1/1379. Transient paralytic attacks of obscure nature: the question of non-convulsive seizure paralysis. Eleven patients with transient paralytic attacks of obscure nature are described. paralysis could involve face or leg alone, face and hand, or face, arm and leg. The duration varied from two minutes to one day. Four patients had brain tumors, six probably had brain infarcts, and one a degenerative process. The differential diagnosis included TIAs, migraine accompaniments, and seizures. In the absence of good evidence for the first two, the cases are discussed from the standpoint of possibly representing nonconvulsive seizure paralysis (ictal paralysis, inhibitory seizure paralysis or somatic inhibitory seizure). Because of the difficulty in defining seizures as well as TIAs and migraine in their atypical variations, a firm conclusion concerning the mechanisms of the spells was not attained. Two cases of the hypertensive amaurosis-seizure syndrome have been added as further examples of ictal deficits. ( info) |
2/1379. Cervicomedullary astrocytoma simulating a neuromuscular disorder. A 12-year-old male developed progressive proximal upper extremity weakness over a 3- to 4-year period. The clinical findings of proximal upper extremity weakness and atrophy, prominent scapular winging, and no sensory deficits or upper motor neuron signs suggested a neuromuscular disorder. electromyography was consistent with a chronic denervating disorder involving the upper cervical anterior horn cells or their axons. A cervical magnetic resonance image revealed a large intramedullary mass extending from the inferior aspect of the fourth ventricle down to the level of T2. A biopsy of the lesion was consistent with a low-grade astrocytoma. ( info) |
3/1379. Secondary glioblastoma remarkably reduced by steroid administration after anaplastic transformation from gliomatosis cerebri--case report. A 45-year-old female presented with gliomatosis cerebri manifesting as hemiballismus-like involuntary movement in the arm, motor weakness in the leg, and hypesthesia in her left side. Computed tomography showed only diffuse swelling of the right cerebral hemisphere, but T2-weighted magnetic resonance imaging revealed a diffuse lesion spreading from the right thalamus to the temporal, parietal, and occipital lobes on the same side. No abnormal enhancement was recognized. cerebral angiography showed no specific finding. A right occipital lobectomy was performed to confirm the diagnosis of gliomatosis cerebri. Anaplastic transformation was recognized 5 months later. The disease did not resolve with radiation or interferon administration, but steroid therapy achieved remarkably effective tumor regression. The patient died due to pneumonia. autopsy showed the features of diffuse glioblastoma. Steroid therapy may be an effective treatment for gliomatosis cerebri before the terminal stage. ( info) |
4/1379. Multiple postoperative intracerebral haematomas remote from the site of craniotomy. A postoperative haemorrhage is a common and serious complication of a neurosurgical procedure. It usually occurs at the site of the surgery, but on occasion a postoperative haematoma is found at a distance from the previous craniotomy. Multiple postoperative haemorrhages are extremely rare. We report the case of a 63-year-old woman, operated on for the removal of a supratentorial astrocytoma, who developed in the early post-operative period multiple bilateral intracerebral haematomas without involvement of the surgical bed. ( info) |
5/1379. Transient peduncular hallucinations secondary to brain stem compression by a cerebellar pilocytic astrocytoma. Almost all peduncular hallucinations have been described in patients with intrinsic lesions of the midbrain. An, as yet, unreported case of peduncular hallucinosis caused by posterior compression of the midbrain by a cerebellar pilocytic astrocytoma is described. The hallucinations and associated symptoms only ceased after removal of the tumour. ( info) |
| OBJECTIVE: The authors describe an ocular lesion combining the characteristics of persistent hyperplastic primary vitreous (PHPV) and a retinal tumor in an infant with tuberous sclerosis complex (TSC). STUDY DESIGN: Case report. methods: immunohistochemistry and cytogenetic studies were performed on TSC cells from an intraocular tumor in a 6-week-old infant. RESULTS: Histopathologic examination showed a thick fibrovascular membrane between the aspect of the lens and the astrocytic component of the mass. glial fibrillary acidic protein (GFAP) showed a variable intracytoplasmic reaction in the astrocytic proliferation, involving approximately 50% of the cells. Tissue culture studies showed a fairly rapid proliferation of fusiform cells, consistent with bipolar astrocytic cells. Cytogenetic studies showed one abnormal clone consisting of three hyperdiploid cells with a loss of chromosome 9 and a gain of chromosomes 6 and 12. CONCLUSION: The atypical localization of the retinal tumor could be explained by the fact that it was trapped during its proliferation by the retinal detachment associated with the PHPV. ( info) |
7/1379. Invasive giant cell astrocytoma of the retina in a patient with tuberous sclerosis. OBJECTIVE: To report an unusual case of giant cell astrocytoma of the retina. DESIGN: Case report. INTERVENTION: A 10-month-old girl with tuberous sclerosis was found to have bilateral astrocytic hamartomas, the right eye being prominently involved by elevated and pedunculated lesions. At 7 years of age, she had posterior subcapsular cataract, retinal detachment, and subretinal exudation develop in the right eye. At 12 years of age, her blind, painful right eye had to be enucleated because of neovascular glaucoma and a spontaneous scleral perforation. RESULTS: Histopathologic examination showed that the entire vitreous cavity was filled with a mixture of tumor, granulation tissue, and necrotic debris. Part of the tumor was composed of spindle-shaped glial cells. The remainder was composed of large gemistocytic cells that contained large atypical nuclei and copious amounts of cytoplasm, which was intensely eosinophilic in some areas. The tumor contained foci of necrosis and rare mitotic figures. It had infiltrated the parenchyma of the retrolaminar nerve and extended to the surgical margin. Areas of unequivocal choroidal invasion were also identified. The tumor cells were intensely immunoreactive for neuron-specific enolase and S-100 protein. In contrast, glial fibrillary acidic protein was only minimally positive. CONCLUSIONS: The histologic and immunohistochemical features of this retinal tumor resemble those of subependymal giant cell astrocytoma, a characteristic lesion in tuberous sclerosis. Although this unusual giant cell astrocytoma of the retina had atypical histopathologic features and local aggressive behavior, the systemic prognosis was excellent. ( info) |
8/1379. 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. ( info) |
9/1379. Early glial tumor metastases through a ventriculo-atrial shunt. A girl of 12 with a pontine polymorphous astrocytoma diffusely metastasizing to the spinal cord leptomeninges suddenly died 20 hours after the insertion of a right ventriculo-atrial shunt. Many severely damaged glial neoplastic cells were found in the blood vessels of the lung and liver. ( info) |
10/1379. Atypical pleomorphic xanthoastrocytoma. A 65-year-old man experienced an ictal episode. CT revealed a left capsulo-thalamic mass, and SPET showed hypoperfusion of the left cerebral emisphere. The lesion was subtotally removed, and postoperative radiotherapy was given. Pathological examination demonstrated an "atypical" pleomorphic xanthoastrocytoma. The patient died of massive regrowth of the tumor 22 months after surgery. This case is discussed in light of the pertinent literature. ( info) |