[Clinical presentations of neurological forms of hepatolenticular degeneration in Primorsky Krai].

Clinical characteristics of neurological forms of hepatolenticular degeneration (HLD) were studied in one of the regions of Russia (Primorsky Krai) in the sample of 111 patients at the wide age range (average 35.6 years); mean time since the diagnosis was 13.7 years. According to N.V. Konovalov's classification, 4 groups of patients were identified: I arrhythmic-hyperkinetic form (n=61), II extra-pyramidal cortical form (n=30), III tremulous form (n=15), IV tremulous-rigid form (n=6). A main body of clinical manifestations of the pathology at the stage of cerebral signal of HLD manifestation was determined; three degrees of the severity of its course were established. Correlation between clinical HLD manifestation severityand the time since the diagnosis of neurological stage of the disease and its form was noted. Age parameters of the development of different extra-pyramidal disturbances and predictors of the severity of the course were specified.