Epilepsia, 44(6):870–871, 2003Blackwell Publishing, Inc.C© 2003 International League Against Epilepsy

Original Letter

Late Language Transfer in Patients with Rasmussen Encephalitis

∗Tobias Loddenkemper, ∗Elaine Wyllie, ∗David Lardizabal, †Lisa D. Stanford, and‡William Bingaman

Departments of ∗Neurology, †Neuropsychology, and ‡Neurosurgery, The Cleveland Clinic Foundation,Cleveland, Ohio, U.S.A.

Language can transfer to the right hemisphere in pa-tients with early left-sided brain injury (1,2). Rare caseswith acquired left hemisphere lesions and language trans-fer after age 9 years have been reported (1,3,4). The pos-sibility of language transfer in adolescent patients mayinfluence decision making in epilepsy surgery and mayalter concepts of language development and plasticity.

We report two adolescents with initially left-hemispheric language dominance proven by intracarotidamobarbital testing (IAT) who developed right-sided lan-guage between 9 and 15 years and between 12 and 14years, respectively. Language was impaired in both pa-tients because of Rasmussen chronic encephalitis begin-ning at 8 and 11 years, with improvement after left hemi-spherectomy at ages 14 and 15 years.

Case one, a right-handed 8-year-old boy, had phar-macologically intractable right clonic seizures associatedwith left frontocentral EEG seizures. Bilateral IAT atage 9 revealed left hemisphere language dominance. Re-peated magnetic resonance imaging (MRI) showed pro-gressive left hemispheric atrophy and increased left tem-porooccipital signal on T2 sequences. At age 15 years,right-sided epilepsia partialis continua and hemiparesisdeveloped. Handedness shifted from right to left. A re-peated IAT at that time showed right-sided language rep-resentation. Left functional hemispherectomy was per-formed. Histopathology was consistent with Rasmussenencephalitis. Postoperatively, no impaired language func-tion was noted. Rare right facial clonic seizures were wellcontrolled. Neuropsychological testing showed languageimprovement as compared with presurgical assessment(Table 1).

Case two, a right-handed 11-year-old girl, had rightclonic seizures and left frontocentral EEG seizures. Re-

Accepted February 8, 2003.Address corresponding and reprint requests to Dr. T. Loddenkemper

at Department of Epilepsy and Sleep Disorders, The Cleveland ClinicFoundation, 9500 Euclid Ave, S-51, Cleveland, OH 44195, U.S.A.E-mail: loddent@ccf.org

peated MRIs demonstrated progressive left-hemisphericvolume loss and hyperintensity on T2-weighted images.IAT at 12 years and 8 months showed left hemisphere lan-guage. Handedness shifted from right to left. The familyinitially declined left hemispherectomy because of con-cerns regarding language function. Steroids and plasma-pheresis were tried instead, without effect. She was read-mitted at age 13 years and 10 months with right-sidedepilepsia partialis continua, dysphasia, right hemipare-sis, dehydration, and inability to swallow. Immediateleft functional hemispherectomy led to seizure freedom.Histopathology was typical of Rasmussen encephalitis.Postoperatively, language was slow, but she could com-municate. Neuropsychological assessment demonstratedimproved verbal performance as compared with preoper-ative assessment (Table 1).

Only seven patients with Rasmussen encephalitis havebeen reported with apparent interhemispheric transfer oflanguage after age 9 years. Transfer was assessed byneuropsychological means, showing initial language dete-rioration after hemispherectomy and progressive improve-ment over time in five patients (1), by late IAT show-ing right hemisphere language dominance at the time ofsurgery (4), and by functional MRI (fMRI) in one pa-tient (3).We add the first patients with late language trans-fer, who had initial left hemisphere language dominanceproven by IAT early in the illness.

Dominant hemispherectomy after age 9 years in pa-tients with Rasmussen chronic encephalitis does notnecessarily produce lasting aphasia, even if IAT ini-tially demonstrated left language dominance. Early hemi-spherectomy also may prevent impending bilateral hemi-spheric involvement of the disease after prolongedduration (5) and early death due to uncontrolled seizureswith epilepsia partialis continua and generalized statusepilepticus (6).

Acknowledgment: T.L. was supported by Innovative Medi-zinische Forschung, WWU Munster (FoeKz. LO 610101) and

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LATE LANGUAGE TRANSFER 871

TABLE 1. Neuropsychological test results in two patients with Rasmussen encephalitis

Wechsler Intelligence Scale for Children–Third edition (WISC III)

Case 1 Case 2

Boy (operation at age 15 yr 3 mo) Girl (operation at age 13 yr 11 mo)

Preoperative Postoperative Preoperative Postoperative

Age at test 9 yr 5 mo 12 yr 1 mo 15 yr 3 mo 15 yr 9 mo 12 yr 11 mo 14 yr 2 mo 14 yr 5 moVerbal IQ 87 82 45 69 65 74 80Performance IQ 110 84 47 79 72 80 81Full-scale IQ 97 82 42 71 66 75 78Verbal comprehension 88 85 50 75 65 76 84Perceptual organization 120 91 50 89 80 90 91

IQ, intelligence quotient.

NRW-Nachwuchsgruppe Kn2000, Federal Ministry of Educa-tion and Research (Foe.1KS9604/0), Interdisciplinary Center ofClinical Research Munster (IZKF Project NWG2).

REFERENCES

1. Boatman D, Freeman J, Vining E, et al. Language recovery after lefthemispherectomy in children with late-onset seizures. Ann. Neurol1999;46:579–86.

2. Satz P, Strauss E, Wada J, et al. Some correlates of intra- and in-terhemispheric speech organization after left focal brain injury. Neu-ropsychologia 1988;26:345–50.

3. Hertz-Pannier L, Chiron C, Jambaque I, et al. Late plasticity for lan-guage in a child’s non-dominant hemisphere: a pre- and post-surgeryfMRI study. Brain 2002;125:361–72.

4. Telfeian AE, Berqvist C, Danielak C, et al. Recovery of languageafter left hemispherectomy in a sixteen-year-old girl with late-onsetseizures. Pediatr Neurosurg 2002;37:19–21.

5. Chinchilla D, Dulac O, Robain O, et al. Reappraisal of Rasmussen’ssyndrome with special emphasis on treatment with high doses ofsteroids. J Neurol Neurosurg Psychiatry 1994;57:1325–33.

6. Oguni H, Andermann F, Rasmussen TB. The natural history of thesyndrome of chronic encephalitis and epilepsy: a study of the MNI se-ries of forty-eight cases. In: Andermann F, ed. Chronic encephalitisand epilepsy: Rasmussen’s syndrome. Boston: Butterworth Heine-mann, 1991:7–21.

Epilepsia, Vol. 44, No. 6, 2003