1/157. Neuro-ophthalmic, radiographic, and pathologic manifestations of adult-onset alexander disease.A 61-year-old woman had a 3-year history of imbalance. Eye movement studies revealed square-wave jerks, gaze paretic nystagmus, rebound nystagmus, impaired smooth pursuit, impaired optokinetic nystagmus, and abnormal fixation suppression of vestibular nystagmus. A brain magnetic resonance imaging study showed extensive areas of increased signal from the middle cerebellar peduncles and dentate nuclei, which enhanced with gadolinium. Histopathological analysis of a needle biopsy specimen of the left cerebellar peduncle revealed diffuse gliosis in the presence of symmetrically distributed areas of demyelination. There were associated Rosenthal fibers. Clinicopathologic correlation supported a diagnosis of alexander disease. An adult patient with a history of progressive imbalance, ocular motility abnormalities consistent with cerebellar and/or brainstem dysfunction, and diffuse, symmetric hyperintense magnetic resonance imaging signals in brainstem and cerebellar white matter should suggest a diagnosis of adult-onset alexander disease.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/157. Hypotonia, congenital nystagmus, ataxia, and abnormal auditory brainstem responses: a report on the first white patient.A white Italian boy, aged 5 years and 8 months, is reported with failure to thrive, hypotonia, truncal ataxia, psychomotor retardation, and congenital horizontal pendular nystagmus with only waves I and II on auditory brainstem responses. Our patient's clinical picture resembles that previously reported in 10 male Oriental patients. He did not manifest spastic diplegia by the age of 2 years, as did the subjects reported in the literature, but knee-jerk hyperreflexia was evident at the most recent clinical reevaluation. Serial brain MRI studies revealed a cystic brain lesion and peritrigonal hyperintensities with no brainstem abnormalities. To date, no other child with a similar syndrome has been described either in europe or in America. The clinical features of this condition are consistent and characteristic. A definitive diagnosis is achieved by demonstrating the absence of all waves following wave I or wave II on auditory brainstem responses as early as 3 months of age. Due to the predominance of males, the occurrence in siblings, the early age at onset, the non-progressive course, and the characteristic auditory brainstem response findings, the syndrome may have a genetic origin and be attributable to a dysgenetic brainstem lesion.- - - - - - - - - - ranking = 3.6666666666667keywords = brain (Clic here for more details about this article) |
3/157. A case of the subacute brainstem encephalitis.A case of brainstem encephalitis of undetermined etiology is reported in 66-year-old woman who had a sudden onset of illness with left abducens palsy, nystagmus and ataxia. The symptoms progressed to complete paralysis of eye movements, dysphagia and left hemiparesis with generalized hyperreflexia. Examination of CSF, CT scan and MRI of the brain were normal. The patient died 4 months after onset of disease. Neuropathologic study disclosed in the brainstem numerous perivascular and nodular inflammatory cell infiltrations composed predominantly of lymphocytes T and B. Most intensive inflammation concerned midbrain and pontine tegmentum and to a lesser degree medulla oblongata, pontine nuclei and cerebellar nuclei. basal ganglia, cerebral and cerebellar cortex were unaffected. Neuropathological finding was reminiscent of brainstem encephalitides related to viral infection or to paraneoplastic syndrome. However, HSV-1, EBV, and CMV antigens were not detected by immunohistochemistry, as well as evidences of malignancy were not present in this case.- - - - - - - - - - ranking = 3keywords = brain (Clic here for more details about this article) |
4/157. Malignant pilocytic astrocytoma in the medulla oblongata: case report.A 27-year-old woman visited our hospital with chief complaints of abducens nerve palsy and cerebellar symptoms. On computerized tomographic scanning and magnetic resonance imaging, a tumor with strong enhancement was found on the dorsal side of the medulla oblongata. A tumor was excised by suboccipital craniotomy and C1 laminectomy. Histologically, many Rosenthal fibers together with pilocytic tumor cells were found in some regions, but a very high Ki-67 labeling rate accompanied by cells with nuclei of irregular size and giant cells was observed in other regions. The tumor was diagnosed as malignant pilocytic astrocytoma originating from pilocytic astrocytoma by transformation. The biological behavior of pilocytic astrocytoma is obscure in several respects. We report our experience of a case of malignant pilocytic astrocytoma that developed in the brain stem and progressed extremely rapidly.- - - - - - - - - - ranking = 0.33333333333333keywords = brain (Clic here for more details about this article) |
5/157. Perinatally ruptured dermoid cyst presenting as congenital oculomotor palsy.An eight-month-old girl presented with congenital exotropia and latent nystagmus. Further evaluation revealed congenital ptosis of the left eye and restriction of the elevation, depression and adduction of the left eye. A diagnosis of congenital oculomotor palsy was made. At the age of three months she had been examined by the neurologist because of retarded psychomotor development. All laboratory investigations were normal. At the age of eight months, a CT scan of the brain and orbit was found to be normal. The patient was treated for amblyopia. At the age of five, strabismus surgery was performed, and a large fibrous tumor encapsulating the superior and lateral rectus muscle was found. A biopsy was taken and pathology showed fibrous tissue containing a hair. Based on the clinical history, the diagnosis of a perinatally ruptured orbital dermoid cyst was made. review of the previous CT and an additional CT showed enlargement of the left lateral orbital wall with a notch in the lateral wall, indicative of a dermoid cyst.- - - - - - - - - - ranking = 0.33333333333333keywords = brain (Clic here for more details about this article) |
6/157. Preservation of the brainstem auditory evoked potential in non-convulsive status epilepticus.Brainstem auditory evoked potentials (BAEPs) were recorded from a patient simultaneously experiencing non-convulsive generalized status epilepticus (NGSE). Waves I, III and V were normal but all subsequent waves were absent. This finding indicates that structures within the brainstem adjacent to the generators for the BAEP are likely not affected by NGSE and also illustrates the resilient nature of the BAEP. This is the first report of the recording of an evoked potential during a naturally occurring generalized seizure.- - - - - - - - - - ranking = 1.6666666666667keywords = brain (Clic here for more details about this article) |
7/157. radiation-induced rhombencephalopathy.We report the case of a patient who underwent radiotherapy of the neck because of an epidermoid carcinoma in Rosenmuller's fossa. Eleven months later, T1-weighted brain magnetic resonance imaging (MRI) revealed a bulbo-pontine lesion, and the clinical course and sequential MRI results led to a diagnosis of radionecrosis-induced rhombencephalopathy. At a distance of more than three years, the lesion is no longer visible on MRI images but the severe neurological deficits remain. The clinical picture has not been improved by treatment with prednisone, hyperbaric oxygen, symptomatic therapies or anticoagulants.- - - - - - - - - - ranking = 0.33333333333333keywords = brain (Clic here for more details about this article) |
8/157. Central nystagmus induced by deep-brain stimulation for epilepsy.PURPOSE: The goal of the present study was to describe the localization of central nystagmus induced as a side effect of electrical deep-brain stimulation for epilepsy. methods: Bilateral deep-brain stimulating electrodes were inserted in the centromedian nucleus of the thalamus to control seizures in a patient with intractable epilepsy. RESULTS: Cathodal high-frequency stimulation through the deepest contact of each electrode elicited cycles of slow ipsiversive conjugate eye deviations, each followed by rapid contralateral jerks. The involved electrode contacts were situated at the mesodiencephalic junction just inferior to the centromedian nucleus of the thalamus and rostral to the superior colliculus. Right-sided stimulation evoked left beating nystagmus and left-sided stimulation evoked right beating nystagmus. Stimulation through other electrode contacts did not induce nystagmus. electronystagmography showed the nystagmus to have constant velocity slow phases. CONCLUSIONS: A central nystagmogenic area exists in humans that appears to be homologous to the nucleus of the optic tract, a region described in nonhuman primates to play a role in the generation of optokinetic nystagmus.- - - - - - - - - - ranking = 2keywords = brain (Clic here for more details about this article) |
9/157. Dorsal midbrain syndrome secondary to a pineocytoma.BACKGROUND: Dorsal midbrain syndrome is a triad of signs consisting of vertical gaze palsy, light-near dissociation of the pupils, and convergence retraction nystagmus. Associated findings may also be present. The most-common etiologies are pineal gland tumors and midbrain infarction. pineal gland tumors are rare tumors that show a predilection for males. The specific tumor reported herein, a pineocytoma, shows no gender predilection and is most common in mid- to late adulthood. CASE REPORT: A 38-year-old Native American man came to us with signs and symptoms consistent with dorsal midbrain syndrome. A neurological evaluation revealed the presence of a pineocytoma, which was later excised unsuccessfully. An explanation of the findings associated with dorsal midbrain syndrome is provided. CONCLUSION: patients who demonstrate signs consistent with dorsal midbrain syndrome should be referred for a neurological evaluation--including an MRI--to rule out any midbrain lesion.- - - - - - - - - - ranking = 3.3333333333333keywords = brain (Clic here for more details about this article) |
10/157. Paroxysmal alternating skew deviation and nystagmus after partial destruction of the uvula.A patient with suspected brain stem glioma involving the area of the left vestibular nuclei and cerebellar peduncle, developed paroxysmal alternating skew deviation and direction changing nystagmus after biopsy of the inferior cerebellar vermis resulting in destruction of the uvula. Between attacks she had right over left skew deviation with asymptomatic right beating horizontal nystagmus. Slow phases of the resting nystagmus showed increasing velocity, similar to congenital nystagmus. At intervals of 40-50 seconds, paroxysmal reversal of her skew deviation occurred, accompanied by violent left beating horizontal torsional nystagmus lasting 10-12 seconds and causing severe oscillopsia. It is proposed that this complex paroxysmal eye movement disorder results from (1) a lesion in the left vestibular nuclei causing right over left skew and right beating resting nystagmus and (2) a disruption of cerebellar inhibition of vestibular nuclei, causing alternating activity in the vestibular system with intermittent reversal of the skew deviation and paroxysmal nystagmus towards the side of the lesion.- - - - - - - - - - ranking = 0.33333333333333keywords = brain (Clic here for more details about this article) |
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