Poster session 7: Ataxia, chorea, drug-induced movement disorders, myoclonus, spasticity, stereotypies, tics, tremor
- Publisher
- John Wiley and Sons
- Year
- 2002
- Tongue
- English
- Weight
- 491 KB
- Volume
- 17
- Category
- Article
- ISSN
- 0885-3185
No coin nor oath required. For personal study only.
✦ Synopsis
Objective: Nigrostriatal dysfunction is a likely substrate for extrapyramidal features that occur in most spinocerebellar ataxias (SCA).
Methods: We evaluated striatal dopaminergic function in patients with SCA-2 using single photon emission computed tomography (SPECT) and radioligands for dopamine transporter function (3I-2-carbomethyl-3-(4iodophenyl)-tropane, -CIT) and dopamine D2 receptor status ( 123 I-iodobenzamide, IBZM). Age-matched healthy subjects and patients with Parkinson's disease (PD) served as controls.
Results: Clinically, cervical dystonia was present in 7 SCA2 patients including torticollis (n ϭ 6, associated with laterocollis in 2 of them) and dystonic head tremor (n ϭ 1). Striato-cerebellar (S/C) ratios of -CIT binding were significantly reduced in SCA2 patients compared to control subjects and they were within the range of matched PD patients. S/C ratios of IBZM binding were significantly reduced in SCA2 patients compared with control and PD subjects.
Conclusions: We conclude that cervical dystonia is a frequent extrapyramidal feature in SCA2, likely reflecting the profound functional disturbance of dopaminergic neurotransmission that was observed both at preand at postsynaptic level.