Clinical and physiological features of epilepsia partialis continua. Cases ascertained in the UK.
Epilepsia partialis continua (EPC) is defined clinically as a syndrome of continuous focal jerking of a body part, usually localized to a distal limb, occurring over hours, days or even years. The anatomical and physiological origin of EPC has been the subject of much speculation. It has been argued that EPC is a form of focal cortical myoclonus, but subcortical mechanisms have also been proposed. We describe a series of 36 patients ascertained over a period of 1 year in the UK using the British Neurological Surveillance Unit. The commonest aetiologies identified were Rasmussen's syndrome (n = 7; 19%) and cerebrovascular disease (n = 5; 14%). Rasmussen's syndrome was the most common diagnosis in patients under 16 years. In seven patients the cause remained unknown. Eight patients (22%) had focal epileptiform scalp EEG abnormalities, and 56% had generalized background scalp EEG disturbances. Lesions on MRI or CT were found in 20 cases (56%), half of whom showed predominant cortical involvement. The muscle jerking resolved in four patients (with no treatment in one), with a partial response to treatment in seven (19%) patients. A cognitive or neurological decline had been noted retrospectively in 13 (36%) patients (and in all of the patients with Rasmussen's syndrome). We personally saw 16 patients who underwent detailed clinical and neurophysiological assessments. Only six of the patients had EEG and EMG evidence for a cortical origin of their jerks; five others had indirect evidence for a cortical origin, from EMG, magnetic stimulation, and other investigations. Two patients did not have myoclonus of cortical origin, but some other source (brainstem and basal ganglia). The origin in the remaining three patients was uncertain. The clinical appearance of the muscle jerks was similar in all patients despite the different origins. We propose that the definition of EPC is best restricted to "continuous muscle jerks of cortical origin'. Continuous muscle jerking that arises from other sites in the nervous system should be termed "myoclonia continua'.
Cockerell
O C
OC
Epilepsy Research Group, National Hospital for Neurology and Neurosurgery, London, UK.
Rothwell
J
J
Thompson
P D
PD
Marsden
C D
CD
Shorvon
S D
SD
D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D004576:Electromyography; D017036:Epilepsia Partialis Continua / Q000473:pathology / Q000503:physiopathology*; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009207:Myoclonus / Q000503:physiopathology; D006113:United Kingdom