An Approach To The Clinical Syptoms Of Huntington's Disease

Objective:Huntington's disease(HD) is a dominantly inherited neurodegenerative disorder, that usually presents with characteristic motor and cognitive disfunction and with variable and diverse psychiatric disturbances. And only the symptomatic therapy is clinically availlable, but still with little effciency. Thus, this artical tried to analysis the symptoms in order to seek the way for symptomatic therapy.Methods:4 normal-onset HD patients and 1 early-onset HD patient were choosen from the database of the neurology department of the second affiliated hospital of zhejiang university. The Unified Huntington Disease Rating Scale(UHDRS) was used to evaluate these five patients.Results:In the 4 normal-onset HD patients:tongue protrusion(3), dysarthria(3), ocular pursuit(2),saccade initiation(1). saccade velocity(1), finger taps(4), LURIA(4), pronate/supinate-hands(2). chorea(33%), rigidity(33%),bradykinesia(17%) and dystonia(17%). In the early-onset HD patient (lyear follow-up ago/now):tongue protrusion(3/4), finger taps(2/6).,rigidity(1/2), bradykinesia(1/2). chorea(1/7). gait(1/1). tandem walking(3/3), retropulsion pull test(2/4),and there is dysarthria(2), pronate/supinate-hands(1) and LURIA(1) after 1-year follow up. The Verbal Fluency Test shows food(7/5), commodity(6/4), people's name(7/7), place name(7/8). The Stroop test has the results:Color Naming 41/40, Word Reading 33/46, Interference 30/25. The behavioral assessment shows that no psychotic symptoms 1 year before, and a little self blaming now.Conclusions:This article foud that there is a high incidence of atypical motor symptoms in HD; the dysfunction of tongue protrusion, dysarthria. and the find activities of the hands are moderate, and makes a fast progress; the abnormal gait exists in most of the patients, but the walking function is somewhat preserved. Meanwhile, there may be immediate memory impairment in HD, and the psychotic symptoms may be associated with the familly carer.