Title: Rasmussen's Encephalitis - Chronic Focal Encephalitis (CFE) - A Rare Entity

Authors: Dr Surender Kumar, Dr Hitender Kumar

 DOI:  https://dx.doi.org/10.18535/jmscr/v5i3.145

Abstract

Rasmussen's encephalitis, also known as chronic focal encephalitis (CFE), is a rare inflammatory neurological disease, characterized by frequent and severe seizures, loss of motorskills and speech, hemiparesis (paralysis on one side of the body), encephalitis (inflammation of the brain), and dementia. The illness affects a single cerebral hemisphere and generally occurs in children under the age of 10.

Advances in neuroimaging suggest that progression of the inflammatory process is seen and MRI might be a good biomarker in Rasmussen’s encephalitis.

Cerebral hemi-spherectomy remains the only cure for seizures, but there are inevitable functional compromises. Decisions of whether or when surgery should be undertaken are challenging in the absence of a dense neurological deficit, and vary by institutional experience. Further, the optimum time for surgery, to give the best language and cognitive outcome, is not yet well understood. Immuno-modulatory treatments seem to slow rather than halt disease progression in Rasmussen’s encephalitis, without changing the eventual outcome.

Keywords: Encephalitis, seizures, hemiparesis, dementia.

References

1.      Tien RD, Ashown BC, Lewis DV et al. Rasmussen encephalitis : neuroimaging findings in 4 patients. AJR Am J Roentgenol. 1992;158(6): 1329-32.

2.      Mclachlan RS, Girvin JP, Blume WT et al. Rasmussen chronic encephalitis in adults. Arch. Neurol. 1993;50 (3): 269-74.

3.      Rasmussen T, Olszewski J, Lloyd SD. Focal seizure due to chronic localized enc-ephalitis. Neurology. 200;8 (6: 435-45).

4.      Bien CG, Granata T, Antozzi C, et al. Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: a European cons-ensus statement. Brain. 2005;128: 454–71.

5.      3. Olson HE, Lechpammer M, Prabhu SP, et al. Clinical application and evaluation of the Bien diagnostic criteria for Rasmussen encephalitis. Epilepsia. 2013;54:1753–60.

6.      Bien CG, Tiemeier H, Sassen R, et al. Rasmussen encephalitis: Incidence and course under randomized therapy with tacrolimus or intravenous immunoglob-ulins. Epilepsia. 2013;54:543–50.

7.      Lamb K, Scott WJ, Mensah A, et al. Prevalence and clinical outcome of Rasmussen encephalitis in children from the United Kingdom. Dev Med Child Neurol. 2013;55(suppl 1):14.

8.      Bien CG, Widman G, Urbach H, et al. The natural history of Rasmussen’s encephalitis. Brain.2002;125:1751–59.

9.      Oguni H, Andermann F, Rasmussen TB. The natural history of the syndrome of chronic encephalitis and epilepsy: a study of the MNI series of forty-eight cases. In: Andermann F, editor. Chronic encephalitis and epilepsy–Rasmussen’s syndrome.  Boston: Butterworth-Heinemann; 1991. pp. 7–35.

10.  Granata T, Gobbi G, Spreafico R, et al. Rasmussen’s encephalitis: early charact-eristics allow diagnosis. Neurology. 2003; 60:422–25.

11.  Thomas JE, Reagan TJ, Klass DW. Epilepsiapartialis continua. A review of 32 cases. Arch Neurol.1977;34:266–75.

12.  Obeso JA, Rothwell JC, Marsden CD. The spectrum of cortical myoclonus. From focal reflex jerks to spontaneous motor epilepsy. Brain. 1985;108:193–24.

13.  Longaretti F, Dunkley C, Varadkar S, et al. Evolution of the EEG in children with Rasmussen’s syndrome. Epilepsia. 2012; 53:1539–45.

14.  Bien CG, Gleissner U, Sassen R, et al. An open study of tacrolimus therapy in Rasmussen encephalitis. Neurology.  2004;62:2106–09.

15.  Thilo B, Stingele R, Knudsen K, et al. A case of Rasmussen encephalitis treated with rituximab. Nat Rev Neurol. 2009; 5:458–62.

16.  Grenier Y, Antel JP, Osterland CK. Immunologic studies in chronic encephalitis of Rasmussen. In: Andermann F, editor. Chronic encephalitis and epilepsy. Rasmussen's syndrome. Boston: Butterworth-Heinemann; 1991. p. 125–34.

17.  Rasmussen T, Andermann F. Update on the syndrome of "chronic encephalitis" and epilepsy. Cleve Clin J Med. 1989;56SupplPt 2 : S181-4.

18.  Farrell MA, Cheng L, Cornford ME et-al. Cytomegalovirus and Rasmussen's enceph-alitis. Lancet. 1991;337 (8756): 1551-2.

19.  Friedman H, Ch'ien L, Parham D. Virus in brain of child with hemiplegia, hemiconv-ulsions, and epilepsy. Lancet 1977; 2: 666.

20.  Rogers SW, Andrews PI, Gahring LC et-al. Autoantibodies to glutamate receptor GluR3 in Rasmussen's encephalitis. Science. 1994;265 (5172): 648-51.

21.  Bien CG, Urbach H, Deckert M, et al. Diagnosis and staging of Rasmussen’s encephalitis by serial MRI and histopat-hology. Neurology. 2002;58:250–57.

22.  Chiapparini L, Granata T, Farina L, et al. Diagnostic imaging in 13 cases of Rasmussen’s encephalitis: can early MRI suggest the diagnosis?  Neuroradiology.  2003;45:171–83.

23.  Yamazaki E, Takahashi Y, Akasaka N, Fujiwara T, Inoue Y. Temporal changes in brain MRI findings in Rasmussen syndrome. Epileptic Disord. 2011;13:229–39.

24.  Wagner J, Schoene-Bake JC, Bien CG, et al. Automated 3D MRI volumetry reveals regional atrophy differences in Rasmussen encephalitis. Epilepsia. 2012;53:613–21.

25.  Larionov S, Konig R, Urbach H, et al. MRI brain volumetry in Rasmussen encephalitis: the fate of affected and “unaffected” hemispheres. Neurology. 2005;64:885–87.

26.  Fiorella DJ, Provenzale JM, Edward CR, Crain BJ, Al Sugair A. 18F-fluorodeox-yglucose positron emission tomography and MR imaging findings in Rasmussen encephalitis. Am J Neuroradiol. 2001;22:1291–99.

27.  Varadkar S, Bien CG, Kruse CA, Jensen FE, Bauer J, Pardo CA, Vincent A, Mathern GW, Cross JH (2014).

28.  Bien CG, Schramm J. Treatment of Rasmussen encephalitis half a century after its initial description: promising prospects and a dilemma. Epilepsy Res.  2009;86:101–12.

29.  Thomas SG, Chacko AG, Thomas MM, et al. Outcomes of disconnective surgery in intractable pediatric hemispheric and subhemispheric epilepsy. Int J Pediatr.  2012;2012:527891.

30.  Althausen A, Gleissner U, Hoppe C, et al. Long-term outcome of hemispheric surgery at different ages in 61 epilepsy patients. J NeurolNeurosurg Psychiatry.  2013;84:529–36.

31.  Vora N, Cross JH, Robinson R, et al. Epilepsy Surgery in Rasmussen Syndr-ome. Epilepsy Currents.2012;12:296.

32.  Takahashi Y, Yamazaki E, Mine J, et al. Immunomodulatory therapy versus surgery for Rasmussen syndrome in early childhood. Brain Devel. 2013;35:778–85.

33.  Vining EP, Freeman JM, Pillas DJ, Uematsu S, Carson BS, Brandt J, et al. Why would you remove half a brain? The outcome of 58 children after hemisphere-ctomy-the Johns Hopkins experience: 1968 to 1996.  Pediatrics 1997; 100: 163–71.

34.  Villemure J-G, Andermann F, Rasmussen TB. Hemispherectomy for the treatment of epilepsy due to chronic encephalitis. In: Andermann F, editor. Chronic encephalitis and epilepsy: Rasmussen's syndrome. Boston: Butterworth-Heinemann; 1991. p. 235–41.

35.  T. Rasmussen, J. Olszweski, and D. Lloyd-Smith, “Focal seizures due to chronic localized encephalitis,” Neurology, vol. 8, pp. 435–445, 1958.

36.  C. A. Pardo, E. P. G. Vining, L. Guo, R. L. Skolasky, B. S. Carson, and J. M. Freeman, “The pathology of rasmussen syndrome: stages of cortical involvement and neuropathological studies in 45 hemispherectomies,” Epilepsia, vol. 45, no. 5, pp. 516–526, 2004.

37.  C. G. Bien, T. Granata, C. Antozzi et al., “Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: a European consensus statement,” Brain, vol. 128, no. 3, pp. 454–471, 2005.

Corresponding Author

Dr Surender Kumar

MD Medicine, Senior Resident, Department of Cardiology

IGMC Shimla, Himachal Pradesh, India