Claude-Bernard-Horner syndrome is an old acquaintance of the MIR exam. We do not see it daily in clinical practice, but it appears in many exams. Characteristic is upper eyelid ptosis and miosis of that eye. This occurs due to a deficit of sympathetic innervation of the orbital territory. The sympathetic nervous system innervates the Müller muscle, which is an accessory muscle to the levator of the upper eyelid. If the Müller ceases to function, the eyelid descends slightly. It also innervates the iris dilator muscle. Therefore, the sphincter muscle, its antagonist, is unopposed and the balance of the pupil shifts toward miosis. It explains ptosis and miosis of the left eye, so we suspect Horner's in that eye. She also describes what may have happened: after a possible sudden movement while playing tennis, she has neck pain and a Horner's. We should suspect an aneurysm. We should suspect a dissecting carotid aneurysm, which is one of the best known (and most serious) causes of an acquired Horner's syndrome. The superior cervical ganglion is a ganglion of the sympathetic nervous system located in the sheath of the carotid artery, and at this level it has been affected. We can also try to get this answer right by ruling out. An alteration of the III cranial nerve can produce ptosis, but the pupil, if affected, should be in mydriasis. And damage to the third cranial nerve should also produce some type of ophthalmoplegia, with diplopia. An involvement of the optic chiasm would produce a bilateral loss of vision (probably a bitemporal hemianopsia). We are told in the statement that he maintains good visual acuity. On the other hand, there would also be no ptosis or anisocoria. And finally, an alteration of the medulla oblongata would produce different neurological symptoms not described, and there should be no ptosis or pupillary alterations, because the third pair is located in the midbrain and the sympathetic nuclei in the spinal cord.

A 25-year-old patient, during a tennis match, has severe pain in his neck and left eye. The next morning he wakes up with a feeling of gait instability and has palpebral ptosis of the left eye and anisocoria, with the left pupil being smaller than the right. The patient maintains good visual acuity, where would you most likely locate the lesion?

[Bilateral internuclear ophthalmoplegia in association with basilar artery occlusive disease].

A 56 year-old man presented with vertigo and the right sided weakness. Neurological examination revealed a lethargic man with good orientation to three spheres. His neck was supple. He had anisocoria, the right pupil being larger than the left by 1.5 mm with sluggish light reaction bilaterally. He had exotropia of the right eye in primary gaze. The abduction of both eyes were full with terminal horizontal nystagmus. The adduction of both eyes were quite limited in each eye. He had a limited upward gaze with poor convergence. These were interpreted as the syndrome of the medial longitudinal fasciculus (MLF) bilaterally. He had a depressed gag reflex on the right side with tongue deviation to the right. He had a mild weakness of the right side limb and also had the right sided hemihyperesthesia including his face to pain and temperature. Twenty four hours after the onset, the left brachial angiography revealed a complete occlusion of the rostral portion of the basilar artery without visualization of the posterior cerebral and superior cerebellar arteries bilaterally. CT scans three days after the onset revealed a low density area in the mid pons with extension rostrally up to the mesencephalon. Four days later he became quadriplegic with bilateral horizontal gaze palsy. No more internuclear ophthalmoplegia is noted on both sides. The midline location of the MLF in the pons, and the separate blood supplies by different paramedian branches of the basilar artery, form the anatomical explanation for the frequent unilaterality of vascular and bilaterality of demyelinating lesions. Bilateral MLF syndrome has been considered almost pathognomonic of multiple sclerosis.(ABSTRACT TRUNCATED AT 250 WORDS)

[A 56-year-old man with fever, backache and tetraparesis].

We report a 56-year-old man who developed progressive paraparesis. He was apparently well, except for left Bell's palsy which developed on May 9 of 1994, for which he received stellate ganglion block on the left side more than ten times until July 2nd of 1994, when he noted pain in his left shoulder and in his lumbar region. On July 5th, he noted some difficulty in urination. On July 6th, he noted tingling sensation in his four extremities and difficulty in gait. He was admitted to another hospital where he was treated with intravenous infusion of glycerol. After this treatment, his gait and sensory disturbance showed some improvement, however, on July 7th, his shoulder and lumbar pain worsened, and he became unable to stand. His temperature went up to 39 degrees C on the next day. Lumbar CSF on that day contained 119 cells/microliters, 112 mg/dl of protein, and 53 mg/dl of sugar. He was transferred to our hospital on July 14th. His past medical history revealed that he had suffered from frequent bouts of osteomyelitis since the age of 13 years. He was operated on several times on osteomyelitis. He had been treated on his tooth ache until shortly before the onset of the present illness. He also received steroid hormone for his Bell's palsy. On admission, his consciousness varied from alert to stupor. His BP was 150/100 mmHg, HR 98/min and regular, BT 39.4 degrees C. The bulbar conjunctiva appeared somewhat icteric. Otherwise, general physical examination was unremarkable. On neurologic examination, there was no apparent dementia. Higher cerebral functions appeared intact. The optic discs were flat. Pupils were round and isocoric reacting to light and accommodation promptly. Ocular movements were full without nystagmus. Some exophthalmos was noted bilaterally. The sensation of the face and facial muscles were intact. The remaining cranial nerves also appeared intact. Nuchal rigidity was present. He was unable to stand or walk. Muscle strength was markedly diminished in all four limbs; manual muscle testing revealed 1 to 2/5 weakness in both upper and lower extremities bilaterally. Muscle stretch reflexes were decreased or lost in both upper and lower limbs, but the plantar response was extensor on the right. Sensation appeared to be diminished in legs, but detail was not clear because of disturbance of consciousness. Pertinent laboratory findings were as follows: WBC 12,800/microliter, GPT 58 IU/l, total bilirubin 2.65 mg/dl, and CRP 16.8 mg/dl. Cerebrospinal fluid contained 34 cells/microliter (approximately two thirds were neutrophils), RBC 1,110/microliter, 2,949 mg/dl of protein, and 119 mg/dl of glucose; stapylococcus aureus was cultured from the CSF. Myelogram showed a filling defect in the anterior epidural space between the low thoracic and the upper lumbar region. The patient was treated with cephotaxim, aminobenzyl penicillin, and chloramphenicol. On the second hospital day, his BT was still 39 degrees C and he was agitated His weakness was worse than the previous day. Spinal MRI was attempted; as he was agitated 5 mg of diazepam was given intravenously at 4 PM. His respiration was rapid and somewhat shallow. At 6 PM, gadolinium DTPA was injected intravenously; at that time, he was breathing and pupils were 3 mm on both sides. At 6:35 PM, an examiner noted that he stopped breathing; the left pupil was dilated to 5 mm. Cardiopulmonary resuscitation was initiated immediately, and intubation was performed. He was placed on a respirator. His blood pressure did not reach 100 mmHg; he was in deep coma. Cardiac arrest occurred at 8:53 AM on the next morning. The patient was discussed in a neurological CPC. Most of the participants thought that the patient had either spinal epidural empyema or spinal subdural abscess. The question was what might be the original focus of infection. Three possibilities were considered, i.e., stellate ganglion block, teeth infection, and osteomyelitis...

An Interesting Case of Pupillary Changes During the Testing of Ocular Movements and its Influence on the Diagnosis?

To describe an unusual case presentation of a decompensating exotropia with an incidental pupil anomaly highlighting the importance of observing pupils during the assessment of ocular movements. A case whose signs included an exotropia and an enlarged tonic pupil which were initially mistaken for an atypical case of Oculomotor nerve palsy, triggering immediate imaging investigations. We discuss how understandable it would be for experienced clinicians to arrive at this case's misdiagnosis and how it could have been avoided thereby helping to preserve where possible costly resources of neuro imaging and inpatient stays. A 71 year old man who was seen as a tertiary referral case by the Queen Elizabeth Hospital Birmingham's (QEHB's) neuro-ophthalmology service, where a second opinion for the patient was sought from a neurology team from another hospital. He was seen by the orthoptist and neuro-ophthalmologist consultant. On presentation at QEHB visual acuity measured 6/9 Snellens in each eye with varifocals, improving to 6/6 with pinhole testing, the fundi and optic discs were normal. Anisocoria was noted with the left pupil being larger than the right pupil. Orthoptic assessment revealed a small angle left exotropia on cover test, increasing in size on alternate cover test with a blink type recovery to its original angle. An orthoptic diagnosis of a decompensating left microexotropia with identity was given. In the left eye there was a slight mechanical restriction in adduction, underacting superior rectus and a larger underacting inferior rectus with an "A" type alphabet pattern with symptoms of horizontal diplopia on all right gaze positions. There was no evidence of ptosis, convergence was intact and saccades were fast and appeared of a normal velocity. The anisocoria appeared more obvious when the patient looked to right gaze where his left pupil seemed to enlarge further. Assessment of the pupillary function led to the patient being diagnosed as having bilateral asymmetrical Adies (tonic) pupils. This case highlights the importance of not only carrying out a detailed pupil reaction assessment but also the necessity of observing the pupils during the assessment of ocular movements. This case highlights how anisocoria can mislead a clinician's attention to believing that only one particular pupil is abnormal where as it could be both. Also it highlights that the classic combination of symptoms and observations of diplopia, exotropia, longstanding incomitance and anisocoria in terms of a dilated pupil may not necessarily be an Oculomotor cranial nerve palsy and requiring the patient to undergo imaging investigations on an urgent basis may be avoided.

[A 62-year-old man with an acute onset of consciousness disturbances].

We report a 62-year-old man who developed coma and died in a fulminant course. The patient was well until May 1, 1996 when he noted chillness, tenderness in his shoulders, and he went to bed without having his lunch and dinner. In the early morning of May 2, his families found him unresponsive and snoring; he was brought into the ER of our hospital. He had histories of hypertension, gout, and hyperlipidemia since 42 years of the age. On admission, his blood pressure was 120/70, heart rate 102 and regular, and body temperature 36.3 degrees C. His respiration was regular and he was not cyanotic. Low pitch rhonchi was heard in his right lower lung field. Otherwise general physical examination was unremarkable. Neurologic examination revealed that he was somnolent and he was only able to respond to simple questions such as opening eyes and grasping the examiner's hand, but he was unable to respond verbally. The optic discs were flat; the right pupil was slightly larger than the left, but both reacted to light. He showed ptosis on the left side, conjugate deviation of eyes to the left, and right facial paresis. The oculocephalic response and the corneal reflex were present. His right extremities were paralyzed and did not respond to pain Deep tendon reflexes were exaggerated on the right side and the plantar response was extensor on the right. No meningeal signs were present. Laboratory examination revealed the following abnormalities; WBC 18,400/ml, GOT 131 IU/l GPT 50 IU/l, CK616 IU/l, BUN 30 mg/dl, Cr 2.1 mg/ dl, glucose 339 mg/dl, and CRP 27.4 mg/dl. ECG showed sinus tachycardia and ST elevation in II, III and a VF leads and abnormal q waves in I, V5, and V6 leads. Chest X-ray revealed cardiac enlargement but the lung fields were clear. Cranial CT scan revealed low density areas in the left middle cerebral and left posterior cerebral artery territories. The patient was treated with intravenous glycerol infusion and other supportive measures. At 2: 10 AM on May 3, he developed sudden hypotension and cardiopulmonary arrest. He was pronounced dead at 3:45 AM. The patient was discussed in a neurological CPC, and the chief discussant arrived at the conclusion that the patient had acute myocardial infarction involving the inferior and the true posterior walls and left internal carotid embolism from a mural thrombus. Post mortem examination revealed occlusion of the circumflex branch of the left coronary artery due to atherom plaque rupture and myocardial infarction involving the posterior and the lateral wall with a rupture in the postero-lateral wall. Marked atheromatous changes were seen in the left internal carotid, the middle cerebral and the basilar arteries; the left internal carotid and the middle cerebral arteries were almost occluded by thrombi and blood coagulate. The territories of the left middle cerebral and the occipital arteries were infarcted; but the left thalamic area was spared. The neuropathologist concluded that the infarction was thrombotic origin not an embolic one as the atherosclerotic changes were severe. Cardiac rupture appeared to be the cause of terminal sudden hypotension and cardiopulmonary arrest. It appears likely that a vegetation which had been attached to the aortic valve induced thromboembolic occlusion of the left internal carotid artery which had already been markedly sclerotic by atherosclerosis. It is also possible that the vegetations in the aortic valve came from mural thrombi at the site of acute myocardial infarction, as no bacteria were found in those vegetations.

[An adult case of congenital Horner's syndrome with heterochromia iridis--with special reference to alteration of Horner's sign associated with development].

It is well known that when the Horner's syndrome is congenital, a defect in pigmentation of the iris is usual; all or part of the iris remains light brown. We reported an adult case of congenital Horner's syndrome with remission and relapse of unilateral ptosis. A 25-year-old man was admitted to our hospital for ophthalmologic surgical treatment of right ptosis. According to the patient's mother, the patient was delivered with the aid of forceps at birth, and the right ptosis was observed during the first few days of his life. At 2 to 3 years of age, his parents noted lighter color of the right eye. The right ptosis was gradually improved as he grew older. However, he developed right ptosis again with left meralgia paresthesia since eighteen age. At age 25 years, he was noted to have right ptosis, right miosis (the left pupil measured 4.5 mm in diameter and the right 3.0 mm), right heterochromia iridis with pigmented iris nevi, and left meralgia paresthesia . Laboratory data of urine, blood and CSF as well as radiological studies of chest X-ray, skull X-ray, spine X-ray, brain MRI and spinal cord MRI showed unremarkable. Sweating test was intact, pharmacologic test to Horner's syndrome with 5% cocaine and 1.25% 1-epinephrine indicated that the damage was pointed to the post ganglionic sympathetic neuron. Ten patients with congenital Horner's syndrome reported in Japan since 1953 were reviewed including our case. Ten of eleven were male and Horner's sign was recorded on the left eye in 8 cases.(ABSTRACT TRUNCATED AT 250 WORDS)

[A 76-year-old man with loss of vision and dementia].

We report a 76-year-old man who developed blurred vision and dementia. He was apparently well until April 4, 1990 (70-year-old at that time) when he had a sudden onset of bilateral loss of vision. Corrected vision was 0.1 (right) and 0.09 (left). He was admitted to the ophthalmology service of our hospital on April 9, 1990, and neurological consultation was asked on April 11. Neurologic examination revealed alert and oriented man without dementia. Higher cerebral functions were intact. He had bilateral large visual field defects with loss of vision; he was only able to count the digit number with his right eye and to recognize hand movement with his left eye. Otherwise neurologic examination was unremarkable. General physical examination was also unremarkable; he had no hypertension. Cranial CT scan was normal on April 11; lumber spinal fluid contained 1 cell/microliter, 63 mg/dl of sugar, and 97 mg/dl of protein; myelin basic protein was detected, however, oligoclonal bands were absent. He was treated with methylprednisolone pulse therapy and oral steroid, however, no improvement was noted in his vision. He started to show gaze paresis to left, ideomotor apraxia, agnosia of the body, and dementia. Cranial CT scan on June 11 revealed a low density area in the deep left parietal white matter facing the trigonal area of the lateral ventricle. He was discharged on July 2, 1990. Hasegawa dementia scale was 2/32.5 upon discharge. In the subsequent course, he showed improvement in his mental capacity and Hasegawa dementia scale was 22.5/32.5 in 1991, however, no improvement was noted in his vision. In 1994, he started to show mental decline in that he became disoriented, and showed delusional ideation of self persecution and depersonalization with occasional confusional state. He also showed unsteady gait. Cranial MRI on February 13, 1996 revealed a T2-high signal intensity lesion on each side of the parietal deep white matter more on the left and another T2-high signal intensity lesion in the left pons as well as in the right thalamus. He complained of right hypochondrial pain and was admitted to another hospital on April 22, 1996. He was markedly confused and demented. He continued to show bilateral loss of vision, but no motor palsy was noted. Cranial CT scan on April 23, 1996 revealed diffuse cortical atrophy and ventricular dilatation in addition to the low density areas in both parietal deep white matter. He developed jaundice in the middle of May. Abdominal CT scan revealed multiple low-to iso-density areas in the liver and marked iso-to high-density swelling of the right kidney. The patient expired on June 9th, 1996. The patient was discussed in a neurological CPC and the chief discussant arrived at the conclusion that the patient had had a carcinomatous limbic encephalitis with optic neuropathy and a choleduct carcinoma. Other opinions entertained included acute disseminated encephalomyelitis with optic neuritis, and granulomatous angiitis of the central nervous system. Some participants thought the primary site of the carcinoma was the right kidney with metastasis to the liver. Post mortem examination revealed a mixed type carcinoma in the right kidney with liver metastases. Neuropathologic examination revealed an incomplete softening in the optic chiasm and the left optic nerve, and in the left parieto-occipital areas. (The right hemisphere was frozen for future biochemical assay.) One of the adjacent cortical arteries had an organized thrombus. Other arteries and arterioles also showed sclerotic changes. Some of the leptomeningeal arteries were positive for Congored staining as well as for beta-amyloid immunostaining. Many senile plaques were seen diffusely in the cerebral cortex and neurofibrillary tangles were seen in the CA1 area and the parahippocampal gylus. No cellular infiltrations or demyelinated foci were seen. The neuropathologic features were consistent with circulatory disturbance based on the amyloid angiopa

[Neuromyelitis optica with high aquaporin-4 expression and positive serum aquaporin-4 autoantibodies].

Disease-specific aquaporin-4 antibodies (NMO-IgG) are the main effector of lesions in neuromyelitis optica (NMO) patients. Brain MRI lesions are detected in 60% of them, with 8% (almost infants) at sites of high aquaporin-4 expression. Patient 1: A fifty-year-old male with loss of vision in the right eye. Empiric treatment with metilprednisolone 1g/d for 3 days was indicated. After 30 days he complained of generalized pain, and a right hemiparesis was evident. The patient received bolus of metilprednisolone 1g/d for 5 days plus IgG 400 mg/kg/d IV for 5 days. He recovered ambulation but persisted with pain and paroxysmal phenomena (Lhermitte). Visual Evoked Potentials (VEP): P100 left eye 123 ms, right eye without response. Brain MRI (FLAIR) showed hyperintensity in the right optic nerve, hypothalamus and anterior white commissure. Cervical MRI showed extensive spinal cord lesion to an extension of 5 vertebral bodies. Patient 2: A fifty-three-year-old female who referred decreased visual acuity in both eyes and paresthesia in lower limbs which subsided spontaneously. One month later the patient evolved with cuadriparesis and sphincter incontinence. No improvement was observed with bolus of metilprednisolone 1g/d for 5 day. VEP: P100 left eye 124 ms, right eye 128 ms. Brain MRI (FLAIR) disclosed hypothalamic and periaqueductal hyperintensity. Cervical MRI showed extensive spinal cord lesion to an extension of 7 vertebral bodies. NMO-IgG antibodies were positive in both patients (indirect immunofluorescence assay). NMO brain lesions at sites of high aquaporin-4 expression, once considered atypical for their topography and infrequency in adults, should be borne in mind when considering differential diagnosis.

Neurology_Adams

Normally, movement of the head at a rate of one to two cycles per second does not cause blurring of vision because of the rapidity with which the VOR accomplishes compensatory eye movements. Dynamic visual acuity is a term applied to testing by having the patient read an eye chart while the head is rotated back-and-forth. A substantial drop in acuity occurring with head movements at this speed is indicative of failure of the VOR. Zee has described yet another means of testing the VOR in which the examiner observes the optic nerve head while the patient rotates the head back and forth. With a normal VOR, the optic disc does not appear to move since the eye’s position in space has remained unchanged. However, if the VOR is impaired, the optic nerve head appears to oscillate. Visual Fixation Reflex (Suppression of the Vestibuloocular Reflex)

[An acute syndrome of opsoclonus and body tremulousness. A case report of benign encephalitis].

A 30-year-old woman was admitted to our hospital in May 1977, suffering from oscillation, body tremulousness and astasia-abasia. Two months prior to admission she had caught a common cold and felt paresthesia on the right arm. Three days before admission, she had photophobia, tremulousness of the trunk and head, and astasia-abasia. A neurological examination revealed difficulty in standing, and jerky movement of the body and right arm, her limbs were well coordinated. An ocular examination resulted in normal findings except for intermittent oscillation of the eyeballs. As the pupils and fundi were normal, so were the routine laboratory tests. The cerebrospinal fluid was clear and colorless under normal pressure, and showed no more than 6 mononuclear cells in any of the three examinations. CSF protein was registered 20 mg/dl, glucose 75 mg/dl, and no virus titers were elevated. Electroencephalogram was slightly abnormal due to a slowing of the Alpha wave in the background activity. Both computerized tomography and vertebral-angiography were normal. A slow and steady abatement of all symptoms started while she was taking steroid treatment in hospital. Electrooculogram showed irregular disconjugation of the eyes rotating or moving in horizontal and vertical direction. There movements conjugated or disconjugated in both eyes with 5-7 Hz of frequency and 10-20 degrees of amplitude. They decreased when the eyelids were closed or staying in a dark room. These ocular movements were regarded as opsoclonus. Then opsoclonus changed to flutter-like oscillation.(ABSTRACT TRUNCATED AT 250 WORDS)

Neurology_Adams

As noted in Chap. 12, about half of patients with optic neuritis recover completely, and most of the remaining ones improve significantly, even those who present initially with profound visual loss (Slamovitis et al). Pain around the eye is short-lived and persistent pain should prompt an evaluation for local disease. In a cohort of 397 patients enrolled in the Optic Neuritis Treatment Trial and examined 5 years after the initial attack of optic neuritis, visual acuity had returned to 20/25 or better in 87 percent of patients and to 20/40 or better in 94 percent—even if there had been a recurrence of optic neuritis during the 5-year period. Moreover, the mode of treatment did not appear to influence the final visual outcome. Dyschromatopsia and impaired contrast sensitivity frequently persist, as does the Pulfrich effect, wherein an object such as a pendulum that is swinging perpendicular to the patient’s line of sight appears to move in a three-dimensional, circular path.

[A rare cause of peracute vision loss: pseudotumor cerebri. Case report of course with recurrence after decompression of the optic nerve].

In primary pseudotumor cerebri (PTC) intracranial pressure is elevated by so far unknown mechanisms. There is a wide range of clinical courses. Therapy is controversial. We present a case of PTC with acute visual loss. After optic nerve sheath decompression a relapse occurred. A 30-year old female patient experienced visual loss within 48 h accompanied by headache and slight neck stiffness. Visual acuity was 1/50 in the right eye; in the left eye just hand movements and light were perceived. Fundoscopy revealed a 9 dptr. prominent optic disc bilaterally. After optic nerve sheath decompression (ONSD) she improved, but underwent a relapse after 3 months. Twenty-four-hour measurement of intracranial pressure revealed elevated values. As a consequence ventriculo-peritoneal shunting was performed, leading to prominent improvement. Primary PTC can cause acute visual loss. If conservative treatment fails, different surgical procedures should be considered.

Consultation section. Cataract surgical problem.

A 68-year-old diabetic Pakistani presented with a 3 year history of progressive bilateral painless loss of vision. Although he was previously untreated for glaucoma, his examination revealed the following: visual acuity, finger counting in the right eye and 20/60 in the left; intraocular pressure (IOP), 41 and 31 mm Hg, respectively. Physical findings included bilateral shallow anterior chambers, minimal nuclear and cortical cataract formation, and extensive glaucomatous optic nerve cupping and atrophy worse in the right eye than in the left. Gonioscopy revealed angles narrowed to grade 1 for 360 degrees in both eyes. Visual field analysis was commensurate with the marked degree of optic nerve damage and reduced visual acuity. Bilateral laser iridotomies and a combination of topical antiglaucoma agents reduced IOP to the mid-20s in both eyes. Subsequently, the right eye had an uneventful trabeculectomy with application of intraoperative mitomycin. Postoperatively, the patient developed posterior aqueous entrapment and was managed with topical cycloplegics, aqueous suppressants, and corticosteroids. After a few weeks, a shallow anterior chamber was present centrally, a filtration bleb was noted, and IOP was 9 mm Hg. Unexpectedly, the patient returned to his native country, discontinued medications, and was lost to follow-up for 1 year. Upon his return, he presented with these findings: visual acuity, hand motion in the right eye and 20/60 in the left eye; IOP, 10 and 30 mm Hg, respectively. Physical findings in the right eye (Figure 1) included a superiorly oriented, thin-walled filtration bleb, a formed anterior chamber with multiple broad peripheral and midperipheral synechias, and a bound pupil covering a dense nuclear sclerotic cataract. The left eye had progressive glaucomatous optic nerve changes. Given the patient's history, now would you manage the glaucoma in the left eye, and what surgical methods would you plan for the right eye, assuming cataract surgery is indicated?

Brain Death Criteria -- Clinical Significance -- I. Physical Examination

CN III, IV, VI: Loss of pupillary light reflex as mentioned above (CN III). For cranial nerves IV, VI, and VIII, the oculocephalic reflex can be tested by holding a patient's eyelids open and turning their head from side to side. A positive oculocephalic reflex is present when the eyes reflexively look in the position opposite to that of the direction the head is turning. When the oculocephalic reflex is absent, the eyes will turn in the same direction in which the head is turning (Doll's eyes). It is imperative to only perform this examination maneuver in patients with a stable cervical spine who are not in cervical spine precautions.

Upper cranial nerve palsy resulting from spontaneous carotid dissection.

Upper cranial nerve palsy has a variety of causes such as cerebral and nerve ischemia, diabetes, infectious and non-infectious meningitis, subarachnoid hemorrhage and intracranial aneurysm. CASE 1 : A 45-year-old man suffered from holocephalic headaches and a right-sided neck pain for two weeks. He presented to our emergency department because of a sudden ptosis of the right eye. On admission neurological examination revealed a right sided Horner's syndrome and hypesthesia of the right side of the face. Magnetic resonance angiography identified a circumscribed dissection of the right extracranial internal carotid artery originating from the carotid bifurcation. Conventional angiography 2 weeks later showed a nearly recanalized artery. CASE 2 : A 55-year-old previously healthy man without cardiovascular risk factors developed right sided neck pain when loading a seeder with several sacks of crop. A few hours later he noticed a left-sided weakness. On admission a severe left sided hemiparesis and a mild neglect were present. Duplex sonography revealed a right-sided distal internal carotid artery (ICA) occlusion. The next morning the patient complained of double vision; he had a right-sided pupil-sparing oculomotor palsy. The diagnosis of ICA dissection was confirmed by conventional angiography, at that time showing a partially recanalized ICA without involvement of the cavernous region by the dissection. ICA dissection must be included in the differential diagnosis of upper cranial nerve palsy and should be assessed by duplex ultrasound and magnetic resonance imaging. A possible explanation is nerve ischemia due to a transient or permanent interruption of the blood supply by compression of the vasa nervorum originating from the intracranial carotid artery.

A cataract, 'a million cut' radial keratotomy, and Marfan syndrome February consultation #1.

A 56-year-old man with an ocular history of 20+ cut radial keratotomy (RK) in both eyes and Marfan syndrome presented with blurred vision in both eyes 2 years previously. He was intolerant of contact lenses and was correctable with spectacles for the past 10 years. His presenting photographs and corneal topographies are shown in Figures 1 and 2JOURNAL/jcrs/04.03/02158034-202102000-00022/figure1/v/2021-04-12T204757Z/r/image-tiffJOURNAL/jcrs/04.03/02158034-202102000-00022/figure2/v/2021-04-12T204757Z/r/image-tiff, respectively. His left eye had greater than 270 degrees of zonulopathy and a visually significant cataract. He underwent a planned pars plana lensectomy/vitrectomy and implantation of a scleral-fixated CZ70BD (Alcon Laboratories, Inc.) intraocular lens (IOL). He has enjoyed adequate vision in the left eye and now has a worsening cataract in his right eye. He is a practicing dentist and requested the fastest visual rehabilitation possible. His corrected distance visual acuity was 20/50 with a manifest refraction of +5.00-4.00 × 90 in the right eye and 20/25 with a manifest refraction of +1.75-2.50 × 180 in the left eye. Intraocular pressure (IOP) was measured at 16 mm Hg in both eyes, and extraocular motility, confrontational visual fields, and pupils were normal in both eyes. On slitlamp examination, he had mild ptosis in both eyes, the corneas had 20+ RK with multiple arcuate incisions at the 3- and 9-o'clock positions in both eyes, the anterior chamber (AC) was deep and quiet in both eyes, both irides had mild iridodonesis, the right lens had a 2 to 3+ nuclear sclerotic cataract with 6 clock hours of superotemporal zonulopathy that was only evident with dilation (Figure 3JOURNAL/jcrs/04.03/02158034-202102000-00022/figure3/v/2021-04-12T204757Z/r/image-tiff) and no phacodonesis. The left lens had a well-positioned CZ70BD IOL fixated at 6 and 12 o'clock without extrusion or exposure of the Gore-Tex suture. The posterior segment examination was unremarkable. What counseling would you provide for this patient in preparation for surgery? How would you plan the IOL calculations? What intraoperative techniques would you use to achieve the safest outcomes given his comorbidities?

Doll's Eyes -- Definition/Introduction

The oculocephalic reflex is performed by holding a patient’s eyelids open and moving their head from side to side. The examination should only be performed on patients with a stable cervical spine without c-spine precautions. With the patient's eyelids open, the examiner briskly rotates the patient's head from side to side while the examiner observes the patient’s eyes. The examiner observes a positive oculocephalic reflex when the patient moves their eyes opposite of the rotation of their head, such that their eyes stay looking forward (like a doll’s eyes). The examiner observes a negative oculocephalic reflex when the patient’s eyes stay midline and do not move while the examiner rotates the head. A similar examination is performable for vertical eye movements. [6] Note that the designation of "positive" (eyes moving in the opposite direction of head movement indicating an intact brainstem function) or "negative" (eyes moving towards the same direction of head movement indicating severe brainstem dysfunction) doll's eyes reflex is an oversimplification and that some eye movement with nystagmus can occur in pathologic states. [7] The reflex is suppressed in a conscious adult with normal neurologic function but is active in a comatose patient with gross brainstem function, absent if there is damage to the reflex arc. [6]

Human brain

The cerebrum has a contralateral organisation with each hemisphere of the brain interacting primarily with one half of the body: the left side of the brain interacts with the right side of the body, and vice versa. The developmental cause for this is uncertain. Motor connections from the brain to the spinal cord, and sensory connections from the spinal cord to the brain, both cross sides in the brainstem. Visual input follows a more complex rule: the optic nerves from the two eyes come together at a point called the optic chiasm, and half of the fibres from each nerve split off to join the other. The result is that connections from the left half of the retina, in both eyes, go to the left side of the brain, whereas connections from the right half of the retina go to the right side of the brain. Because each half of the retina receives light coming from the opposite half of the visual field, the functional consequence is that visual input from the left side of the world goes to the

Neurology_Adams

Figure 12-2 illustrates the visual field defects caused by lesions of the retina, optic nerve and tract, lateral geniculate body, geniculocalcarine pathway, and striate cortex of the occipital lobe. In the alert, cooperative patient, the visual fields can be plotted fairly accurately at the bedside. With one of the patient’s eyes covered and the other fixed on the corresponding eye of the examiner (patient’s right with examiner’s left), a target—such as a moving finger, a cotton pledget, or a white disc mounted on a stick—is brought from the periphery toward the center of the visual field (confrontation testing). With the target at an equal distance between the eye of the examiner and that of the patient, the fields of the patient and examiner are then compared. Similarly, the patient’s blind spot can be aligned with the examiner’s, and its size determined by moving a small target outward from the blind spot until it is seen. For reasons not known, red-green test objects are more

A case of Brown-Sequard syndrome with associated Horner's syndrome after blunt injury to the cervical spine.

A 26 year old motorcyclist was received by the trauma team in our accident and emergency department after a head on collision with a motor vehicle. He had been correctly immobilised and his primary survey was essentially normal. He was alert and orientated with a Glasgow Coma score of 15 and had no symptoms or signs of spinal injury. His cervical spine radiography was also normal. Neurological examination however, revealed anisocoria, his left pupil being smaller than his right, and a Brown-Sequard syndrome, with a sensory level at C6. Immobilisation was maintained and he was transferred to the regional neurosurgical centre where magnetic resonance imaging revealed a contusion of the left half of the spinal cord adjacent to the 6th cervical vertebrae. Computed tomography revealed no bony injury but spinal column instability was demonstrated after flexion-extension spinal views and he underwent surgery to fuse his spine at the C5-C6 level. This report highlights the necessity to observe strict ATLS guidelines. This must include a thorough examination of the central and peripheral nervous system where spinal injury is suspected, even in the absence of radiographic abnormality and neck pain. This article also presents the unusual phenomena of Brown-Sequard syndrome and unilateral Horner's syndrome after blunt traumatic injury to the cervical spine.

InternalMed_Harrison

The range of eye movements and whether they are equal in each eye should be observed. Peripheral eye movement disorders (e.g., cranial neuropathies, eye muscle weakness) are usually disconjugate (different in the two eyes). One should check pursuit (the ability to follow a smoothly moving target) and saccades (the ability to look back and forth accurately between two targets). Poor pursuit or inaccurate (dysmetric) saccades usually indicates central pathology, often involving the cerebellum. Finally, one should look for spon taneous nystagmus, an involuntary back-and-forth movement of the eyes. Nystagmus is most often of the jerk type, in which a slow drift (slow phase) in one direction alternates with a rapid saccadic movement (quick phase or fast phase) in the opposite direction that resets the position of the eyes in the orbits. Except in the case of acute vestibulopathy (e.g., vestibular neuritis), if primary position nystagmus is easily seen in the light, it is probably due to a

Isolated Horner syndrome as a rare initial presentation of nasopharyngeal carcinoma: a case report.

Horner syndrome refers to a set of clinical presentations resulting from disruption of sympathetic innervation to the eye and adnexa. Classically, the clinical triad consists of ipsilateral blepharoptosis, pupillary miosis, and facial anhidrosis. Ocular sympathetic denervation may signify life-threatening causes. Timely investigation and accurate diagnosis are essential in patients with oculosympathetic denervation. A 33-year-old Asian man with a heavy smoking habit presented with a 3-week history of left ptosis and no other complaints. His visual acuity was 20/20 bilaterally. An ophthalmic examination was significant for mild ptosis of his left eyelid and anisocoria (smaller left pupil), which was greater in the dark. Both pupils reacted to light briskly without an afferent pupillary defect. Anhidrosis was found on the medial side of the left forehead. A 10% cocaine test was positive. At his first visit, neurologic examination was unremarkable. Comprehensive radiological investigations were scheduled for a left-sided isolated Horner syndrome. Two weeks after his first visit, he experienced a left-sided headache along with ipsilateral Horner syndrome. Neurologic examination revealed hypoesthesia in the left cranial nerve V<sub1-3</sub territories. Emergent computed tomography angiography was suspected for petrous part of the left internal carotid artery (ICA) dissection. Magnetic resonance imaging demonstrated an enhancing infiltrative lesion with its epicenter at the left sphenoid bone. The lesion encased the left ICA and invaded the left Meckel cave. Rhinoscopy with incisional biopsy revealed squamous cell nasopharyngeal carcinoma. This case involved an unusual initial presentation of nasopharyngeal carcinoma: isolated Horner syndrome with clinical progression to adjacent structures. Infiltration involving the Meckel cave and ICA at the foramen lacerum can present as postganglionic Horner syndrome associated with trigeminal pain and hypoesthesia. These clinical findings may mimic carotid artery dissection on computed tomography angiography. Detailed magnetic resonance imaging with careful attention to the skull base should be performed.

Visual loss in one eye after spinal surgery.

To report a patient who developed an unusual combination of central retinal artery occlusion with ophthalmoplegia following spinal surgery in the prone position. A 60-year-old man underwent a cervical spinal surgery in the prone position. Soon after recovery he could not open his right eye and had ocular pain due to the general anesthesia. Upon examination, we determined that he had a central retinal artery occlusion with total ophthalmoplegia. Despite medical treatment, optic atrophy was still present at the following examination. Ptosis and the afferent pupillary defect disappeared and ocular motility was recovered, but visual loss persisted until the last follow-up. A prolonged prone position during spinal surgery can cause external compression of the eye, causing serious and irreversible injury to the orbital structures. Therefore, if the patient shows postoperative signs of orbital swelling after spinal surgery the condition should be immediately evaluated and treated.

Anti-aquaporin-4 antibody-positive optic neuritis treated with double-filtration plasmapheresis.

The anti-aquaporin-4 (AQP4) antibody was recently reported to be associated with neuromyelitis optica (NMO). Optic nerve involvements in many NMO cases are bilateral and the prognosis is poor. However, it has been suggested that plasma exchange is effective for those patients when steroid pulse therapy is ineffective. Herein, we report successful treatment of a patient with NMO using double-filtration plasmapheresis (DFPP). A 22-year-old woman consulted a neurologist for neck pain in March 2008. High-intensity lesions were shown in the cervical spinal cord by T2-weighted magnetic resonance imaging. On July 15, the patient was referred to our department for a headache and pain and blurred vision in the left eye. The best-corrected visual acuity was 20/50 and 20/500 in the right and left eyes, respectively, with visual field defects observed in both. After 3 courses of steroid pulse therapy, anti-AQP4 antibodies were positive. In November, the patient again noticed visual acuity loss in the left eye and was treated by additional steroid pulse therapy, which was not effective. Next, she underwent plasma exchange therapy, though it was stopped due to hypotension and dyspnea. The next day, the patient underwent DFPP treatment and visual function gradually recovered. It is important to consider NMO when steroid pulse therapy is not effective. We successfully and safely treated NMO in a young adult patient using DFPP.

Syringomyelia presenting with unilateral optic neuropathy: a case report.

In this case report, we present two cases of syringomyelia with optic neuropathy. In Case 1, a 36-year-old Malay lady presented to our clinic with acute onset of blurring of vision in her left eye that she experienced since past 1 month. She was diagnosed with syringomyelia 12 years ago and was on conservative management. Her visual acuity was 6/6 in the right eye and counting fingers at 1 m in the left. There was a positive relative afferent pupillary defect in her left eye. Optic nerve functions of her left eye were reduced. Visual field showed a left inferior field defect. Her extraocular muscle movements were full. Magnetic resonance imaging of the brain and spine showed syringomyelia at the level of C2-C6 and T2-T9. Both of her optic nerves were normal. Her condition improved with intravenous and oral corticosteroids. In Case 2, a 44-year-old Malay lady presented to our clinic with a progressive central scotoma in her right eye that she experienced since past 1 month. She had previous history of recurrent episodes of weakness in both of her lower limbs from past 8 months. Visual acuity in her right and left eye was 6/9 and 6/6, respectively. The relative afferent pupillary defect in her right eye was positive. Optic nerve functions of her right eye were affected. Visual field showed a central scotoma in her right eye. Her extraocular muscle movements were full. Fundoscopy of her right eye showed a pale optic disc. Her left eye fundus was normal. Magnetic resonance imaging of the brain and spine showed syringomyelia at T3-T6. Both of her optic nerves were normal. A diagnosis of syringomyelia with right optic atrophy was performed. Her condition improved with intravenous and oral corticosteroids. Optic neuropathy is a rare neuro-ophthalmic manifestation in patients with syringomyelia. Prompt diagnosis and timely management are essential to avoid a poor visual outcome. Intravenous corticosteroids are beneficial in the treatment of early optic neuropathy in syringomyelia.

[A 29-year-old man with diabetes insipidus and cerebellar ataxia and development of spinal cord swelling 15 years after the onset].

We report a 29-year-old man with diabetes insipidus and cerebellar ataxia who developed spinal cord swelling 15 years after the onset. He was well until 14 years of the age when he noted dizziness. Two years after there was an onset of gait disturbance and slurred speech. He also noted polydipsia and polyuria. He was evaluated at the neurosurgery service of our hospital when he was 17 years of the age. Neurologic examination at that time revealed memory loss, horizontal nystagmus, cerebellar ataxic gait, dysmetria and decomposition more on the left. Cranial CT scan revealed a mass lesion involving the left subthalamic region and the head of the caudate area. Spinal fluid was unremarkable, however, human chorionic gonadotropin was increased to 27 mIU/ml. He was treated by radiation therapy (3,000 rads for total brain area and 5,460 rads for focal region). His CT scan and memory loss improved, however, cerebellar ataxia was unchanged. Three years after the radiation, he started to show choreic movement in his neck and left upper extremity. He was admitted to our service in August 14, 1995 when he was 29 years of the age. On admission, he was alert but disoriented to time; calculation was also poor. Higher cerebral functions were intact. The optic fundi were normal without papilledema. Visual field appeared intact. Gaze nystagmus was observed in all the directions, but more prominent in the horizontal direction. Speech was slurred. Otherwise, cranial nerves were unremarkable. Motor wise, he showed marked truncal and gait ataxia; he was unable to walk because of ataxia. Muscle atrophy and marked weakness was noted in both upper extremities more on the left side. Deep tendon reflexes were diminished in the upper extremities but active in the lower extremities. He was polyuric; urinary specific gravity was low. Spinal fluid contained 6 cells/cmm and 113 mg/ dl of protein; Queckenstedt was positive. MRI revealed swelling of the cervical cord; in addition, the entire cervical region and the medullar oblongata appeared as high signal intensity areas. No mass lesion was noted in the supratentorial structures but the third ventricle was markedly enlarged. Surgical biopsy was performed on the cervical lesion. The patient was discussed in neurologic CPC, and the chief discussant arrived at the conclusion that the patient had germinoma with syncytiotrophoblastic giant cells in the diencephalic region which appeared to have been cured by radiation therapy; he thought that the cervical lesion was the seeding of germinoma. Cerebellar ataxia was ascribed to the remote effect of germinoma. Most of the participants thought that the original tumor was germinoma and the cervical lesion was its spread. Some participants thought that his ataxia was caused by germinoma cells involving the medulla and the inferior cerebellar peduncles. Histologic observation of the biopsied tissue from the spinal cord revealed the typical two cell patterned germinoma. Most of the tumor cells were not stained for an antibody against HCG, but some tumor cells were positively stained. Germinoma is very radio-sensitive; this patient showed T2 high signal lesion involving the medulla oblongata and cervical cord continuously. Probably, tumor cells in the lower brain stem escaped radiation, and gradually spread to the spinal cord over many years. At the time of operation, the surface of the spinal cord was free from tumor cells. Therefore, tumor cells invaded the spinal cord continuously from the medulla oblongata. He was treated with cervical radiation, and his neurologic as well as radiologic findings showed marked improvement.

[A 50-year-old man with metamorphopsia and optic nerve swelling].

We report a 50-year-old male having metamorphopsia associated with retinal edema. He was well until one month prior to the present admission when he developed occipital headache and backache. Three weeks later, he noted a sudden onset of twisting of visual images. On admission, he was in no acute distress with well preserved general conditions. Only neurological abnormalities were bilateral papilledema, retinal edema and horizontal nystagmus with a rotatory component. Routine blood chemistries were unremarkable. The CSF contained 28 cells/cmm with 60% consisting of large atypical cells. Cranial CT scans revealed no mass, however, the magnified orbital CT showed bilateral swelling of the optic nerve. He was treated with ventriculoperitoneal shunt and Ommaya tube placement through which methotrexate, cytarabine and prednisolone were administered. He was also treated with systemic cisplatin, mitomycin-C and 5-fluorouracil. With these therapy, his headache, metamorphopsia and papilledema improved. He was discharged for out-patient follow-up, however, he had to admitted again because of progressive difficulty of gait and loss of appetite. On admission, he complained of severe backache, and his gait disturbance appeared to be in part due to his backache. A slight weakness was noted in all four limbs with loss of deep reflexes. Mentally he was alert and cranial nerves appeared intact without papilledema. But nuchal rigidity was noted. Cranial CT scan revealed attenuation of all the cortical sulci and marked diffuse low density changes in the cerebral white matter, and his chest film revealed a ring-shape shadow in his left lung field. He deteriorated progressively with terminal gastrointestinal hemorrhage. He expired three weeks after his second admission.(ABSTRACT TRUNCATED AT 250 WORDS)

Management of hypotony-related maculopathy after combined phacoemulsification and trabeculectomy: January consultation #1.

A 59-year-old man with mild to moderate pigmentary glaucoma was referred for management of hypotony-related maculopathy 3 years after combined phacoemulsification and trabeculectomy in his right eye. His ocular history is significant for retinal detachment in the right eye that was surgically treated with a pars plana vitrectomy and scleral buckle 5 years prior to the current presentation and 1 year prior to his combined phacoemulsification-trabeculectomy procedure. After trabeculectomy, he reportedly had a wound leak with hypotony and choroidal effusion. The patient was referred to a second surgeon who performed a bleb revision with a pericardial patch graft, but the patient had a severe intraocular pressure (IOP) spike in the immediate postoperative period requiring suture removal. This resulted in recurrent hypotony with maculopathy. A second bleb revision with pericardial patch graft was performed but was not successful in raising the IOP out of the single digits or in resolving the maculopathy. Accordingly, the patient was referred for further assessment. At presentation, the patient's corrected distance visual acuity was 20/100 in the right eye and 20/20 in the left eye. Applanation tonometry IOP was 4 mm Hg and 16 mm Hg for the right and left eyes, respectively. Central corneal thickness was 609 μm in the right eye and 574 μm in the left eye. The right pupil was noted to be slightly irregular with a relative afferent pupillary defect. Slitlamp examination of the right eye was notable for a moderately elevated, Seidel test-negative bleb, and deep anterior chamber. The left eye had a Krukenberg spindle and mild nuclear sclerotic cataract but was otherwise unremarkable. Fundus examination of the right eye was notable for significant macular folds with edematous nerve fiber layer (NFL) and optic nerve. The left optic nerve and fundus examination were unremarkable. Gonioscopy revealed open angles with dense trabecular meshwork pigment in both eyes. There was a nicely patent superior sclerostomy in the right eye. There was no cyclodialysis cleft. Optical coherence tomography (OCT) of the macula showed chorioretinal folds and NFL edema in the right eye and was normal in the left eye (Figure 1JOURNAL/jcrs/04.03/02158034-202101000-00022/figure1/v/2021-01-04T143903Z/r/image-tiff). The OCT of the optic nerve in the right eye was distorted secondary to the chorioretinal folds, whereas the left eye was notable for mild superior thinning of the NFL (Figure 2JOURNAL/jcrs/04.03/02158034-202101000-00022/figure2/v/2021-01-04T143903Z/r/image-tiff). Humphrey visual field revealed a superior nasal step in the right eye (Figure 3JOURNAL/jcrs/04.03/02158034-202101000-00022/figure3/v/2021-01-04T143903Z/r/image-tiff). Axial lengths were 25.33 mm and 26.53 mm in the right and left eye, respectively; it is notable that the right eye had a shorter axial length despite the scleral buckle, which demonstrates the degree of axial shortening from hypotony (Figure 4JOURNAL/jcrs/04.03/02158034-202101000-00022/figure4/v/2021-01-04T143903Z/r/image-tiff). What would be your approach for managing this patient's hypotony? Does the fact that the referring surgeon had performed 2 failed bleb revision procedures prior to referral influence your approach? Given that a previous attempt at revision resulted in an extreme IOP spike would you also recommend a glaucoma procedure? If so, what procedure would you perform? Would you do it coincident with the revision or in a staged procedure later on an as-needed basis?

Human ocular torsional position before and after unilateral vestibular neurectomy.

The static ocular torsional position of both eyes of 23 patients was measured by means of fundus photographs one day before and one week after unilateral vestibular neurectomy for the treatment of acoustic neuroma, Ménière's disease or paroxysmal vertigo. The results showed that in all patients the vestibular neurectomy caused both eyes to tort (i.e. to roll around the visual axis) toward the side of the neurectomy when measured one week after operation. The extent of this torsion was an average of 9.5 degrees one week after operation and there was no statistically significant difference in the average magnitude of the torsion in the two eyes. In 8 of these patients, additional measurements were made at intervals up to one year after operation and it was found that in these patients there is a significant reduction in torsion over time from an average of 10.2 degrees one week after operation to an average of 2.8 degrees by 16 weeks after operation. The change in torsional eye position following the neurectomy was accompanied by a change in the perceived visual orientation of a small (9.5 degrees visual angle) illuminated horizontal line at a straight ahead eye level position in an otherwise completely darkened room. One week after operation when asked to adjust the line to the perceived gravitational horizontal by rotating it in roll (i.e. around an X axis), patients who had had a right vestibular neurectomy consistently set the line so that the right side of the line (from the patient's point of view) was below the true gravitational horizontal. Similarly patients after a left neurectomy consistently set the line so that the left side of the line was below the true gravitational horizontal. There is a high correlation (r = 0.95) between the direction and magnitude of the change in torsional eye position and the direction and magnitude of the change in the perceived visual horizontal one week after operation.(ABSTRACT TRUNCATED AT 250 WORDS)

Unilateral progressive epitheliopathy after LASIK.

A 36-year-old man was referred back to our clinic after an uneventful laser in situ keratomileusis (LASIK) surgery 6 months previously, with a chief complaint of declining vision in the left eye for the last several months. His pertinent ocular history prior to the LASIK surgery was significant for episodic left eye irritation and inflammation of the superior part of the left cornea secondary to contact lens-induced keratopathy, according to his optometrist.This was a femtosecond-assisted LASIK surgery using the WaveLight FS200 femtosecond laser (Alcon) with the Allegretto EX500 excimer laser (Alcon) for attempted correction of -4.50 diopter (D) of myopia in both eyes under an 8.8 mm flap with a thickness of 100 μm. The uncorrected distance visual acuity (UDVA) was 20/15 in both eyes for the first 2 weeks; at the 1-month follow-up visit, a well-demarcated, wavelike plaque of coarse, thickened corneal epithelium along the superior aspect of the cornea was noted. The UDVA had declined to 20/30 in the left eye. The right eye remained clear with a UDVA of 20/15. The patient was treated for presumed interface inflammation with 1% topical prednisolone acetate for 3 weeks on a tapering regimen and it was noted that there was some improvement in the vision subjectively although the epithelial ridge involving the superior one third of the LASIK flap was present without any change.Over the course of the next 2 months, a progressive decline in vision was noted in the left eye. At no point did his right eye experience symptoms or visual decline. Four months postoperatively the UDVA had declined to 20/60 in the left eye. A linear epithelial ridge extending from limbus at 11 to 2 o'clock position was noted superiorly; staining showed the ridge involved the superior aspect of the flap. The patient was again given corticosteroid treatment, which would improve the symptom of irritation without any change in the visual acuity. Over the course of the next few months, a new wave of abnormal epithelium involving the inferior aspect of the limbus and the flap, encompassing the 2 o'clock position of the flap from the 4 to 6 o'clock positions, was also noted ().(Figure is included in full-text article.)Conjunctival staining using lissamine green and fluorescein dye showed staining of the well-demarcated epithelial ridge superiorly and inferiorly involving the flap encroaching the visual axis. On examination, the patient's corrected distance visual acuity was now 20/80 in the left eye. , , and show the tomographic, topographic, and optical coherence tomography (OCT) results at the 6-month follow-up post-LASIK surgery.(Figure is included in full-text article.)(Figure is included in full-text article.)(Figure is included in full-text article.)Preoperatively the patient denied any ocular history but on further questioning at the 6-month follow-up visit, he provided information about the use of systemic isotretinoin (Accutane) for juvenile acne for 1 year at age 17 and potential exposure to chemicals during military service although he denied any specific ocular exposure. He is a barbeque enthusiast and brews his own alcohol but denies any thermal or direct injury to either eye. His mother had recently told him that he might have had battery acid exposure when he was very young, before age 10, but never required medical attention.What is the most likely diagnosis? What medical or surgical interventions would you recommend for this patient? What is the prognosis for this patient?

[Superior oblique myokymia--a case report and pathogenetic consideration].

A 30-year-old male visited us with complaints of dizziness and diplopia of abrupt onset in September, 1988. Neurological studies demonstrated paroxysmal rotatory to-and-fro oscillations of the left eye. There has been the same clinical episode at 25 years of age, lasting approximately 6 months. The characteristics of his ocular involuntary movement with the mild IVth cranial nerve paresis were as follows: quick, nonrhythmic, initially counterclockwise-rolling, more prominent in moving the left eye to the lower medial side, persisting for 1-10 seconds and rapidly repeated oscillations. When oscillations were prominent, he complained of faintness in addition to double vision. Except for the ocular signs, other neurological and laboratory examinations including cerebrospinal fluid, brain MRI and brain stem auditory evoked response, failed to disclose the precise location and nature of the lesion. Abnormal ocular movement was fluctuating for 4 months and gradually disappeared without any medication. In conclusion, the faintness could be considered to result from a lesion of reticular activating system adjacent to the IVth cranial nerve nucleus and its rostral (excitatory or inhibitory) supranuclei, and it suggests that a responsible lesion of the abnormal ocular movement is located at a region of the dorsomedial midbrain.

Rapid, painless unilateral vision loss in a 37-year-old healthy woman.

A 37-year-old woman experienced painless, progressive vision loss to no light perception in the left eye over the course of 3 days. The right eye was unaffected. On examination, the only other abnormal finding was a +4 left afferent pupillary defect. She was initially diagnosed with retrobulbar optic neuritis and admitted for treatment with intravenous methylprednisolone. Neuro-imaging revealed a large right anterior cerebral artery aneurysm that crossed the midline to compress the left optic nerve. The aneurysm was treated with coil embolization, which was technically successful but which did not lead to significant improvement in vision.

[Subacute sensory neuropathy manifestated by tonic pupils associated with small cell carcinoma of the lung].

Only a few cases of carcinomatous neuropathy with tonic pupils have been reported. In the present paper we described a 53-year-old woman with subacute sensory neuropathy who had presented with bilateral tonic pupils. She noticed numbness over the medial aspect of the left thigh in March, 1988, and then developed dysesthesia over the left forearm and hand, mild weakness of left upper and lower extremities and urinary disturbance. Neurological symptoms were subacutely progressive and she was bed-ridden in May. She was admitted to our hospital in June, 1988. On examination, she had body-weight loss of 6 kg during the last six months and general status was otherwise unremarkable. She had anisocoria; the left pupil was larger in daylight than the right, while smaller in dim light. The left pupil scarcely reacted to light, but promptly constricted to near vision. The right pupil constricted normally to light and near vision. An instillation of 0.0625% pilocarpine solution showed supersensitive response of both pupils. An instillation of 1.25% epinephrine solution demonstrated mild dilation of both pupils. Thus, it was conceivable that she had postganglionic ciliary nerve damage characteristic of tonic pupil as well as the lesion of sympathetic nerve innervating pupillary dilator. She had severe sensory ataxia and pseudoathetosis of the hands. Weakness was mild to moderate in extremities. Almost all deep tendon reflexes were absent. All modalities of sensation, particularly on deep sense, were severely involved with sea-level-type distribution below Th7, and over C2 to C3 regions on the left side.(ABSTRACT TRUNCATED AT 250 WORDS)