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1/1387. Anterior pontine hydatid cyst: case report.

    We report a rare case of anterior pontine hydatid cyst. diagnosis was established on magnetic resonance imaging (MRI) findings preoperatively and was confirmed during surgery and by laboratory findings of cyst fluid, and section of cyst wall. A classical suboccipital craniectomy was performed, followed by an approach through the floor of the fourth ventricle. After a failed attempt at the Dowling technique, we punctured the cyst, aspirated the contents, then extirpated the cyst membrane. The patient survived suffering only minor morbidity. The surgical technique and pontine myelotomy are discussed.
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2/1387. Encephalopathy associated with haemophagocytic lymphohistiocytosis following rotavirus infection.

    A 2-year-old Japanese boy with a haemophagocytic lymphohistiocytosis (HLH) associated encephalopathy which developed after rotavirus infection is described. The neurological symptoms consisted of coma, seizures and spastic quadriplegia. On therapy with steroids, etoposide and cyclosporin A, the patient recovered without any neurological deficits. The interferon-gamma levels in serum and CSF were elevated at onset of the disease but had returned to normal at the time of clinical remission. brain MRI revealed diffuse white matter abnormalities and parenchymal volume loss. Proton magnetic resonance spectroscopy revealed elevated lactate in the abnormal lesions observed on MRI, indicating that macrophages not exhibiting aerobic metabolism had infiltrated the CNS. At the time of clinical remission, the white matter abnormalities and brain lactate had disappeared. These findings suggested that the neurological symptoms resulted from the overproduction of cytokines by activated T-cells and macrophages. The pathophysiology of a HLH associated encephalopathy was considered to be a local immune response within the CNS, because interferon-gamma can induce the expression of major histocompatibility complex class I and II antigens on glial cells in the CNS. CONCLUSION: Haemophagocytic lymphohistiocytosis associated encephalopathy should be considered early in the differential diagnosis of cases with acute onset neuropathy.
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3/1387. Two similar cases of encephalopathy, possibly a reversible posterior leukoencephalopathy syndrome: serial findings of magnetic resonance imaging, SPECT and angiography.

    Two young women who had encephalopathy that resembled reversible posterior leukoencephalopathy syndrome are presented. The brain magnetic resonance imaging (MRI) of these patients exhibited similar T2-high signal lesions, mostly in the white matter of the posterior hemispheres. Xe-SPECT during the patients' symptomatic period showed hypoperfusion in the corresponding areas, and angiography demonstrated irregular narrowing of the posterior cerebral artery. Clinical manifestations subsided soon after treatment, and the abnormal radiological findings also were almost completely resolved. Thus, we concluded that transient hypoperfusion followed by ischemia and cytotoxic edema might have had a pivotal role in these cases.
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4/1387. Intracranial Castleman's disease of solitary form. Case report.

    This 62-year-old woman presented with clumsiness in her right hand. magnetic resonance imaging demonstrated a small lesion mimicking a meningioma, which had arisen from the tentorium and contained notable edema. Full recovery was achieved by total removal of the lesion, which was diagnosed as a lymphoid mass resembling giant lymph node hyperplasia on histological examination. The lack of notable findings on whole-body and laboratory studies was compatible with a rare case of intracranial Castleman's disease of solitary form. The authors document clinical, neuroradiological, and pathological features of this rare disease.
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5/1387. 18Fluoro-2-deoxyglucose (18FDG) PET scan of the brain in type IV 3-methylglutaconic aciduria: clinical and MRI correlations.

    The clinical, 18fluorodeoxyglucose positron emission tomography (18FDG PET) and the magnetic resonance imaging (MRI) brain scan characteristics of four patients diagnosed to have 3-methylglutaconic aciduria were reviewed retrospectively. The disease has a characteristic clinical pattern. The initial presentations were developmental delay, hypotonia, and severe failure to thrive. Later, progressive encephalopathy with rigidity and quadriparesis were observed, followed by severe dystonia and choreoathetosis. Finally, the patients became severely demented and bedridden. The 18FDG PET scans showed progressive disease, explaining the neurological status. It could be classified into three stages. Stage I: absent 18FDG uptake in the heads of the caudate, mild decreased thalamic and cerebellar metabolism. Stage II: absent uptake in the anterior half and posterior quarter of the putamina, mild-moderate decreased uptake in the cerebral cortex more prominently in the parieto-temporal lobes. Progressive decreased thalamic and cerebellar uptake. Stage III: absent uptake in the putamina and severe decreased cortical uptake consistent with brain atrophy and further decrease uptake in the cerebellum. The presence of both structural and functional changes in the brain, demonstrated by the combined use of MRI and 18FDG PET scan, with good clinical correlation, make the two techniques complementary in the imaging evaluation of 3-methylglutaconic aciduria.
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6/1387. Abrupt exacerbation of acute subdural hematoma mimicking benign acute epidural hematoma on computed tomography--case report.

    A 75-year-old male was hit by a car, when riding a bicycle. The diagnosis of acute epidural hematoma was made based on computed tomography (CT) findings of lentiform hematoma in the left temporal region. On admission he had only moderate occipitalgia and amnesia of the accident, so conservative therapy was administered. Thirty-three hours later, he suddenly developed severe headache, vomiting, and anisocoria just after a positional change. CT revealed typical acute subdural hematoma (ASDH), which was confirmed by emergent decompressive craniectomy. He was vegetative postoperatively and died of pneumonia one month later. Emergent surgical exploration is recommended for this type of ASDH even if the symptoms are mild due to aged atrophic brain.
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7/1387. 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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8/1387. Coronal computed tomography: indications and accuracy.

    The application of wide aperture scanners to neuroradiology permits improved anatomic definition and localization of intracranial and intraorbital lesions. Coronal scans are most useful in demonstrating lesions of the skull base and apex, distinguishing between infra- and supratentorial lesions, and in determining if a lesion is intra- or extraaxial. Limitations of coronal scans include discomfort in positioning, high spatial frequency artifacts, and additional radiation exposure.
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9/1387. Discourse deficits following right hemisphere damage in deaf signers.

    Previous findings have demonstrated that hemispheric organization in deaf users of American sign language (ASL) parallels that of the hearing population, with the left hemisphere showing dominance for grammatical linguistic functions and the right hemisphere showing specialization for non-linguistic spatial functions. The present study addresses two further questions: first, do extra-grammatical discourse functions in deaf signers show the same right-hemisphere dominance observed for discourse functions in hearing subjects; and second, do discourse functions in ASL that employ spatial relations depend upon more general intact spatial cognitive abilities? We report findings from two right-hemisphere damaged deaf signers, both of whom show disruption of discourse functions in absence of any disruption of grammatical functions. The exact nature of the disruption differs for the two subjects, however. Subject AR shows difficulty in maintaining topical coherence, while SJ shows difficulty in employing spatial discourse devices. Further, the two subjects are equally impaired on non-linguistic spatial tasks, indicating that spared spatial discourse functions can occur even when more general spatial cognition is disrupted. We conclude that, as in the hearing population, discourse functions involve the right hemisphere; that distinct discourse functions can be dissociated from one another in ASL; and that brain organization for linguistic spatial devices is driven by its functional role in language processing, rather than by its surface, spatial characteristics.
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10/1387. Provision of a neuroendoscopy service. The Southampton experience.

    BACKGROUND: A series of 21 patients (aged 1 week to 80 years) underwent a total of 22 neuroendoscopic procedures in our Unit in the period July 1993 to January 1996. methods: The procedures were performed by one surgeon familiar with the technique using the Stortz rigid neuroendoscope system. The most common indication for neuroendoscopy was obstructive hydrocephalus. The most frequently performed procedure was third ventriculostomy and tumor biopsy. The intended surgical procedure was successfully performed in all but two of the cases (attempted septostomy and internal cyst drainage) were both abandoned due to unrecognisable anatomy. RESULTS: Of the 19 patients treated by fenestration or ventriculostomy to relieve hydrocephalus, 5 eventually required definitive shunting procedures. CONCLUSIONS: Complications relating directly to the neuroendoscopy occurred in 2 patients (bleeding requiring temporary external ventricular drain) and there were no surgical deaths.
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