Sulfamethoxazole-trimethoprim double-blind, placebo-controlled, crossover trial in Machado-Joseph disease: sulfamethoxazole-trimethoprim increases cerebrospinal fluid level of biopterin. 1995

T Sakai, and T Matsuishi, and S Yamada, and H Komori, and H Iwashita
Department of Neurology, National Chikugo Hospital, Fukuoka, Japan.

We performed a double-blind, placebo-controlled, crossover trial of sulfamethoxazole-trimethoprim (S-T) in 8 patients with Machado-Joseph disease (MJD), and measured the blood and cerebrospinal fluid levels of biopterins, biogenic amines or metabolites, and folate. The clinical results were as follows; mild improvements of hyperreflexia of knee jerks and of rigospasticity of the legs during S-T treatment period. In addition, S-T significantly reduced the times of 8 motor activities on the timed tests. The biochemical results showed that basal levels of all biopterins and homovanillic acid in the cerebrospinal fluid (CSF) were reduced to less than half the levels of those of controls with other neurological diseases. After S-T treatment, total and oxidized form of biopterins in the CSF increased significantly. Therefore, S-T may be effective to neurologic deficits through its mechanism of increasing the level of brain biopterins.

UI MeSH Term Description Entries
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D004311 Double-Blind Method A method of studying a drug or procedure in which both the subjects and investigators are kept unaware of who is actually getting which specific treatment. Double-Masked Study,Double-Blind Study,Double-Masked Method,Double Blind Method,Double Blind Study,Double Masked Method,Double Masked Study,Double-Blind Methods,Double-Blind Studies,Double-Masked Methods,Double-Masked Studies,Method, Double-Blind,Method, Double-Masked,Methods, Double-Blind,Methods, Double-Masked,Studies, Double-Blind,Studies, Double-Masked,Study, Double-Blind,Study, Double-Masked
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D001708 Biopterins Pterin derivatives based on 2-amino-6-(1,2-dihydroxypropyl)-4(1H)-pteridinone. Biopterins are natural products that have been considered as growth factors for some insects. Biopterins are cofactors for the AROMATIC AMINO ACID hydroxylases and NITRIC OXIDE SYNTHASE. Deficiencies in BIOPTERINS metabolism (e.g., lowered TETRAHYDROBIOPTERIN) are associated with neurological deterioration (e.g., HYPERPHENYLALANINAEMIA). 2-Amino-6-((1S,2R)-1,2-dihydroxypropyl)-4(1H)-pteridinone,2-Amino-6-((1S,2S)-1,2-dihydroxypropyl)-4(1H)-pteridinone,2-Amino-6-(1,2-dihydroxypropyl)-4(8H)-pteridinone,2-amino-6-((1R,2R)-1,2-dihydroxypropyl)-4(3H)-pteridinone,4(1H)-Pteridinone, 2-amino-6-(1,2-dihydroxypropyl)-, (S-(R*,S*))-,6-Biopterin,Biopterin,D-threo-Biopterin,L-Biopterin,L-erythro-Biopterin,L-threo-Biopterin,2-Amino-6-(1,2-dihydroxypropyl)-4(1H)-pteridinone,Dictyopterin,Orinapterin,6 Biopterin,D threo Biopterin,L Biopterin,L erythro Biopterin,L threo Biopterin
D013997 Time Factors Elements of limited time intervals, contributing to particular results or situations. Time Series,Factor, Time,Time Factor
D015662 Trimethoprim, Sulfamethoxazole Drug Combination A drug combination with broad-spectrum antibacterial activity against both gram-positive and gram-negative organisms. It is effective in the treatment of many infections, including PNEUMOCYSTIS PNEUMONIA in AIDS. Trimethoprim-Sulfamethoxazole Combination,Abactrim,Bactifor,Bactrim,Biseptol,Biseptol-480,Centran,Centrin,Co-Trimoxazole,Cotrimoxazole,Drylin,Eslectin,Eusaprim,Insozalin,Kepinol,Kepinol Forte,Lescot,Metomide,Oriprim,Septra,Septrin,Sulfamethoxazole-Trimethoprim Combination,Sulprim,Sumetrolim,TMP SMX,TMP-SMX,Trimedin,Trimethoprim-Sulfamethoxazole,Trimethoprimsulfa,Trimezole,Trimosulfa,Biseptol 480,Biseptol480,Co Trimoxazole,Sulfamethoxazole Trimethoprim Combination,Trimethoprim Sulfamethoxazole,Trimethoprim Sulfamethoxazole Combination
D017827 Machado-Joseph Disease A dominantly-inherited ATAXIA first described in people of Azorean and Portuguese descent, and subsequently identified in Brazil, Japan, China, and Australia. This disorder is classified as one of the SPINOCEREBELLAR ATAXIAS (Type 3) and has been associated with a mutation of the MJD1 gene on chromosome 14. Clinical features include progressive ataxia, DYSARTHRIA, postural instability, nystagmus, eyelid retraction, and facial FASCICULATIONS. DYSTONIA is prominent in younger patients (referred to as Type I Machado-Joseph Disease). Type II features ataxia and ocular signs; Type III features MUSCULAR ATROPHY and a sensorimotor neuropathy; and Type IV features extrapyramidal signs combined with a sensorimotor neuropathy. (From Clin Neurosci 1995;3(1):17-22; Ann Neurol 1998 Mar;43(3):288-96) Azorean Disease,Joseph Disease,Spinocerebellar Ataxia Type 3,Striatonigral Degeneration, Autosomal Dominant,Autosomal Dominant Striatonigral Degeneration,Azorean Ataxia,Azorean Disease (Machado-Joseph),Azorean Disease, Nervous System,Azorean Neurologic Disease,Joseph Azorean Disease,Machado-Joseph Azorean Disease,Machado-Joseph Disease Type I,Machado-Joseph Disease Type II,Machado-Joseph Disease Type III,Machado-Joseph Disease Type IV,Nervous System Azorean Disease,Nigrospinodentatal Degeneration,Spinocerebellar Ataxia 3,Spinocerebellar Ataxia-3,Spinocerebellar Atrophy III,Spinocerebellar Atrophy Type 3,Type 3 Spinocerebellar Ataxia,Type I Machado-Joseph Disease,Type II Machado-Joseph Disease,Type III Machado-Joseph Disease,Type IV Machado-Joseph Disease,3s, Spinocerebellar Ataxia,Ataxia 3, Spinocerebellar,Ataxia 3s, Spinocerebellar,Atrophy III, Spinocerebellar,Atrophy IIIs, Spinocerebellar,Azorean Disease (Machado Joseph),Degeneration, Nigrospinodentatal,Degenerations, Nigrospinodentatal,Disease, Azorean,Disease, Azorean (Machado-Joseph),Disease, Azorean Neurologic,Disease, Joseph,Disease, Joseph Azorean,Disease, Machado-Joseph,Disease, Machado-Joseph Azorean,III, Spinocerebellar Atrophy,Machado Joseph Azorean Disease,Machado Joseph Disease,Machado Joseph Disease Type I,Machado Joseph Disease Type II,Machado Joseph Disease Type III,Machado Joseph Disease Type IV,Neurologic Disease, Azorean,Nigrospinodentatal Degenerations,Spinocerebellar Ataxia 3s,Spinocerebellar Atrophy IIIs,Type I Machado Joseph Disease,Type II Machado Joseph Disease,Type III Machado Joseph Disease,Type IV Machado Joseph Disease

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