Improvement of SSPE after carbamazepine: natural course or therapeutic effect?

Introduction: Subacute sclerosing panencephalitis (SSPE) is a progressive lethal neurodegenerative disease caused by persistent infection with mutated virus after measles infection. With a latency of 2–10 years clinical manifestation are behavioral changes, cognitive decline (stage 1), myoclonus (st. 2), progressive spastic/extrapyramidal movement disorder (st. 3) and dementia up to decerebration (st. 4). We present a 17-year-old girl with an uncommon favorable course until now.

Patient: At age 12 years she developed encephalopathy with somnolence, myoclonus and ataxia up to incapacity to walk and eat independently. There was a preceding episode of 2–3 months with amnesia and deterioration in school performance. Just before moving to Germany at the age of 14 the diagnosis SSPE was established by the doctors in Kosovo. There was only little improvement under levetiracetam and valproate. After confirming the diagnosis we changed the therapy to carbamazepine (CBZ) (Titomanlio et al. 2007). Within a few days we observed a marked decrease of myoclonus still accompanied by continued periodic complexes on EEG as well as improvement of memory, motor function and vigor. After 8 months on therapy there was a progressive deterioration of all functions, with disastrous continuation after exchange from CBZ to lamotrigine (confinement to bed). After restarting CBZ the response was unexpectedly good and better than before discontinuing CBZ. In the following 6 months clinical improvement continued with complete disappearance of myoclonus and the Radermecker complexes in EEG. Also motor and cognitive functions improved. After 12 months of further observation she shows no clinical signs of deterioration.

Conclusion: In the literature there are no evidence-based recommendations for the therapy of SSPE. The clinical spectrum of the disease is vast. In the majority of cases the course is rapidly progressive. There are cases, however, of slow progression and intermittent improvements which makes judgement of therapeutic intervention difficult. In our case we observed that the first use of CBZ caused marked improvement. The second use, after deterioration and stopping the drug, shows a very favorable course indeed for 18 months now. The patient improved from stage 2 to stage 1. This maybe is the natural course of the disease rather than caused by our intervention.