Striatal Dopaminergic Functioning in Patients with Sporadic and Hereditary Spastic Paraplegias with Parkinsonism
A 64-yr-old male presented with a progressive spastic gait and pain in both legs starting 4 yr prior. On neurological examination, he revealed spasticity with brisk DTRs and ankle clonus. He exhibited postural hand tremor and rigidity, primarily on the right side, as well as subtle bradykinesia on rapid alternating movement. On cranial nerve function test, the patient had sensory neural hearing loss and showed head-shaking nystagmus suggestive of left vestibulopathy. His arm motor function was normal, and the motor strength of his legs was mildly decreased (Rt hip 3, Lt hip 4+, Rt knee 4, Lt knee 4+). He complained of urinary hesitancy and difficulty on initial examination; however, approximately 2 months after the initial examination, voiding symptoms disappeared spontaneously. Other autonomic dysfunctions were not observed. He was taking anti-parkinsonian medications for 13 months (last medications were levodopa 600 mg, pramipexole 1.5 mg, amantadine 100 mg per day); however the medications were discontinued due to ineffectiveness and continuous aggravation. SPG4 was normal and SCA genetic tests were not performed. On his last visit (at the age 67 yr), neurological symptoms were worse. He was wheelchair-bound and showed severe dysarthria, dysphagia, and generalized myalgia. He died at the age of 68 yr.