Autoimmune basal ganglia encephalitis (BGE)

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Autoimmune basal ganglia encephalitis (BGE). Dopamine 2 receptor (D2R) antibody: this also causes. Fully referenced neurology checklist.

Causative antibodies

  • Dopamine 2 receptor (D2R) antibody: this also causes
  • Anti N-methyl-D-aspartate receptor (NMDAR) antibody
  • Anti LGI1
  • Anti-recoverin antibodies (Abs) 
  • Seronegative

Movement disorders

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Other neurological features

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Psychiatric features

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Magnetic resonance imaging (MRI) brain: features

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Investigations

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Differential diagnosis

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Acute treatment

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Chronic treatment

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References

  1. Dale RC, Merheb V, Pillai S, et al. Antibodies to surface dopamine-2 receptor in autoimmune movement and psychiatric disorders. Brain 2012; 135:3453-3468.
  2. Dai X, Kuang L, Feng L, et al. Anti-dopamine receptor 2 antibody-positive encephalitis in adolescent. Front Neurol 2020; 11:471.
  3. 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; 59:445-448.
  4. Giri A, Andhale A, Acharya S, Kumar Singh R, Talwar D. Seronegative autoimmune basal ganglia encephalitis presenting as acute parkinsonism and refractory faciobrachial seizures: a case report. Cureus 2022; 14:e21351.
  5. Liu Y, Hao X, Zhou D, Hong Z. Relapsing and immune-responsive paroxysmal jaw clonus with blepharospasm and sialorrhea associated with D2R autoantibodies. Neurol Neuroimmunol Neuroinflamm 2022; 9:e1172.
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