Abstracts

RATIONALE: Jakob[ndash]Creutzfeldt disease (CJD) is a form of acquired spongiform encephalopathy that results in rapidly progressive cognitive decline, involuntary movements such as myoclonus, and pyramidal and extrapyramidal involvements, which ultimately lead to grave outcome. The myoclonus can be very prominent and worsens in severity with disease progression. There have been recent reports of CJD presenting as non-convulsive status epilepticus.1 In addition, the myoclonus of CJD is often refractory to most of the antiepileptic medications other than benzodiazepines. Zonisamide, a novel anticonvulsant, has been reported to be effective in the treatment of the often refractory myoclonic seizures seen in progressive myoclonic epilepsy, therefore, it was thought that zonisamide may be effective in treating the myoclonus of CJD. This report describes two patients with CJD and intractable myoclonus who were recently treated with zonisamide.
METHODS: Retrospective chart analysis was performed for two patients diagnosed with CJD admitted to University of Southern California (USC) University Hospital between 2000 and 2001.
RESULTS: Two patients, a 58-year-old male and 72-year-old female, presented with similar complaints of sudden and rapid decline in cognition, diminished attention span, speech disorders, excessive myoclonic jerks, and gait and balance problems. The patients were admitted to local hospitals and then transferred to USC. The 72-year-old female patient had continuous myoclonus, which caused her to continuously fall. At the local hospital, she was treated with valproic acid, followed by carbamazepine, both of which were ineffective. On transfer, video telemetry electroencephalogram monitoring showed the patient to be in myoclonic status epilepticus. She was started on zonisamide, and the myoclonus was markedly reduced. The 58-year-old male had classical stimulus-sensitive myoclonus and was also given zonisamide, resulting in decreased myoclonus. Both patients were evaluated for the 14-3-3 protein at the National Prion Center at Case Western University; the female patient was negative, and the male patient was positive. The male patient[scquote]s hospital course was very short and his brain autopsy showed spongioform changes characteristic of CJD. The female patient had a skin biopsy for Lafora bodies and other tests for progressive myoclonic epilepsy, which were negative.
CONCLUSIONS: Myoclonus in CJD can be varied in presentation from classic stimuli-sensitive myoclonus to myoclonic status epilepticus, which is refractory to other antiepileptic medications, but both types appear to respond well to zonisamide.
Reference:
1. Schwinn PJ, Krumholz A, Seiden LG. Jakob-Creutzfeldt disease presenting as non-convulsive status epilepticus. Epilepsia 2001;42(Suppl 7):146-147.