Journal of the neurological sciences
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Polymorphism at codon 219 lysine in prion protein (PrP) is considered to affect the clinicopathological features of prion diseases including Creutzfeldt-Jakob disease (CJD) and to have an inhibiting effect on the pathogenesis of these diseases. We describe the first autopsied case of dura mater graft-associated CJD (dCJD) with heterozygosity of lysine at codon 219 in PrP observed in a Japanese subject. ⋯ These findings in this case were atypical of the non-plaque type of dCJD and MM1 subgroup of CJD. Thus, these findings can be unique to dCJD with codon 219 lysine allele, and this allele may influence the clinicopathological features and PrP profiles in dCJD.
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The presence of a hyperdense middle cerebral artery sign (HMCAS) on baseline brain CT is associated with poor clinical outcome in stroke patients treated with intravenous recombinant tissue plasminogen activator (tPA). It remains uncertain whether the presence of HMCAS is associated with acute neurological deterioration after tPA treatment. ⋯ The HMCAS is associated with early neurological deterioration and poor functional outcome, but not with symptomatic ICH.
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Cerebrovascular reactivity (CVR) testing with CO2 challenge is used clinically as a measure of cerebrovascular reserve. However it is not known whether CVR measures the same physiological process as spontaneous cerebral autoregulation (CA). ⋯ We showed a decrease in spontaneous CA after vasodilatation. However, the lack of correlation between baseline CA and degree of CVR suggests that cerebrovascular reserve and CA are based in part on distinct physiological mechanisms. Further studies are needed to determine which of these parameters is most useful to guide treatment decisions in pathological states.
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Case Reports
Reversible metronidazole-induced cerebellar toxicity in a multiple transplant recipient.
Metronidazole-induced central nervous system (CNS) toxicity causes a spectrum of neurological symptoms including ataxia, encephalopathy and peripheral neuropathy. It is associated with characteristic MRI changes of high signal intensity in the dentate nuclei. Given the increasing use of metronidazole, it is import to recognise this drug as a cause of ataxia, as it is entirely reversible on drug withdrawal.
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Randomized Controlled Trial
Efficacy and tolerability of pregabalin in essential tremor: a randomized, double-blind, placebo-controlled, crossover trial.
We performed a double-blind, crossover-design study to assess the tolerability and efficacy of pregabalin (PGB) in patients with essential tremor (ET). Twenty patients (11 women; mean age of 62.2+/-12.7 years, mean ET duration of 25.5+/-14.9 years) with ET were randomized for treatment with PGB (150-600 mg/day) or placebo, titrated over 6 weeks. ⋯ We found no improvement in any of the TRS measures and a statistically significant worsening of QUEST scores while patients were taking PGB. Adverse events were similar in frequency to previously published studies of PGB, the most common being drowsiness and dizziness.