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The activation of the c-Jun N-terminal kinase (JNK) pathway is critical for naturally occurring neuronal cell death during development and may be important for the pathological neuronal cell death of neurodegenerative diseases. The small molecule inhibitor of the mixed-lineage kinase (MLK) family of kinases, CEP-1347, inhibits the activation of the JNK(More)
Sporadic Creutzfeldt-Jakob disease (sCJD) is a rapidly progressive dementia (RPD) that can be difficult to identify antemortem, with definitive diagnosis requiring tissue confirmation. We describe the clinical, magnetic resonance imaging (MRI), cerebrospinal fluid (CSF), and electroencephalogram (EEG) measures of a small cohort of 30 patients evaluated for(More)
INTRODUCTION This study characterizes the nature of pain in hereditary neuropathy with liability to pressure palsy (HNPP). METHODS This retrospective study was performed to assess duration, nature, location, and intensity of pain on initial presentation of subjects with HNPP, including the degree and type of analgesic medication use and electrodiagnostic(More)
The radial nerve is the major nerve serving the extensor compartment of the arm. This article describes the anatomic features, clinical features based on lesion location, electrodiagnostic assessment, prognosis, and treatment of radial neuropathies. Numerous traumatic causes have been reported to result in radial neuropathies, including compression or(More)
MIXED LINEAGE KINASE INHIBITOR CEP1347 FAILS TO DELAY DISABILITY IN EARLY PARKINSON DISEASE To the Editor: We read the Parkinson Study Group’s article with interest.1 The disappointing result in PRECEPT that used mixed-lineage kinase (MLK) inhibitor CEP-1347 for treatment of Parkinson disease (PD) may be because 1) the hypothesis that MLK inhibitors retard(More)
The hemodialyzability of cyclophosphamide was investigated in four patients on long-term hemodialysis. Cyclophosphamide 100 mg was given intravenously over 10 min before hemodialysis. Blood and dialysate samples were collected periodically during the 4 hr dialysis and measured by gas-liquid chromatography (GLC) for cyclophosphamide. Dialysis clearance(More)
The carotid body is a neural crest-derived neuroendocrine organ that detects the oxygen level in blood and regulates ventilation. Unlike many other neural crest derivatives, the trophic factors mediating survival and differentiation of neuroendocrine cells of the carotid body are unknown. Given that many neural crest derivatives rely on the glial cell(More)
Small-molecule mixed-lineage kinase (MLK) inhibitors, such as CEP-1347 [3,9-bis[(ethylthio)methyl]-(8R*,9S*,11S*)-(-)-9-hydroxy-9-methoxycarbonyl-8-methyl-2,3,9,10-tetrahydro-8,11-epoxy-1H,8H, 11H-2,7b,11a-triazadibenzo(a,g)cycloocta(cde)trinden-1-one] and CEP-11004(More)
Facioscapulohumeral muscular dystrophy (FSHD) is a clinically recognizable and relatively common muscular dystrophy. It is inherited mostly as an autosomal dominant disease or in a minority of cases, in a digenic pattern. The disease manifestation is variable and most likely dependent on genetic and epigenetic factors. We review the history, epidemiology,(More)