Arch Neurol Chicago
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Arch Neurol Chicago · Jul 2006
Case ReportsChildhood onset in familial prion disease with a novel mutation in the PRNP gene.
Up to 15% of cases of prion diseases are due to the autosomal dominant inheritance of coding PRNP mutations. ⋯ A novel mutation in the PRNP gene was identified in all of the available, clinically affected members of this family with a rapidly progressive neurodegenerative disease. To our knowledge, the propositus represents the youngest individual with inherited prion disease described to date.
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Arch Neurol Chicago · Jul 2006
Comparative Study Clinical TrialLong-duration response to levodopa in patients with advanced Parkinson disease treated with subthalamic deep brain stimulation.
Long-duration response (LDR) to levodopa is supposed to decrease with Parkinson disease (PD) progression, but direct observation of this response in advanced PD has never been performed. ⋯ Our results suggest that the LDR to levodopa remains significant even in advanced PD, and that subthalamic DBS compensates for the short-duration response and LDR to levodopa.
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Arch Neurol Chicago · Jul 2006
Improvement of postherpetic neuralgia after treatment with intravenous acyclovir followed by oral valacyclovir.
Postherpetic neuralgia (PHN) is a complication of shingles (herpes zoster), a painful rash due to varicella-zoster virus reactivation. Studies of patients with PHN and zoster sine herpete (radicular pain without rash) support the notion that low-grade viral ganglionitis contributes to pain. If chronic pain reflects active infection, then antiviral therapy may help patients with PHN. ⋯ Clinical improvement reported by most of our patients warrants further investigation in a larger, randomized, double-blind, placebo-controlled trial.