Wilson’s Disease, Huntington’s Disease (AAN 2006)

Subject: Wilson’s Disease, Huntington’s Disease (AAN 2006)

Date: 4/25/2006

E-MOVE reports from the 58th Annual Meeting of the American Academy of Neurology, San Diego California, April 1-8, 2006. Poster, session, and page numbers are from Neurology 2006;66 (supplement 2)

Treatment of Wilson’s disease with ammonium tetrathiomolybdate: IV. Comparison of tetrathiomolybdate and trientine in a double blind study of treatment of the neurologic presentation of Wilson’s disease
MT Lorincz, F Askari, M Carlson, M Schilsky, P Hedera, P Moretti, R Tankanow, RB Dick, J Sitterly, GJ Brewer
S33.003, A252

Ammonium tetrathiomolybdate (ATM) is superior to trientine in treatment of the neurologic symptoms of Wilson’s disease, according to this study. ATM binds with cooper and protein, and may be administered with food to prevent copper absorption, or without food to enter the bloodstream and bind toxic copper.

Forty-eight patients were randomized to a standard regimen of trientine or to ATM, 3 times daily with meals and 3 times daily between meals. Six patients on trientine and 1 on ATM deteriorated over the course of the eight-week trial. Over 3 years of follow-up, 3 patients on trientine died following neurological worsening, versus 1 on ATM. ATM-treated patients had an overall 80% improvement on a semi-quantitative neurological exam, versus 51% for trientine.

“Tetrathiomolybdate is a better choice than trientine for preserving neurologic function in patients who present with neurologic disease,” the authors conclude.

This study is now available in Archives of Neurology, 2006;63:521-527

Microglial activation in presymptomatic Huntington’s disease gene carriers: A combined 11C-PK1195 and 11C-raclopride PET study
YF Tai, N Pavese, A Gerhard, S Tabrizi, RA Barker, DJ Brooks, P Piccini
S20.003, A134

Microglia are activated in regions of striatal neuronal dysfunction before onset of Huntington’s disease symptoms, according to this study.

Eleven presymptomatic HD gene carriers (average CAG repeat length of 43) and 10 controls underwent PET with raclopride for dopaminergic dysfunction imaging, and PK1195 for imaging of activated microglia. The mean striatal binding potential for PK1195 was twice as high in gene carriers as in controls (p=0.001), while striatal raclopride binding was 20% lower (p=0.025). Abnormal raclopride binding was found in 9 of 11 gene carriers; of these, 7 had abnormal PK1195 binding, “suggesting neuronal dysfunction may precede microglial activation,” according to the authors.

“Our findings support the notion that microglial activation is an early event in the pathogenic processes of HD,” which may have important therapeutic implications, they conclude.

E-MOVE Editor: Richard Robinson, NASW, WE MOVE

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