Annals of neurology
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Annals of neurology · Sep 1996
Case ReportsProgressive encephalomyelitis with rigidity responsive to plasmapheresis and immunosuppression.
The stiff-man syndrome is reported in a 55-year-old woman who also had signs of severe bulbar musculature hypercontraction. These clinical features correspond to the progressive encephalomyelitis with rigidity syndrome. Diazepam was minimally beneficial, but after treatment with plasmapheresis and corticosteroids, she was still asymptomatic when seen 5 years later.
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Annals of neurology · Jun 1996
Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease.
We sequenced the prion protein gene and studied the biochemical characteristics and the intracerebral distribution of protease-resistant prion protein with Western blot and immunohistochemistry in 19 cases of sporadic Creutzfeldt-Jakob disease. We identified four groups of subjects defined by the genotype at codon 129 of the prion protein gene, the site of a common methionine/valine polymorphism, and two types of protease-resistant prion proteins that differed in size and glycosylation. The four Creutzfeldt-Jakob disease groups showed distinct clinicopathological features that corresponded to previously described variants. ⋯ Our data indicate that the sporadic form of Creutzfeldt-Jakob disease comprises a limited number of variants. The methionine/valine polymorphism at codon 129 of the prion protein gene and two types of protease-resistant prion proteins are the major determinants of these variants. These findings suggest the existence of prion strains in humans and provide the molecular basis for a novel classification of sporadic Creutzfeldt-Jakob disease.
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Annals of neurology · Mar 1996
Randomized Controlled Trial Multicenter Study Comparative Study Clinical TrialIntramuscular interferon beta-1a for disease progression in relapsing multiple sclerosis. The Multiple Sclerosis Collaborative Research Group (MSCRG)
The accepted standard treatment of relapsing multiple sclerosis consists of medications for disease symptoms, including treatment for acute exacerbations. However, currently there is no therapy that alters the progression of physical disability associated with this disease. The purpose of this study was to determine whether interferon beta-1a could slow the progressive, irreversible, neurological disability of relapsing multiple sclerosis. ⋯ There were no major adverse events related to treatment. Interferon beta-1a had a significant beneficial impact in relapsing multiple sclerosis patients by reducing the accumulation of permanent physical disability, exacerbation frequency, and disease activity measured by gadolinium-enhanced lesions on brain magnetic resonance images. This treatment may alter the fundamental course of relapsing multiple sclerosis.
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Annals of neurology · Jan 1996
Proton magnetic resonance spectroscopic imaging in patients with cerebellar degeneration.
Using proton magnetic resonance spectroscopic imaging, we studied the cerebellum of 9 patients with cerebellar degeneration and of 9 age-matched normal control subjects. This technique permits the simultaneous measurement of N-acetylaspartate, choline-containing compounds, creatine/phosphocreatine, and lactate signal intensities from four 15-mm slices divided into 0.84-ml single-volume elements. ⋯ Our method of analysis suggests the presence of a neurodegenerative process in cerebellar areas that do not appear to be atrophic on the MRI. Some limitations of proton magnetic resonance spectroscopic imaging in the present study were related to the partial field inhomogeneity characteristics of the posterior fossa, the anatomical location of the cerebellum, and the particularly severe cerebellar atrophy in some of the patients.
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Annals of neurology · Nov 1995
Comparative StudySignificance of spikes recorded on electrocorticography in nonlesional medial temporal lobe epilepsy.
Whether spikes recorded by intraoperative electrocorticography imply active epileptogenicity has not been adequately addressed. We performed preresection and postresection electrocorticography on 47 patients with nonlesional medial temporal lobe epilepsy who were undergoing surgery for the treatment of medically refractory epilepsy. A standard anteromedial temporal lobectomy was performed on all patients, with no additional resection, regardless of electrocorticographic findings. ⋯ The majority of these had a low-frequency spike discharge rate and were localized to the margin of resection. We found no correlation between residual spikes on preresection and postresection electrocorticography and outcome. These findings do not support the role of intraoperative electrocorticography in guiding mesial temporal lobe resection.