Neurology
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Randomized Controlled Trial Clinical Trial
Branched-chain amino acids and amyotrophic lateral sclerosis: a treatment failure? The Italian ALS Study Group.
We initiated a double-blind, placebo-controlled trial to test the efficacy and safety of branched-chain amino acids (BCAA) (L-leucine 12 g, L-isoleucine 6 g, and L-valine 6 g daily) in amyotrophic lateral sclerosis (ALS) patients. There was an excess mortality in subjects randomized to active treatment (24 BCAA, 13 placebo) when a total of 126 ALS patients had been recruited. This finding, associated with the lack of efficacy of BCAA (measured by comparing the disability scales in the two treatment groups), led the Data Monitoring Committee to require cessation of the trial.
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More than 360 children with intractable epilepsy have been treated with vigabatrin in single-blind or open, add-on studies. Approximately 50% or more of patients with West syndrome and partial seizures have shown a 50% or greater reduction in seizure frequency with the use of vigabatrin. ⋯ The use of vigabatrin in idiopathic localization-related epilepsy, idiopathic generalized epilepsy, and the Landau Kleffner syndrome have not been reported, but its evaluation in these conditions may be warranted based on the relatively excellent safety profile of vigabatrin. Vigabatrin has been shown to aggravate "nonprogressive myoclonic epilepsies." Vigabatrin has been well tolerated in children, with mild drowsiness and agitation being the most commonly reported side effects.
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Although generally effective, various antiepileptic drugs have been reported on occasion to increase seizure frequency, result in seizure relapse, or elicit new types of seizures. Some seizure types and epilepsy syndromes appear more prone than others to exacerbation by a given drug. Vigabatrin is rarely associated with seizure worsening, and then mainly in patients with resistant generalized epilepsies.
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Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) may occur in association with a monoclonal gammopathy of undetermined significance (MGUS) or a variety of other systemic illnesses. It is not known if the clinical features of CIDP are altered by the presence of an MGUS. We compared demographic features, clinical presentation, improvement and outcome after initial treatment, and electrodiagnostic features of a group of 77 patients with idiopathic CIDP (CIDP-I, no associated systemic illness) with 26 patients with CIDP in whom an MGUS was found during evaluation of the neuropathy (CIDP-MGUS). ⋯ CIDP-MGUS patients also demonstrated less functional impairment, more frequent sensory loss, and more abnormal sensory conduction studies than patients with CIDP-I. Because of the greater improvement of CIDP-I patients with treatment, both groups had similar outcomes from their initial episodes of weakness. Subgroup analysis of CIDP-MGUS patients did not demonstrate differences between groups with IgM and IgG or IgA gammopathies.
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We evaluated sensorimotor processing in patients with writer's cramp using PET and H2(15)O blood flow scans. The study included six right-handed patients with unilateral writer's cramp and eight right-handed normals. Subjects had blood flow scans at rest and during vibration of either the "affected" or "unaffected" hand. ⋯ Both responses were significantly reduced approximately 25% in patients with writer's cramp (PSA, p = 0.002; SMA, p = 0.02) whether vibrating the affected or unaffected hand. This indicates that patients with unilateral writer's cramp have bilateral brain dysfunction. These data provide objective evidence of abnormal central sensorimotor processing in writer's cramp.