Autoimmune basal ganglia encephalitis

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Clinic

  • BGE is characterized by the relatively rapid development of symptoms of parkinsonism such as akinesia, rigidity, and tremors.
  • Autoimmune inflammation of the basal ganglia structures.
  • Various autoantibodies, namely, dopamine D2 receptor (D2R), N-methyl-D-aspartate receptor (NMDAR), and anti-LGI1, trigger it.
  • When it occurs as a paraneoplastic manifestation usually due to ovarian teratomas, then anti-recoverin antibodies are classically associated. In these cases, there is additional development of retinopathy, also termed cancer-associated retinopathy (CAR)
  • The clinical presentation can also include other features such as
    • Cognitive abnormalities
    • Seizures


PANDAS

PANS and PANDAS are a type of basal ganglia encephalitis.

  • Acute movement disorders
  • Executive dysfunction
  • Fine motor difficulties
  • Anxiety [1]


Causation[2]

  • Respiratory viruses (INFL, RSV, PIV , HMPV, RNV, COV-2, COV-19, ADV, HBOV)
  • CJD
  • Arbovirus (DHF, RVF, JE, WNE, TBE, YF)
  • MTB



Entities / Miasms

CJD JE WNE TBE
Parkinsonism

(Rigidity / Bradikinesia / Tremor)

3+ 3+ 3+ 1+
Cognition impaired (executive function) 2+ 3+ 2+ 3+
Emotional lability / Anxiety / Depression -- 1+ -- 3+
Awakward, Inccordination 1+ 3+ 2+ 3+
Seizure 2+ 3+ -- 2+
  1. 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.
  2. G. C. Beattie, C. A. Glaser, H. Sheriff, S. Messenger, C. P. Preas, M. Shahkarami, A. Venkatesan, Encephalitis With Thalamic and Basal Ganglia Abnormalities: Etiologies, Neuroimaging, and Potential Role of Respiratory Viruses, Clinical Infectious Diseases, Volume 56, Issue 6, 15 March 2013, Pages 825–832, https://doi.org/10.1093/cid/cis990