LAUSR

Rasmussen syndrome is a subacute inflammatory encephalitis and one of the causes of continual partial seizures. The onset of convulsive attacks is usually preceded by an episode of non-specific fever, usually of viral origin (eg, CMV infection). Attacks are usually partial, long-lasting and frequent. It most often occurs in children under 10 years of age. EEG findings show diffuse paroxysmal activity. The disease is progressive, with development of various neurological deficits (hemiplegia, hemianopsia, and aphasia). Here we present a case of a 3-year-old girl, with normal early psychomotor development, who had the first absence-type convulsive attack during her holidays in Greece in 2016. Treatment with valproic acid was initiated. In the next two months, two more seizures followed, accompanied with fever (eyes fixation, arm and leg swings, squinting, non-response to calls for 10 seconds). Both times she was hospitalized, and during the second hospitalization, EEG showed the existence of generalized epileptiform changes. MR of the endocranium was normal. Due to poor control of attacks and their frequency, new antiepileptics (lamiktal, carbamazepine, topamax) have been introduced into the therapy. In March 2017, new MR of the endocranium showed the excistence of a hypotrophy of the left hemisphere and descreet left-sided Walerian degeneration, when diagnosis of Rasmussen’s syndrome was suspected. Further diagnostic procedures included immunological examination of the liquor and the presence of oligoclonal IgG strains in the liquor (LGI1, AMPA, GABA-B) was confirmed. Anti-NMDA autoantibodies were negative, and therapy with specific immunoglobulins was administered. Corticosteroids were also administered during the exacerbations. In a further course of the disease, a clinical finding reveals a worsening of neurological status (spastic right-sided hemiparesis, facial nerve paralysis and dysarthria). At the end of 2017, the girl had an average of 4–5 convulsive attacks per day. With medical therapy, physical and speech therapies were regularly performed. Because of progression of the disease and poor response to therapy, surgical treatment was proposed. In September 2018, left functional hemispherotomy was performed at the clinic of neursciences in Paris, after which the convulsions completely disappeared. From all of the above, we conclude that functional hemispherotomy is a therapy of choice in the treatment of resistant attacks in Rasmussen’s syndrome, which stops progression of the disease and prevents further neurological damage.