We describe a picture of typical peripheral facial paralysis, with dysgeusia, hyperacusis and mastoid pain. In this case the treatment is corticoid. The first one would not be correct because although within the age range of multiple sclerosis debut, the presentation is usually gradual over days and the nuclear involvement of the VII cranial nerve (which could simulate a peripheral facial) would not cause dysgeusia, hyperacusis or mastoid pain. In the third answer they propose us that it is a vascular picture and therefore that the facial paralysis is central, so it is discarded and the last one is not correct because most of the idiopathic peripheral facial paralysis (Bell's palsy) heal without sequelae.

We describe a picture of typical peripheral facial paralysis, with dysgeusia, hyperacusis and mastoid pain. In this case the treatment is corticoid. The first one would not be [HIDDEN] because although within the age range of multiple sclerosis debut, the presentation is usually gradual over days and the nuclear involvement of the VII cranial nerve (which could simulate a peripheral facial) would not cause dysgeusia, hyperacusis or mastoid pain. In the third answer they propose us that it is a vascular picture and therefore that the facial paralysis is central, so it is [HIDDEN] and the last one is not [HIDDEN] because most of the idiopathic peripheral facial paralysis (Bell's palsy) heal without sequelae.

A 20-year-old girl comes to the emergency department because she has noticed after getting up, when looking in the mirror, weakness in the right half of the face (including the forehead, closing the eyelid and smiling). She is accompanied by dysgeusia, with a sensation of metallic taste of food as well as hyperacusis and ipsilateral mastoid pain. On examination there is no evidence of strength deficits or sensory deficits in the extremities or speech or language disorders. In this case, which of the following statements is correct?

The most probable diagnosis is a demyelinating plaque in the contralateral hemiprotuberance, the most necessary complementary exploration would be a cranial MRI.

Oral corticosteroids are the treatment of choice for the patient.

Intravenous fibrinolysis should be considered if the evolution time is less than 3h.

Most likely the picture is irreversible.

Paraneoplastic Encephalopathy in a Patient With Metastatic Lung Cancer: A Case Study.

<bCASE STUDY</b RS, a 36-year-old female, presented to the emergency department (ED) of a large academic medical center upon the advice of her primary care provider because of 3 weeks of progressive mental status changes, weakness, and decreased oral intake. According to her husband, RS was diagnosed with stage IIIA large cell lung cancer 8 months earlier and was treated with concurrent chemotherapy (carboplatin, pemetrexed, and bevacizumab) and radiation therapy that was completed 4 months prior to admission. No other specific information about her treatment or outside health records was available. According to her husband, RS had been in her usual state of health until approximately 3 weeks prior, when she began having significant mental status changes. She first exhibited some difficulty finding words and later was noted to be putting food in a coffee maker. This spontaneously resolved after approximately 1 week; however, she rapidly developed slurred speech and began to make nonsensical statements. These manifestations also slowly improved but were followed by worsening speech deficit, difficulty walking, and impaired balance. During one of these episodes, she had an occurrence of incontinence. Her husband also noted an incident where her "eyes were beating back and forth and the left side of her face was twitching." RS also had periods (according to her husband) where she "did not seem to be interacting with her environment." These progressively worsened during the last week, and she completely stopped walking and talking 2 days prior to coming to the ED. According to her husband, RS had rheumatoid arthritis and no surgical history. Her family history was unknown except that RS's mother had "seizures." RS had reportedly not used tobacco, alcohol, or drugs, and she was sexually active with her husband. Home medications included transdermal fentanyl 12 μg/hr patch changed every 72 hours; oxycodone-acetaminophen tablets 5-325 mg, two every 4 hours as needed for pain; prednisone 10 mg, one tablet daily; and megestrol 40 mg/mL suspension, 20 mL once daily for appetite stimulation. RS was admitted to an inpatient medical oncology service and evaluated by the oncology advanced practitioner (AP) on her second inpatient day. Upon exam, RS was nonverbal except for moaning in response to painful stimuli and to her sister's voice. Her vital signs were normal. She appeared ill but well-nourished, and she was mildly diaphoretic. Neurologic examination revealed that her pupils were slightly sluggish but equal, round, and reactive to light. Extraocular muscle movements were intact, but she did not move her eyes in response to commands. She tracked the AP and family members around the room with her eyes. Cranial nerve examination was intact with the exception of cranial nerves IX, X, and XI, which were difficult to examine given her inability to cooperate and open her mouth. Motor examination revealed increased tone throughout and intermittent, inconsistent resistance to passive movement. She was seen to move all four extremities spontaneously although not in response to commands. Deep tendon reflexes were intact and equal in all extremities. Examination of other body systems was as follows: there was dry, peeling skin on her lips, but her mucous membranes were moist and free of erythema or lesions. Her lungs were clear to auscultation bilaterally. Her heart rate and rhythm were regular, there were no murmurs, rubs, or gallops, and distal pulses were intact. Her abdomen was nondistended with normally active bowel sounds in all four quadrants. Her abdomen was soft, nontender to palpation, and without palpable masses. There was no peripheral discoloration, temperature changes, or edema, and examination of her skin was benign. <bWorkup</b On admission to the emergency department, serum laboratory studies were unrevealing for any potential causes of encephalopathy. Kidney and liver function were normal, making diagnoses of uremic and hepatic encephalopathies less likely. Cultures of the urine and blood were negative. Samples of cerebrospinal fluid (CSF) were obtained via lumbar puncture and were unrevealing for any abnormalities. Computed tomography (CT) of the head without contrast was negative for any acute intracranial process. Ultrasound of the right upper quadrant revealed a single, nonspecific, hypoechoic hepatic lesion. Computed tomography scans of the chest, abdomen, and pelvis demonstrated the primary malignancy in the upper lobe of the left lung, as well as possible metastatic disease within the left lung, right lung, and liver, and widespread osseous metastatic disease. Magnetic resonance imaging (MRI) of the brain performed 1 day after admission demonstrated numerous scattered punctate foci of enhancement throughout the supratentorial and infratentorial brain parenchyma, measuring at most 3 to 4 millimeters in diameter. There was no significant mass effect or midline shift. A paraneoplastic panel was sent to an outside laboratory and returned positive for antivoltage-gated potassium channel (VGKC) autoantibodies. <bDifferential Diagnosis</b Clinically, RS was exhibiting signs of encephalopathy, a broad term that indicates general brain dysfunction, the hallmark of which is altered mental status. Diagnosing encephalopathy is challenging, as many differential diagnoses must be considered. The clinician must consider metabolic derangements, toxic and infectious etiologies, psychiatric disorders, and less commonly, prion disorders and progressive dementia. Cultures of RS's blood and urine as well as other specialized endocrine tests were negative, decreasing the likelihood of a metabolic or infectious cause for her presentation. The abnormalities on her brain MRI were reviewed by a neuro-oncology team, who felt that the faint, nondescript nature of the visualized lesions was not suspicious for metastatic disease. Sequelae of seizures was also considered by neuro-oncology but dismissed given a grossly normal prolonged electroencephalogram. Some encephalopathies are caused by autoimmune or inflammatory mechanisms, which are confirmed by the presence of autoantibody markers and/or clear response to immunomodulatory treatment (Vernino, Geschwind, & Boeve, 2007). These types of encephalopathies have been seen in patients with cancer and have thus been termed paraneoplastic. The presence of anti-VGKC antibodies on RS's paraneoplastic panel directed the inpatient medical oncology team toward a paraneoplastic neurologic disorder (PND) as the most likely diagnosis.

A Case of Steroid-Associated Expressive Aphasia.

Expressive aphasia (non-fluent aphasia) is characterized by the inability to produce words or sentences. The most common cause of expressive aphasia is stroke, usually due to thrombus or emboli in the middle cerebellar artery or internal carotid artery affecting Broca's area. We present an important, reversible, and previously undescribed cause of a purely expressive aphasia secondary to steroid use. A case of a steroid-induced expressive aphasia has not yet been described in the medical literature. Recognition of this presentation is critical to appropriate therapy and excess morbidity, particularly as steroid (dexamethasone) utilization has increased since the COVID-19 pandemic.

A case of neuropsychiatric lupus Erythematosus characterized by the Owl's eye sign: a case report.

Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disorder characterized by multiple affected systems. More than half of SLE patients will suffer from neuropsychiatric lupus erythematosus (NPSLE) during the course of their disease. Although nearly half of the NPSLE patients have normal MRI manifestations, the abnormalities found in the remainder can be located anywhere in the brain, and especially in the subcortical white matter of the frontal and temporal lobe. However, NPSLE involving the medulla oblongata and spinal cord which presents as the "owl's eye" sign has to our best knowledge not been reported to date. A 19-year-old girl presented at our hospital with a 7-day history of fever and headache since a one day's exertion, accompanied by 2 days of weakness. The patient had slurred speech. Neurological examination revealed the presence of horizontal nystagmus and a limitation of bilateral eye movement when looking up and down. At the same time, she showed difficulty in raising the jaw, accompanied by a weak pharyngeal reflex. Muscle strength was remarkably decreased in all four extremities: the MRCS grade of the upper limbs was 4/5, while in the lower limbs it was 0/5. Hypotonia was apparent in the lower extremities. Regarding subjective sensation, the patient appeared to be experiencing an increased sense of pain in the whole body, and especially in the cervical region, abdomen, and feet. An examination of shallow reflex documented the reinforcement of the abdominal reflex. Deep tendon reflexes were symmetric: absent in lower, normal in upper extremities. The patient also had a stiff neck with a positive Kernig's sign. The laboratory examination showed elevated C - reactive protein and rheumatoid factor, as well as complement components 3 and 4. Symptomatic treatments were applied, but she did not respond well, after which we did immunological laboratory examinations. The results showed the presence of anti-nRNP/Sm, anti-dsDNA and anti-AMA M2 antibodies. An MRI scan and enhancement of the cervical and thoracic regions displayed abnormal signs in the medulla and bilateral anterior horn of the lower thoracic spine. Following the exclusion of other possible diseases, neuropsychiatric lupus was diagnosed. High-dose intravenous gamma-globulin combined with methylprednisolone gradually improved her condition. We report the first case of NPSLE presenting with medulla oblongata and spinal cord involvement, manifesting as the "owl's eye" sign in MRI.

[The importance of neurological examinations in the age of the technological revolution].

Neurologic practice and care have been modified in many important ways during the past ten years, to adapt to the explosion of new information and new technology. Students, residents and practicing physicians have been continuing programs to a model that focuses almost exclusively on the applications to neurologic disorders of the new knowledge obtained from biomedical research. On the other hand high demand for outpatient neurologic care prevents adequate patient's evaluation. Case 1: 65 years old female. Occipital headache diagnosed of tensional origin (normal computerized tomography). Two months later is re-evaluated due to intractable pain and hypoglossal lesion. An amplified computerized tomography revealed a occipital condyle metastasis. Case 2: 21 years old female. Clinical suspicion of demyelinating disease due to repeated facial paresis and sensitive disorder. General exploration and computerized tomography revealed temporo-mandibular joint. Case 3: 60 years old female. Valuation of anticoagulant therapy due to repeated transient ischemic attacks. She suffered from peripheral facial palsy related to auditory cholesteatoma. Neurologic education is nowadays orientated to new technologies. On the other hand, excessive demand prevents adequate valuation and a minute exploration is substituted by complementary evaluations. These situations generate diagnostic mistakes or iatrogenic. It would be important a consideration of the neurologic education profiles and fulfillment of consultations time recommendations for outpatients care.

Lateral medullary syndrome

Lateral medullary syndrome is a neurological disorder causing a range of symptoms due to ischemia in the lateral part of the medulla oblongata in the brainstem. The ischemia is a result of a blockage most commonly in the vertebral artery or the posterior inferior cerebellar artery. Lateral medullary syndrome is also called Wallenberg's syndrome, posterior inferior cerebellar artery (PICA) syndrome and vertebral artery syndrome. Signs and symptoms This syndrome is characterized by sensory deficits that affect the trunk and extremities contralaterally (opposite to the lesion), and sensory deficits of the face and cranial nerves ipsilaterally (same side as the lesion). Specifically a loss of pain and temperature sensation if the lateral spinothalamic tract is involved. The cross body finding is the chief symptom from which a diagnosis can be made.

[Really drunk?].

We report about a 42-year-old patient who was admitted to the emergency department because of suspected alcohol abuse. He declared himself to be drunk. He stated in his case history that he had suffered from right sided neck and facial pain for several days. The clinical examination revealed a left sided hemiparesis. Together with the demonstrated right hemispherical brain ischemia by computed tomography, a presumptive diagnosis of a dissection of the right internal carotid artery was made. This diagnosis was finally confirmed by ultrasound and magnetic resonance imaging. A therapy with full dose heparin was begun and oral anticoagulation was subsequently initiated. After two weeks of follow-up, the neurological deficiencies were partially regredient.

Morbihan Disease - An Old and Rare Entity Still Difficult to Treat.

Morbihan disease (MD), also known as Morbihan syndrome, "solid persistent facial edema and erythema", "rosacea lymphedema", and "solid facial edema in acne", is a rare and often unrecognizable entity, that presents with a slow occurrence of persistent lymphoedema of the upper two-thirds of the face (1,2). A 30-year-old woman presented to our Department with persistent, asymptomatic face edema and erythema lasting for 18 months. She was previously treated for rosacea with doxycycline (100 mg/day for four months) without improvement. Dermatological examination revealed erythematous, nonpitting, solid edema located on the mid-forehead, nose, and cheeks with sparse erythematous papules and pustules on the entire face including the chin and comedones, papules, and pustules on the back (Figure 1 and Figure 2). She was otherwise healthy and was not taking any medication. Laboratory tests with immunological tests and Quantiferon test together with MRI of the orbits, chest X-ray, chest high-resolution computed tomography, cranial X-ray, and abdominal ultrasound were all within normal limits. Histopathology revealed dermal edema, perivascular and peri-adnexal lymphohistiocytic infiltrate, and sebaceous gland hyperplasia. Based on the typical clinical picture, histopathological findings, and the exclusion of several differentials the diagnosis of MD was established. The patient was treated with oral isotretinoin (20 mg/day for eight months) without regression of solid edema and erythema on the face but with complete regression of acne on the trunk. She was started on oral corticosteroids (prednisolone, 20 mg/day for two months followed by reduction of the dose over three months), again with only slight short transient improvement and rapid relapse of facial erythema and edema. The patient refused any other suggested treatment. We treated our patient for a total 2 years and followed up for 5 years. The pathogenesis of MD is still unknown. It is considered a clinical variety or a complication of rosacea or acne which does not tend to regress spontaneously. It is believed that chronic inflammation in patients with MD is due to acne or rosacea causing structural damage to blood and lymph vessels (1,3,4). However, cases of MD without previous history of rosacea and acne have been reported supporting the distinct disease theory (3,4). Edema and erythema are localized on the upper half of the face affecting the forehead, glabella, eyelids, nose, and cheeks. Although the symptoms may come and go, MD usually does not improve without treatment (5). Several therapeutic options have been reported, although there is no established standard treatment for MD. Reported therapy includes short-term oral isotretinoin (0.5 mg/kg/day), long-term oral isotretinoin (40-80 mg/day, 10-24 months), long-term doxycycline, combination of systemic corticosteroids and antibiotic (prednisolone 20 mg/day for 2 weeks and doxycycline 200 mg/day for 12 weeks), slow-releasing doxycycline monohydrate (40 mg/day for 6 months), long-term minocycline (50 mg/day for 4 months), and a combination of both oral retinoid and ketotifen (isotretinoin 0.7 mg/kg/day for 4 months, ketotifen 2 mg/day for 4 months) (1,2,6,8). The disease is frequently recalcitrant to therapy, and only several cases of successfully treated patients with MD have been reported (1,2,4, 6-8). We presented a patient with characteristic features of MD, which is a persistent, cosmetically disturbing condition, unfortunately mostly refractory to therapeutic measures.

Neurology_Adams

The transient neurologic deficits were mainly sensorimotor, often involving the hand and lips, and aphasia; only 6 patients had visual symptoms. The patients were asymptomatic between attacks and in none did the entire illness persist beyond 7 weeks. We have observed several cases, all in otherwise healthy middle-aged men and we found corticosteroids to be helpful.

Brown-Séquard syndrome

In addition, if the lesion occurs above T1 of the spinal cord it will produce ipsilateral Horner's syndrome with involvement of the oculosympathetic pathway. Diagnosis Magnetic resonance imaging (MRI) is the imaging of choice in spinal cord lesions. Brown-Séquard syndrome is an incomplete spinal cord lesion characterized by findings on clinical examination which reflect hemisection of the spinal cord (cutting the spinal cord in half on one or the other side). It is diagnosed by finding motor (muscle) paralysis on the same (ipsilateral) side as the lesion and deficits in pain and temperature sensation on the opposite (contralateral) side. This is called ipsilateral hemiplegia and contralateral pain and temperature sensation deficits. The loss of sensation on the opposite side of the lesion is because the nerve fibers of the spinothalamic tract (which carry information about pain and temperature) crossover once they meet the spinal cord from the peripheries.

[Bilateral ageusia caused by right thalamic infarction].

A 58-year-old man noticed left hemiparesis at 01:00 pm on a particular day in March 2006. Because his symptoms developed gradually, he was referred to the emergency room of our hospital at 05:00 pm and was admitted with the diagnosis of cerebral infarction. While he presented slight left hemiparesis involving the face, impairment of sensation was not apparent. Diffusion-weighted magnetic resonance imaging of the head showed a high-intensity area in the ventromedial area in the right thalamus. The patient was treated with anticoagulant and edaravone, and his symptoms resolved on hospital day 3. When he began eating, he noticed that he was unable to distinguish tastes. On day 5, we performed taste examination using a commercial kit. The taste sensation on both sides of his tongue was severely affected, while the touch sensations in the mouth and olfaction were preserved. His symptoms improved spontaneously and resolved on hospital day 15. This is the second case report of bilateral ageusia caused by right thalamic infarction. Our study indicates the importance of the right thalamus in taste sensation involving both sides of the tongue.

Neurology_Adams

The onset of Bell’s palsy is acute; about one-half of cases attain maximum paralysis in 48 h and practically all within 3 or 4 days. Pain behind the ear may precede the paralysis by a day or two and in a few patients is intense and persistent. Although a report by the patient of fullness or numbness in the face is common, in a small number there is hypesthesia in 1 or more branches of the trigeminal nerve. The explanation of this finding is not clear. Impairment of taste is present in most patients but it rarely persists beyond the second week of paralysis. This indicates that the lesion has extended proximal to the point at which the chorda tympani joins the facial nerve. Hyperacusis or distortion of sound is then experienced in the ipsilateral ear and, as mentioned, indicates paralysis of the stapedius muscle.

Optic neuritis

When an inflammatory recurrent optic neuritis is not demyelinating, it is called "Chronic relapsing inflammatory optic neuropathy" (CRION) When it is anti-MOG related, it is demyelinating and it is considered inside the anti-MOG associated inflammatory demyelinating diseases. Some reports point to the possibility to establish a difference via OCT Treatment In most MS-associated optic neuritis, visual function spontaneously improves over 2–3 months, and there is evidence that corticosteroid treatment does not affect the long term outcome. However, for optic neuritis that is not MS-associated (or atypical optic neuritis) the evidence is less clear and therefore the threshold for treatment with intravenous corticosteroids is lower. Intravenous corticosteroids also reduce the risk of developing MS in the following two years in patients with MRI lesions; but this effect disappears by the third year of follow up.

[Opercular syndrome of vascular aetiology].

Facio linguo masticatory paralysis resulting from bilateral lesion of the anterior opercular region, known today as Foix Chavany Marie syndrome, appears very frequently in adult patients as a consequence of generally ischemic vascular lesions, which directly affect the Rolandic opercula or the subcortical area surrounding them. However, forms that are secondary to infections, neoplasias and even unilateral lesions have been reported. Female patient, aged 70, with unknown auricular fibrillation, which started suddenly with right faciobrachial paresis secondary to a left striated ischemic infarction. Approximately 24 hours after beginning intravenous heparinization, the patient presents anarthria, facial hypomimia without asymmetries, dysphagia above all for liquids and bilateral lingual paresis. Extraoccular movements, blinking and corneal reflex were normal. Understanding of spoken and written language was maintained and there were no apraxias or agnosias. Neurological exploration was compatible with an opercular syndrome (OS). Urgent brain CT revealed the existence of a right cortiico subcortical temporal haemorrhage, in addition to the prior contralateral ischemia. The association of both lesions, ischemic and haemorrhagic, mirrored, justified all the symptomatology. Anterior OS is characterised by a bilateral voluntary central paresis of the muscles innervated by the 5th, 7th, 9th, 10th and 12th cranial pairs with preservation of emotional or automatic mobility. Underlying mechanisms are also discussed.

Spontaneous spinal epidural abscess in pregnancy: a case series.

Spontaneous spinal epidural abscess (SEA) is a rare diagnosis; only eight cases have been reported during pregnancy. Diagnosis of SEA can be difficult, especially when the classic triad of fever, back pain, and neurologic deficits are not present. Early diagnosis and treatment are necessary to reduce potential morbidity and mortality. We report two separate cases of SEA in pregnancy and summarize the existing literature. Case 1: A 20-year-old G1P0 presented at 35-week gestation with low back pain and lower extremity (LE) weakness. Magnetic resonance imaging (MRI) revealed thoracic SEA. The patient underwent cesarian delivery followed by posterior thoracic laminectomy and fusion (T9-11), abscess decompression, and antibiotic therapy. Unfortunately, there was a recurrence of her infection requiring a second irrigation and debridement 1 month after index procedure. At final follow-up, the patient had complete neurologic recovery. Case 2: A 38-year-old G10P0 presented at 36-week gestation in labor with LE weakness and difficulty ambulating. After delivery, she had significant LE neurologic deficits. MRI demonstrated thoracic osteodiscitis with associated epidural abscess. She underwent thoracic laminectomy and fusion (T7-12), abscess decompression, and antibiotic therapy. Unfortunately, despite aggressive treatment, she has persistent LE neurologic deficits. Pregnancy complicates the diagnosis and treatment strategies of SEA: back pain is very commonly underestimated, especially in the absence of fever and gross neurologic deficits. Prompt diagnosis and treatment are paramount to prevent neurologic decline and facilitate recovery. It is important to perform a focused physical exam noting motor strength, sensation, and reflexes. Coordinated management between the Emergency Department, OB-GYN, and spinal surgery team is required for best possible patient outcomes. Typically, management consists of aggressive surgical decompression and antibiotic therapy.

[A case of ipsilateral ageusia, sensorineural hearing loss and facial sensorimotor disturbance due to pontine lesion].

We report a 58-year-old woman with pontine lesion presented with subacute onset of unilateral gustatory disturbance accompanied by facial numbness, and hearing loss. Neurologic examination revealed superficial hypesthesia and paresthesia on the right side of the face, right peripheral type facial paresis, ageusia on the right half of the tongue and right sensorineural deafness. No other neurologic signs were observed, and laboratory data were all normal. Brain MRI revealed a small lesion in the right dorsolateral tegmentum of the middle pons. Electrogustometry showed marked reduction in the sense of taste on the right half of the tongue. ABR showed diminished amplitude in the IV-V wave of the right side, while SEP and VEP were normal. The clinical diagnosis was demyelinating lesion and intravenous methylprednisolone (1 g/day) was administered for 3 consecutive days, resulting in prompt improvement in the symptoms. The lesion was suspected of affecting ipsilateral side of the spinal trigeminal nerve tract and the nucleus, the intraaxial infranuclear facial nerve fiber, the lateral lemniscus adjacent to the superior olivary nucleus and the central gustatory tract. Our case suggests that the central gustatory pathway projecting from the nucleus of the solitary tract to the parabrachial nucleus, presumed to be pontine taste area, ascends ipsilaterally and is located laterally from the medial lemniscus.

Brown-Séquard syndrome in a 11-year-old girl due to penetrating glass injury to the thoracic spine.

Injuries in children are one of the most frequent causes of high morbidity and mortality, and they present a challenge to the treating physician. Fortunately, spinal trauma in pediatric patient is relatively rare. Brown-Séquard syndrome is a rare form of incomplete spinal cord injury consisting of ipsilateral upper motor neuron paralysis (hemiplegia) and loss of proprioception with contralateral pain and temperature sensation deficits resulting from hemisection or lateral injury to the spinal cord. A 11-year-old girl was admitted to our Pediatric Trauma Emergency Department after she had suffered a penetrating back injury. Neurological examination demonstrated left lower extremity paresis and moderate spastic paralysis of the right lower extremity. The examination showed loss of temperature sensation contralateral to and below the lesion. The examination of the pain sensation was difficult because the patient was in pain shock, but it was diminished on the side opposite to the damage. Multislice spiral computed tomography (MSCT) demonstrated a triangular foreign body in spinal canal at the level of the Th11-Th12. After a Th11-L2 laminectomy and retrieval of foreign bodies, dura repair was performed. Patient was discharged from the hospital with partial recovery. Operative decompression of the neural elements in case of spinal canal compromise is the treatment of choice. Indication for surgical intervention in existing cerebrospinal fluid fistula includes closure of the dura and reducing neural elements compression and lowering the risk of infectious complications by removing bone or foreign body fragments. Patients with Brown-Séquard syndrome have good prognosis for functional recovery.

The Brain of Morbius

of Radio Times awarded it five stars out of five. He noted that Solon's insistence that he only use the Doctor's head was "a fundamental lapse in logic", but otherwise said that the serial was "a salivating treat". The A.V. Club reviewer Christopher Bahn found some minor problems in the script, but gave a positive review of the story, pointing out how it did not rip off classic stories but repurposed them. DVD Talk's David Cornelius gave the serial four out of five stars, saying that it "allows for a wide range of storytelling tones without feeling cluttered or uneven", though at points the "silliness" of the Morbius costume threatened to "overtake" the story.

Trichotillomania: Bizzare Patern of Hair Loss at 11-Year-old Girl.

Trichotillomania (TTM) is defined by the Diagnostics and Statistic Manual of Mental Disorders, 4th edition (DMS-IV) as hair loss from a patient`s repetitive self-pulling of hair. The disorder is included under anxiety disorders because it shares some obsessive-compulsive features. Patients have the tendency towards feelings of unattractiveness, body dissatisfaction, and low self-esteem (1,2). It is a major psychiatric problem, but many patients with this disorder first present to a dermatologist. An 11-year-old girl came to our department with a 2-month history of diffuse hair loss on the frontoparietal and parietotemporal area (Figure 1). She had originally been examined by a pediatrician with the diagnosis of alopecia areata. The patient`s personal history included hay fever and shortsightedness, and she suffered from varicella and mononucleosis. Nobody in the family history suffered from alopecia areata, but her father has male androgenetic alopecia (Norwood/Hamilton MAGA C3F3). The mother noticed that the child had had changeable mood for about 2 months and did not want to communicate with other persons in the family. The family did not have any pet at home. At school, her favorite subjects were Math and Computer Studies. She did not like Physical Education and did not participate in any sport activities during her free time. This was very strange because she was obese (body-mass index (BMI) 24.69). She was sometimes angry with her 13-year-old sister who had better results at school. The girl had suddenly started to wear a blue scarf. The parents did not notice that she pulled out her hair at home. Dermatological examination of the capillitium found a zone of incomplete alopecia in the frontoparietal and parietotemporal area, without inflammation, desquamation, and scaring. Hairs were of variable length (Figure 1). There was a patch of incomplete alopecia above the forehead between two stripes of hair of variable length (Figure 2). The hair pull test was negative along the edges of the alopecia. Mycological examination from the skin capillitium was negative. The trichoscopy and skin biopsy of the parietotemporal region of the capillitium (Figure 3) confirmed trichotillomania. Laboratory tests (blood count, iron, ferritin, transferrin, selenium, zinc, vitamin B12, folic acid, serology and hormones of thyroid gland) were negative. We referred the girl for ophthalmologic and psychological examination. Ophthalmologic examination proved that there was no need to add any more diopters. The psychological examination provided us with a picture in which she drew her family (Figure 4). The strongest authority in the family was the mother because she looked after the girls for most of the day. She was in the first place in the picture. The father had longer working hours and spent more time outside the home. He worked as a long vehicle driver. He was in the second place in the picture. There was sibling rivalry between the girls, but the parents did not notice this problem and preferred the older daughter. She was successful at school and was prettier (slim, higher, curly brown hair, without spectacles). Our 11-years-old patient noticed all these differences between them, but at her level of mental development was not able to cope with this problem. She wanted to be her sister's equal. The sister is drawn in the picture in the third place next to father, while the patient's own figure was drawn larger and slim even though she was obese. Notably, all three female figures had very nice long brown hair. It seemed that the mother and our patient had better quality of hair and more intense color than the sister in the drawing. The only hairless person in the picture was the father. The girl did not want to talk about her problems and feelings at home. Then it was confirmed that our patient was very sensitive, anxious, willful, and withdrawn. She was interested in her body and very perceptive of her physical appearance. From the psychological point of view, the parents started to pay more interest to their younger daughter and tried to understand and help her. After consultation with the psychiatrist, we did not start psychopharmacologic therapy for trichotillomania; instead, we started treatment with cognitive behavioral therapy, mild shampoo, mild topical steroids (e.g. hydrocortisone butyrate 0.1%) in solution and methionine in capsules. With parents' cooperation, the treatment was successful. The name trichotillomania was first employed by the French dermatologist Francois Henri Hallopeau in 1889, who described a young man pulling his hair out in tufts (3-5). The word is derived from the Greek thrix (hair), tillein (to pull), and mania (madness) (5). The prevalence of TTM in the general adult population ranges from 0.6% to 4%, and 2-4% of the general psychiatric outpatient population meet the criteria for TTM (2-5). The prevalence among children and adolescents has been estimated at less than 1% (5). The disease can occur at any age and in any sex. The age of onset of hair pulling is significantly later for men than for women (3). There are three subsets of age: preschool children, preadolescents to young adults, and adults. The mean age of onset is pre-pubertal. It ranges from 8 to 13 years (on average 11.3 years) (2-5). The occurrence of hair-pulling in the first year of life is a rare event, probably comprising <1% of cases (5). The etiology of TTM is complex and may be triggered by a psychosocial stressor within the family, such as separation from an attachment figure, hospitalization of the child or parent, birth of a younger sibling, sibling rivalry, moving to a new house, or problems with school performance. It has been hypothesized that the habit may begin with "playing" with the hair, with later chronic pulling resulting in obvious hair loss (2). Environment is a factor because children usually pull their hair when alone and in relaxed surroundings. The bedroom, bathroom, or family room are "high-risk" situations for hair-pulling (5). Men and women also differed in terms of the hair pulling site (men pull hair from the stomach/back and the moustache/beard areas, while women pull from the scalp) (3). Pulling hair from siblings, pets, dolls, and stuffed animals has also been documented, often occurring in the same pattern as in the patient (5). Genetic factors contributing to the development of TTM are mutations of the SLITRK1 gene, which plays a role in cortex development and neuronal growth. The protein SAPAP3 has been present in 4.2% of TTM cases and patients with obsessive-compulsive disorder (OCD). It may be involved in the development of the spectrum of OCD. A significantly different concordance rate for TTM was found in monozygotic (38.1%) compared with dizygotic (0%) twins in 34 pairs (3). The core diagnostic feature is the repetitive pulling of hairs from one`s own body, resulting in hair loss. The targeted hair is mostly on the scalp (75%), but may also be from the eyebrows (42%), eyelashes (53%), beard (10%), and pubic area (17%) (3,5). There are three subtypes of hair pulling - early onset, automatic, and focused. Diagnostic criteria for TTM according to DSM-IV criteria are (2,3,5): 1) recurrent pulling of one`s hair resulting in noticeable hair loss; 2) an increasing sense of tension immediately prior to pulling out the hair or when attempting to resist the behavior; 3) pleasure, gratification, or relief when pulling out the hair; 4) the disturbance is not better accounted for by another mental disorder and is not due to a general medical condition (e.g., a dermatologic condition); 5) the disturbance causes clinically significant distress or impairment in social, occupational, or other important areas of functioning. The differential diagnosis includes alopecia areata (Table 1) (6), tinea capitis, telogen effluvium, secondary syphilis, traction alopecia, loose anagen syndrome, lichen planopilaris, alopecia mucinosa, and scleroderma (2-5). Biopsy of an involved area (ideally from a recent site of hair loss) can help to confirm the diagnosis (5). On histologic examination, there are typically increased numbers of catagen and telogen hairs without evidence of inflammation. Chronic hair pulling induces a catagen phase, and more hairs will be telogen hairs. Pigment casts and empty anagen follicles are often seen. Perifollicular hemorrhage near the hair bulb is an indicator of TTM (2). Complications of TTM are rare, but they comprise secondary bacterial infections with regional lymphadenopathy as a result of picking and scratching at the scalp. Many patients play with and ingest the pulled hairs (e.g. touching the hair to lips, biting, and chewing). Trichophagia (ingestion of the hair) can lead to a rare complication named trichobezoar (a "hair ball" in stomach). This habit is present in approximately 5% to 30% of adult patients, but it is less frequent in children. Patient with trichophagia present with pallor, nausea, vomiting, anorexia, and weight loss. Radiologic examination and gastroscopy should not be delayed (2,4,5). The management of the disease is difficult and requires strong cooperation between the physician, patient, and parents. The dermatologist cannot take part in the therapy, strictly speaking, but without the psychological, psychopharmacologic, and topic dermatologic treatment a vicious circle will be perpetuated.

Bertha Pappenheim

Throughout the two years of her illness, she developed a wide spectrum of symptoms: Language disorders (aphasia): on some occasions she could not speak at all, sometimes she spoke only English, or only French, or Italian. She could however always understand German. The periods of aphasia could last for days, and sometimes varied with the time of day. Neuralgia: she suffered from facial pain which was treated with morphine and chloral and led to addiction. The pain was so severe that surgical severance of the trigeminus nerve was considered. Paralysis (paresis): signs of paralysis and numbness occurred in her limbs, primarily on only one side. Although she was right-handed, she had to learn to write with her left hand because of this condition. Visual impairments: she had temporary motor disturbances in her eyes. She perceived objects as being greatly enlarged and she squinted.

Acupuncture Therapies for Addressing Post Odontectomy Neuropathy.

<bBackground:</b Third-molar extraction is a common oral surgical procedure that can cause complications. Although rare, nerve injuries that result in permanent neuropathy can occur and include a variety of complaints such as sensory disorders, taste-sensation disorders, speech articulation disorders, etc. Acupuncture is one of several nonpharmacologic therapies that has played a role in managing neuropathic lesions and has been proven to produce good results. <bCase:</b A 44-year-old woman with postodontectomy neuropathy developed paresthesia, dysarthria, xerostomia, dysgeusia, pain in the gums and lower right jaw, a chewing disorder, and cephalgia. Her numerical rating scale (NRS) results were: lower right gum pain, 3/10; numbness of the tongue, 4/10; and headache, 1/10. A physical examination revealed dysarthria, a decreased sense of sharpness and dullness in the right mandibular nerve branches, decreased right masseter muscle contractions, and tenderness on the right GB 20 point. Electromyography revealed partial functional lesions in the postganglion at the right fifth cranial nerve. She had body acupuncture therapy at GV 20, GB 20, ST 5, ST 6, ST 7, CV 23, LI 4, HT 5, ST 36, LU 7, and KI 6; ear acupuncture at the Parotid and <iShenmen</i points; and treatment with the Tan Balance Method. <bResults:</b After 3 consecutive sessions of acupuncture therapy, this patient's symptoms were reduced. <bConclusions:</b Acupuncture was helpful for reducing paresthesia, dysarthria, xerostomia, dysgeusia, gum and lower right jaw pain, a chewing disorder, and cephalgia in this patient with postodontectomy neuropathy. Clinical trials are needed to support the findings in this case.

Neurology_Adams

The first step in diagnosis is to recognize that the patient is confused. This is obvious in most cases but, as pointed out earlier, the mildest forms, particularly when some other alteration of personality is prominent, may be overlooked. Sometimes, the patient’s attention can be best engaged by speaking softly or whispering rather than shouting or using a conversational amplitude of voice. A subtle disorder of orientation may be betrayed by an incorrect response regarding dates (off by more than one day of the month or day of the week), or in misnaming the hospital. The ability to retain a span of digits forward (normally 7) and backward (normally 5), spelling a word such as “world” or “earth” forward and then backward, reciting the months of the year in their reverse order, and serial subtraction of 3s from 30 or 7s from 100 are useful bedside tests of the patient’s capacity for attentiveness and sustained mental activity, though some of these presuppose that the patient is

Spontaneous spinal epidural hematoma mimicking Guillain-Barre Syndrome.

The initial symptoms of Guillain-Barre Syndrome (GBS) can be similar to a case of spontaneous spinal epidural hematoma (SSEH) located at the cervicothoracic junction. Therefore, SSEH may be misdiagnosed as GBS. A previously healthy 6-year-old girl presented with a 2-day history of progressive pain in the lower extremities and an inability to walk. On initial evaluation, she was completely paraparetic in the lower extremities. Deep tendon reflexes were absent in the lower extremities, and Babinski reflexes were positive on both sides. She exhibited reduced response to light touch and pinprick with a sensory level below T10, and experienced difficulty during urination. However, the strength, sensation and flexion of upper extremities were normal. Because her presentation and examinations were consistent with GBS, we initiated intravenous immunoglobulin therapy. The next day, she also developed pain and muscle weakness of the right upper extremity. Three days after admission, respiratory depression progressed rapidly. Spinal MRI showed a mass extending from the level of C7-T3, with spinal cord compression. The patient underwent an emergency laminectomy with evacuation of hematoma, and was diagnosed with SSEH. Sixty days after admission, she was transferred to the rehabilitation hospital with severe neurologic sequelae of paralysis in both legs. SSEH might have severe consequences, including neurologic deficits and risk of death. This case report serves to raise the awareness of SSEH that mimics the initial presentation of GBS.

[The early hemiparesis of stroke in evolution of basilar artery--case report].

In basilar artery thrombosis, hemiparesis without obvious brainstem signs may precede the tetraplegia with coma or a locked-in state. This premonitory hemiparesis was called as "herald hemiparesis" by Fisher (1988). Its early detection is important because immediate anticoagulant therapy may prevent its evolution. We reported two patients with such hemiparesis. The first case was a 71-year-old diabetic and hypertensive man who was admitted because of right hemiparesis, dysarthria and decreased spontaneous speech. Initially, his symptoms looked like those of a left cerebral lesion. CT scans revealed no fresh cerebrovascular lesion. A few hours later, a myoclonic movement appeared in his left lower limb, and right MLF syndrome developed. We thought he had basilar artery thrombosis and we started intravenous administration of heparin. But he developed tetraplegia with locked-in state. The second case was a 76-year-old diabetic and hypertensive man with a confusional state, right hemiparesis, dysarthria and conjugate gaze deviation to left. After admission, he rapidly became comatose and developed respiratory arrest. CT scans revealed low density areas in the brainstem, cerebellum and the occipital lobe. He died ten days later. Early detection of herald hemiparesis is by no means easy. However, severe dysarthria and myoclonic jerks in a patient with hemiparesis should be considered as warning signs to indicate the herald hemiparesis with subsequent severe brainstem infarction.

[Ciguatera: clinical relevance of a marine neurotoxin].

A 45-year-old woman complained of gastrointestinal symptoms followed by paraesthesiae of the mouth, face, hands, feet, and trunk after she had eaten fish while she was on a journey to the red sea. Additionally, she suffered from headaches and muscle aches, weakness of the arms and legs, instability of gait as well as troubles of vision, taste, speech, and swallowing. Neurological examination revealed a cerebellar syndromE with mild dysarthria, multifocal dysaesthesiae, paresis of the arms and legs, generalized hyporeflexia, bilaterally reduced vision, and ataxic gait. Laboratory work-up provided no diagnostic clues. Cranial and spinal magnetic resonance imaging were normal. The cerebrospinal fluid showed a normal cell count and a moderately impaired barrier function. Electrophysiological evaluation suggested a sensory and motor axonal neuropathy. Based on the history and the characteristic clinical findings we made a diagnosis of ciguatera, a marine food poisoning caused by the ingestion of fish contaminated with ciguatoxin. Symptomatic treatment with mannitol, amitriptyline, carbamazepine, and corticosteroids started 3 weeks after the onset of symptoms proved inefficient, however, during the 21 months of follow-up there was a marked spontaneous clinical and electrophysiological reversal of symptoms. In the age of growing foreign tourism marine food intoxications have to be considered in the differential diagnosis of the frequently occurring travellers-diarrhea. The diagnosis of ciguatera is based on the typical history of a fishmeal followed by characteristic clinical findings with predominant neurological symptoms, often including dysaesthesiae, besides gastrointestinal disturbances. In the early phase of the disease intravenous therapy with mannitol has emerged as an efficient treatment.

Verbum dicendi

Diagnostics of direct speech in Japanese

Middle cerebral artery syndrome

Signs and Symptoms Hemiparesis or hemiplegia of the lower half of the contralateral face Hemiparesis or hemiplegia of the contralateral upper and lower extremities* Sensory loss of the contralateral face, arm and leg* Ataxia of contralateral extremities* Speech impairments/aphasia: Broca's area, Wernicke's or Global aphasia as a result of a dominant hemisphere lesion (usually the left brain) Perceptual deficits: hemispatial neglect, anosognosia, apraxia, and spatial disorganization as a result of a non-dominant hemisphere lesion (usually the right brain) Visual disorders: déviation conjuguée, a gaze preference towards the side of the lesion; contralateral homonymous hemianopsia Note: *faciobrachial deficits greater than that of the lower limb Diagnosis Diagnosis can be confirmed by CT Scan or MRI for advanced investigations. References External links Stroke Syndromes affecting the nervous system

Neurology_Adams

This pattern of speech is caused by weakness or paralysis of the articulatory muscles, the result usually of disease of the motor nuclei of the medulla and lower pons or their intramedullary or peripheral extensions (lower motor neuron paralysis). In advanced forms of this disorder, the shriveled tongue lies inert and fasciculating on the floor of the mouth, and the lips are lax and tremulous. Saliva constantly collects in the mouth because of dysphagia, and drooling is troublesome. Dysphonia, an alteration of the voice to a rasping monotone because of vocal cord paralysis, is often an additional feature. As this condition evolves, speech becomes slurred and progressively less distinct. There is special difficulty in the enunciation of vibratives, such as r, and as the paralysis becomes more complete, lingual and labial consonants are finally not pronounced at all. In the past, bilateral paralysis of the palate, causing nasality of speech, often occurred with diphtheria and

Oral and Vulvar Lichen Sclerosus.

Lichen sclerosus (LS) is a chronic, inflammatory, mucocutaneous disorder of genital and extragenital skin (1). Simultaneous involvement of the oral mucosa is extremely rare, but it may be the only affected area (2). A 55-year-old woman was referred to the Department of Oral Medicine, School of Dental Medicine University of Zagreb due to whitish lesions on the right ventrolateral part of the tongue and buccal mucosa with desquamative gingivitis (Figure 1, a-c). The lesions were asymptomatic but indurated on palpation. Histology was conclusive for oral lichen sclerosus (OLS). The lesions on gingiva were successfully treated with betamethasone ointment, three times a day for two weeks. One year earlier, she had been referred to the Department of Dermatology and Venereology with progressive pruritus and dyspareunia, white patches, obliteration of the labia minora, and stenosis of the introitus (Figure 2). Histology was conclusive for vulvar LS (Figure 3, a and b). She was successfully treated for 5 months with clobetasol propionate 0.05% ointment. The patient was taking levothyroxine to treat hypothyroidism associated with Hashimoto's thyroiditis and was otherwise healthy. Oral LS is clinically characterized by the appearance of white macules, papules, or plaques mostly appearing on labial mucosa but also on buccal, palate mucosa and on the lower lip (2,3). On the genitals, it typically manifests as atrophic white plaques, which may be accompanied by purpura or fissuring (1). While vulvar LS is often associated with pruritus, dyspareunia, and dysuria, OLS is often asymptomatic, although pain, soreness, pruritus, and tightness when opening the mouth can be present (1,2). Oral manifestations of LS, as well as association of anogenital and oral LS, are rarely reported in the literature (4-6). Tomo et al. searched the Medline database for papers reporting oral LS cases with histological diagnosis confirmation from 1957 to 2016 and found only 34 cases of oral LS with histopathologic confirmation of the diagnosis (4). Kakko et al. reported 39 histologically proven cases of OLS (2). Attilli et al. (5) reviewed the clinical and histologic features of 72 cases of LS with oral/genital involvement. They reported that LS was diagnosed with exclusive genital lesions in 45, exclusive lip involvement in 20, and orogenital involvement in only 7 cases (5). Some believe that many cases of clinically diagnosed lichen planus may actually be LS and that isolated oral mucosal LS may not be as rare as is generally thought (2). While vulvar LS can occur at any age with increasing incidence with age, the median age of patients with OLS was 34 years and most of the patients were female (1,2,5). Due to the small number of patients in the literature, treatment recommendations for OLS are not available. In case of symptomatic oral lesions, topical or intralesional corticosteroids are considered to be the first-line treatment (2). First-line treatment for anogenital LS is a potent to very potent topical corticosteroid ointment, and second-line therapies include topical calcineurin inhibitors 1% pimecrolimus and 0.1% and 0.03% tacrolimus (1). For treatment-resistant genital LS, oral retinoids, methotrexate, and possibly local steroid injections for single lesions are mainly applicable for women (1). There is limited evidence for systemic treatments for both conditions. If it is not treated, genital LS is associated with a greater degree of scarring and an elevated risk of progression to squamous cell cancer; however, malignant transformation of OLS has not been reported (1-6). Due to the very rare presentation in the oral cavity, it is important to notice these lesions during a dental exam.

Neurology_Adams

Writing is, of course, an integral part of language function, but a less essential and universal component, for a segment of the world’s population speaks but does not read or write. It might be supposed that all the rules of language derived from the study of aphasia would be applicable to agraphia. In large part, this is true. One must be able to formulate ideas in words and phrases in order to have something to write as well as to say; hence, disorders of writing, like disorders of speaking, reflect all the basic defects of language. But there is an obvious difference between these two expressive modes. In speech, only one final motor pathway coordinating the movements of lips, tongue, larynx, and respiratory muscles is available, whereas if the right hand is paralyzed, one can still write with the left one, or with a foot, and even with the mouth by holding a pencil between the teeth (a contrivance used by individuals whose arms are paralyzed by cervical root avulsion from

Moyamoya Syndrome after Radiation Therapy: a Clinical Report.

Moyamoya syndrome (MMS) is a rare, chronic and progressive vasculopathy with a characteristic angiographic pattern and well-recognized predisposing conditions, such as cranial therapeutic radiation. We report the case of a 36-year-old Caucasian female with a history of craniopharyngioma treated with whole-brain radiotherapy 20 years previously. She was admitted to the emergency department with disorientation and imperceptible speech lasting for 1 hour. Upon examination, she had slight motor aphasia, without sensory or motor deficits. However, the neurological deficits worsened on standing position. The computed tomography (CT) angiogram and transcranial Doppler ultrasonography revealed occlusion of the distal portion of the left internal carotid artery (ICA). Mechanical thrombectomy was attempted without success. Head CT was repeated, revealing left periventricular acute ischaemic stroke. The cerebral angiography showed total occlusion of the left ICA with an exuberant network of transdural collateral vessels, confirming MMS. The patient completed a functional rehabilitation program with progressive improvement of deficits and maintained a multidisciplinary follow-up. MMS is a serious late complication from cranial radiation therapy and a well-described risk factor for ischaemic stroke in younger patients. Therefore, early detection and prompt treatment are mandatory, as the consequences can be disastrous, such as cognitive and neurologic decline due to repeated strokes. Moyamoya syndrome should be considered in younger patients presenting with acute neurologic deficits, particularly those treated with cranial radiotherapy.The treatment of acute stroke in patients with moyamoya syndrome should include intravenous hydration and avoidance of hypotension.The benefit of antiplatelet agents is limited and equivocal for patients with moyamoya syndrome and oral anticoagulants are not recommended for long-term treatment.

Neurology_Adams

In the most advanced form of the syndrome, patients lose all power of speaking. Not a word can be uttered in conversation, in attempting to read aloud, or in trying to repeat words that are heard. One might suspect that in this mutism the lingual and phonatory apparatus is paralyzed, until patients are observed to have no difficulty chewing, swallowing, clearing the throat, crying or shouting, and even vocalizing without words. Occasionally, the words yes and no can be uttered, usually in the correct context. Or patients may repeat a few stereotyped utterances over and over again, as if compelled to do so, a disorder referred to as monophasia (Critchley), recurring utterance (Hughlings Jackson), verbal stereotypy, or verbal automatism. If speech is possible at all, certain habitual expressions, such as “hi,” “fine, thank you,” or “good morning,” seem to be the easiest to elicit, and the words of well-known songs may be sung hesitantly, or counting by consecutive numbers may remain

Seropositive Neuromyelitis Optica in a Case of Undiagnosed Ankylosing Spondylitis: A Neuro-Rheumatological Conundrum.

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune astrocytopathy against foot processes of aquaporin-4 (AQP4) water channels. Patients with NMOSD tend to have other coexisting autoimmune/connective tissue diseases. However, AQP-4-antibody-positive NMOSD coexisting with ankylosing spondylitis (AS) is rare. AS is an immune-mediated disorder, a subset of axial spondyloarthropathies, which commonly manifests as chronic inflammatory back pain in young people, and it has a strong association with HLA-B27. In this study, a 35-year-old Indian man with an undiagnosed progressive axial spondyloarthropathy (i.e., AS) is reported presenting with acute-onset longitudinally extensive transverse myelitis, a clinical subset of NMOSD. Neuromyelitis optica spectrum disorder (NMOSD), a primary demyelinating disorder of the central nervous system (CNS), is an autoimmune astrocytopathy against foot processes of aquaporin-4 (AQP4) water channels, which manifests with optic neuritis, longitudinally extensive transverse myelitis (LETM), area-postrema syndrome, brainstem syndrome diencephalic syndrome, and cerebral syndrome.<sup1-4</sup Ankylosing spondylitis (AS) is an immune-mediated disorder, a subset of axial spondyloarthropathies, which commonly manifests as chronic inflammatory back pain in young people, and it has a strong association with HLA-B27.<sup5,6</sup AS characteristically targets the axial skeleton, peripheral joints, entheses (connective tissues between tendons/ligaments and bones), and gut.<sup5,6</sup Patients with NMOSD tend to have other coexisting autoimmune/connective tissue diseases.<sup7</sup For example, cases with NMOSD and multiple sclerosis, which are other autoimmune primary demyelinating disorders of the CNS, have been reported.<sup8,9</sup However, concurrent existence of AS and NMOSD in the same patient even over years of disease course is rare.<sup10,11</sup In addition, studies describing neurological manifestations of AS are limited,<sup12</sup and they focus on joint inflammation and long-standing bony pathology (ankylosis) related to compressive myelopathy, myelo-radiculopathy, and cauda equina syndromes.<sup12,13</sup The authors present a case of a young Indian man with an undiagnosed progressive AS (misdiagnosed and mismanaged by an indigenous medical practitioner) presenting with acute-onset LETM variant of AQP4-positive NMOSD. A 35-year-old healthy, non-comorbid man from rural India came to the outpatient department with complaints of persistent tingling, numbness, and weakness of both lower limbs (right more than left) for 10 days. The clinical picture showed acute-onset urinary retention, which was relieved by urinary catheterization. An indigenous medical practitioner had prescribed drugs to treat a urinary tract infection. His weakness gradually progressed over the following week, causing him to become bedridden. During the removal of the catheter, he felt urgency, increased frequency of micturition, and overt urinary incontinence. He gave no history suggestive of any girdle-like sensations, root/radicular/tract pain, vertebral pain, trauma, recent vaccination, and diarrheal or febrile illness. For the last 8 months, he had a complaint of an insidious-onset, persistent, bilateral, dull aching pain in the gluteal region accompanied by low-back pain and morning stiffness up to 1 h, which markedly improved with activity and reoccurred following long periods of inactivity. He sometimes had to rise in the middle of the night because of excruciating pain, which could be relieved after moving around the room and corridors for half an hour. He was taking over-the-counter diclofenac tablets for pain relief prescribed by some indigenous medical practitioners who told him that it was due to overwork in agricultural fields, that is, mechanical back pain. He also had a normal X-ray of the lumbosacral spine. He had no addiction liabilities, and none of the family members had ever suffered from a similar kind of illness. He had never consulted any trained medical practitioner, as his previous back-pain-related symptoms responded well to the tablets prescribed by the indigenous medical practitioner(s). During examination, he was found to have recent-onset, asymmetric spastic paraparesis (right more than left) with upper motor neuron-type urinary bladder symptoms. Cognitive assessment (assessed by the Montreal cognitive assessment test) was normal, and posterior column sensations were preserved. Sensory system examination revealed no definite sensory level. Except for the paretic lower limbs, cerebellar functions were normal in other regions. Neuro-ophthalmological examinations were also normal, and no signs of meningeal irritation were observed. The history and course of the disease and clinical examinations were analyzed. Selective tractopathy (early and predominant motor and autonomic tract affection) was suggested for an intramedullary demyelinating pathology affecting the anterior central cord. This case was initially classified as acute-onset non-compressive myelopathy at the lower cervical/upper dorsal region level in a patient with a pre-existing axial spondyloarthropathy. Complete blood cell count; liver, kidney, and thyroid function tests; and plasma glucose and electrolytes were normal, except for an increased erythrocyte sedimentation rate (66 mm in the first hour). Magnetic resonance imaging (MRI) of the spinal cord revealed a demyelinating LETM from C5 to D4 level (Figure 1). Meanwhile, an MRI of the sacroiliac joints revealed bilateral sacroiliitis. Brain and orbital MRIs were devoid of any lesions. Anti-aquaporin 4 (AQP-4) antibodies were tested by cell-based assay in serum and cerebrospinal fluid (CSF), and both were positive. CSF further revealed lymphocytic pleocytosis and increased intrathecal protein production. Visually evoked potential recordings were also normal. In addition, anti-myelin oligodendrocyte glycoprotein antibodies were negative. Anti-nuclear antibody (ANA), ANA-profile, autoimmune vasculitis profile (c-ANCA, p-ANCA), neurovirus panel (i.e., polymerase chain reaction for adenovirus, Epstein-Barr virus, herpes simplex viruses 1 and 2, human herpesviruses 6 and 7, cytomegalovirus, enteroviruses, varicella-zoster virus, Japanese encephalitis, and dengue virus), CSF-polymerase chain reaction for <iMycobacterium tuberculosis</i, angiotensin-converting enzyme, anti-phospholipid, and anti-thyroid antibodies were negative. Anti-CCP-antibody and rheumatoid factor were also negative, including creatine phosphokinase level and serum vitamin B12. Moreover, serologies for hepatitis B, C, human immunodeficiency virus, and scrub typhus were negative. However, HLA-B27 assay was positive. The final diagnosis was AQP4-positive NMOSD associated with AS. He was placed on pulse intravenous methylprednisolone (1 g/day for 5 days). Consequently, his lower limb power improved remarkably. Cyclical rituximab therapy was initiated to prevent relapses. At 3-month follow-up, he had no residual neurological deficit except for persistence of paresthesias. Neuroimaging and visually evoked potential studies revealed no active or new lesions. After 6 months of therapy, a subjective and objective improvement was observed in disease severity based on the Ankylosing Spondylitis Disease Activity Score. Our patient satisfied the new Assessment of SpondyloArthritis International Society diagnostic/classification criteria for AS and the Wingerchuk criteria for NMOSD,<sup4,14</sup an association that has been rarely reported.<sup10,11</sup Amid the extra-articular complications of long-standing AS, neurological manifestations are considered infrequent.<sup15</sup However, subclinical neurological complications may be frequent in AS.<sup12</sup Common neurological manifestations result from bony (vertebral) ankylosis, subluxation of joints, ossification of anterior and posterior longitudinal ligaments, secondary spinal canal stenosis, bony (vertebral) fractures, and subsequent compressions over nerve radicles/roots/cauda equina, and inflammation-related (entrapment) peripheral neuropathies.<sup12,16,17</sup Acute transverse myelitis can occur as a subset of several primary demyelinating disorders of the CNS (i.e., multiple sclerosis, NMOSD, myelin oligodendrocyte glycoprotein antibody disease, and acute disseminated encephalomyelitis) and various systemic autoimmune connective tissue disorders (i.e., systemic lupus erythematosus, mixed connective tissue disease, Sjögren syndrome, inflammatory bowel disease, and neurosarcoidosis).<sup18</sup Acute transverse myelitis (short or long segment) is an infrequent extra-articular complication of AS.<sup18</sup It has been reported to evolve either as a distinct neurological complication of AS, or it may develop secondary to TNF-alpha-inhibitor therapy for the treatment of AS.<sup18,19</sup AS is a heritable inflammatory spondyloarthropathy that primarily affects the axial skeleton, which is mediated by T-cells; B-cells only play a minor role.<sup5</sup On the contrary, the key for the pathogenesis of NMOSD is the production of autoantibodies against AQP-4 channels expressed on astrocytes, leading to complement-mediated damage, with ensuing demyelination. Myelitis usually shows high signal intensity on the tbl2-weighted image and contrast enhancement in the spinal cord.<sup1-4</sup Despite the difference in molecular mechanisms, the diagnosis of these diseases in the same individual may not be coincidental. Recent evidence has shown T-cell-mediated inflammatory responses in cases of NMOSD.<sup20</sup In particular, Th17 and Th2-related cytokines are elevated in the CSF of NMO patients.<sup20</sup Environmental factors such as <iEscherichia coli</i have also been proven to aggravate autoimmunity in AS and NMOSD (however, body fluid cultures for <iEscherichia coli</i, performed in our patient, showed similar association, and they were found negative two times).<sup21,22</sup Although large-scale epidemiological studies investigating the underlying pathogenesis related to these diseases are lacking, studies have demonstrated an increased incidence of optic neuritis among patients with AS.<sup23</sup Systemic sclerosis and mixed and undifferentiated connective tissue diseases were excluded after expert opinions (from two board-certified rheumatologists and two dermatologists) because of the lack of suggestive clinical findings (e.g., absence of skin thickening, salt-and-pepper appearance, nail changes, Mauskopf facies, sclerodactyly, calcinosis cutis, Raynaud's phenomenon, other cutaneous manifestations, pulmonary arterial hypertension/interstitial lung disease, dysphagia, muscular pain/weakness renal impairments, absence of ANA, anti-centromere antibodies, anti-Scl-70, PM-Scl antibodies, anti-ds DNA, PCNA, CENP-B, anti-nucleosomes, anti-Smith, anti-U1-RNP, anti-Jo1, anti-Mi2, anti-Ro52, anti-La antibodies, and normal C3 and C4 complement levels) (The European League Against Rheumatism and the American College of Rheumatology classification criteria 2019).<sup24</sup Finally, our patient was treated with intravenous steroids followed by rituximab infusions, a monoclonal anti-CD20 antibody directed against B-cells. In particular, this patient clinically and radiologically responded to immunomodulatory drugs, which might support a possible common pathogenic basis of the two processes. TNF-alpha inhibitors are commonly used as novel therapeutics in AS; however, they can potentially result in serious complications, that is, secondary demyelinating disorders.<sup25</sup However, such inhibitors in this patient were not used. When used in cases of AS, they show satisfactory results.<sup25,26</sup Therefore, it was decided to treat him with rituximab only without adding any second immunomodulatory. Other possible therapeutic options include cyclophosphamide and mycophenolate mofetil, but they were not used because of their low efficacy-safety balance. Moreover, plasmapheresis was not available in our specific setting, despite solid evidence that early treatment with therapeutic strategy (5-7 courses) provides good long-term outcomes in patients with NMOSD.<sup27</sup Therefore, when dealing with a case of acute non-compressive myelopathy, history and clinical examination are important to determine the potential underlying etiology and identify an undermined systemic disorder with apparently unrelated non-specific features. Connective tissue disorders should always be considered as a differential diagnosis and be ruled out in all cases of either seropositive or seronegative NMOSD. A diagnosis of AS should be considered in relevant circumstances when dealing with a case of isolated seronegative LETM. Moreover, early diagnosis and treatment of AS are quintessential to prevent lifelong distressing disabilities. However, whether patients with AS have any extra predilection to develop NMOSD throughout their life requires further studies.