1/109. Neuro-ophthalmologic manifestations of Maffucci's syndrome and Ollier's disease.patients with Ollier's disease (multiple skeletal enchondromas) and Maffucci's syndrome (multiple enchondromas associated with subcutaneous hemangiomas) may develop skull base chondrosarcomas or low-grade astrocytomas as a delayed consequence of these disorders. We report three patients with Ollier's disease and Maffucci's syndrome who had diplopia as the initial manifestation of intracranial tumors. Since patients with Maffucci's syndrome and Ollier's disease are at risk for the delayed development of brain and systemic neoplasms, neuroophthalmologists must be aware of the need for long-term surveillance in patients affected by these conditions.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/109. Creutzfeldt-Jakob disease presenting with visual blurring, diplopia and visual loss: Heidenhain's variant.Focal electroencephalographic abnormalities as described in Heidenhain's variant of Creutzfeldt-Jakob disease are uncommon. We report a 73-year-old male presenting with visual symptoms, right hemianopia and rapidly progressive dementia. myoclonus was synchronous with generalised periodic epileptiform discharges on electroencephalography (EEG). In addition, there were periodic focal sharp waves at the left occipital region. diffusion-weighted magnetic resonance brain images showed slightly increased signal intensity in the occipital parasagittal area, left more than right. 14-3-3 protein was detected in the cerebrospinal fluid. The patient died within 5 months of presentation.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
3/109. Spontaneous intracranial hypotension.PURPOSE: To describe a patient with classic presentation of spontaneous intracranial hypotension and subsequent improvement with targeted epidural blood patch. methods: Report of one case and review of the literature. RESULTS: Examination of cerebrospinal fluid after lumbar puncture disclosed a reduced opening pressure, an increased level of protein, and lymphocytic pleocytosis. magnetic resonance imaging of the brain with gadolinium showed diffuse enhancement of the pachymeninges, no evidence of leptomeningeal enhancement, and chronic subdural fluid collection. Radionuclide cisternography demonstrated reduced activity over the cerebral convexities, early accumulation of radiotracer in the urinary bladder, and direct evidence of leakage at the cervicothoracic junction (C7-T1). Clinical, laboratory, and radiologic features were consistent with the diagnosis of spontaneous intracranial hypotension. Therapy with a targeted epidural blood patch resulted in the rapid resolution of symptoms. CONCLUSIONS: In this report, we describe a classic case of spontaneous intracranial hypotension in a 63-year-old man with an initial presentation of postural headaches, blurred vision, pain in the left eye, diplopia on left gaze, and neck soreness.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
4/109. Rosai-Dorfman disease presenting multiple intracranial lesions with unique findings on magnetic resonance imaging. Case report.Rosai-Dorfman disease (RDD) is a rare idiopathic histoproliferative disease affecting the systemic lymph nodes. Although an extranodal lesion has also been recognized, central nervous system involvement is extremely rare. To the authors' knowledge, only 20 cases of intracranial lesions have been reported previously. Intracranial RDD is clinically and radiologically difficult to distinguish from meningioma, and histological examination is essential for a definitive diagnosis. The authors treated a large frontal lobe tumor associated with multiple meningeal nodules in a 67-year-old patient presenting with diplopia and headache. Radiological examination indicated that the mass was an inflammatory lesion rather than a meningioma. Microscopically the lesion consisted of mixed inflammatory infiltrate with marked emperipolesis, which is characteristic of RDD. A review of the literature and a discussion of the differential diagnosis of this rare lesion are also presented.- - - - - - - - - - ranking = 2.3111904122954keywords = central nervous system, nervous system (Clic here for more details about this article) |
5/109. Intact sensory fusion in a child with divergence paresis caused by a pontine glioma.PURPOSE: To describe a child with divergence paresis esotropia caused by a brain tumor with intact sensory and motor fusion. METHOD: Case report. RESULTS: A 9-year-old boy who had one episode of double vision was initially seen with a small, variably present esophoria at near vision, an intermittent 10 prism diopter esotropia at distance, and stereopsis of 80 arc seconds. A magnetic resonance imaging examination disclosed a 4.0 x 4.5-cm pontine glioma. CONCLUSIONS: Ophthalmologists should recognize that the presence of intact sensory and motor fusion in a child with acute, comitant esotropia of the divergence paresis type does not preclude intracranial abnormality. If immediate neuroimaging is deferred, repeated thorough ocular motility examinations are warranted to detect progression.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
6/109. 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 = 1keywords = brain (Clic here for more details about this article) |
7/109. Asymmetric sweating in a child with multiple sclerosis.A 10-year-old male with multiple sclerosis complained of excessive sweating on the right side of the forehead and shoulder on relapse 3 months after the onset of multiple sclerosis. Because the neurologic evaluation revealed no abnormalities in the sudomotor function, it is likely that the hyperhidrosis resulted from a lesion in the central or preganglionic sympathetic nervous system. magnetic resonance imaging demonstrated a high-intensity lesion involving the left hypothalamus on T(2)-weighted imaging. Thus hypothalamic involvement might be the reason for the hyperhidrosis in this patient.- - - - - - - - - - ranking = 0.71605588796977keywords = nervous system (Clic here for more details about this article) |
8/109. Isolated nuclear oculomotor nerve syndrome due to mesencephalic hematoma.Unilateral third nerve palsy with bilateral superior rectus paresis and bilateral ptosis is a typical condition for nuclear oculomotor nerve syndrome. We report a case of nuclear oculomotor nerve syndrome due to midbrain hemorrhage, as a rare cause. A 73-year-old man presented with an abrupt onset of double vision and difficulty opening his eyes. He had uncontrolled hypertension in his history. Neurological examination revealed right oculomotor palsy with impairment of bilateral upward gaze and bilateral ptosis. MRI showed a mesencephalic area of increased T1 signal and decreased T2 signal consistent with a subacute hematoma. It is emphasized that isolated mesencephalic hemorrhage may be the cause of the nuclear oculomotor nerve syndrome without associated neurological signs.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
9/109. Subarachnoid cyst and ascent to high altitude--a problem?A 31-year-old man suffered diplopia and ataxia on two occasions when he ascended from sea level to 4,000 m. Evaluation revealed a moderate-sized subarachnoid cyst in the left frontal region, which did not communicate with the cerebral ventricles. The cyst might have acted as a space-occupying lesion, and caused symptoms on ascent due to hypoxic brain swelling, brain compression against the cyst, and elevated intracranial pressure. Subarachnoid cysts are common, and they should be considered in the differential diagnosis of neurological problems at high altitude.- - - - - - - - - - ranking = 2keywords = brain (Clic here for more details about this article) |
10/109. multiple sclerosis with caudate lesions on MRI.A 31-year-old woman displayed sleepiness and impairment of recent memory. T2-weighted MRI revealed high signal intensity lesions in the bilateral basal ganglia, thalamus, and brainstem. Although remission was achieved with corticosteroid therapy, she again displayed memory dysfunction and emotional disturbance one year later, at which time MRI disclosed new lesions in the right caudate nucleus and left frontal white matter. Corticosteroid therapy lead to improvement, and she suffered no recurrence on maintenance steroid therapy. These findings suggest that caudate lesions do occur in multiple sclerosis, the manifestations of which can be abulia and memory dysfunction, as in the present case.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
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