Psychopathology in Patients With Degenerative Cerebellar Diseases: A Comparison to Huntington's Disease

• Published in: The American journal of psychiatry 2002-08, Vol.159 (8), p.1306-1314
• Main Authors: Leroi, Iracema, O'Hearn, Elizabeth, Marsh, Laura, Lyketsos, Constantine G., Rosenblatt, Adam, Ross, Christopher A., Brandt, Jason, Margolis, Russell L.
• Format: Article
• Language: English
• Subjects: Adult and adolescent clinical studies; Biological and medical sciences; Cerebellar Diseases - diagnosis; Cerebellar Diseases - epidemiology; Cerebellar Diseases - psychology; Cognition & reasoning; Cognition Disorders - diagnosis; Cognition Disorders - epidemiology; Comorbidity; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Female; Humans; Huntington Disease - diagnosis; Huntington Disease - epidemiology; Huntington Disease - psychology; Huntington's chorea; Male; Medical sciences; Mental Disorders - diagnosis; Mental Disorders - epidemiology; Middle Aged; Miscellaneous; Neurodegenerative Diseases - diagnosis; Neurodegenerative Diseases - epidemiology; Neurodegenerative Diseases - psychology; Neurological disorders; Neurology; Personality Disorders - diagnosis; Personality Disorders - epidemiology; Prevalence; Psychiatric Status Rating Scales; Psychiatry; Psychology. Psychoanalysis. Psychiatry; Psychopathology; Psychopathology. Psychiatry; Severity of Illness Index
• ISSN: 0002-953X; 1535-7228
• DOI: 10.1176/appi.ajp.159.8.1306

OBJECTIVE: This study estimated the psychiatric morbidity of patients with degenerative cerebellar diseases. METHOD: The study included a series of 31 patients with degenerative cerebellar diseases, compared with 21 patients with Huntington's disease and 29 neurologically healthy comparison subjects. Comprehensive psychiatric evaluations, including the Structured Clinical Interview for DSM-IV and psychopathology rating scales, were administered. RESULTS: The overall rate of noncognitive psychiatric disorders was 77% in the patients with degenerative cerebellar diseases, nearly identical to that in the patients with Huntington's disease (81%) and about double that seen in the neurologically healthy subjects (41%). There were high rates of all mood disorders in both the degenerative cerebellar diseases group (68%) and the Huntington's disease group (43%); the rate in the degenerative cerebellar diseases group was significantly higher than that in the neurologically healthy subjects (31%). The frequency of personality change in the three groups was striking: change was present in 26% of the degenerative cerebellar diseases patients, 48% of the Huntington's disease patients, and none of the neurologically healthy comparison subjects. A total of 19% of the degenerative cerebellar diseases subjects and 71% of the Huntington's disease subjects met DSM-IV criteria for either cognitive disorder or dementia. CONCLUSIONS: The high rate of psychiatric and cognitive disorders in the patients with degenerative cerebellar diseases suggests that many, if not most, patients with degenerative cerebellar diseases may benefit from psychiatric interventions. These results also support previous findings that the cerebellum may have a role in modulating emotion and cognition.