A syndrome of chronic partial epileptic seizures attended by progressive focal sensorimotor neurological deficit and cognitive decline, and with neuropathological features of a localized chronic encephalitis, was first defined by Rasmussen and his colleagues in 1958 . Diagnosis of Rasmussen's syndrome (RS) is suggested by the typical clinical features of epilepsia partialis continua, episodes of complex partial status and intractability to standard anti-epileptic drugs. Support for the diagnosis is provided by interictal electroencephalographic evidence of multiple independent lateralized epileptiform abnormalities, and radiological evidence of focal brain atrophy. T2-weighted magnetic resonance imaging may demonstrate high intensity signals in the white matter, consistent with gliosis, and positron emission tomography and single photon emission computed tomography may demonstrate localized changes in glucose metabolism and cerebral perfusion. Ultimately, however, diagnosis is dependent on the neuropathological appearances at brain biopsy or hemispherectomy. These show some variability , ranging from an ongoing inflammatory process with microglial nodules, neuronophagia, perivascular lymphocytic cuffing and glial scarring to “non-specific” changes with very few or no microglial nodules, mild perivascular inflammation and various degrees of neuronal loss and glial scarring.
Most predominantly an affliction of children, RS frequently presents before the age of 5 years [3, 4]. Adults may also be affected, albeit very rarely [4–7]. The syndrome is uncommon; the largest series reported to date is of 48 cases  and only 11 of 510 patients operated on at one centre for intractable epilepsy between 1974 and 1992 had clinical and pathological findings consistent with the diagnosis . However, these may be underestimates . The finding of focal encephalitic changes in 5–10% of patients undergoing temporal lobectomy for intractable complex partial seizures  may represent a non-progressive variant of RS.
Prognosis is poor, for although the neurological deficit usually stabilizes ultimately, seizures may continue, necessitating surgical intervention. Functionally complete but anatomically subtotal hemispherectomy is the operation of choice [10, 11] early surgery in children to pre-empt intellectual decline has been advocated . Although the syndrome is uncommon, it merits attention because of the significant handicap suffered by affected individuals.
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Rasmussen T, Olszewski J, Lloyd-Smith D (1958) Focal seizures due to chronic localized encephalitis. Neurology 8:435–445
Robitaille Y (1991) Neuropathologic aspects of chronic encephalitis. In: Andermann F (ed) Chronic encephalitis and epilepsy: Rasmussen's syndrome. Butterworth-Heinemann, Boston, pp 79–110
Piatt JH, Hwang PA, Armstrong DC, Becker LE, Hoffman HJ (1988) Chronic focal encephalitis (Rasmussen's syndrome): six cases. Epilepsia 29:268–279
Oguni H, Andermann F, Rasmussen TB (1991) The natural history of the syndrome of chronic encephalitis and epilepsy: a study of the MNI series of forty-eight cases In: Anderman F (ed) Chronic encephalitis and epilepsy: Rasmussen's syndrome. Butterworth-Heinemann, Boston, pp 7–35
Gray F, Serdaru M, Baron H, Daumas-Duport C, Loron P, Sauron B, Poirier J (1987) Chronic localized encephalitis (Rasmussen's) in an adult with epilepsia partialis continua. J Neurol Neurosurg Psychiatry 50:747–751
McLachlan RS, Girvin JP, Blume WT, Reichman H (1993) Rasmussen's chronic encephalitis in adults. Arch Neurol 50:269–274
Larner AJ, Smith SJM, Duncan JS, Howard RS (1995) Late-onset Rasmussen's syndrome with first seizure during pregnancy. Eur Neurol (in press)
Rasmussen TB (1991) Chronic encephalitis and seizures: historical introduction. In: Andermann F (ed) Chronic encephalitis and epilepsy. Rasmussen's syndrome. Butterworth-Heinemann, Boston, pp 1–4
Laxer KD (1991) Temporal lobe epilepsy with inflammatory pathologic changes. In: Andermann F (ed) Chronic encephalitis in epilepsy: Rasmussen's syndrome. Butterworth-Heinemann, Boston, pp 135–140
Rasmussen T (1983) Hemispherectomy for seizures revisited. Can J Neurol Sci 10:71–78
Tinuper P, Andermann F, Villemure JG, Rasmussen T, Quesney LF (1988) Functional hemispherectomy for treatment of epilepsy associated with hemiplegia: rationale, indications, results, and comparison with callosotomy. Ann Neurol 24:27–34
Vining EPG, Freeman JM, Brandy J, Carson BS, Uematsu S (1993) Progressive unilateral encephalopathy of childhood (Rasmussen's syndrome): a reappraisal. Epilepsia 34:639–650
Anderson M (1993) Virus infections of the nervous system. In: Walton J (ed) Brain's diseases of the nervous system, 10th edn. Oxford University Press, Oxford, pp 317–350
Friedman H, Ch'ien L, Parham D (1977) Virus in brain of child with hemiplegia, hemiconvulsions, and epilepsy. Lancet 11: 666
Mizuno Y, Chou SM, Estes ML, Erenberg G, Cruse RP, Rothner AD (1985) Chronic localized encephalitis (Rasmussen's) with focal cerebral seizures (abstract). J Neuropathol Exp Neurol 44:351
Harvey AS, Andermann F, Hopkins IJ, Kirkham TH, Berkovic SF (1992) Chronic encephalitis (Rasmussen's syndrome) and ipsilateral uveitis. Ann Neurol 32:826–829
Laxer KD, Gilden DH, Burkhardt C, Vafai A (1986) Detection of herpes simplex virus nucleic acid in human cortex from patients with complex partial seizures (abstract). Epilepsia 27:594
Walter GF, Renella RR (1989) Epstein-Barr virus in brain and Rasmussen's encephalitis. Lancet 1:279–280
Walter GF, Renella RR, Hori A, Wirnsberger G (1989) Nachweis von Epstein-Barr-Viren bei Rasmussen's Encephalitis. Bericht über zwei Fälle. Nervenarzt 60:168–170
Power C, Poland SD, Blume WT, Girvin JP, Rice GPA (1990) Cytomegalovirus and Rasmussen's encephalitis. Lancet 336:1282–1284
Vinters HV, Wang R, Wiley CA (1993) Herpesviruses in chronic encephalitis associated with intractable childhood epilepsy. Hum Pathol 24:871–879
Maria BL, Ringdahl DM, Mickle JP, Smith LJ, Reuman PD, Gilmore RL, Drane WE, Quisling RG (1993) Intraventricular alpha interferon therapy for Rasmussen's syndrome. Can J Neurol Sci 20:333–336
Rasmussen T, Andermann F (1989) Update on the syndrome of “chronic encephalitis” and epilepsy. Cleve Clin J Med 56 [Suppl, part 2]: S181-S184
Ragazzo PC, Zanini LA, Cendes F, deAzevedo L (1991) Control of continuous partial motor seizures in Rasmussen's encephalitis during therapy with acyclovir (abstract). Epilepsia 32 [Suppl 1]:91
DeToledo JC, Smith DB (1994) Partially successful treatment of Rasmussen's encephalitis with zidovudine: symptomatic improvement followed by involvement of the contralateral hemisphere. Epilepsia 35:352–355
Lyon G, Griscelli C, Fernandez-Alvarez E, Prats-Vinas J, Lebon P (1980) Chronic progressive encephalitis in children with X-linked hypogamma-globulinemia. Neuropaediatrie 11:57–71
Grenier Y, Antel JP, Osterland CK (1991) Immunologic studies in chronic encephalitis of Rasmussen. In: Andermann F (ed) Chronic encephalitis and epilepsy: Rasmussen's syndrome. Butterworth-Heinemann, Boston, pp 125–134
Andrews JM, Thompson JA, Pysher TJ, Walker ML, Hammond ME (1990) Chronic encephalitis, epilepsy, and cerebrovascular immune complex deposits. Ann Neurol 28:88–90
Dulac O, Robain O, Chiron C, Plouin P, Pinel JF, Vigevano F, Raynaud C, Ponsot G (1991) High-dose steroid treatment of epilepsia partialis continua due to chronic focal encephalitis. In: Andermann F (ed) Chronic encephalitis and epilepsy: Rasmussen's syndrome. Butterworth-Heinemann, Boston, pp 193–199
Walsh PJ (1991) Treatment of Rasmussen's syndrome with intravenous gammaglobulin. In: Andermann F (ed) Chronic encephalitis and epilepsy: Rasmussen's syndrome. Butterworth-Heinemann, Boston, pp 201–204
Rogers SW, Andrews PI, Gahring LC, Whisenand T, Cauley K, Crain B, Hughes TE, Heinemann SF, McNamara JO (1994) Autoantibodies to glutamate receptor GluR3 in Rasmussen's encephalitis. Science 265:648–651
Hollmann M, Heinemann S (1994) Cloned glutamate receptors. Annu Rev Neurosci 17:31–108
Larner AJ (1993) Recent advances in the understanding of the immunological basis of peripheral neuropathies. Br J Clin Pract 47:262–265
Larner AJ (1995) Axonal sprouting and synaptogenesis in temporal lobe epilepsy: possible pathogenetic and therapeutic roles of neurite growth-inhibitory factors. Seizure (in press)
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Larner, A.J., Anderson, M. Rasmussen's syndrome: pathogenetic theories and therapeutic strategies. J Neurol 242, 355–358 (1995). https://doi.org/10.1007/BF00868389
- Rasmussen's syndrome
- Chronic encephalitis