DOUBLE PATHOLOGY IN RASMUSSENS-SYNDROME - A WINDOW ON THE ETIOLOGY

The syndrome of chronic encephalitis with epilepsy (Rasmussen's syndro me) typically occurs in children and is characterized by the developme nt of intractable focal seizures, progressive hemiparesis and intellec tual deterioration. The etiology is unknown, and the pathological abno rmalities vary from those of active disease, with numerous microglial nodules, with or without neuronophagia, perivascular round cells and g lial scarring, to those of remote disease, demonstrated by neuronal lo ss, gliosis and perivascular round cells but few microglial nodules. W e describe five patients presenting with clinical features typical of Rasmussen's syndrome, in whom pathological examination showed a second , previously unsuspected pathology in addition to the changes of chron ic encephalitis. Two of the patients had vascular abnormalities bearin g some resemblance to cavernous angiomata, one had a tumor, one had tu berous sclerosis, and one the forme fruste of tuberous sclerosis. The coexistence of a second pathology in these patients may provide inform ation about the underlying mechanism of this rare condition.