Table 1.

known neuropathology in hyperkinetic disorders described in this article.

Hyperkinetic disorder	Disease	Description of neuropathology	Immunohistochemistry	Future directions
Chorea	HD	Degeneration in striatum, frontal cortex, brain stem, cerebellum + gliosis + protein inclusions	1C2+/huntingtin+
	Neuroacanthosis	Striatal degeneration + gliosis (no protein inclusions)	1C2−
	DRPLA	Degeneration in pallidum, STN, dentate, and red nucleus + protein inclusions	1C2+/atrophin-1
	SCA17	Degeneration in striatum, ventral thalamus, SN, inferior olive, cerebellum + protein inclusions	1C2+/TBP
	HDL2	Degeneration and protein inclusions largely comparable to HD	1C2+
	HDL1	??		Neuropathology?
	HDL3	??		Neuropathology?
Dystonia	DYT1	Degeneration of SNc, LC, raphe nuclei, PPN, and dentate	Ub+, TorsinA+	Concomitant tangles/Lewy bodies?
	DYT6 and DYT11	??		Neuropathology?
	DYT5/DYT14	SN neurodegeneration; 1 case with Lewy bodies		Concomitant Lewy bodies?
	LUBAG/DYT3	Mosaic neuronal loss in caudate and lateral putamen + gliosis
	PD	Diffuse degeneration throughout the CNS (six stages) with Lewy bodies, pale bodies, and Lewy neurites	Alfa-synuclein
	PSP	Degeneration of SN, locus coeruleus, pons, midbrain, pallidum + neuronal and glial protein inclusions	tau
	CBD	Degeneration in SN, pallidum, striatum, STN, thalamus, and red nucleus + neuronal and glial protein inclusions	tau
	MSA	Variable degeneration of olivo-ponto-cerebellar system and basal ganglia + neuronal + glial protein inclusions	Alfa-synuclein
Tremor	ET	Cerebellar cortical degeneration		LB in brainstem concomitant? Ub+/p62+ nii specific?
	PD	See above	Alfa-synuclein
	Cerebellar tremor	Lesions in cerebellum or fiber tracts from deep cerebellar nuclei
	Holmes tremor	Lesions in ventrolateral thalamus/red nucleus or surrounding fiber tracts
	Palatal tremor	Degeneration of dentate-olivary pathway + secondary hypertrophy of inferior olive
	Li induced tremor	Neuronal loss + gliosis in cerebellar cortex and dentate nucleus