Gene imprinting explains the transmission of the childhood form primarily through the father. Eventually progresses to include dementia and slowed eye movements. In juvenile HD, rigidity and parkinsonian tremor may be the primary manifestation. The most significant neuropathological change is a preferential loss of medium spiny neurons in the neostriatum. Biochemically there is marked loss of GABA, substance P, enkephalin, angiotension converting enzyme.
Neuronal changes begin very early in life, perhaps even from birth.
There is no known treatment that will stop progression, but there are symptomatic treatments such as haloperidol. Huntingtons can be diagnosed on MRI by caudate atrophy with appropriate history, and also by genetic testing. Differential diagnosis includes hepatocerebral degeneration, schizophrenia with tardive dyskinesia, other chorea's, and drug reactions. Sapp et al. Huntingtin localization in brains of normal and huntington's disease patients.