The Role of Medical Treatment in Rasmussen's Encephalitis
Abstract number :
2.132
Submission category :
Year :
2000
Submission ID :
1284
Source :
www.aesnet.org
Presentation date :
12/2/2000 12:00:00 AM
Published date :
Dec 1, 2000, 06:00 AM
Authors :
Lucia Fusco, Tiziana Granata, Giuseppe Gobbi, Giuseppe Capovilla, Bernardo Dalla Bernardina, Laura Tassi, Carlo Antozzi, Renato Mantegazza, Marina Casazza, Gianna Bertani, Lucio Giordano, Federico Vigevano, Children's Hosp Bambino Gesu', Rome, Italy; Neur
RATIONALE: The etiopathogenesis of Rasmussen's encephalitis (RE) is still unknown and the role of viral infection and immunomediated disorders still under study. In addition to antiepileptic drugs that are of little benefit, the different proposed etiopathogeneses led clinicians to try a number of medical treatments, mainly antiviral or immunosuppressant agents, in the attempt to avoid or at least delay surgery. METHODS: In the context of a collaborative study, we reviewed medical and surgical treatment in 13 patients with RE followed up for a mean period of 8.2 ? 6.5 years (range 1-25 years). RESULTS: Age at onset ranged between 14 months and 11 years (mean 5.2 ? 2.9 years). Antiepileptic drug resistance was evident in all cases within a few months. Epilepsia partialis continua was present in 12 cases. The first neurological deficits appeared after a mean period of 6.5 (? 6.6) months from the first seizures (range 0 days-2 years) and invariably worsened, although the rate of progression varied in the different cases. Anti GluR3 antibodies were detected in 7 of 10 tested patients. Different therapeutic approaches were tried, including steroids (12 cases), ganciclovir (3 cases), interferon (1 case), plasma exchange (7 cases), immunoglobuline (8 cases) and immunosuppressant agents (6 cases). In only one case Ig immuno- adsorption was effective and prevented the need for surgery. In all other patients, medical treatment provided only transient improvement. Ten patients underwent surgery: eight patients underwent hemispherotomy, one patient underwent anatomical hemispherectomy and one functional hemispherectomy. During post surgical follow-up (mean 3.3 ? 3.4 years), 7 patients were seizure-free, and three patients had significant reduction in seizure frequency and disappearance of EPC. Disappearance of seizures was associated with significant improvement in neurological and mental status. CONCLUSIONS: Medical treatment, including antiepiletpic drugs and other pharmacological therapies, is of very little value in RE, but can delay the need for radical surgery that is inevitable in most cases.