Basal ganglia Encephalitis entities

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Viral miasms

  • Parkinsonism + Movement disorders such as dystonia, chorea, myoclonus and Stereotypy is a very rare manifestation following DHF encephalitis. [1]


Clinic

  • Autoimmune basal ganglia encephalitis causes Parkinsonism (Akinesia + Rigidity + Tremors).
  • Here, we report a case with Starting symptoms of Anorexia, Parkinsonism, and Disturbance of consciousness [2]
  • Basal ganglia encephalitis is frequently caused by an underlying infection and can trigger Neurologic / Psychiatric symptoms such as Obsessions/ Compulsions, Anxiety, Depression, mood swings and other changes in behavior [3]
  • BGE is a rare but distinct entity of putative autoimmune aetiology, with specific basal ganglia inflammation and acute movement disorders. Here four pediatric cases of BGE is presented. Although all patients made significant motor recoveries, all four cases displayed executive dysfunction, fine motor difficulties, and anxiety. Three of them displayed attention deficits. [4]
  • BGE is associated with chorea and other movement disorders. Seizures are still rare. Here a 45-year-old male BGE is presented with cognitive dysfunction + Slowness of activities + faciobrachial seizures. [5]

BGE vs NMDR encephalitis vs SC

  • NMDAR encephalitis, Autoimmune basal ganglia encephalitis (BGE), and Sydenham's chorea (SC) are comparatively investigated in 2014. NMDAR encephalitis (n = 10), BGE (n = 12), and SC (n = 9).
    • Stereotypy was only seen in NMDAR encephalitis (8/10) and not in BGE and SC (P < 0.001).
    • Perseveration was only seen in NMDAR encephalitis (5/10) and not in BGE and SC (P < 0.001).
    • Akinesia was more commonly seen in BGE (5/12) than in NMDAR encephalitis (1/10, P = 0.097).
    • Tremor was more commonly seen in BGE (5/12) than in NMDAR encephalitis (1/10, P = 0.097).
    • Chorea was seen in all groups: NMDAR encephalitis (4/10), BGE (3/12), and SC (9/9).
    • Dystonia was seen in all groups: NMDAR encephalitis (6/10), BGE (7/12), and SC (2/9).

Conclusion

  • Stereotypies or perseveration are suggestive of NMDAR encephalitis, whereas their absence and the presence of akinesia and tremor is more suggestive of BGE.
  • Chorea and dystonia are least discriminating. [6]


Related disease

Basal ganglion disease

Miasms

  1. Ganaraja VH, Kamble N, Netravathi M, Holla VV, Koti N, Pal PK. Stereotypy with Parkinsonism as a Rare Sequelae of Dengue Encephalitis: A Case Report and Literature Review. Tremor Other Hyperkinet Mov (N Y). 2021 Jun 23;11:22. doi: 10.5334/tohm.630. PMID: 34221697; PMCID: PMC8231449.
  2. uki Kitazaki, Norimichi Shirafuji, Naoko Takaku, Tomohisa Yamaguchi, Soichi Enomoto, Masamichi Ikawa, Osamu Yamamura, Yasunari Nakamoto, Tadanori Hamano, Autoimmune basal ganglia encephalitis associated with anti-recoverin antibodies: A case report, eNeurologicalSci, Volume 25, 2021, 100382, ISSN 2405-6502,
  3. Chain JL, Alvarez K, Mascaro-Blanco A, et al. Autoantibody Biomarkers for Basal Ganglia Encephalitis in Sydenham Chorea and Pediatric Autoimmune Neuropsychiatric Disorder Associated With Streptococcal Infections. Front Psychiatry. 2020;11:564. Published 2020 Jun 24. doi:10.3389/fpsyt.2020.00564 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7328706/
  4. Pawela C, Brunsdon RK, Williams TA, Porter M, Dale RC, Mohammad SS. The neuropsychological profile of children with basal ganglia encephalitis: a case series. Dev Med Child Neurol. 2017 Apr;59(4):445-448. doi: 10.1111/dmcn.13351. Epub 2016 Dec 19. PMID: 27991656.
  5. Giri A, Andhale A, Acharya S, et al. (January 17, 2022) Seronegative Autoimmune Basal Ganglia Encephalitis Presenting as Acute Parkinsonism and Refractory Faciobrachial Seizures: A Case Report. Cureus 14(1): e21351. doi:10.7759/cureus.21351
  6. Mohammad SS, Fung VS, Grattan-Smith P, Gill D, Pillai S, Ramanathan S, Brilot F, Dale RC. Movement disorders in children with anti-NMDAR encephalitis and other autoimmune encephalopathies. Mov Disord. 2014 Oct;29(12):1539-42. doi: 10.1002/mds.25999. Epub 2014 Aug 22. PMID: 25154478.