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BACKGROUND Central core disease (CCD) and nemaline rod myopathy are generally considered two genetically and histologically distinct disorders. CCD is defined by the presence of well-demarcated round cores within most myofibers. Nemaline rod myopathy is distinguished by the presence of characteristic nemaline bodies within myofibers. The simultaneous(More)
Earlier we have shown alterations in immunoreactivity (IR) to the calcium-binding proteins parvalbumin (PV) and calbindin D-28k (CaB) in surviving Purkinje cells of patients with spinocerebellar ataxia-1 (SCA-1). In the present study we determined PV and CaB expression (by immunohistochemical and immunoblot analyses) in Purkinje cells of transgenic mice(More)
Metastatic tumors to the choroid plexus are rare. We report a case of renal cell carcinoma metastasizing to the choroid plexus of the lateral ventricular atrium. This tumor was shown by magnetic resonance imaging, followed by craniotomy and histologic confirmation. Computed tomography of the abdomen showed the primary tumor. We also reviewed 40 years of the(More)
Deposits of amyloid beta-protein (A beta) form the cores of the pathological plaques which characterize Alzheimer's disease. The mechanism of formation of the deposits is unknown; one possibility is failure of a clearance mechanism that would normally remove the protein from brain parenchyma. This study has investigated the capacity of the central nervous(More)
Neuroprotective effects of a Group II metabotropic glutamate receptor agonist, LY379268, were examined in a neonatal rat model of hypoxia-ischemia (unilateral common carotid artery ligation followed by hypoxic exposure for 1.5h in 7-day-old rat pups). LY379268 administered 5 min after hypoxic exposure (2, 5, or 10 mg/kg, i.p.) significantly reduced brain(More)
We present a two-generation family consisting of a father and two daughters, who had an adult-onset leukodystrophy characterized by widespread destruction of cerebral white matter with neuroaxonal spheroids. The mode of inheritance appears to be autosomal dominant. All three patients presented with a variety of motor and cognitive symptoms, including(More)
Most mass lesions of the internal auditory canal and cerebellopontine angle are neoplastic, expansile, and eventually symptomatic. We report two cases of neurogenic hamartomas at these anatomic sites. In a patient with unrelated auditory or vestibular symptoms, such a developmental lesion might result in false-positive posterior fossa contrast results.
BACKGROUND AND AIM Severe dysautonomia may be secondary to viral infections, resulting in impaired autoimmune, cardiovascular, urinary and digestive dysfunction. Herein, we present a case of a 31-year-old white female patient who had severe gastroparesis related to autonomic failure following an episode of acute gastroenteritis. This seems to be the first(More)
Computerized tomography (CT)-guided stereotactic biopsies of intracranial lesions in 29 patients were analyzed, paying particular attention to the correlation between core biopsies and aspiration smears. Seven percent were nondiagnostic due to sampling error. Discrepancies occurred between core biopsies and aspiration smears in 10% of the cases. In two(More)