Rasmussen’s encephalitis (RE) is a rare chronic inflammatory brain disorder resulting in progressive neurodegeneration in one cerebral hemisphere. The inflammatory process is accompanied by progressive loss of function of the affected hemisphere, associated with drug-resistant partial epilepsy. The diagnosis is based on a range of clinical, electroencephalographic, radiological and biochemical arguments, without any specific formal marker, which makes the diagnosis of the disease complex, especially in its initial phase. Seizures are refractory to anti-seizures medication (ASM) and to classical immunomodulatory treatments.
Clinical criteria for Rasmussen´s encephalitis
| Part A | Need 3/3 = 5 criteria |
| Clinical | Focal seizures (±epilepsia partialis continua) AND unilateral cortical deficit(s) |
| EEG | Unihemispheric slowing ± epileptiform activity AND unilateral focal seizure onset |
| MRI | Gray or white matter T2/Flair hyperintense signal, hyperintense signal or atrophy of ipsilateral caudate head |
| Part B | Need 2/3 |
| Clinical | Epilepsia partialis continua or progressivea unilateral cortical deficit(s) |
| MRI | Progressivea unihemispheric focal cortical atrophy |
| Histopathology | T-cell dominated encephalitis with activated microglial cells (cerebral biopsy) |
Patient has to meet A or B criteria. a Progressive means that at least two sequential clinical examinations and MRI studies are necessary to conclude.
References:
- Villeneuve N, Lépine A, Girard N, Guedj E, Daquin G. Rasmussen’s encephalitis: Early diagnostic criteria in children. Rev Neurol (Paris). 2022 May 11:S0035-3787(22)00583-5. [Medline]
- Cay-Martinez KC, Hickman RA, McKhann Ii GM, Provenzano FA, Sands TT. Rasmussen Encephalitis: An Update. Semin Neurol. 2020 Apr;40(2):201-210. [Medline]
Created Jun 06, 2022.
