Granulomatous amoebic encephalitis (GAE), also referred to as cerebral amoebiasis, is a rare and usually fatal subacute-chronic CNS infection in immunocompromised patients caused by free-living amoebae such as Acanthamoeba spp (main cause), Balamuthia mandrillaris and Sappinia pedata.
This is a distinct clinical syndrome to primary amoebic meningoencephalitis, which is an acute illness that presents like a severe bacterial meningoencephalitis and is caused by Naegleria fowleri.
GAE has a gradual and highly variable subacute-chronic course over weeks and months 1-6. Patients with GAE have been described with symptoms that include: headache, low fever, seizures, hemiparesis, and coma leading to eventual death 2. This presentation is often more aggressive in cases due to Balamuthia mandrillaris when compared to cases due to Acanthamoeba spp 4.
It has been posited that the skin and olfactory neuroepithelium serve as portals of entry, and occasionally, an inflammatory process may indeed be observed at these primary foci 5. Skin lesions, such as plaques over the face, may especially be prominent in patients with GAE due to Balamuthia mandrillaris 4.
Furthermore, as GAE commonly coincides with other CNS infectious diseases, the clinical presentation could be even more variable due the other associated comorbidities.
Although lumbar puncture is generally contraindicated for patients who present with a focal intracranial lesion, when CSF can be obtained, the following may be seen 3:
- pressure slightly elevated
- presence of lymphocytes and plasma cells
- glucose levels are usually low
- moderately high protein levels
- PCR of the cerebrospinal fluid can be diagnostic
Multifocal parenchymal lesions characterised by foci of chronic granulomatous inflammation centred around arteries and veins and also infiltrating the meninges 1,2. Trophozoites and cysts may be found in and around walls of affected blood vessels and also in areas relatively free of inflammation 1,2.
Imaging findings are nonspecific and are rarely described in the literature, but can include the following brain lesions 6:
- multiple parenchymal lesions
- pseudotumoural lesions
- meningeal exudates
- haemorrhagic infarcts (more common in Balamuthia mandrillaris)
- necrosis (more common in Balamuthia mandrillaris)
Treatment and prognosis
The approach to treatment is uncertain and is based on case report-level evidence 6,7. A variety of antifungal and antiparasitic agents may be trialled in combination 6,7. Neurosurgical intervention may also be sought 6.
- 1. Ellison D, Love S, Chimelli LMC et-al. Neuropathology. Elsevier Health Sciences. (2012) ISBN:0723437467. Read it at Google Books - Find it at Amazon
- 2. Lu D, Luo L, Xu Q et-al. A clinico-pathological study of granulomatous amoebic encephalitis. Zhonghua Bing Li Xue Za Zhi. 2003;28 (3): 169-73. Pubmed citation
- 3. Solaymani-Mohammadi S, Lam MM, Zunt JR et-al. Entamoeba histolytica encephalitis diagnosed by PCR of cerebrospinal fluid. Trans. R. Soc. Trop. Med. Hyg. 2007;101 (3): 311-3. doi:10.1016/j.trstmh.2006.05.004 - Pubmed citation
- 4. Reed RP, Cooke-Yarborough CM, Jaquiery AL, Grimwood K, Kemp AS, Su JC, Forsyth JR. Fatal granulomatous amoebic encephalitis caused by Balamuthia mandrillaris. The Medical journal of Australia. 167 (2): 82-4. Pubmed
- 5. Walochnik J, Aichelburg A, Assadian O et-al. Granulomatous amoebic encephalitis caused by Acanthamoeba amoebae of genotype T2 in a human immunodeficiency virus-negative patient. J. Clin. Microbiol. 2008;46 (1): 338-40. doi:10.1128/JCM.01177-07 - Free text at pubmed - Pubmed citation
- 6. P. Singh, R. Kochhar, R.K. Vashishta, N. Khandelwal, S. Prabhakar, S. Mohindra, P. Singhi. Amebic Meningoencephalitis: Spectrum of Imaging Findings. American Journal of Neuroradiology. 27 (6): 1217. Pubmed
- 7. Zamora A, Henderson H, Swiatlo E. Acanthamoeba encephalitis: A Case Report and Review of Therapy. Surgical neurology international. 5: 68. doi:10.4103/2152-7806.132239 - Pubmed