RASMUSSEN´S ENCEPHALITIS. CASE REPORT.

Encefalitis de Rasmussen

INTRODUCTION: Rasmussen's encephalitis is a rare, chronic, progressive, inflammatory, neurodegenerative disease that affects a single cerebral hemisphere and usually appears during childhood. A case of Rasmussen's encephalitis is presented.

CASE PRESENTATION: A 4-year-old female patient presented with focal tonic-clonic seizures with loss of left hemibody strength. Patient was afebrile, conscious, oriented, but with slow cognitive development and slurred speech. Left facial and upper motor paralysis was found. Neurological examination showed complete left hemiparesis with global aphasia, muscle atrophy, spasticity, dystonia and ipsilateral Babinski's sign. MRI demonstrated diffuse atrophy of the right cerebral hemisphere with ipsilateral ventricular dilatation and prominence of the Sylvian fissure. The findings were consistent with the diagnosis of Rasmussen's encephalitis.

DISCUSSION: Rasmussen's encephalitis is characterized by progressive unilateral focal neurologic deterioration and drug-refractory focal seizures, associated with hemiparesis, cognitive dysfunction, and hemispheric atrophy. Refractory continuous partial epilepsy is the common denominator of the disease. Diagnosis is based on clinical, electroencephalographic and neuroimaging features. MRI may demonstrate focal or unihemispheric involvement, excluding other pathologies. Various immunomodulatory treatments can slow, but not stop, disease progression. The treatment of choice is surgical hemispheric disconnection to decrease neurological deficits.

CONCLUSION: Rasmussen's encephalitis is of unknown etiology. Its variable presentations make accurate diagnosis and timely treatment difficult.

1. Yang Z, Sun G. Headache maybe the initial symptom in Rasmussen's syndrome: A child case report. Epilepsy Behav Case Rep. 2016;6:75-77. doi: 10.1016/j.ebcr.2016.09.001.
2. Noordin NS, Deyo LJ, Ryon CW, Anderson WT. Atypical presentation of cerebral palsy and seizures: A case report on Rasmussen's encephalitis in an adolescent. Cureus. 2021;13(3):e13705. doi: 10.7759/cureus.13705.
3. Hammed A, Badour M, Baqla S, Amer F. Diagnosis and treatment of Rasmussen's encephalitis pose a big challenge: Two case reports and literature review. Ann Med Surg (Lond). 2021;68:102606. doi: 10.1016/j.amsu.2021.102606.
4. Tang C, Luan G, Li T. Rasmussen's encephalitis: mechanisms update and potential therapy target. Ther Adv Chronic Dis. 2020;11:2040622320971413. doi: 10.1177/2040622320971413.
5. Pinto AL, Lohani S, Bergin AM, Bourgeois BF, Black PM, Prabhu SP, Madsen JR, Takeoka M, Poduri A. Surgery for intractable epilepsy due to unilateral brain disease: a retrospective study comparing hemispherectomy techniques. Pediatr Neurol. 2014;51(3):336-43. doi: 10.1016/j.pediatrneurol.2014.05.018.
6. Kebir H, Carmant L, Fontaine F, Béland K, Bosoi CM, Sanon NT, Alvarez JI, Desgent S, Pittet CL, Hébert D, Langlois MJ, Rébillard RM, Nguyen DK, Cieuta-Walti C, Holmes GL, Goodkin HP, Mytinger JR, Connolly MB, Prat A, Haddad E. Humanized mouse model of Rasmussen's encephalitis supports the immune-mediated hypothesis. J Clin Invest. 2018;128(5):2000-2009. doi: 10.1172/JCI97098.
7. Liu L, Yu J, Li L, Zhang B, Liu L, Wu CH, Jong A, Mao DA, Huang SH. Alpha7 nicotinic acetylcholine receptor is required for amyloid pathology in brain endothelial cells induced by Glycoprotein 120, methamphetamine and nicotine. Sci Rep. 2017;7:40467. doi: 10.1038/srep40467.
8. Gataullina S, Dulac O. Rassmussen's encephalitis: are there new, better therapeutic medical and surgical guidelines? Epilepsia. 2010;51 Suppl 1(suppl 5):92-3. doi: 10.1111/j.1528-1167.2009.02459.x.
9. Hammed A, Badour M, Baqla S, Amer F. Diagnosis and treatment of Rasmussen's encephalitis pose a big challenge: Two case reports and literature review. Ann Med Surg (Lond). 2021;68:102606. doi: 10.1016/j.amsu.2021.102606.
10. Assogba K, Apetse KM, Waklatsi KP, Douti K, Kombate D, Balogou KA. Rasmussen's epileptogenic encephalitis in a tropical country. J Pediatr Neurosci. 2018;13(4):490-495. doi: 10.4103/JPN.JPN_51_18.
11. Ai J, Wang Y, Liu D, Fan D, Wang Q, Li T, Luan G, Wang P, An J. Genetic factors in Rasmussen's encephalitis characterized by whole-exome sequencing. Front Neurosci. 2021;15:744429. doi: 10.3389/fnins.2021.744429.
12. Klaa H, Ben Younes T, Benrhouma H, Nagi S, Rouissi A, Kraoua I, Ben Youssef-Turki I. Rasmussen's encephalitis: A report of a Tunisian pediatric case and literature review. Case Rep Neurol Med. 2020;2020:6810237. doi: 10.1155/2020/6810237.
13. Bauerschmidt A, Rubinos C, Claassen J. Approach to managing periodic discharges. J Clin Neurophysiol. 2018;35(4):309-313. doi: 10.1097/WNP.0000000000000464.
14. Ravindra VM, Mazur MD, Mohila CA, Sweney MT, Hersh A, Bollo RJ. Rasmussen encephalitis with dual pathology in a patient without seizures: case report and literature review. Childs Nerv Syst. 2015;31(11):2165-71. doi: 10.1007/s00381-015-2757-8.
15. Li TR, Zhang YD, Wang Q, Shao XQ, Li ZM, Lv RJ. Intravenous methylprednisolone or immunoglobulin for anti-glutamic acid decarboxylase 65 antibody autoimmune encephalitis: which is better? BMC Neurosci. 2020;21(1):13. doi: 10.1186/s12868-020-00561-9.