Handbook of epilepsy treatment : forms, causes, and therapy by S D Shorvon

By S D Shorvon

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Sample text

It is an uncommon condition with a male predominance and usually without a family history. Onset occurs at between 18 months and 13 years, in most cases between 4 and 7 years. The aetiology and pathogenesis of the syndrome, if indeed these are unitary, are unknown. The condition develops in children who were previously developmentally normal, and presents with a progressive aphasia, developing gradually over months or subacutely over weeks, although acute presentations are also encountered. Verbal comprehension and expressive speech both become severely affected.

Sexual feelings can occur sometimes with erection or ejaculation. Gustatory seizures have their origin in the suprasylvian region (adjacent to the mouth and throat primary sensory region). Ictal vertigo also originates in the suprasylvian region. Transient postictal sensory deficits or spatial disorientation occur. Seizures from the occipital, parieto-occipital, and temporal-occipital cortex are usually characterized by visual symptoms. Elementary visual hallucinations (sensations of colours, shapes, flashes and patterns) are most common, which can be intermittent, stationary or appear to move across the visual field and to grow.

Apparently generalized tonic–clonic seizures, without lateralizing features, are particularly characteristic of seizures arising in the cingulate or dorsolateral cortex, but can occur from other frontal lobe locations. Version of the head and eyes is common in many types of frontal lobe (and less frequently in temporal lobe epilepsy), and is sometimes the only seizure manifestation (‘versive seizures’). When version occurs in full consciousness at the onset of a seizure, this is useful evidence of a focus in the contralateral frontal dorsolateral convexity, but in other situations the direction of version is of little lateralizing value.

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