[An improved case of bedridden mental impairment with normal pressure hydrocephalus associated with acoustic neurinoma after tumor resection].

A 67-year-old woman developed gait disturbance, dysarthria, cognitive impairment and incontinence at age 65, and became bedridden. She showed mutism, stupor and lower limb spasticity. Cranial CT and MRI revealed marked ventricular enlargement and a cerebellopontine angle tumor. CSF study showed normal pressure (125 mmH2O) and elevated protein (143 mg/dl). Radionuclide cisternography showed redistribution of radionuclide to the ventricles and intraventricular residual radionuclide after 72 hours, which allowed a diagnosis of normal pressure hydrocephalus. After removal of the tumor, ventricle size and CSF protein decreased, and the symptoms of cognitive impairment and motor dysfunction resolved. Histological examination showed acoustic neurinoma. Over the half of hydrocephalus following acoustic neurinoma shows a tendency to improve by surgical resection of the tumor. Neurologists who see cognitively impaired spastic bedridden patients should not overlook this pathology.

Imaging the ischemic penumbra with 18F-fluoromisonidazole in a rat model of ischemic stroke.

BACKGROUND AND PURPOSE: The ischemic penumbra is a major focus of stroke research. 18F-fluoromisonidazole (18F-FMISO), a positron emission tomography (PET) marker of hypoxic cells, has shown promise as a technique to image the penumbra in humans. Our aim was to delineate the pattern of 18F-FMISO binding in a rat middle cerebral artery transient thread-occlusion model, and correlate this with tissue outcome at 24 hours. We hypothesized that the pattern of 18F-FMISO binding would mimic that seen in humans. METHODS: Thirty-eight rats underwent 2 hours transient middle cerebral artery (MCA) occlusion, and then received 18F-FMISO at time points from 0.5 to 22 hours post-MCA occlusion and were killed 2 hours later. Autoradiographic assessment of 18F-FMISO binding and assessment (triphenyltetrazolium chloride) of the area of infarction were performed on tissue slices. RESULTS: Until 1 hour after MCA occlusion, 18F-FMISO binding was increased in the entire MCA territory, with little or no infarction visible. Over the next 5 hours, the pattern of binding evolved to a small rim of intensely binding tissue surrounding the infarct core, which itself showed reduced binding compared with the contralateral hemisphere. By 24 hours, there was minimal accumulation of 18F-FMISO binding and a large area of infarction. CONCLUSIONS: The pattern of 18F-FMISO binding rats reproduced the pattern seen in humans, consistent with this tracer being a marker of the ischemic penumbra in both species. This technique may have application in studying the ischemic penumbra in animal models, and correlating this with similar studies in humans.

Clinical features and neuropathological findings of familial amyotrophic lateral sclerosis with a His46Arg mutation in Cu/Zn superoxide dismutase.

We examined the characteristic clinical features of one family of familial amyotrophic lateral sclerosis (FALS) with a His46Arg mutation in the enzyme Cu/Zn superoxide dismutase-1 (SOD1). The disease duration for this family was 18.1 +/- 13.2 (mean +/- S.D.) years, with the age at onset being 39.7 +/- 10.5 years old (mean +/- S.D.). The initial sign was distal weakness of the unilateral lower limb, extending to the lower limb of the other side. A wheel chair became necessary at 9.8 +/- 3.2 years after the onset. Upper limb weakness started at 15.5 +/- 8.9 years following from the onset. An autopsy was performed on a 71-year-old woman of the family with the mutation. Her disease duration was 47 years, and she died of pneumonia. She had no clear upper motor neuron involvement. Bulbar sign and respiratory muscle weakness had developed 2 years before her death. Neuropathological findings showed degeneration of corticospinal tracts, anterior/posterior spinocerebellar tracts, posterior columns, and Clarke's columns. There were few anterior horn cells in the lumbar spinal cord and no Lewy body-like hyaline inclusion bodies in these remaining anterior horn neurons. This is the first autopsy report of FALS with a His46Arg mutation in the SOD1 enzyme.