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Q1: What is this uncommon epilepsy syndrome called?
Epilepsia partialis continua (EPC).
Q2: What is the connection between the abnormal biochemistry and the neurological presentation?
Hyperglycaemia is a well recognised metabolic cause of EPC.1
Q3: In what proportion of cases of this syndrome will computed tomography or magnetic resonance imaging of the brain be abnormal?
EPC is defined as clonic muscular twitching repeated at fairly short intervals in one part of the body for a period of days or weeks. It is a form of focal status epilepticus manifesting as continuous focal motor seizures.2 It is associated with a wide range of electroencephalographic abnormalities including focal spikes and focal slow waves2. However in some cases the electroencephalogram may be normal.3 Causes of EPC include structural abnormalities such as central nervous system tumours, trauma, cerebral infarction, intracerebral haemorrhage, cerebral abscess, neuronal migration disorders, and vascular malformation.2 However in up to 50% of cases conventional brain imaging may be normal.3 Causes where brain imaging may be normal include hyponatraemia, hyperglycaemia, hepatic encephalopathy, and encephalitis.2
The association between hyperglycaemia and EPC is well recognised. In some cases patients have an underlying structural abnormality such as cerebral infarction. In others hyperglycaemia can cause EPC in the absence of any neurological disease. In these patients treatment of the seizures and reversal of the metabolic disorder prevents any long term neurological sequelae.1 In the majority of patients with EPC associated with hyperglycaemia EPC occurs before impairment of consciousness and coma; it is important therefore to recognise this association. All patients presenting with EPC should have immediate determination of blood glucose concentrations. This could lead to the detection of previously undiagnosed diabetes mellitus and prompt reversal of the neurological condition. Our patient was treated with insulin, intravenous fluids, and a carbamezepine 400 mg daily. His seizures quickly resolved and he has not had any recurrence. Neurological examination at a follow up neurology outpatient clinic three months later did not reveal any abnormalities.
Epilepsia partialis continua, secondary to hyperglycaemia.
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