Rasmussen encephalitis (RE), also known as chronic focal encephalitis (not to be confused with a Rasmussen aneurysm), is a chronic inflammatory disease of unknown origin, usually affecting one brain hemisphere.
Most cases (85% cases) occur in children under the age of 10 years 1. However, detection in adults is increasing with routine MRI investigations for intractable seizures 5.
Patients frequently have episodes of epilepsia partialis continua or generalised status epilepticus, although this is less common. The seizures are intractable despite aggressive medical management 1.
Apart from seizures, patient may have hemiparesis and speech disturbances, as well as hemianopia (pertaining to unilateral cerebral involvement). Mental deterioration over time may also be seen, especially in patient presenting later in adolescence.
Pathologic features are similar to viral encephalitis, with lymphocytes around round cells and diffuse proliferation of microglia. However, later spongiform degeneration and cortical atrophy sets in.
The observed inflammatory changes in RE include perivascular cuffing, microglial nodules, T-lymphocytic infiltration gliosis, meningeal inflammation, and neuronal injury or loss 9,10.
An autoimmune mechanism has also been proposed describing antibodies against GluR3 subunit of the α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor in a few patients 11.
The exact cause of the disease is unknown. However, various viral (SSPE-like, EBV or CMV) or inflammatory episodes have been implicated by different authors 3,4,6.
Radiographic features are usually isolated to a single hemisphere, however bilateral Rasmussen encephalitis has also been rarely described in numerous case reports 12-14.
CT may not show any specific feature in early imaging; however, patchy hypodense attenuation areas (similar to viral encephalitis) may be seen. Late stage disease may show unilateral cortical atrophy. CT perfusion may show decreased cerebral blood flow (CBF) 1.
- T1: unilateral cortical atrophy with ex-vacuo ventricular dilatation 15
- T2: hyperintense signal areas in the affected hemisphere 15
- DWI/ADC: restricted diffusion may be seen in altered signal areas 15
- T1 C+ (Gd): no significant post-contrast enhancement 15
Treatment and prognosis
Treatment with high-dose methylprednisolone and intravenous immunoglobulin 16 has been successful, further supporting the autoimmune nature of the disease.
Functional hemispherectomy is the only definitive treatment in refractory cases, with most patients having either no or less frequent seizures 16.
History and etymology
It was first described by American neurologist Theodore Brown Rasmussen (1910-2002) in 1958 2.
General imaging differential considerations include:
- 1. Tien RD, Ashdown BC, Lewis DV et-al. Rasmussen's encephalitis: neuroimaging findings in four patients. AJR Am J Roentgenol. 1992;158 (6): 1329-32. AJR Am J Roentgenol (abstract) - Pubmed citation
- 2. Rasmussen T, Olszewski J, Lloyd Smith D. Focal seizures due to chronic localized encephalitis. Neurology. 2000;8 (6): 435-45. Pubmed citation
- 3. Rasmussen T, Andermann F. Update on the syndrome of "chronic encephalitis" and epilepsy. Cleve Clin J Med. 1989;56 Suppl Pt 2 : S181-4. - Pubmed citation
- 4. Farrell MA, Cheng L, Cornford ME et-al. Cytomegalovirus and Rasmussen's encephalitis. Lancet. 1991;337 (8756): 1551-2. Lancet (link) - Pubmed citation
- 5. Mclachlan RS, Girvin JP, Blume WT et-al. Rasmussen's chronic encephalitis in adults. Arch. Neurol. 1993;50 (3): 269-74. Arch. Neurol. (link) - Pubmed citation
- 6. Atkins MR, Terrell W, Hulette CM. Rasmussen's syndrome: a study of potential viral etiology. Clin. Neuropathol. 14 (1): 7-12. - Pubmed citation
- 7. Oguni H, Andermann F, Rasmussen TB. The syndrome of chronic encephalitis and epilepsy. A study based on the MNI series of 48 cases. Adv Neurol. 1992;57: 419-33. Pubmed citation
- 8. Sethi NK, Tenney JR. Child Neurology: Hemiconvulsion-hemiplegia-epilepsy syndrome. Neurology. 2012;79 (24): 2367. doi:10.1212/01.wnl.0000424157.07211.bf - Pubmed citation
- 9. Rasmussen T, Olszewski J, Lloydsmith D. Focal seizures due to chronic localized encephalitis. Neurology. 2000;8 (6): 435-45. Pubmed citation
- 10. Bien CG, Bauer J, Deckwerth TL et-al. Destruction of neurons by cytotoxic T cells: a new pathogenic mechanism in Rasmussen's encephalitis. Ann. Neurol. 2002;51 (3): 311-8. Pubmed citation
- 11. Rogers SW, Andrews PI, Gahring LC et-al. Autoantibodies to glutamate receptor GluR3 in Rasmussen's encephalitis. Science. 1994;265 (5172): 648-51. Pubmed citation
- 12. Guan Y, Luan G, Zhou J, Liu X. Bilateral Rasmussen encephalitis. Epilepsy & behavior : E&B. 20 (2): 398-403. doi:10.1016/j.yebeh.2010.12.004 - Pubmed
- 13. Tobias SM, Robitaille Y, Hickey WF, Rhodes CH, Nordgren R, Andermann F. Bilateral Rasmussen encephalitis: postmortem documentation in a five-year-old. Epilepsia. 44 (1): 127-30. Pubmed
- 14. Peariso K, Standridge SM, Hallinan BE, Leach JL, Miles L, Mangano FT, Greiner HM. Presentation, diagnosis and treatment of bilateral Rasmussen's encephalitis in a 12-year-old female. Epileptic disorders : international epilepsy journal with videotape. 15 (3): 324-32. doi:10.1684/epd.2013.0594 - Pubmed
- 15. Faria AV, Reis F, Dabus GC, Zanardi VA, Guerreiro MM, Cendes F. MRI findings in the diagnosis and monitoring of Rasmussen's encephalitis. Arquivos de neuro-psiquiatria. 67 (3B): 792-7. Pubmed
- 16. Bien CG, Granata T, Antozzi C et-al. Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: a European consensus statement. Brain. 2005;128 (3): 454-71. doi:10.1093/brain/awh415 - Pubmed citation
Infections of the central nervous system
- classification by aetiology
- eastern equine encephalitis
- enterovirus rhomboencephalitis
- flavivirus encephalitis
herpes virus family
- herpes simplex virus 1 (HSV-1) encephalitis
- herpes simplex virus 2 (HSV-2) encephalitis
- varicella zoster virus (VZV) encephalitis
- Epstein-Barr virus (EBV) encephalitis
- cytomegalovirus (CMV) encephalitis
- human herpesvirus 6 (HHV-6) encephalitis
- HIV CNS manifestations
- HTLV-1-associated myelopathy
- JC virus
- measles encephalitis
- Nipah virus (NiV) encephalitis
- rabies encephalitis
- CNS listeriosis (Listeria monocytogenes)
- CNS nocardiosis (Nocardia spp)
- CNS tuberculosis (Mycobacterium tuberculosis)
- Lyme disease (Borrelia burgdorferi)
- neurosyphilis (Treponema pallidum)
- Rocky Mountain spotted fever (Rickettsia rickettsii)
- cerebral amoebiasis
- cerebral malaria (Plasmodium falciparum)
- cerebral sparganosis (Spirometra mansonoides)
- neurocysticercosis (Taenia solium)
- neurohydatidosis (Echinococcus spp)
- neurotoxoplasmosis (Toxoplasma gondii)
- others or those with possible infectious aetiologies
- classification by location
- meninges and ventricular system
- brain parenchyma, brainstem, and spinal cord
- classification by aetiology