Striatal Dopaminergic Functioning in Patients with Sporadic and Hereditary Spastic Paraplegias with Parkinsonism
This 50-yr-old male was admitted because of a progressive gait disturbance that developed at age 40. Five years later, he became wheelchair-bound. Neurological examination showed severe spasticity in both legs, with marked enhanced tendon reflexes and extensor plantar responses. Muscle strength was decreased to grade 4 (Medical Research Council) in both lower extremities. The patient showed intermittent myoclonus in both legs that started 10 yr prior. He did not have cerebellar dysfunction. Eye movement examination revealed square wave jerk. He had a cystostomy because of urinary retention. Genetic studies for SCA types 1, 2, 3, 6, and 7 and SPG4 were all negative. His mother exhibited spastic gait in her 40s; however her gait disturbance were progressed minimally, and she could walk independently for 30 yr. Other family members were healthy and without any neurological problems.