Cases reported "Reflex, Abnormal"

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1/82. Hyperactive rhizopathy of the vagus nerve and microvascular decompression. Case report.

    A 37-year-old woman underwent microvascular decompression of the superior vestibular nerve for disabling positional vertigo. Immediately following the operation, she noted severe and spontaneous gagging and dysphagia. Multiple magnetic resonance images were obtained but failed to demonstrate a brainstem lesion and attempts at medical management failed. Two years later she underwent exploration of the posterior fossa. At the second operation, the vertebral artery as well as the posterior inferior cerebellar artery were noted to be compressing the vagus nerve. The vessels were mobilized and held away from the nerve with Teflon felt. The patient's symptoms resolved immediately after the second operation and she has remained symptom free. The authors hypothesize that at least one artery was shifted at the time of her first operation, or immediately thereafter, which resulted in vascular compression of the vagus nerve. To the authors' knowledge, this is the first reported case of a hyperactive gagging response treated with microvascular decompression. The case also illustrates the occurrence of a possibly iatrogenic neurovascular compression syndrome.
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2/82. Hypertonia, hyperreflexia, and excessive startle response in a neonate.

    Following an uneventful gestation, a newborn girl presented with hypertonia, hyperreflexia, tremor, and excessive startle response. nose tap elicited a dramatic head recoil. Her mother had similar symptoms beginning as a child that improved but persisted into adulthood. In addition, several members of mother's family died unexpectedly in infancy. Hypertonia in the newborn period indicates central nervous system dysfunction of several possible causes, most of which are associated with severe cognitive deficits and limited neurological development.
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ranking = 3.4893129907003
keywords = central nervous system, nervous system
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3/82. Sequestrum-like appearance of a multiple sclerosis brain lesion on serial magnetic resonance images.

    Using serial magnetic resonance imaging, we monitored an unique lesion of the brain in a 15-year-old girl with clinically definite and laboratory-supported remitting-relapsing multiple sclerosis. During initial phases of the disease course, cystic necrosis around the plaque was observed. Later, remyelination of the central core of the lesion was speculated, as similarities in signal intensity between the core and the normal appearing white matter were partially recovered both on the T1- and the T2-weighted images.
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keywords = brain
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4/82. Loss of ability to sneeze in lateral medullary syndrome.

    Four consecutive patients with lateral medullary syndrome reported reversible inability to complete a reflex sneeze, despite retaining the urge to do so and the ability to mimic the motor act. This previously undescribed feature of a relatively common syndrome is in keeping with the known location of a "sneeze center" in the lateral medulla of cat. In man, unilateral brainstem lesion is sufficient to abolish the sneeze reflex temporarily.
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5/82. Hyperreflexia in a patient with motor axonal guillain-barre syndrome.

    We report a patient who presented after an episode of diarrhoea with ascending, symmetrical weakness without sensory loss, and without sphincter or other autonomic dysfunction. On clinical examination there were no cranial nerve deficits. Hyperreflexia of tendon jerks without other upper motor neurone signs was found. Electrophysiological examination demonstrated acute distal symmetrical motor axonal polyneuropathy. No electrophysiological signs of peripheral nerve demyelination or central nervous system involvement were found. Albuminocytologic dissociation was present in the cerebrospinal fluid. Stool culture and serological tests were inconclusive. Our patient's clinical picture was, apart from hyperreflexia of tendon jerks throughout the disease, characteristic of guillain-barre syndrome. This is the first such patient reported in europe. The aetiology remained unclear. We suggest that selective axonal motor fibre affection, with possible mild pyramidal involvement, caused tendon jerk hyperreflexia.
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ranking = 3.4893129907003
keywords = central nervous system, nervous system
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6/82. Susac's syndrome: beneficial effects of corticosteroid therapy in a Japanese case.

    Susac's syndrome is a rare disorder characterized by the triad of microangiopathy of the brain and retina with hearing loss. More than 50 affected individuals have been reported worldwide, all Caucasians. We herein identify the first Japanese patient with Susac's syndrome. A 36-year-old man developed recurrent subacute encephalopathy, bi- a lateral sensorineural hearing loss, and retinal arteriolar occlusions, caused by microangiopathy from a year previously. T2-weighted MRI showed multiple high-signal lesions ti predominantly in the periventricular white matter. During the exacerbated phase both high-dose intravenous methyl-prednisolone and oral prednisone therapy produced beneficial effects. He showed definite remission within 2 years from the disease onset.
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7/82. central nervous system involvement following type I aviator's bends complicated by complacency.

    A false sense of security surrounds the possibility of post-flight complications resulting from "aviator's bends." The accepted clinical clue that a patient is at risk for serious complications is the presence of some form of dysbarism at altitude. This principle has been inappropriately extended to imply that serious post-flight complications of the evolved gas syndrome only follow serious in-flight symptoms. This paper, in addition to reporting the occurrence of post-flight neurologic signs in a patient after Type I pain-only bends during an altitude chamber flight, also identifies a broader subtle complacency in the professional community that routinely deals with hypobarics.
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ranking = 6.9320592086972
keywords = nervous system
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8/82. Meniere's disease in the elderly.

    OBJECTIVES: To assess the prevalence, presentation, treatment, and evolution of Meniere's disease in elderly patients (> or = 65 yr old). STUDY DESIGN: Retrospective clinical study and case report. SETTING: A neuro-otology referral center at the university hospital in Bern, switzerland, and a neurotologic practice in Geneva, switzerland. patients: patients were selected from the hospital clinic and otolaryngologic practice registers. Among 8423 neurotologic checkups performed between 1988 and 1998, 432 (5.1%) patients had definite Meniere's disease and 66 (15.3%) of these patients were > or = 65 years old. INTERVENTION: The files of these 66 patients were analyzed. RESULTS: Meniere's disease in the elderly had 2 modes of presentation: reactivation of longstanding Meniere's disease (40.9%) and de novo Meniere's disease (59.1%), where the first dizzy spells, tinnitus, and hearing loss appeared after 65 years of age. Drop attacks occurred in 11.1 and 25.6% of the cases, respectively, and could be responsible for misdiagnosis of strokes of the brainstem. The patients were treated by mild antivertiginous drugs (betahistine, cinnarizine). Twenty-seven patients (41%) underwent surgery: transtympanic ventilation tubes (19 patients), sacculotomy (4 patients), vestibular neurectomy (3 patients), surgical labyrinthectomy (1 patient). The vertigo spells disappeared in 73.3 to 100% of the cases according to the type of surgery performed. CONCLUSION: Our study shows that Meniere's disease in the elderly is not at all uncommon. It can appear as a de novo disease or as a reactivated longstanding disease. Drop attacks are more frequent than reported in general patient populations with Meniere's disease and can mimic a stroke of the brainstem. Medical and surgical treatments have to be cautious because many of these elderly patients are fragile.
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ranking = 2
keywords = brain
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9/82. Presigmoid transpetrosal approach for the treatment of a large trochlear nerve schwannoma--case report.

    A 61-year-old man presented with a rare, large trochlear nerve schwannoma manifesting as left-sided weakness and hypesthesia, bilateral bulbar pareses, and trochlear nerve paresis persisting for 3 months. T1-weighted magnetic resonance imaging with gadolinium revealed an intensely enhanced, well-circumscribed lesion with multicystic formation occupying the prepontine and interpeduncular cisterns and compressing the pons and midbrain with greater extension to the right. The mass was completely removed through the presigmoid transpetrosal approach with preservation of the posterior cerebral, superior cerebellar, and basilar arteries and their branches. Neuroradiological examination after 3 years demonstrated no recurrence. Enlargement of a tumor in the cisternal portion is inclined to involve and/or encase the adjacent major arteries and their branches. The presigmoid transpetrosal approach is one of the best surgical routes to remove a large trochlear nerve schwannoma safely and completely.
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10/82. Constant involvement of the Betz cells and pyramidal tract in amyotrophic lateral sclerosis with dementia: a clinicopathological study of eight autopsy cases.

    We investigated clinicopathologically pyramidal signs, including hyperreflexia, Babinski sign, and spasticity, and the involvement of the primary motor cortex and pyramidal tract, in eight Japanese autopsy cases of amyotrophic lateral sclerosis (ALS) with dementia. Pyramidal signs were observed in seven (88%) of the eight autopsy cases. Hyperreflexia and Babinski sign were evident in seven (88%) and three (38%) patients, respectively, but spasticity was not observed in any of the eight patients. Loss of Betz cells in the primary motor cortex was evident in the seven cases in which this structure was examined. Astrocytosis in the fifth layer of the primary motor cortex was noticed in three cases. In all eight cases, involvement of the pyramidal tract was obvious in the medulla oblongata, but no involvement of the pyramidal tract was found in the midbrain. Involvement of the pyramidal tract in the spinal cord, particularly of large myelinated fibers, was observed in all six cases in which the spinal cord was examined. In ALS with dementia, pyramidal signs were shown to be present more frequently than previously believed, and the clinicopathological correlation between pyramidal signs and involvement of the pyramidal tract was obvious. Constant involvement of Betz cells and the pyramidal tract in ALS with dementia has not been reported. Our clinicopathological findings may make a contribution to the understanding of the clinicopathological hallmarks of this disorder. Furthermore, we believe that this study will also contribute to the elucidation of the nosological status of ALS with dementia.
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