Corticosteroids, IV immunoglobulins (IVIg), cyclophosphamide, therapeutic plasma exchange (TPE), and protein A IgG immunoadsorption (PAI) were evaluated in 15 patients with confirmed Rasmussen’s encephalitis (RE) and reported from various centers in Italy. Steroids were used for status epilepticus or worsening seizures. In 6/11 patients steroid treatment temporarily reduced seizure frequency. All attempts to withdraw prednisone resulted in seizure exacerbation, and treatment was maintained until surgery. IVIg in 11 patients was partially effective in 6. Cyclophosphamide was ineffective in 4 patients treated, 1 developing severe leukopenia. TPE was effective in the control of status epilepticus in 1 of 5 patients treated. PAI given chronically initially controlled status epilepticus and improved the neurologic condition in 3 patients treated, but the effect was not sustained. Surgery (hemispherotomy or hemispherectomy) in 13 patients at 5.2 +/- 3.9 years after disease onset controlled seizures initially, and antiepileptic drugs were withdrawn in 4 and reduced in 7. After surgery, the neurologic condition improved in all patients. Surgery is the only treatment that halts disease progression. Immunomodulatory therapy is considered when surgery is not feasible, in late-onset patients with slow progression, and in the infrequent case of bilateral disease. [1]

COMMENT. The authors propose immunotherapy to delay surgery in patients with 1) late onset (adolescent or adult) RE with slower and milder course than the typical childhood-onset form; 2) dominant hemisphere involvement and slow progression, when surgery is contraindicated; 3) suspected cases of RE without deterioration and atrophy; 4) bilateral RE, and 5) severe cases requiring stabilization before surgery. Surgery is the preferred and optimal treatment to control seizures and halt neurologic deterioration. Bolus corticosteroids and PAI block status epilepticus. IVIg may have a role in adult patients but is ineffective in childhood.