A case of progressive supranuclear palsy with late-onset supplementary motor area seizure

Abstract

We report a 75-year-old, right-handed man, presenting with supplementary motor area (SMA) seizure. The patient had suffered from frequent attacks of transient inability to speak and move without loss of awareness. On admission, he presented with vertical gaze paresis, axial rigidity, paratonia of extremities and gait disturbance. The attacks were preceded by discomfort on the head, followed by inability to move the whole body and arrest of vocalization with tonic posture and exaggerated breathing. Consciousness and cognitive function were preserved throughout the attacks. Electroencephalography recorded intermittently slow theta waves in the bifrontal regions. Brain MRI showed atrophy of the midbrain tegmentum with lacunar state suggesting progressive supranuclear palsy. SPECT with 123I-iomazenil revealed decreased uptake in the medial frontal areas including SMA, bilaterally. The seizures resolved completely following treatment with carbamazepine. Based on clinical features and neuroimagings, we speculated that the negative motor area within SMA was responsible for his seizure. Physicians should keep in mind that SMA seizure comprising negative motor phenomenon can occur in the elderly.