Striatal Dopaminergic Functioning in Patients with Sporadic and Hereditary Spastic Paraplegias with Parkinsonism
This 59-yr-old male was healthy until the age of 49 yr, when he experienced unsteadiness and gait imbalance. He complained of sensory paresthesia in his feet and orthostatic dizziness. Neurological examination revealed spastic ataxia and postural instability. The patient showed spasticity of the legs with motor weakness. He had autonomic dysfunction on breath test (expiration/inspiration ratio=1.04, normal range>1.08), and the other autonomic functions were normal. He was taking amantadine (200 mg per day) without effect. Orthostatic dizziness disappeared during the follow-up period. Genetic evaluation for SCA types 1, 2, 3, 6, 7, and 17, SPG 4, and Friedreich ataxia (frataxin) were all negative. The patient is now 63 yr old and shows spastic gait, leg weakness, and paresthesia. Orthostatic dizziness, and other dysautonomic symptoms were not present.