Clinical manifestations and thyrotropin releasing hormone therapy in cerebellar degenerations

Twenty-nine documented cases of cerebellar degeneration at the National Taiwan University Hospital during a 8-year period (1980 through 1987) were reviewed. They were classified as familial (N = 14) or sporadic (N = 15) to compare the clinical signs and symptoms between both groups. The familial cases had earlier onset, and gaze paresis was found only in this group. Patients younger than 30 years old were more often familial cases and had more frequent deep sensation involvement. Oculomotor abnormalities on electrooculographic (EOG) tracings of 21 patients were analysed. The defects included abnormal OKN (86%), jerky pursuit (76%), ocular dysmetria (57%), slow saccades (43%), abnormal VOR or VVOR (43%), and fixation instability (19%). Intravenous TRH therapy was used in 17 patients, and resulted in 8 (47%) subjectively and 6 (35%) objectively improved cases. The use of TRH therapy in cerebellar degeneration was discussed.