introducing coil embolization for the total management of and outcome in patients with subarachnoid hemorrhage (SAH). In southern Sweden, a prospective analysis of all patients with SAH of verified aneurysmal origin was conducted during the 3 years when coiling was gradually being introduced. The incidence of acute or chronic hydrocephalus, vasospasm, delayed ischemic deterioration, and outcome at 3 to 6 months after bleeding was analyzed. Coiling of aneurysms was used progressively more compared with clipping during the study period. The number of patients subjected to coiling was 26 (36%) of 73 patients during calendar year 1997, 43 (53%) of 81 patients in 1999, and 55 (74%) of 74 patients in 2001 (P < 0.0001). Gradually, more elderly patients were admitted during the study period. Virtually all aneurysms located in the posterior circulation were treated by coil embolization, even at the start of the study, whereas aneurysms at all other locations were progressively more likely to be treated similarly. The incidence of hydrocephalus in the acute (average for all 3 yr, 39%) or chronic (16%) phase, vasospasm as measured by Doppler sonography (33%), and delayed ischemic deterioration (29%), as well as outcome at 3 to 6 months (61% good recovery, 13% deceased), did not change significantly during the study. The main cause of unfavorable outcome was the severity of the SAH. The increasing use of coil embolization for ruptured aneurysms in the anterior circulation did not have any significant impact on the overall incidence of SAH-related complications or outcome. The main determinant for outcome after SAH is still the severity of the SAH.

Anterior cerebral artery syndrome

The crucial step in the evaluation of stroke patients is to obtain brain imaging to ascertain the type and characteristics of the stroke. In this regard, non-contrast CT of the head is the imaging modality of choice. Ischemic changes may classify as acute, subacute, and chronic, depending on the time in which they present after the onset of stroke. CT scan can also rule out intracranial hemorrhage.[18] If an intracranial hemorrhage is present, aneurysmal rupture should be investigated given its association with arterial vasospasm resulting in stroke.[3] Anterior cerebral artery strokes could be missed on imaging studies depending on their location or size. One case series found that 37.5% (6 of 16) of ACA infarcts evaluated by CT were identifiable only after using contrast injection or angiography. If the area of hypodensity is small and localized over a sulcus, the infarct could be overlooked.[1] [13] Noncontrast head CT should be quickly followed by CT angiography of the head and

Subarachnoid hemorrhage

People with poor clinical grade on admission, acute neurologic deterioration, or progressive enlargement of ventricles on CT scan are, in general, indications for the placement of an external ventricular drain by a neurosurgeon. The external ventricular drain may be inserted at the bedside or in the operating room. In either case, strict aseptic technique must be maintained during insertion. In people with aneurysmal subarachnoid hemorrhage the EVD is used to remove cerebrospinal fluid, blood, and blood byproducts that increase intracranial pressure and may increase the risk for cerebral vasospasm. Preventing rebleeding Efforts to keep a person's systolic blood pressure below somewhere between 140 and 160 mmHg is generally recommended. Medications to achieve this may include labetalol or nicardipine.

[New clinical grading in ruptured cerebral aneurysm].

A new clinical grading of subarachnoid hemorrhage (SAH) due to ruptured aneurysm, classified by the presence or absence of vomiting, and by the duration of initial unconciousness at the time of bleeding, is proposed. Grade I: headache without vomiting, Grade II: headache, vomiting, and/or loss of consciousness lasting less than one hour, Grade III: loss of consciouness for over one hour. Grade IV: permanent unconsciousness or cerebral herniation signs. Based on the clinical records, 142 cases of ruptured cerebral aneurysms directly operated on in phases varying from peracute phase (within 72 hours) to delayed phase (22 days or over) were retrospectively analyzed. They included 99 cases which were operated on under microscope. The Hunt & Hess grading was applied just before surgery. Outcome at the 6 month to 1 year follow-up was rated as good, fair, poor and dead. Correlations between the severity and the outcome were calculated using the chi-square test and the levels of significance were compared with those between the recent Hunt and Hess grading and the outcome. In the total of 142 cases, correlation between the clinical severity and the outcome was significant (P less than 0.0005), whereas correlation between the Hunt & Hess grading and the outcome was not significant. In the analysis of cases classified by the operative timing, the clinical severity showed good correlation in the peracute (within 72 hours after SAH) (P less than 0.05) alone, while Hunt & Hess grading showed correlation in delayed phase alone. Neither of the gradings was significant in the acute phase or subacute phase.(ABSTRACT TRUNCATED AT 250 WORDS)

[Multiple bacterial aneurysms: case report].

A 59-year-old man presented with an internal carotid artery (ICA) bacterial aneurysm which ruptured during surgery for treatment of another bacterial aneurysm. He had been admitted to our hospital because of the recurrence of colon cancer. He had undergone aortic and mitral valve replacement because of closure incompetence due to bacterial endocarditis two months previously. Two months after treatment for colon cancer, he developed fever, and arterial blood culture demonstrated. Staphylococcus epidermidis. A few days later, he suddenly suffered severe headache and vomiting, followed by deterioration of consciousness. CT showed subarachnoid hemorrhage and angiography showed a saccular aneurysm at the opercular portion of the left middle cerebral artery (MCA). Immediate clipping of the aneurysm was attempted. The carotid cistern was opened via a left frontotemporal craniotomy, but an ICA aneurysm, which had not been previously recognized, ruptured suddenly. The ICA aneurysm was wrapped with Vascwrap with some difficulty. The MCA aneurysm was then trapped. Postoperatively, the patient continued to be stuporous for a few days. Two weeks later, he died of complications caused by pneumonia. Bacterial aneurysm is more likely to be located in the distribution of the distal arterial tree, mainly in the distribution of the MCA. The difficulty of preoperative diagnosis and the unpredictable clinical course of bacterial aneurysms are emphasized.

[Spontanious subarachnoidal hemorrhage: report on 67 surgicaly treated patients].

The aneurysmal subarachnoid hemorrhage (SAH) is a syndrom with an extremely complex pathophysiological course. Although treatment outcomes in patients with intracranial aneurysms of the circle of Willis and SAH have improved over time, the mortality and morbidity rates remain unacceptably high. The clinical course and the outcome were evaluated in a group of patients who underwent surgery. Patient characteristics, including age, sex, preexisting medical conditions, aneurysm location and size, time to admission, admission neurological status, computerized tomography and angiography findings were analyzed to determine relationship to outcome. A total of 67 patients with the intracranial aneurysms were treated by the microsurgical technique for a direct aneurysm surgery. The data were analyzed in two aspects: the overall management and the surgical results. An occlusion of aneurysm neck was done in 97% and 3% of patients were treated by aneurysm wrapping. There was a significant relationship between the neurological status on admission and the outcome (p < 0.005). Out of 67 patients, 50% had a good result, 16% were moderately disabled, 18% were severely disabled, and 12% were dead at the six month post-SAH evaluation. The results were significantly better according to the better neurological status on admission (p = 0.0001). The disastrous natural history of subarachnoid hemorrhage secondary to ruptured intracranial aneurysm has largely remained unaffected by even the best medical and surgical care because of the natural history of the disease, as well as mistakes in diagnosis and delays in treatment. Among the patients who were underwent surgery, 50% returned to their premorbid state, and 12% died. The factors associated with poor results were direct effects of the initial hemorrhage, vasospasm and rebleeding. Although the results of management of patients with ruptured aneurysms seem to be improved with time, there is opportunity for substantial additional improvement.

[Postoperative hemorrhage due to normal pressure hyperperfusion breakthrough after a trapping of VA-PICA dissecting aneurysm].

We described a case of cerebellar hemorrhage after trapping of a vertebral artery dissecting aneurysm. A forty-eight-year-old man had suffered from severe headache, vomiting and disturbance of consciousness. He was transferred to our hospital in an ambulance. Emergency CT scan showed subarachnoid hemorrhage in the posterior fossa predominantly, intraventricular hemorrhage and hydrocephalus change. Chest X-ray showed radiological evidence of pulmonary edema. The initial blood-gas determinations demonstrated a marked reduction in PaO2 and increased PaCO2. Five days after admission, the patient's condition was improving. Cerebral angiography was performed using the Seldinger method. It revealed a right vertebral artery dissecting aneurysm just distal to the posterior inferior cerebellar artery. We performed an operation to trap the VA dissecting aneurysm. Blood pressure was well controlled under 140 mmHg during the operation and he recovered from anesthesia completely. On the day after the operation, suddenly the patient's consciousness began to deteriorate. Emergency CT scan was performed and it showed SAH, cerebellar hemorrhage and diffuse swelling of the cerebellum on the same side as the operation. We suspected rebleeding of the aneurysm due to a clip's having slipped. Reoperation was performed, but the clip was not displaced and there were no definite bleeding vessels on the operative field. Consequently only external decompression and resection of the right cerebellum were performed. We discuss pathogenesis of the occurrence of hemorrhage in this particular case after trapping. We also review the relevant literature.

[Natural history and operative treatment of intracranial aneurysms after subarachnoid haemorrhage (author's transl)].

This report presents an evaluation of prognosis in 227 patients with subarachnoid haemorrhage caused by intracranial aneurysm, comparing operative treatment in 118 patients with conservative treatment in 109. In all operable cases, the prognosis following early surgery was better than that of conservative treatment. Statistical comparison of patient groups according to the clinical condition allowed the formation of guidelines for the optimal timing of surgery: patients in good clinical condition should be operated on at the earliest possible moment. The overall mortality with such a policy is lower than with a waiting policy, since the majority of recurrences occurred within 2 weeks. Patients with disturbed consciousness and neurological deficit are best operated on either within 24 hours or following stabilization or improvement of the clinical picture -- mostly after 2 weeks. The risk of rebleeding is much lower in these cases, which justifies awaiting optimum clinical conditions. Surgery on comatose patients is contraindicated.

[The foramen of Monro--blockage caused by a giant aneurysm of the basilar artery. A case report and review of the literature].

A case of a giant aneurysma of the basilar artery bifurcation with occlusive hydrocephalus due to bilateral foramen of Monro occlusion is reported. The patient presented with progressive neurological deterioration which led to computerized tomography examination and implantation of a ventricular shunt as an emergency procedure. After the patient had recovered to a certain extent, four-vessel angiography was performed that demonstrated a giant basilar tip aneurysm. As the posterior communicating, the posterior cerebral as well as the superior cerebellar arteries originated from the aneurysm sac direct surgical attack as well as endovascular treatment was considered to be impossible. A permanent biventricular-abdominal shunt system was implanted and the patient was discharged. Three weeks later the patient was readmitted comatose after he had suffered a massive subarachnoid hemorrhage from which he finally died. The clinical and radiological signs, the differential-diagnostic considerations as well as the few cases of foramen of Monro occlusion by a giant basilar artery tip aneurysm, presented in the literature so far, are discussed.

[Management for subarachnoid hemorrhage with negative initial angiography].

Subarachnoid hemorrhage (SAH) is commonly caused by ruptured aneurysm or arteriovenous malformation which is detected by cerebral angiogram. However, since angiograms sometimes cannot show the origin of SAH, we review 12 cases (6.3% of total SAH) to assess the management of these cases. We divided the 12 cases into three groups. Group A (occulted aneurysm group) consisted of six cases of which aneurysms were detected by repeated angiographies in four, and by surgical procedures in two. Group U (unknown etiology group) consisted of five cases. Their follow up periods varied from 7 months to 7 years 11 months, and all of them recovered well and had no episode of rebleeding. Group R (rebleeding group) consisted of one case which fatally re-bled on the second day. Group A tended to be Hunt and Hess grade 3, and Fisher group 3 or 4. In contrast, group U tended to be H and H grade 1 or 2, and Fisher group 2. However by their clinical and neuroradiological findings alone, it was not possible to distinguish the two groups certainly. This means that the patients whose initial angiography does not show the origin of bleeding must be cared for as an occult aneurysm case. Twice repeated angiograms should be programmed. In our cases the first was carried out on the seventh day in the hope that the reason for vasospasm of the parent artery might be shown to be a hidden aneurysm. The second was carried out sometime between the 14th and 21st day because of thrombolysis in the aneurysm, and because it was necessary to relieve vasospasm.(ABSTRACT TRUNCATED AT 250 WORDS)

Relationship of Pressure Reactivity Index and Delayed Cerebral Ischemia in Aneurysmal Subarachnoid Hemorrhage.

Patients with aneurysmal subarachnoid hemorrhage (SAH) continue to have poor functional outcome due to the occurrence of delayed cerebral ischemia (DCI). Although vasospasm represents the primary therapeutic target for mitigating DCI, DCI occurs through multifocal etiologies that involve impaired cerebral autoregulation. Worse pressure reactivity index (PRx) values, which consists of a moving correlation coefficient between intracranial pressures and mean arterial pressures, have been shown to be associated with DCI in non-randomized clinical trials. We discuss two patients that presented with high-grade SAH and comatose exams. Patient one was a 34-year-old male diagnosed with SAH from a ruptured right middle cerebral artery aneurysm. He had intact PRx values (Mean: -0.07 during hospital days 9-19), while having severe, refractory vasospasm. At the conclusion of his hospitalization, he was functionally independent, had negligible DCI, and was successfully discharged home. Patient two was a 78-year-old female diagnosed with SAH from a ruptured anterior communicating artery aneurysm. She had an improving PRx ranging from -0.1 to 0.1 early in her hospitalization. However, upon developing severe vasospasm, her PRx increased to 0.6 (overall PRx from hospital days 4-16 was 0.3), and she suffered from extensive DCI in bilateral middle cerebral and anterior cerebral artery distributions that ultimately resulted in malignant cerebral edema and brain death. Cerebral autoregulation as measured by PRx may represent a viable target for neuroprognostication by evaluating DCI risk in patients with SAH who develop severe or refractory vasospasm. Further studies evaluating the role of cerebral autoregulation, PRx, and its pathophysiological role in DCI are warranted.

Subarachnoid hemorrhage

In general, the decision between clipping and coiling is made on the basis of the location of the aneurysm, its size and the condition of the person. Aneurysms of the middle cerebral artery and its related vessels are hard to reach with angiography and tend to be amenable to clipping. Those of the basilar artery and posterior cerebral artery are hard to reach surgically and are more accessible for endovascular management. These approaches are based on general experience, and the only randomized controlled trial directly comparing the different modalities was performed in relatively well people with small (less than 10 mm) aneurysms of the anterior cerebral artery and anterior communicating artery (together the "anterior circulation"), who constitute about 20 percent of all people with aneurysmal SAH. This trial, the International Subarachnoid Aneurysm Trial (ISAT), showed that in this group the likelihood of death or being dependent on others for activities of daily living was reduced

[Ruptured distal anterior cerebral artery aneurysms presenting with acute subdural hematoma: report of two cases].

Two cases of ruptured distal anterior cerebral-artery aneurysms presenting with acute subdural hematoma are reported. Case 1 was a 55-year-old male, who showed abrupt disturbance of consciousness. An emergency CT revealed acute subdural hematoma at the right parietal convexity and interhemispheric fissure with moderate midline shift. There was no evidence of subarachnoid hemorrhage. Right carotid angiography showed an aneurysm at the right distal anterior cerebral artery. An emergency external decompression was performed and the aneurysm was clipped successfully through the interhemispheric fissure. In the operative field, subarachnoid hemorrhage could not been seen, and the patient had uneventful recovery. Case 2 was a 66-year-old female, who complained of severe headache. She deteriorated rapidly and become comatous with development of anisocoria. An emergency CT revealed acute subdural hematoma on the bilateral parietal convexities and interhemispheric fissure with severe midline shift. There was no evidence of subarachnoid hemorrhage. Carotid angiography showed right distal anterior cerebral artery aneurysm. An emergency external decompression was performed, then the aneurysm was clipped successfully. She recovered with disorientation and hemiparesis. Ruptured distal anterior cerebral artery aneurysms presenting with acute subdural hematoma without subarachnoid hemorrhage are rare. It is suggested that CT scans and history of patients are most important but an emergency angiography was prerequisite for correct diagnosis. Surgical treatment should be the best management in such cases.

Interdisciplinary treatment selection in admitted patients with acute subarachnoid hemorrhage.

To review the angiographic and clinical outcomes from acute (first 6 hours after bleeding) interdisciplinary treated patients with ruptured intracranial aneurysm. An interventional neuroradiologist and a neurosurgeon set the emergent treatment of 100 consecutive patients with subarachnoid hemorrhage (SAH) harboring 111 cerebral aneurysms. Decisions were based on the patient's clinical condition, aneurysm's morphology and location, presence of intracranial mass-occupying process (IMOP), angiographic flow distribution patterns and treatment-related risk profit. The benefits of a joined case assessment were retrospectively analyzed, comparing our clinical and radiological results with those of the averaged literature series. Initially, 39 aneurysms in 34 patients were endovascularly (EV) treated and 68 aneurysms in 66 patients were surgically (S) treated. Four nonruptured aneurysms remain under control. Complete radiological aneurysm occlusion was initially achieved in 35 (89.7%) EV and 62 (95.4%) S treated aneurysms. Twenty-six patients in poor clinical condition with IMOP as well as ten patients with distally located, 18 with EV unfavorable and 12 with highly expected recanalization rate aneurysms required S treatment. Four aneurysm remnants (two EV and two S) remain unchanged. Two EV treated aneurysms grew and were surgically occluded, and another three patients required an additional coiling procedure after recanalization. Favorable evolution was observed in 27 (79.4%) EV and 55 (83.3%) S treated patients. Computed tomography-documented rebleedings (7:1), angiographic vasospasm (3:5) and occlusion of main vessels (2:2) were the method-related complications in the EV and S groups, respectively. After comparing the literature data, acute interdisciplinary treatment of patients with SAH increases the rate of aneurysm occlusion, improves patient's evolution and reduces the number of complications.

Cerebromeningeal hemorrhage. Analysis of autopsies performed over a 10-year period.

A study was conducted on the medical records of 353 patients who died of a subarachnoid hemorrhage (SAH) and who were submitted to autopsy over the last 10 years. SAH was associated with arterial hypertension in 180 (51%) cases, with ruptured aneurysms in 102 (28.9%), and with other pathologies in 71 (20.1%). The patients with hemorrhage associated with arterial hypertension were mostly males, and those with hemorrhage due to aneurysms were mostly females. Of the patients with aneurysms, 36 (35.3%) had aneurysms in the anterior communicating artery, 30 (29.4%) in the internal carotid artery, and 23 (22.6%) in the middle cerebral artery. Among the patients with aneurysms who suffered rebleeding and vasospasm, 59.1% and 61.5%, respectively, were classified as grade I and II upon admission, and all evolved toward grade IV after these complications. Vasospasm predominated from the 3rd to the 10th day after hemorrhage, and rebleeding from the 9 to 16th day and both were most frequent among patients with aneurysms of the anterior communicant artery. Sixty eight percent of the patients with aneurysms died during the first 9 days after hemorrhage. Because of our conduct was to operate systematically late, a considerable number of patients lost the opportunity to be treated surgically with possible favorable evolution due to vasospasm or rebleeding.

[Value of the measurement of cerebral blood flow before and after diamox injection in predicting clinical vasospasm and final outcome in aneurysmal subarachnoid hemorrhage].

The timing for surgery on ruptured intracranial aneurysms remains a difficult question and the choice of the day of operation depends greatly from the occurrence of a vasospasm. On a previous paper, the value of the cerebral blood flow (CBF) measurement by intravenous injection of Xenon 133 was demonstrated to be efficient for the prediction of vasospasm only when done between day 4 and day 8 after bleeding. Moreover the efficiency of the measurement was much greater if the evolution of the CBF values between D0 and D8 was considered, but this method was incompatible with early surgery. It suggested the interest of a dynamic study of the CBF by the same method. On a series of 43 patients, the comparison between basal CBF values and reactivity of CBF values to intravenous injection of 1 gram of acetazolamide for the prediction of clinical vasospasm is presented. The series comprises 32 early admitted patients (74%) and 31 operated patients (16 of them between D0 and D3 after bleeding, 15 others after D4). The efficiency of the CBF reactivity study for the prediction of the clinical vasospasm appears much greater than that of the basal CBF value, even during the first three days after bleeding, but not for the prediction of the final clinical outcome. The method is immediate and compatible with early surgery. What precisely is evaluated by this method on the physiopathology of the vasospasm remains disputable, but the operability of the patients seems to be explored.

Saccular Aneurysm -- Treatment / Management -- 3. Surgical Clipping

Those patients with a ruptured cerebral aneurysm should be admitted to the intensive care unit for close monitoring. Endovascular coiling or surgical clipping of the ruptured aneurysm should be performed as early as possible in the majority of the cases to reduce the risk of rebleeding. Patients need to be monitored for the signs and symptoms of clinical deterioration due to vasospasm, rebleeding, seizures, cerebral edema, hydrocephalus, and hyponatremia. In the first 48 hours after a SAH, the major cause of morbidity or mortality is rebleeding. Blood pressure should be maintained near 120/80 mmHg before the definite procedure to occlude the aneurysm, but after the treatment, the mean arterial pressure should be maintained above 100 mmHg to reduce ischemic changes secondary to vasospasm. Cerebral vasospasm resulting in cerebral ischemic and neurologic deterioration starts around day 3 to 5 and peaks at day 7. Oral nimodipine at a dose of 60 mg every 4 hours is given for 21 days to improve outcomes. [34] [35] Daily transcranial doppler is recommended to monitor vasospasm. After aneurysm repair, cerebrovascular imaging is generally recommended to look for remnants or recurrence of the aneurysm that may require re-intervention. This can be performed intraoperatively or a few days later.

[A case of multiple anomalies of cerebral vessels--fenestration of the middle cerebral artery aneurysm of the anterior communicating artery and arteriovenous malformation on the frontopolar region (author's transl)].

A case associated with multiple cerebral vascular anomalies, which consisted of fenestration of the middle cerebral artery, arteriovenous malformation and aneurysm of the anterior communicating artery, was reported. A 48 year-old male has been suffering from the left paralysis and mental disorder after the initial attack of subarachnoid hemorrhage, and the second attack resulted in the deterioration of the symptoms. He was admitted to our clinic on October 28, 1974. On neurological examination, mental disorders, such as disorientation, emotional incontinence, amnesia and acalculia, hemiplegia on the left and meningeal irritation signs were observed in admission period. Physical examination was negative. Cerebral angiographic findings were as follows: 1) Moderate vasospasm of the right internal carotid artery at the terminal segment, mild bowing of the anterior cerebral artery and stretching of the frontparietal opercular branches of the middle cerebral artery were observed. 2) Right frontpolar arteriovenous malformation fed by the frontobasal artery and the frontopolar artery, and drained via the aberrant cortical vein into the superior sagittal sinus. 3) Aneurysm of the anterior communicating artery was opacified by left carotid angiography. 4) An abnormal vessel derived from the terminal segment of the right internal carotid artery and terminated at the portion of the sphenoidal segment of the middle cerebral artery. Complete loop was formed between genuine middle cerebral artery and this abnormal artery. He was operated with dissecting microscope on November 11, 1974. The arteriovenous malformation at right frontopolar region was totally removed and aneurysm of the anterior communicating artery was clipped. According to the operative findings, the arachnoid membrane over the right frontopolar region was turbid and adhered to the adjacent tissues. On the contrary, no abnormal findings suggestive of previous subarachnoid hemorrhage were observed around the region of the anterior communicating artery aneurysm. These findings showed that subarachnoidal bleeding was caused by rupture of the arteriovenous malformation of right frontopolar region, but not by the aneurysm on the anterior communicating artery. The postoperative course was uneventful and during the hospitalization the patient starts on rehabilitation therapy. The authors discussed the genesis of fenestration of the middle cerebral artery and relation among these combined vascular anomalies. We inferred that fenestration of the middle cerebral artery arose from the in complete fusion of procursor vascular network in embryonic stage. Additionally, we emphasized that it was necessary to make a distinction between these two terms "fenestration" and "duplication".

[Late complications of subarachnoid hemorrhage from ruptured aneurysms treated with early and delayed surgery].

Result of studies on remote sequele of SAH from ruptured brain aneurysms are presented. The investigation have been pointed at the character, range and intensity of some changes in CNS initiated by SAH and resulting from this bleeding. The material comprised of 110 patients: 48 out of this number having been operated on within 72 hours from initial bleed, while 62 have had surgery in delayed term. Three methods of investigations were applied: 1. Angioscintigraphy of the brain. 2. Computerized tomography. 3. Clinical evaluation according to GOS (Glasgow Outcome Scale). The results obtained seem to give reasons to assumption that SAH initiates the gradual process of degradation of the CNS tissues. The blood extravasated to the subarachnoid space following aneurysm burst sets in motion a chain of events leading to: 1) impairment of brain arteries reactivity on elevation of pCO2, 2) intensification of brain atrophy. The intensity of the above mentioned phenomena is proportional to the severity of clinical course of SAH. It came out that timing of surgery has influenced the remote postoperative results less than it had been expected.

Management of a ruptured cerebral aneurysm in infancy. Report of a case of a ten-month-old boy.

A ten month old unconscious boy with hemiplegia (Hunt and Hess IV) was first admitted to a district hospital without a CT scanner or a neurosurgical service (Glasgow-Coma-Score 4, no pathological pupillary signs). Therefore, he was transferred to the Pediatric Department of the University Hospital the same night. An emergency CT scan that night showed intracerebral and subarachnoid hemorrhage with enlarged ventricle (Fisher grade 5). Angiography was not available within reasonable time. Thus in the stage of progressively increasing clinical deterioration, still without pupillary signs, an external ventricular drain-age was placed. Immediately after reduction of the cerebrospinal fluid volume, arterial hypertension was noticed--the right pupil was mydriatic and fixed. Without further apparative diagnosis an emergency craniotomy was performed for decompression under the suspicion of a secondary hemorrhage due to a rerupture of a middle cerebral artery aneurysm. A bleeding aneurysm of the right middle cerebral artery was found and clipped. A mass transfusion was necessary and a pulmonary air embolism occurred. The infant died in tabula. The histological specimens revealed disruption of the internal elastic membrane of both MCA. This emphasizes a congenital nature of the aneurysm. We conclude that cerebral arterial aneurysms have to be considered in the differential diagnosis of stroke-like symptoms in infancy and early childhood, although the incidence of reported cases is less than one case per year. Since no valid screening parameter is available, diagnosis is often made only after rupture of the aneurysm. This causes problems for emergency management. Infants and children with stroke or stroke-like symptoms should immediately be transferred to a hospital with a neurosurgical unit.

[Ruptured intracranial aneurysm. Modification of the surgical method based on the state of consciousness and age of patients. Personal experience with 468 patients admitted during 2 periods (1972-84 and 1985-89)].

The management of the ruptured intracranial aneurysm (clinical grade I to IV only) is studied in a series including 140 patients admitted from 1985 through 1989, in which selected patients were submitted to early surgery and other patients were postponed for delayed surgery. The results are compared to those of an earlier series including 328 patients admitted from 1972 through 1984, for which the general attitude was the delayed surgery: in the later series; the selection for the timing of surgery was based on two main parameters: the clinical status and the patient's age. When we compare both series, the overall management results demonstrate an improvement of 10% of satisfactory results and a decrease of 10% in the death rate in favour of the later series; for the surgical results, the figures are respectively 6% and 5% in favour of the later series. The relationship between age and outcome shows a considerable improvement: over 50 years of age, we observed plus 25% of satisfactory results and minus 22% in death in favour of the later series. Similarly, the relationship between state of consciousness and outcome, demonstrated a great improvement: for drowsy and stuporous patients the figures are respectively plus 22% and minus 21% in favour of the later series. When we consider the later series alone, the patients were admitted at 4 intervals of time from S.A.H. (D0-3, D4-6, D7-15, D16 and over). The most favorable outcome was observed for those patients admitted late (after D7) and already stabilized.(ABSTRACT TRUNCATED AT 250 WORDS)

Delayed cerebral ischemia causing cortical blindness due to repeat cocaine use weeks subsequent to aneurysmal subarachnoid hemorrhage.

Cocaine is a known vasoactive drug associated with poor clinical outcomes and high in-hospital mortality related to aneurysmal subarachnoid hemorrhage; however, the association of prior cocaine use with the incidence of vasospasm and delayed cerebral ischemia remains controversial. We report a case of a 42-year-old male with a history of active cocaine use who presented with a severe headache. Imaging demonstrated diffuse cisternal subarachnoid hemorrhage due to a ruptured basilar apex aneurysm, which was successfully treated with endovascular coil embolization. Despite expedited endovascular treatment and an initially benign clinical course, he suffered from delayed cerebral ischemia resulting in cortical blindness due to bilateral posterior cerebral artery vasospasm secondary to repeat cocaine use weeks after his initial ictus. To our knowledge, the present case is the first to describe delayed cerebral ischemia resulting in a severe neurologic deficit due to repeat cocaine use weeks subsequent to aneurysm rupture. We review the current literature on the association of cocaine use with the incidence of vasospasm and delayed cerebral ischemia as well as the effects of cocaine on the cerebrovasculature.

Rescue endovascular treatment for rapid regrowth of aneurysm remnant on middle cerebral artery trunk after unsuccessful surgical clipping in patients with a ruptured cerebral aneurysm: A report of two cases.

We report two rare cases treated with coiling after rapid regrowth (within a month) of an aneurysm remnant on the middle cerebral artery (MCA) trunk after incomplete surgical clipping. The first case, a 47-year-old man with subarachonoid hemorrhage (SAH) (Hunt-Hess grade II, Fisher grade III) underwent clipping of a ruptured saccular aneurysm with a wide neck on the right early frontal branch arising from the MCA trunk. Incomplete clipping with a 1 mm sized remnant neck was performed to avoid sacrificing the lenticulostriate artery. In a follow-up cerebral angiogram on postoperative day 30, a rapid regrowth of the aneurysm remnant was observed, and on that day, complete obliteration was obtained by rescue endovascular treatment. The second case, a 48-year-old healthy woman with SAH (Hunt-Hess grade II, Fisher grade III) underwent clipping of an anteroposteriorly projecting bilobulated aneurysm on the left M1. Incomplete clipping with a minimal remnant neck was performed. In follow-up digital subtraction angiogram on postoperative day 30, a rapid regrowth of an aneurysm remnant involving only a part of the initial aneurysm near the neck was observed, and on that day, complete obliteration was obtained by rescue coiling. These patients were both discharged without any neurological deficits.

Case of unusual presentation of fusiform aneurysm of the basilar artery.

To describe a case of a fusiform aneurysm of the basilar artery presented as a pontine infarct and 2 days later as a subarachnoid hemorrhage caused by the rupture of the same aneurysm. Case report. Tertiary-care hospital. Fusiform aneurysm of cerebral vessels is a rare pathology that presents with ischemic stroke, subarachnoid hemorrhage, or mass effect. Ischemia and subarachnoid hemorrhage in the same patient 2 days apart, to our knowledge, was not reported before. A 55-year-old Hispanic man with history of untreated hypertension and alcohol abuse presented with acute onset of right hemiparesis and dysarthria. On day 2 of his hospital admission, he developed arrhythmia and loss of consciousness. The patient expired from cardiac arrest on day 4 of his hospitalization. Initial head computed tomography scan showed dolichoectatic basilar artery and marked calcification of internal carotid arteries (ICA) and middle cerebral arteries (MCA). Brain magnetic resonance imaging showed left upper pons infarct 2 cm in diameter. Magnetic resonance angiography confirmed presence of a fusiform aneurysm of the basilar artery. Head computed tomography scan at the time of clinical deterioration on day 2 of hospital admission showed subarachnoid bleeding and significant brain edema. Autopsy revealed a ruptured basilar artery aneurysm with thrombus in the lumen and left pontine infarct. Microscopic examination of fusiform aneurysm showed atherosclerosis of the aneurysmal wall and attenuation and inflammation at the rupture site. We hypothesize that in our case, pontine infarct had developed because of occlusion of pontine perforators by a thrombus within an aneurysm, and subarachnoid hemorrhage had developed secondary to a rupture of weakened by inflammatory changes aneurysmal wall. We also hypothesize that in our case, ischemic stroke and subsequent subarachnoid hemorrhage may represent different stages of the same process of atherosclerosis and inflammation in an aneurysmal wall confirmed by autopsy. Origin as well as management of fusiform cerebral aneurysms are unclear. Antiplatelet agents and anticoagulation are recommended by some for stroke prophylaxis in patients with unruptured fusiform cerebral aneurysms. Our case shows that caution should be exercised in prescribing anticoagulants or even antiplatelet agents to a patient with fusiform aneurysm of cerebral arteries due to a possibility of rupture of an aneurysm. Randomized prospective study may be necessary to clarify this issue.

[Time course of blood velocity changes and clinical symptoms related to cerebral vasospasm and prognosis after aneurysmal surgery].

Cerebral vasospasm is a major complication associated with subarachnoid hemorrhage. In spite of extensive research, the pathogenesis of vasospasm remains obscure, and clinical management has so far been extremely difficult. For the evaluation of the efficacy of any treatment and the timing of operation, the development and resolution of the arterial narrowing should be monitored. It is important to know the correlation between the change in flow velocity and the course of clinical symptoms. In this study, the blood flow velocity in the different arteries was measured at short intervals (at least every third day) with transcranial Doppler ultrasonography, in eighteen patients with ruptured cerebral aneurysm. Flow velocity changes began to accelerate from 3 days after surgery, and reached the maximum value between 7 and 10 days, with normalization occurring within the following 2 weeks. The changes showed a significant relationship to the severity of vasospasm, the side of the operative approach, and the administration of calcium antagonist. Based on our results, we categorized the flow velocity changes of vasospasm for clinical practice as follows. (1) A slight acceleration of up to 120 cm/s in MCA and 90 cm/s in ACA was defined as within the normal range. In this state vasospasm was never observed angiographically. (2) Flow velocity changes between 120 and 140 cm/s were a subcritical state. These changes were seen in vasospastic patients who did not develop ischemic deficits. (3) Flow velocity changes of over 140 cm/s in MCA and 100 cm/s in ACA were critical vasospasm, since all of the patients who developed ischemic symptoms were in this group. Velocity changes higher than 170 cm/s in MCA and 130 cm/s in ACA seem to indicate a critical condition with a tendency to develop into brain infarction. The comparison between the time course of the flow velocity changes and clinical status showed that in symptomatic vasospasm the increase in velocity occurred before the manifestation of clinical symptoms. The transcranial Doppler measurements help to identify those patients who have a high risk of developing neurological deficits due to vasospasm, and help to select those who would benefit from preventive treatment in asymptomatic stage.

[Two cases of subarachnoid hemorrhage associated with neurofibromatosis type I: a case of multiple cerebral aneurysms and arteriovenous malformation, and another case of an anterior communicating artery aneurysm].

Two cases of subarachnoid hemorrhage associated with neurofibromatosis type I (von Recklinghausen's disease) are reported. A 30-year-old male patient (case 1) had been diagnosed as having neurofibromatosis type I due to neurofibroma and café-au-lait spot. He suffered from subarachnoid hemorrhage and angiography showed multiple aneurysms in the right and left middle cerebral arteries and left internal carotid artery. He also had arteriovenous malformation in the left temporal lobe. Case 2 was that of a 62-year-old female patient with neurofibroma and café-au-lait spot. She suffered from subarachnoid hemorrhage and angiography showed an aneurysm in the anterior communicating artery. Both patients were discharged with no deficits after neck clipping, however they both suffered from large-sized hematoma in the punctured site of the femoral artery after angiography. The cerebral aneurysms associated with neurofibromatosis type I are often multiple and may coexist with arteriovenous malformation.

[Correlation of vasospasm with operative results in ruptured aneurysms].

The present study is a retrospective clinicoradiological correlative investigation of subarachnoid hemorrhage associated with ruptured aneurysms. There were 360 patients in whom the source of subarachnoid hemorrhage was shown by angiography to be a ruptured aneurysm, and 302 of them underwent surgery. Arterial vasospasm was demonstrated in 113 of 360 patients (31%). When the time relationship between the subarachnoid hemorrhage and vasospasm was considered, vasospasms were revealed in 35 patients (31%) to ten days after a hemorrhage. In these cases with preoperative vasospasm, 17 patients were operated within 14 days and 43 patients later than two weeks. On the examination, their clinical state was graded according to Hunt and Hess. The surgical results were also grade in five groups (excellent, good, fair, poor and died) according to the functional results at discharge from the hospital. Of 35 patients of grade I and II at operation, 23 patients (66%) were of excellent or good results. However, of 14 patients of grade IV and V at operation, only on patient took good result. Of 12 patient in whom severe vasospasm was demonstrated and surgery was delayed more than two weeks, 8 patients (66%) had good result. Postoperative vasospasm was recognized in 2 patients, who took poor result. Surgery was carried out in 32 patients on the first or second day after subarachnoid hemorrhage, and 7 patients (22%) of them took good result. The overall mortality was 59% (19 patients). In ten patients, surgery was carried out on the third or fourth day after subarachnoid hemorrhage. Five patients had good result (50%) and four (40%) died. Considering the relationship between the timing and result of surgery, the mortality was 35% in patients operated on in the first week, whereas there were only 38 deaths among the 252 patients operated on after that interval. Among the death patients who were in grade IV or V at admission, intracranial hematoma was seen in 73%. It was concluded that in patients classified as grade I or II, the operation should be performed as soon as possible to prevent another rupture. In grade IV and V patients with severe damage by large hematoma, the mortality and morbidity were high even after evacuation of the hematoma. Patients with marked and diffuse vasospasm should be given conservative treatment at first and then operated on when the patients condition becomes good.

International Subarachnoid Aneurysm Trial

Updated data from the ISAT group in March 2008 shows that the higher aneurysm rate of recurrence is also associated with a higher rebleeding rate, given that the rebleed rate of coiled aneurysms appears to be 8 times higher than that of clipping treated aneurysms in this study. The ISAT authors conclude that "when treating ruptured cerebral aneurysms, the advantage of coil embolization over clip ligation cannot be assumed for patients < 40 years old." Other subsequent studies have questioned the ISAT's conclusions directly. This conclusion is based on a number of methodological assumptions itself and other authors have cautioned about extending it to other patient populations. It appears that although endovascular coiling is associated with a shorter recovery period as compared to surgical clipping, it is also associated with a significantly higher recurrence rate after treatment. The long-term data for unruptured aneurysms are still being gathered.

Early aneurysm surgery: a 7 year clinical practice report.

One hundred and fifty patients with intracranial aneurysms, operated on consecutively in the early stage in our department, were re-evaluated retrospectively. Seven surgeons operated on 159 aneurysms in 150 patients. Seventy-nine percent of the patients were in grades I-III (scale of Hunt and Hess), 21% in grades IV-V. Seventy-one percent had a severe haemorrhage (classification of Fisher et al.), 21% had an intracerebral haematoma. Intraoperative CSF drainage was an almost indispensable tool while postoperative external drainage did not prove to be helpful in preventing vasospasm and/or hydrocephalus. Induced hypotension was abandoned in favour of temporary clipping. Thirteen percent of the patients suffered a permanent or fatal immediate postoperative deterioration, while 11% developed delayed neurological deficits. Five percent were related to vasospasms alone, they were all transient. Five percent had vasospasm combined with other complications. One of them had permanent and the other one fatal deficits. One percent deteriorated due to embolism or occluded vessels. The results improved with the introduction of the calcium channel blocker nimodipine, induced hypertension and transcranial Doppler sonographic control of the vasospasm. Patients in good preoperative condition had a good early outcome in 69%. The result was fair in 21% and poor in 4%, while 6% of the patients died. In the poor condition group 22% of the patients made a good, 13% a fair, and 59% a poor recovery, 16% of whom died.(ABSTRACT TRUNCATED AT 250 WORDS)


The key in the question is "stopping the clinical course", as Hungtinton's disease, Tourette's and Parkinson's disease do not have treatments that can stop the clinical course. Sydenham's chorea is typical of younger children with a history of streptococcal infection and resolution is usually spontaneous. On the other hand, in clinical cases it is recommended to rule out Wilson's disease in young patients with movement and neuropsychiatric disorders.

In a patient presenting in the second decade of life with a progressive picture of parkinsonism, tremor, dystonia and behavioral alteration, you should always perform a study to rule out the presence of a disease whose appropriate treatment can improve neurological symptoms and stop the clinical course. What disease is it?

[Tourettism, hemiballism and juvenile Parkinsonism: expanding the clinical spectrum of the neurodegeneration associated to pantothenate kinase deficiency (Hallervorden Spatz syndrome)].

Pantothenate kinase deficiency (Hallervorden-Spatz syndrome, HSS) triggers cerebral neurodegeneration with iron deposition in the basal ganglia. The classical form has an early onset in infancy, a progressive course, the presence of extrapyramidal symptoms (dystonia, chorea, rigidity) and pigmentary retinitis. There are atypical late onset forms with predominance of symptoms of Parkinsonism and dementia, which progress slowly and course somewhat less progressively. We describe three patients with HSS and an atypical presentation, with onset during the second decade of life. In all cases magnetic resonance imaging showed areas of hyposignal in T2 sequences in medial globus pallidus, with central hypersignal, which gave rise to a tiger's eye image. Other aetiologies, such as Wilson's disease, gangliosidosis GM1, hypoprebetalipoproteinemia, hexosaminidase A deficiency, aminoacidurias and infantile Huntingdon's chorea, were precluded. In the 20-year-old male the initial manifestations at the age of 17 were superposed over Gilles de la Tourette syndrome, with complex motor and vocal tics, palilalia, behavioural disorders and postural instability. The 13-year-old patient presented symptoms of chorea, hemiballic movements and dystonia in the lower limbs, which limited walking at the age of 12. The 28-year-old female patient presented a progressive rigid akinetic syndrome, with dementia and partial response to levodopa. The clinical spectrum of HSS is broad and its differential diagnosis must include hemiballism, Tourette syndrome and juvenile Parkinsonism.

FINGERPRINT PATTERNS IN HUNTINGTON'S CHOREA AND PARKINSON'S DISEASE.

In the course of a continuing search for means of predicting Huntington's chorea before the onset of neurological symptoms, a study of fingerprint patterns was undertaken, using the technique employed by Hodges and Simon in the investigation of patients with Wilson's disease. Fingerprint patterns of 61 patients with Huntington's chorea and 50 with Parkinson's disease were compared with norms established by Scotland Yard. Although an increased incidence of the "whorl" pattern was seen in the left second and third fingers in patients with Huntington's chorea, this finding could not be interpreted as having diagnostic or prognostic value as it was found also in some normal subjects and in occasional cases of Parkinson's disease. The pattern supposedly characteristic of Wilson's disease was also seen in persons with Huntington's chorea.

Nosology and Phenomenology of Psychosis in Movement Disorders.

Psychotic symptoms, such as delusions and hallucinations, are part of the clinical picture of several conditions presenting movement disorders. Phenomenology and epidemiology of psychosis in Parkinson's disease have received wide attention; however, the presence of psychosis in other movement disorders is, comparatively, less well known. To review psychotic symptoms present in different movement disorders. A comprehensive and structured literature search was performed to identify and analyze data on patients with movement disorders and comorbid psychosis. In monogenic parkinsonisms, such as PARK-GBA, PARK-LRRK2, and PARK-SNCA, visual hallucinations related to dopamine replacement therapy are frequent as well as are delusions in PARK-LRRK2 and PARK-SNCA, but not in PARK-GBA. Different types of delusions and hallucinations are found in Huntington's disease and other choreic disorders. In Tourette's syndrome, paranoid delusions as well as visual, olfactory, and auditory hallucinations have been described, which usually develop after an average of 10 years of disease. Delusions in ataxias are more frequent in ATX-TBP, ATX-ATN1, and ATX-ATXN3, whereas it is rare in Friedreich's ataxia. Psychosis is also a prominent and frequent clinical feature in Fahr's disease, Wilson's disease, neurodegeneration with brain iron accumulation, and some lysosomal storage disorders, whereas it is uncommon in atypical parkinsonisms and dystonia. Psychosis usually occurs at late disease stages, but may appear as onset symptoms of the disease, especially in Wilson's disease, Huntington's disease, late-onset Tays-Sachs, and Niemann-Pick. Psychosis is a frequent comorbidity in most hyper- and hypokinetic movement disorders. Appropriate recognition is relevant both in the early and late disease stages.

Profile of patients enrolled in a new movement disorder clinic.

To assess the need for a regional expertise in movement disorders, the numbers of patients, clinic visits, and medication changes for a new movement disorder clinic were recorded. During 3 1/2 years, 355 patients were seen, with 1,329 clinic visits. Idiopathic Parkinson's disease was the most common diagnosis, comprising 36% of the population, followed by dystonia (17%), tremor (12%), parkinsonism (i.e., Parkinson's plus syndromes, drug-induced parkinsonism, etc.) (10%), chorea (10%), Tourette's syndrome (6.5%), and tardive dyskinesia (3.4%). Distribution of follow-up visits was similar, with Parkinson's disease (52%) being most frequent and Tourette's syndrome (3.1%) least frequent. The relative utilization of medical care by each patient group was assessed by determining the number of medication changes and the number of clinic visits per follow-up year. No differences in these measures were found using a one-way analysis of variance. Of the Parkinson's disease patients, 67% had Hoehn and Yahr stages III-IV and 77% of the clinic visits were made by this subgroup. When considered in light of the prevalence of each of the diseases, these data show a need for an expertise in movement disorders for a population base of the size we have served.

Movement disorders in childhood.

The aim of this article is to review movement disorders in children. They are common but have etiology and phenomenology different than in adults. Tics are the most common phenomena although in most instances they are mild and have a favorable long-term prognosis. Dystonia is the second most common phenomena but when present it is usually genetic or idiopathic and causes meaningful disability. Sydenham's chorea is the most common cause of chorea in children worldwide. Systemic lupus erythematosus is a much rarer cause of chorea but it is always to be ruled out given the lack of a specific diagnostic marker for Sydenham's chorea. Tremor, usually caused by drugs or essential tremor, is regarded as rather uncommon in children. Arguably, most pediatric patients with tremor do not seek medical attention because of the lack of disability. Stereotypies are relatively uncommon but their recognition is clinically relevant since they are usually associated with severe conditions such as autism and Rett syndrome. Parkinsonism is quite rare in children and either results from encephalitis or is a side effect of medications. Wilson's disease must be ruled out in all children with movement disorders.

Pharmacology_Katzung

Huster D: Wilson disease. Best Pract Res Clin Gastroenterol 2010;24:531. Jankovic J: Medical treatment of dystonia. Mov Disord 2013;28:1001. Kalia LV, Kalia SK, Lang AE: Disease-modifying strategies for Parkinson’s disease. Mov Disord 2015;30:1442. Kimber TE: An update on Tourette syndrome. Curr Neurol Neurosci Rep 2010;10:286. Kordower JH et al: Transplanted dopaminergic neurons develop PD pathologic changes: A second case report. Mov Disord 2008;23:2303. LeWitt PA et al: AAV2-GAD gene therapy for advanced Parkinson’s disease: A double-blind, sham-surgery controlled, randomised trial. Lancet Neurol 2011;10:309. Lorincz MT: Neurologic Wilson’s disease. Ann N Y Acad Sci 2010;1184:173. Lyons KE, Pahwa R: Outcomes of rotigotine clinical trials: Effects on motor and nonmotor symptoms of Parkinson’s disease. Neurol Clin 2013;31(3 Suppl):S51. Olanow CW et al: A double-blind, delayed-start trial of rasagiline in Parkinson’s disease. N Engl J Med 2009;361:1268.

Wilson's disease

Neuropsychiatric symptoms About half of the people with Wilson's disease have neurological or psychiatric symptoms. Most initially have mild cognitive deterioration and clumsiness, as well as changes in behavior. Specific neurological symptoms usually then follow, often in the form of parkinsonism (cogwheel rigidity, bradykinesia or slowed movements and a lack of balance are the most common parkinsonian features) with or without a typical hand tremor, masked facial expressions, slurred speech, ataxia (lack of coordination) or dystonia (twisting and repetitive movements of part of the body). Seizures and migraine appear to be more common in Wilson's disease. A characteristic tremor described as "wing-beating tremor" is encountered in many people with Wilson's; this is absent at rest but can be provoked by abducting the arms and flexing the elbows toward the midline.

Huntington's disease

Huntington's disease (HD), also known as Huntington's chorea, is a neurodegenerative disease that is mostly inherited. The earliest symptoms are often subtle problems with mood or mental abilities. A general lack of coordination and an unsteady gait often follow. It is also a basal ganglia disease causing a hyperkinetic movement disorder known as chorea. As the disease advances, uncoordinated, involuntary body movements of chorea become more apparent. Physical abilities gradually worsen until coordinated movement becomes difficult and the person is unable to talk. Mental abilities generally decline into dementia. The specific symptoms vary somewhat between people. Symptoms usually begin between 30 and 50 years of age but can start at any age. The disease may develop earlier in each successive generation. About eight percent of cases start before the age of 20 years, and are known as juvenile HD, which typically present with the slow movement symptoms of Parkinson's disease rather than

[Contributions of Charcot and Marsden to the development of movement disorders in the 19th and 20th centuries].

Charcot described many neurological diseases in the 19th century, particularly in movement disorders. Charcot contributed in the clinical description of Parkinson's disease, and introduced its first pharmacological treatment. He also studied the hyperkinesias, e.g. of Tourette syndrome, differential diagnosis of tremors, dystonias, choreas and startle disease. Marsden, who died recently, was an exponent in the study of Movement Disorders, with many publications in this field in the 20th century. His most important contributions are definitions and classifications of movement disorders, such as Parkinson's disease, dystonia, myoclonus, essential tremor, the description of the syndromes "Painful Legs Moving Toes", "Gait Ignition Failure" and "Primary Writing Tremor". The contributions of Charcot in the 19th century and Marsden in the 20th century to the movement disorders allow us to conclude that both of them were the most representative icons in this field in the past two centuries.

Motor disturbances: These include the characteristic unwanted involuntary movements which initially begins in the distal extremities and are of a smaller degree but could go on to affect the facial muscles as well. The movements then spread gradually to the more proximal and axial muscles and are of greater amplitude. Motor symptoms tend to be progressive. Early in the disease, they are mostly hyperkinetic with involuntary chorea. In later stages, however, hypokinesia with bradykinesia and dystonia predominate. The balance between chorea and hypokinesia varies from patient to patient and ranges from overwhelming rigidity in the younger patients, also known as the Westphal variant to older patients being severely affected in the later stages of the disease with rigidity and contractures in the extremities leading them to be bedridden. Dysarthria and dysphagia develop during the course of the disease, which could lead to aspiration in patients, with pneumonia being a common cause of death. Dystonia, characterized by increased muscle tone with slower movements, leads to abnormal posturing such as torticollis and can be the first sign of motor involvement in HD. Other involuntary features include tics and cerebellar signs such as ataxia. Pyramidal signs such as the Babinski sign are present. The motor disturbance on daily activities progresses over time and can lead to difficulties in walking, standing, and frequent falls.

Neurology_Adams

In terms of pathologic anatomy and physiology, bradykinesia may be caused by any process or drug that interrupts the cortico-striato-pallido-thalamic circuit. Clinical examples include reduced dopaminergic input from the substantia nigra to the striatum, as in Parkinson disease; dopamine receptor blockade by neuroleptic drugs; extensive degeneration of striatal neurons, as in striatonigral degeneration and the rigid form of Huntington chorea; and destruction of the medial pallidum, as in Wilson disease. As illustrated in Fig. 4-4B, which gives a schematic representation of the hypokinetic state of Parkinson disease, changes in the cortico-striato-pallido-thalamic circuit (in this case mainly the direct striatopallidal pathway) can be interpreted in terms of altered neurochemical and resultant physiologic connectivity within the basal ganglia. The reciprocal situation, enhanced motor activity, is summarized in the analogous diagram for Huntington disease (Fig. 4-4C), in which a

Huntington Disease -- History and Physical -- Clinical course and classification:

Overall, the mean age of onset is approximately 45 years. Patients who often present with neurological symptoms also exhibit psychiatric changes. Early in the course of the disease following diagnosis, symptoms such as changes in eye movements, mental planning, depressed or irritable mood as well as mild involuntary movements can be observed. The affected patients are generally able to perform their daily activities. Eventually, chorea progresses, and it becomes increasingly difficult to perform voluntary activities. There are intermittent bursts of aggressive behavior and social disinhibitions. Although patients can maintain some degree of independence, most depend on their caregiver for help. In the later stages of the disease, severe motor disability is noted, and patients are completely dependent on caregivers. The median survival after the onset of the disease is 15 to 18 years. In around 25% of patients, a delayed onset is seen, and these patients exhibit symptoms after the age of 50 and some after the age of 70. Chorea and disturbances in gait are noted on them and often exhibit a benign and more prolonged course than typical patients. [3]

Inherited movement disorders.

A high proportion of neurological diseases characterized by movement disorders are caused by single genes; in others, such as Parkinson's disease, there appears to be a genetic component. Gene mapping studies have made substantial progress in unravelling the aetiology of dystonias and Huntington's disease, and are underway in other disorders such as essential tremor and Gilles de la Tourette syndrome. These advances are already applicable to clinical practice, particularly in Huntington's disease where identification of the disease mutation has led to the possibility of improved diagnosis and more widespread availability of predictive testing for asymptomatic family members.

Age of onset

The age of onset is the age at which an individual acquires, develops, or first experiences a condition or symptoms of a disease or disorder. For instance, the general age of onset for the spinal disease scoliosis is "10-15 years old," meaning that most people develop scoliosis when they are of an age between ten and fifteen years. Diseases are often categorized by their ages of onset as congenital, infantile, juvenile, or adult. Missed or delayed diagnosis often occurs if a disease that is typically diagnosed in juveniles (such as asthma) is present in adults, and vice versa (such as arthritis). Depending on the disease, ages of onset may impact features such as phenotype, as is the case in Parkinson's and Huntington's diseases. For example, the phenotype for juvenile Huntington's disease clearly differs from adult-onset Huntington's disease and late-onset Parkinson's exhibits more severe motor and non-motor phenotypes.

Central nervous system disease

Tourette's Tourette's syndrome is an inherited neurological disorder. Early onset may be during childhood, and it is characterized by physical and verbal tics. Tourette's often also includes symptoms of both OCD and ADHD indicating a link between the three disorders. The exact cause of Tourette's, other than genetic factors, is unknown. Neurodegenerative disorders Alzheimer’s Alzheimer's is a neurodegenerative disease typically found in people over the age of 65 years. Worldwide, approximately 24 million people have dementia; 60% of these cases are due to Alzheimer's. The ultimate cause is unknown. The clinical sign of Alzheimer's is progressive cognition deterioration. Huntington's disease

Parkinson-Plus Syndrome -- Introduction

Less prevalent disorders are frontotemporal dementia, Pick disease, pallidonigral degeneration, parkinsonian-dementia complex of Guam, Wilson disease, and a rigid variant of Huntington disease. [5] [6] This review will discuss the most common PPS conditions, focusing on epidemiology, etiology, pathophysiology, evaluation, and treatment options.

List of MeSH codes (C10)

– high pressure neurological syndrome – meningitis – meningoencephalitis – lupus vasculitis, central nervous system – movement disorders – angelman syndrome – choreatic disorders – chorea gravidarum – huntington disease – dystonic disorders – dystonia musculorum deformans – meige syndrome – torticollis – essential tremor – hallervorden-spatz syndrome – hepatolenticular degeneration – multiple system atrophy – olivopontocerebellar atrophies – shy-drager syndrome – striatonigral degeneration – parkinsonian disorders – lewy body disease – parkinson disease – parkinson disease, secondary – mptp poisoning – parkinson disease, postencephalitic – supranuclear palsy, progressive – Tic disorders – Tourette syndrome – ocular motility disorders – pneumocephalus

Tourette's syndrome in the year 2000.

To examine the current status of knowledge of Tourette's syndrome (TS) and to highlight those areas of research that are most likely to have the most significant advances in the next few years into the 21st century. Index Medicus was consulted from its beginning in 1885 until 1964 (inclusive), looking initially under the title 'tics' and subsequently under 'Tourette's Disorder'. From 1965 and the advent of MEDLINE, a search was performed looking for 'Tourette's'. A chronological examination of TS in the medical literature is presented. Some issues surrounding Huntington's Disease (HD) research are pertinent to TS and may serve as a guide in the future direction of TS research; these issues have been identified and illustrated in the context of TS. Where relevant, other medical disorders are also commented on. There has been a steady increase in the volume of TS literature since 1885, with a marked increase since 1980, changing in focus from a psychological to a neurobiological viewpoint. Current areas of interest include genetics, comorbid psychopathologies, neuroimaging, treatments, epidemiology and educational considerations. Issues raised by HD and other movement disorders such as Parkinson's Disease (PD) are predictive testing, gene therapy and neural transplants. The year 2000 is likely to herald a significant increase in our knowledge of the genetics and neuroimaging of TS, with new developments in therapy. A broadening of awareness of TS among health workers and teachers in particular is likely to increase the number of diagnosed patients and, hence, new challenges will be posed to existing resources for health and educational provision.

Joseph Jankovic

Past president of the International Parkinson and Movement Disorder Society Past president of the International Neurotoxin Association Honorary Member of the American Neurological Association, Australian Association of Neurologists, French Neurological Society, and the International Parkinson’s Disease and Movement Disorders Society Selected as “Great Teacher” by the National Institute of Health Fellow of the American Academy of Neurology American Academy of Neurology Movement Disorders Research Award First National Parkinson Foundation Distinguished Service Award Huntington’s Disease Society of America Guthrie Family Humanitarian Award Tourette Syndrome Association Lifetime Achievement Award Dystonia Medical Research Foundation Distinguished Service Award Texas Neurological Society Lifetime Achievement Award Baylor College of Medicine Alumni Association Distinguished Faculty Award Baylor College of Medicine Fulbright & Jaworski Faculty Excellence Award

Parkinson's disease

Multiple causes can occur for parkinsonism or diseases that look similar. Stroke, certain medications, and toxins can cause "secondary parkinsonism" and need to be assessed during visit. Parkinson-plus syndromes, such as progressive supranuclear palsy and multiple system atrophy, must also be considered and ruled out appropriately due to different treatment and disease progression (anti-Parkinson's medications are typically less effective at controlling symptoms in Parkinson-plus syndromes). Faster progression rates, early cognitive dysfunction or postural instability, minimal tremor, or symmetry at onset may indicate a Parkinson-plus disease rather than PD itself.

Eye Movement Disorders in Movement Disorders.

Oculomotor assessment is an essential element of the neurological clinical examination and is particularly important when evaluating patients with movements disorders. Most of the brain is involved in oculomotor control, and thus many neurological conditions present with oculomotor abnormalities. Each of the different classes of eye movements and their features can provide important information that can facilitate differential diagnosis. This educational review presents a clinical approach to eye movement abnormalities that are commonly seen in parkinsonism, ataxia, dystonia, myoclonus, tremor, and chorea. In parkinsonism, subtle signs such as prominent square wave jerks, impaired vertical optokinetic nystagmus, and/or the "round the houses" sign suggest early progressive supranuclear gaze palsy before vertical gaze is restricted. In ataxia, nystagmus is common, but other findings such as oculomotor apraxia, supranuclear gaze palsy, impaired fixation, or saccadic pursuit can contribute to diagnoses such as ataxia with oculomotor apraxia, Niemann-Pick type C, or ataxia telangiectasia. Opsoclonus myoclonus and oculopalatal myoclonus present with characteristic phenomenology and are usually easy to identify. The oculomotor exam is usually unremarkable in isolated dystonia, but oculogyric crisis is a medical emergency and should be recognized and treated in a timely manner. Gaze impersistence in a patient with chorea suggests Huntington's disease, but in a patient with dystonia or tremor, Wilson's disease is more likely. Finally, functional eye movements can reinforce the clinical impression of a functional movement disorder.

Wilson's disease: two treatment modalities. Correlations to pretreatment and posttreatment brain MRI.

Brain magnetic resonance imaging (MRI) studies on Wilson's disease (WD) show lack of correlations between neurological and neuroimaging features. Long-term follow-up reports with sequential brain MRI in patients with neurological WD comparing different modalities of treatment are scarce. Eighteen patients with neurological WD underwent pretreatment and posttreatment brain MRI scans to evaluate the range of abnormalities and the evolution along these different periods. All patients underwent at least two MRI scans at different intervals, up to 11 years after the beginning of treatment. MRI findings were correlated with clinical picture, clinical severity, duration of neurological symptoms, and treatment with two different drugs. Patients were divided into two groups according to treatment: D: -penicillamine (D-P), zinc (Zn), and Zn after the onset of severe intolerance to D-P. MRI scans before treatment showed, in all patients, hypersignal intensity lesions on T2- and proton-density-weighted images bilaterally and symmetrically at basal nuclei, thalamus, brain stem, cerebellum, brain cortex, and brain white matter. The most common neurological symptoms were: dysarthria, parkinsonism, dystonia, tremor, psychiatric disturbances, dysphagia, risus sardonicus, ataxia, chorea, and athetosis. From the neurological point of view, there was no difference on the evolution between the group treated exclusively with D-P and the one treated with Zn. Analysis of MRI scans with longer intervals after the beginning of treatment depicted a trend for neuroimaging worsening, without neurological correspondence, among patients treated with Zn. Neuroimaging pattern of evolution was more favorable for the group that received exclusively D-P.

X-linked dystonia parkinsonism

X-linked dystonia parkinsonism (XDP), also known as Lubag Syndrome or X-linked Dystonia of Panay, is a rare x-linked progressive movement disorder with high penetrance found almost exclusively in males from Panay, Philippines. It is characterized by dystonic movements first typically occurring in the 3rd and 4th decade of life. The dystonic movements often either coexist or develop into parkinsonism within 10 years of disease onset. Symptoms and signs Symptoms typically present in the 3rd or 4th decade of life, but have been seen as early as the age of 14. It presents with torsion dystonia, particularly when presenting at a younger age, which then progresses to parkinsonism with or without ongoing dystonia. Often the two symptoms coexist. The parkinsonian features of x-linked dystonia parkinsonism include festinating gait, bradykinesia, blepharospasm, and postural instability. It often lacks a resting tremor, helping to differentiate it from Parkinson's disease. Genetics

Dystonia

An accurate diagnosis may be difficult because of the way the disorder manifests itself. Sufferers may be diagnosed as having similar and perhaps related disorders including Parkinson's disease, essential tremor, carpal tunnel syndrome, temporomandibular joint disorder, Tourette's syndrome, conversion disorder or other neuromuscular movement disorders. It has been found that the prevalence of dystonia is high in individuals with Huntington's disease, where the most common clinical presentations are internal shoulder rotation, sustained fist clenching, knee flexion, and foot inversion. Risk factors for increased dystonia in patients with Huntington's disease include long disease duration and use of antidopaminergic medication.

Neurological features and outcomes of Wilson's disease: a single-center experience.

Wilson's disease (WD) is an autosomal recessive genetic disorder of copper metabolism, and WD patients can present with neurologic symptoms. We aimed to report the general characteristics and prognosis of a Turkish series of WD patients with neurological manifestations. A total of 12,352 patients were screened from the patient database, and 53 WD patients were included. Patients were classified based on the predominant neurological syndrome type including tremor, dystonia, parkinsonism, or discrete neurological signs and were classified as having "good outcome," "stable," and "poor outcome" according to their treatment response. There were 32 male and 21 female patients, aged 20-66 years. The mean follow-up was 11.3 ± 4.56 years. Sixty-two percent of patients presented predominantly with neurological symptoms. Neurological WD diagnosis was established after a mean time delay of 2.3 years from the WD diagnosis. The most common neurological manifestation was dystonia, followed by tremor and parkinsonism. Fifteen patients had a family history of WD. Consanguinity was present in 20 patients. Patients were treated with D-penicillamine, trientine, zinc salts, or their combinations. Besides the main treatments, 41 patients were on symptomatic treatment for neurologic symptoms. Thirty-six patients had a "good outcome," five patients were stable, and six patients had "poor outcome." Post-chelation neurological worsening was observed in 11 patients. WD should be considered in differential diagnosis in any patient with unexplained neurologic symptoms. Early diagnosis is important, and appropriate treatment should be promptly initiated to prevent progressive and irreversible damage, with good prognosis and stable disease in the majority of the patients with treatment compliance.

Tetrahydrocannabinol

Neurodegenerative disorders Huntington disease. No reliable conclusions could be drawn regarding the effectiveness of THC or oral cannabis extract in treating the symptoms of Huntington disease as the available trials were too small to reliably detect any difference Parkinson's disease. Based on a single study, oral CBD extract was rated probably ineffective in treating levodopa-induced dyskinesia in Parkinson's disease. Alzheimer's disease. A 2009 Cochrane Review found insufficient evidence to conclude whether cannabis products have any utility in the treatment of Alzheimer's disease. Other neurological disorders Tourette syndrome. The available data was determined to be insufficient to allow reliable conclusions to be drawn regarding the effectiveness of oral cannabis extract or THC in controlling tics. Cervical dystonia. Insufficient data was available to assess the effectiveness of oral cannabis extract of THC in treating cervical dystonia.

Psychiatric and behavioral abnormalities in Wilson's disease.

From the literature and our experience, a relatively consistent picture of psychiatric and behavioral abnormalities in Wilson's disease emerges. The essential elements of this picture are as follows: 1. Psychiatric and behavioral abnormalities are frequent manifestations of WD. The estimates range from 30% (18) to 100% (2) of symptomatic patients. As Wilson himself was the first to state in reference to "mental change," "its importance should not be underestimated." 2. Psychiatric and behavioral abnormalities are often the initial manifestations of WD. Two thirds of our patients first presented with psychiatric symptoms and one third received psychiatric treatment before the diagnosis of WD was made. In the early stages of the disease, when psychiatric and behavioral symptoms predominate, the diagnosis is often missed. Of our 124 patients, WD was diagnosed in only one during this phase. Until the psychiatric presentation of WD is recognized, and the disease is included in the differential diagnosis of psychiatric symptoms, its diagnosis will be missed or delayed. In our patients, and others' (13,15), the delay in diagnosis ranged from 1 to 5 years. Such a delay is particularly tragic as favorable outcome depends upon early discovery. 3. The most common of the psychiatric and behavioral manifestations of WD include: personality changes such as irritability and low threshold to anger, depression sometimes leading to suicidal ideation and attempts, deteriorating academic and work performance that is present in almost all neurologically affected patients. We (1) have also observed, as did Scheinberg and Sternlieb (2) that WD patients exhibit increased sexual preoccupation and reduced sexual inhibition. Finally, cognitive impairment, psychosis, anxiety, and other psychiatric disorders, although less frequent, also occur. 4. Some of the psychiatric and behavioral symptoms are reversible with WD-specific therapy, whereas others are not. We are impressed with the frequency with which the behavioral and "cognitive" symptoms are reversed over 1 to 2 years of treatment.(ABSTRACT TRUNCATED AT 250 WORDS)

Internal globus pallidus

Clinical significance Dysfunction of the internal globus pallidus has been correlated to Parkinson's disease, Tourette syndrome, and tardive dyskinesia. The internal globus pallidus is the target of deep brain stimulation (DBS) for these diseases. Deep brain stimulation sends regulated electrical pulses to the target. In patients with tardive dyskinesia treated with DBS, most people reported more than a 50% improvement in symptoms. Tourette syndrome patients have also benefited from this treatment, showing over 50% improvement in tic severity (compulsive disabling motor tics are symptoms of Tourette patients). The GPi is also considered a "highly effective target for neuromodulation" when using deep brain stimulation on Parkinson's disease patients. There is seen to be only some involvement in Huntington's disease with mostly the external globus pallidus being affected. References Basal ganglia

The representation of movement disorders in fictional literature.

This review considers novels, plays and poems dealing with movement disorders in order to show the relevance in the literary context. The motifs are arranged and compared following a modern neurological nosology according to Parkinson syndromes, dystonia, myoclonus, tics, hemifacial spasm, Tourette syndrome, Huntington's disease and hyperekplexia. There is considerable variety in how movement disorders are depicted and how much influence they have on the plot structures. Their usage ranges from a brief reference in order to accentuate aspects of a character's personality or social position, such as in Shakespeare, Dickens, Tolstoy or Galdós; to truly constituting one of the plot's main themes as, for example, with the representation of Lewy body disease in Franzen's The Corrections and Huntington's disease in Vonnegut's Galápagos, Sawyer's Frameshift or McEwan's Saturday. The symbolic connotation of the disease is of major importance, as is its social and psychological impact. Some 20th century authors transfer rhythm patterns of specific movement disorders into the textual structure, including, among others, Beckett.

Tourettism

Conditions that may manifest tics or stereotyped movements include developmental disorders; autism spectrum disorders and stereotypic movement disorder; Sydenham's chorea; idiopathic dystonia; and genetic conditions such as Huntington's disease, neuroacanthocytosis, pantothenate kinase-associated neurodegeneration, Duchenne muscular dystrophy, Wilson's disease, and tuberous sclerosis. Other possibilities include chromosomal disorders such as Down syndrome, Klinefelter syndrome, XYY syndrome and fragile X syndrome. Acquired causes of tics include drug-induced tics, head trauma, encephalitis, stroke, and carbon monoxide poisoning. The symptoms of Lesch–Nyhan syndrome may also be confused with Tourette syndrome. Tic mimickers Chorea (disease) Myoclonus Dystonia Torsion dystonia Idiopathic dystonia Genetic/chromosomal

Juvenile Huntington's disease: two case reports and a review of the literature.

Huntington's disease is a rare, autosomal dominant neurodegenerative disease characterized by motor, cognitive, and psychiatric symptoms. Usually, the disease symptoms first appear around the age of 40, but in 5-10% of cases, they manifest before the age of 21. This is then referred to as juvenile Huntington's disease. According to the small number of cases reported in the literature, the course of juvenile Huntington's disease significantly differs from adult onset and shows significant interpatient variability, making every case unique. Our study aims to highlight the complexity and diversity of rare juvenile Huntington's disease. We report cases of two Caucasian patients with chronic tics referred to the Huntington's Disease Competence Center of Vilnius University Hospital Santaros Klinikos with suspicion of juvenile Huntington's disease due to the appearance of chronic motor tics, and behavior problems. The diagnosis of juvenile Huntington's disease was confirmed on both clinical and genetic grounds. In both cases described, the patients developed symptoms in all three main groups: motor, cognitive, and psychiatric. However, the first patient was experiencing more severe psychiatric symptoms; in the second case, motor symptoms (rigidity, tremor) were more prominent. In both cases, apathy was one of the first symptoms and affected patients' motivation to participate in treatment actively. These two case descriptions serve as an important message for clinicians seeing patients with chronic tics and gradually worsening mood and behavior, indicating the need to investigate them for rare genetic disorders. Description of these two clinical cases of juvenile Huntington's disease provides insight into how differently it manifests and progresses in young patients and the difficulties the patients and their families face. There were different but painful ways for families to accept the diagnosis. Because the disease inevitably affects the patient's closest ones, it is crucial to also provide adequate psychological and social support to all the family members. Establishment of multidisciplinary specialist centers for Huntington's disease, as demonstrated by our experience, not only allows timely diagnosis and treatment plans but also ensures thorough disease management and care for patients and systematic support for their families.

Whose name is it anyway? Varying patterns of possessive usage in eponymous neurodegenerative diseases.

There has been long-standing debate over whether use of the possessive form of the names of eponymous neurological disorders should be abandoned. Which view has actually predominated in practice? We empirically assessed current and historical usage in the scientific literature. The PubMed database was queried for the percentage of titles published each year from 1960-2012 which contained the possessive form of Parkinson's (PD), Alzheimer's (AD), Huntington's (HD), Wilson's (WD), and Gaucher's (GD) diseases (e.g. Huntington's disease or chorea vs Huntington disease or chorea). Down syndrome (DS), well known for its changes in terminology, was used as a reference. The possessive form was nearly universal in all conditions from 1960 until the early 1970s. In both DS and GD it then declined at an approximately constant rate of 2 percentage points per year to drop below 15%. The possessive forms of both PD and AD began to decline at the same time but stabilised and have since remained above 80%, with a similar but more volatile pattern in HD. WD, meanwhile, is intermediate between the DS/GD and PD/AD/HD patterns, with a slower decline to its current value of approximately 60%. Declining possessive form usage in GD and DS papers has been remarkably uniform over time and has nearly reached completion. PD and AD appear stable in remaining predominantly possessive. The larger volume of papers published in those fields and their possibly greater public recognition and involvement may make that unlikely to change in the short-term. In a secondary analysis restricted to PD, we found that practices have switched dramatically several times in each of three US-published general neurology journals. Meanwhile, in two UK-published journals, and in the specialist title "Movement Disorders", the possessive form has been maintained consistently. The use of eponyms in neurology shows systematic variation across time, disorders, and journals.