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Accession number;06A0654485
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| Title;Review/Advances in Neurological Therapeutics (2005). Spinocerebellar Degeneration |
| Author;
TSUNEMI TAIJI
(Tokyo Med. and Dent. Univ.)
MIZUSAWA HIDEHIRO
(Tokyo Med. and Dent. Univ.)
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Journal Title;Neurological Therapeutics
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Journal Code:X0110A
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ISSN:0916-8443
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VOL.23;NO.4;PAGE.377-381(2006)
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| Figure&Table&Reference;REF.29 |
| Pub. Country;Japan |
| Language;Japanese |
| Abstract;We reviewed reports on new therapies for spinocerebellar degeneration (SCD) which were published in 2005. In clinical trials, anti-oxidant therapy for Freisreich ataxia was reported. A combined treatment using coenzyme Q10 (400mg/dl) and vitamin E (2,100IU/dl) was carried out for 47 months. The significant improvement in cardiac function and cardiac and muscle bioenergetics was observed. Moreover, international co-operative Ataxic Rating Scale and kinetic scores were better than predicted in 7 out of 10 patients. Open labeled clinical studies or a case report were publishied. Lamotrigine is new anti-convulsants and may reduce NMDA associated neurotoxicity. Results of open labeled trial showed the improvement gait disturbance in spinocerebellar ataxia type 3 (SCA3). Low dose piracetam was reported to be effective for myoclonus in SCA3. One of the potassium channel blocker 3,4-diaminopyridine was reported to be effective for postural instability. Repattive transcranial magnetic stimulation was applied for patients with pure cerebellar ataxia and showed significant effect. The cost of intrathecal baclofen therapy for spastic paraparesis became to be covered by the Japanese Health Insurance in April 2006. In basic research using animal models, several studies succeeded to clarify the mechanism of neurodegeneration. Previous studies showed that ataxin-1 may regulate gene expression. Ataxin-1 has a AXT domain, which conserves among several species, interacts with transcription factor Senseless/Gfi-1. Gfi-1 levels were reduced in ataxin-1 transgenic mice and a loss of Purkinje cells was found in Gfi-1 null mice, indicating that Gfi-1 contributes to survival of Purkinje cells. Ataxin-3 with normal polyglutamine (polyQ) repeat was reported to suppress neurodegeneration induced by ataxin-3 with mutant polyQ repeat. This suppressor activity required ubiqutin activity of ataxin-3 and depend on proteosome function.... (author abst.) |
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