Epilepsia partialis continua

Epilepsia partialis continua (EPC) can be defined as spontaneous regular or irregular clonic twitching of cerebral cortical origin, sometimes aggravated by action or sensory stimuli, confined to one part of the body, and continuing for hours, days or weeks. It is a remarkable condition, with highly characteristic features, and has a number of underlying causes (Table 4.7).

The clonic jerks in EPC can affect any group of muscles. In some individuals they are confined to a single muscle or muscle group, but in others the distribution is more widespread, and the distribution of the jerks can vary over time.

Table 4.7 Causes of epilepsia partialis continua.

Cerebral tumour Cerebral infection

Cerebral inflammatory disease

Cerebrovascular disease

Other cerebral disorders

Metabolic disturbance Drugs, toxins, poisoning

Primary (benign/malignant) Metastasis

Bacterial abscess, tuberculoma Parasitic infection (e.g. cysticercosis) Viral encephalitis, meningitis, HIV Whipple disease

Cerebral vasculitis (any type) Rasmussen encephalitis Granuloma (any type) Paraneoplastic disease, coeliac disease

Cerebral infarction/haemorrhage Arterio-venous malformation Venous thrombosis

Cortical dysplasia (many types) Mitochondrial disease Anoxic brain damage

Acute metabolic disturbance Hereditary or congenital (many types)

Many types

Agonists and antagonists are affected together, and distal muscles are more commonly involved than the proximal musculature. The jerks are spontaneous, and often exacerbated by action, startle or sensory stimuli. They can be single or cluster, and may have a rhythmic quality with a wide range of frequencies and amplitudes. Some jerks recur only every few minutes and others are more frequent. In chronic cases the jerks can continue relentlessly for months or years.

Treatment should be largely directed at the underlying cause. The seizures can remit spontaneously in acute cases. In a well-established case, however, epilepsia partialis continua can be particularly resistant to therapy, and IV antiepileptic therapy even to the point of anaesthesia can produce only temporary respite. It is usual to prescribe oral antiepileptic drugs, to prevent secondary generalization, even if the EPC itself is not controlled. Any of the antiepi-leptic drugs can be used and treatment follows conventional lines. In addition, the oral corticosteroids are sometimes helpful. Where there is an inflammatory or post-infective cause, courses of high-dose IV IgG have been used, sometimes with startling benefit. Plasma exchange has been tried with little effect, as have other immunosuppressive therapies and zidovudine. The long-term outcome depends on the underlying cause, but in many cases the clonic movements continue in spite of medical therapy. Very occasionally, there is resort to surgical therapy, either resective or by multiple subpial transection.

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