PT  - JOURNAL ARTICLE
AU  - Ryuji Kaji
AU  - Kailash Bhatia
AU  - Ann M Graybiel
TI  - Pathogenesis of dystonia: is it of cerebellar or basal ganglia origin?
AID  - 10.1136/jnnp-2017-316250
DP  - 2017 Oct 31
TA  - Journal of Neurology, Neurosurgery & Psychiatry
PG  - jnnp-2017-316250
4099  - http://jnnp.bmj.com/content/early/2017/10/31/jnnp-2017-316250.short
4100  - http://jnnp.bmj.com/content/early/2017/10/31/jnnp-2017-316250.full
AB  - Dystonia is a disorder of motor programmes controlling semiautomatic movements or postures, with clinical features such as sensory trick, which suggests sensorimotor mismatch as the basis. Dystonia was originally classified as a basal ganglia disease. It is now regarded as a ‘network’ disorder including the cerebellum, but the exact pathogenesis being unknown. Rare autopsy studies have found pathology both in the striatum and the cerebellum, and functional disorganisation was reported in the somatosensory cortex in patients. Recent animal studies showed physiologically tight disynaptic connections between the cerebellum and the striatum. We review clinical evidence in light of this new functional interaction between the cerebellum and basal ganglia, and put forward a hypothesis that dystonia is a basal ganglia disorder that can be induced by aberrant afferent inputs from the cerebellum.