PubMed1985
The marked variability of psychiatric and neurological features of Huntington's chorea is described in a large family consisting of 31 members of two filial generations and the parenteral generation, of which 13 members showed manifest signs of the disease, while two further members died probably in a preliminary stage of the disease. It is of interest that a few members of the family had a large number of children, while the majority remained without offspring. Analysis of the psychiatric symptoms and signs shows that the course of the disease may take either a quiet demential, or a turbulent form. In the latter the features of manic-depressive psychosis or of schizophrenia occur, as well as a syndrome resembling psychopathic states with explosive-violent features comparable to the pseudo-psychopathia syndrome of U. H. Peters. The quiet demential course is correlated to progressive cerebral atrophy. The different forms of the turbulent course may be due to additional, genetically determined radicals of both groups of psychoses, to localized differences in the progression of the cerebral atrophy or to unspecific noxious influences from the external or internal milieu. With regard to neurological features, apart from the signs of chorea, akinesic rigidity and tic-like hyperkinesias with transition into stereotypes and primitive motor patterns were observed, as well as an apallic syndrome in the terminal phase. As a rare psychiatric variant, a syndrome characterized by compulsive pedantry combined with tic-like hyperkinesias was observed. The possibility of a striatal lesion causing motor and psychiatric impulsive features is pointed out.