Examination And Investigations

On presentation in 1996 the patient had an expressive aphasia, making grunts, and communicating by pointing out things with his eyes and hands. He understood most of what happened around him in spite of having brief absence seizures with eye blinking that occurred almost every minute. I estimated that his IQ, except for the aphasia, was in the 60s. He weighed 85 kg and was ataxic. There were no other physical or neurological abnormalities.

An EEG showed continuous slowing in the left frontocentral region, which frequently spread widely through the left hemisphere (Figure 35.1) and eventually evolved to frequent absence seizures with blinking and generalized irregular spike-wave discharges. The seizures lasted 4-20 s and occurred every 10-30 s.

The mother refused to allow further investigations because of the anesthetic accidents in childhood. Follow-up EEGs showed progressively less frequent abnormalities. Eventually, in late 1996, a waking background rhythm of 5-6 Hz was observed.

A very old CT scan was reportedly normal. In mid-1999, a 99-technetium ECD cerebral single photon emission CT scan showed decreased perfusion in the left peri-sylvian region. A 2.0 T magnetic resonance imaging (MRI) study showed a very large cranial vault hemangioma in the left parietal region. Five months later, a contrast

MRI scan followed by a bone-density CT scan showed that the hemangioma was unchanged, had no intracranial extension, and did not involve the meninges or cortex.

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