Cases reported "Brain Diseases"

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1/60. 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.
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2/60. Infantile and juvenile presentations of Alexander's disease: a report of two cases.

    We describe 2 new cases of Alexander's disease, the first to be reported in belgium. The first patient, a 4-year-old girl, presented with progressive megalencephaly, mental retardation, spastic tetraparesis, ataxia and epilepsy: post-mortem examination showed widespread myelin loss with Rosenthal fibers (RFs) accumulation throughout the neuraxis. She was the third of heterozygotic twins, the 2 others having developed normally and being alive at age 5 years. The second patient developed at age 10 years and over a decade spastic paraparesis, palatal myoclonus, nystagmus, thoracic hyperkyphosis and thoraco-lumbar scoliosis with radiological findings of bilateral anterior leukoencephalopathy. brain stereotactic biopsy at age 16 years demonstrated numerous RFs. With these 2 cases, we review the literature on the various clinico-pathological conditions reported as Alexander's disease. We discuss the nosology of this entity and the pathogeny of RFs formation and dysmyelination. Clues to the diagnosis of this encephalopathy in the living patient are briefly described.
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3/60. Oculopalatal tremor with tardive ataxia.

    Oculopalatal tremor consists of palatal tremor and pendular nystagmus and may develop in a delayed fashion after an acute brainstem lesion. Delayed sequelae are generally restricted to the eyes and branchial-derived muscles, such as those of the palate. We report three cases of oculopalatal tremor that subsequently developed disabling delayed-onset ataxia and emphasize the potential for this significant complication after larger bilateral acute brainstem lesions with sparing of the inferior olive.
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4/60. Ocular tilt reaction due to a mesencephalic lesion in juvenile polyarteritis nodosa.

    PURPOSE: To describe a case of ocular tilt reaction caused by vasculitic lesions in the midbrain in a child with polyarteritis nodosa. DESIGN: Observational case report. methods: A 5-year-old girl with a chronic illness developed diplopia associated with a left head tilt, right hypertropia, torsional nystagmus, slowed vertical saccades and poor convergence. Fundoscopic examination demonstrated conjugate leftward torsion of the eyes consistent with a sustained ocular tilt reaction.Renal angiography confirmed polyarteritis nodosa and cerebral magnetic resonance imaging demonstrated mesencephalic pathology. CONCLUSIONS: polyarteritis nodosa is a difficult condition to diagnose in a child and can cause brainstem lesions. This rare case of ocular tilt reaction of midbrain origin highlights that a sustained head tilt in a child can be due to brainstem pathology, rather than a fourth nerve palsy.
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5/60. Cerebromeningeal haemophagocytic lymphohistiocytosis.

    We describe 3 children with a progressive encephalopathy that was characterised by irritability, convulsions, cranial nerve palsies, ataxia, nystagmus, walking difficulties, delayed psychomotor development, hemiplegia/tetraplegia, visual disturbance, vomiting, neck stiffness, and non-specific signs of raised intracranial pressure. A final diagnosis was made in all 3 patients from necropsy material. The clinical features were ascribed to multiple inflammatory, predominantly lymphocytic, reactions and raised intracranial pressure. This condition is an atypical form of haemophagocytic lymphohistiocytosis, which normally presents with fever, hepatosplenomegaly, and cytopenias. By contrast, the disease pattern in our 3 children was dominated by cerebromeningeal involvement, which can precede the typical systemic symptoms of haemophagocytic lymphohistiocytosis. An awareness of this condition is important because treatments are available.
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6/60. Medullary hemorrhage causing vertigo and gaze nystagmus.

    Brainstem hematomas carry a poor prognosis. There are rare reports of excellent outcome with hemorrhages in the midbrain. We report a 43-year-old woman with vertigo and minimal neurological symptoms, whose symptoms were initially mistaken for "inner ear disease," but subsequent investigations revealed a medullary hemorrhage. Recovery was complete within two weeks. While rare, medullary hemorrhage should be considered in the differential diagnosis of vertigo.
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7/60. A Turkish case of subcortical/subependymal heterotopia associated with corpus callosum dysgenesis, craniofacial dysmorphism, severe eye abnormalities, and growth-mental retardation.

    The patient is a 12-year-old boy with a history of learning disability, growth retardation, and strabismus. Weight, height and head circumference were below the 3rd percentile. A cafe-au-lait spot, 1x1 cm a diameter, on the back region and pectus excavatum deformity were diagnosed. He had facial asymmetry, a broad nose, sparse eyebrows and eyelashes, a rudimentary frontal sinus, deviation of the nasal septum, and bilateral small maxillary bones. The left orbital fossa was also mildly rudimentary. On eye examination the movements of the left globe to the upward and lateral side were limited and internal strabismus was noted at this side. visual acuity was 1/10, bilaterally. Bilateral choroid coloboma, glaucoma, vertical and horizontal nystagmus were diagnosed. Fundoscopic examination revealed bilateral optic atrophy and macular and paramacular granulation tissues on the left side. intelligence quotient was 46. electroencephalography revealed bilateral frontal slow-wave activity. Visual evoked potential revealed prolonged p100 wave latencies bilaterally. magnetic resonance imaging of the brain demonstrated corpus callosum dysgenesis, bilateral subcortical heterotopia in the frontal lobes and subependymal heterotopia in the posterior horn of the left ventricle. Chromosomal analysis revealed a normal male karyotype, 46, XY. Although several cases of heterotopia in association with mental retardation, craniofacial dysmorphism, cerebral, and eye abnormalities have been described the combination of abnormalities diagnosed in our case has not previously been reported. We hypothesize that the combination of subcortical/subependymal heterotopia, corpus callosum dysgenesis, craniofacial dysmorphism, severe eye abnormalities, and growth-mental retardation may be a new syndrome.
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8/60. hearing loss and vertigo in superficial siderosis of the central nervous system.

    PURPOSE: Otoneurologic findings in patients with superficial siderosis (SS) of the central nervous system are described. A 20-year-old man with acute vertigo, dizziness, with a history of head trauma in childhood; an 87-year-old woman with severe disequilibrium appearing after peridural anesthesia; and a 55-year-old woman with recurrent episodes of positional vertigo and progressive ataxia, suffering from a lumbar ependymoma are described; all patients complained of progressive bilateral hearing loss and tinnitus.methods and results Otoneurologic examination showed bilateral sensorineural hearing loss, disturbed ocular pursuit and optokinetic nystagmus, incomplete visual suppression of the vestibulo-ocular reflex during rotatory pendular testing, right hyporeflexia, and bilateral caloric areflexia. Cerebral magnetic resonance imaging (MRI) revealed rims of hypointensity surrounding the brainstem and linear hypointensities following the surface of the cerebellar folia. CONCLUSIONS: The etiology stems from subarachnoid hemorrhage, but the source of bleeding may remain obscure. Bilateral hearing loss is described in 95% and disequilibrium in 90% because of peripheral vestibular deficit and cerebellar ataxia. In patients with progressive bilateral cochleo-vestibular deficit of unknown etiology, MRI is the examination of choice to confirm SS.
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9/60. Enterogenous cyst of the cervicomedullary junction. A case report.

    Enterogenous cyst of the cervicomedullary junction extending from C2 level to the anterior aspect of the medulla oblongata was diagnosed in a 23-year-old male with a one month history of episodic tetraparesis and bilateral horizontal nystagmus. The cyst was removed subtotally except for a small portion at the point of the attachment to the anterior aspect of the medulla oblongata by a laminectomy of C1-C2 and a suboccipital craniectomy. Histopathological and immunohistochemical examinations revealed that the cyst originated exclusively from the endoderm.
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10/60. Mapping of brainstem lesions by the combined use of tests of visually-induced eye movements.

    To determine the diagnostic value of visually-induced eye movements for indicating the lateralization of the lesion, optokinetic nystagmus (OKN), fixation-suppression of caloric nystagmus and pursuit eye movements were investigated in 28 patients who showed discrete unilateral brainstem lesions. In all patients, pursuit gains decreased in parallel with the direction of the impairment of slow-phase OKN velocity with a significant left/right difference. Decreases of gains were predominantly towards the affected side in 22 patients, whereas they were predominantly towards the contralateral side in 2 patients with dorsal tegmental pontine lesions and in 4 out of 8 patients with Wallenberg's syndrome. The relationship of pursuit gains to percentage reduction of fixation-suppression of caloric nystagmus (%FS) showed no correlation. The following four groups were classified. In group A, which was the largest, pursuit gains and %FS decreased predominantly towards the lesioned side. This group consisted of 5 patients with lesions in the midbrain, 6 patients with lesions in the ventral pons and 4 out of 8 patients with Wallenberg's syndrome. The opposite of group A was group D, showing reverse electro-oculographic (EOG) patterns to those in group A in relation to the lesioned side. Group D consisted of 2 patients with lesions in the dorsal pontine tegmentum. This observation, taken together with the decreases of pursuit and OKN gains in relation to the lesioned side, might indicate that lesions of ascending fibres are responsible for pursuit and OKN abnormalities. Group B, consisting of 7 patients with lesions either in the superior or middle cerebellar peduncles, showed decreased pursuit gains predominantly towards the lesioned side and %FS reduced predominantly towards the side contralateral to the lesion. EOGs of these patients were consistent with the results of floccular ablation in the monkey. In group C, these two parameters showed patterns opposite to those in group B in relation to the lesioned side and this group consisted of the other 4 patients with Wallenberg's syndrome. In patients of this group, the inferior cerebellar peduncle might be involved. The combined use of these tests is thus useful for lateralizing the lesion in the brainstem.
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