Limbic encephalitis - VGKC

Case contributed by Royal Melbourne Hospital

Presentation

One week history of decreased cognitive and memory function, including difficulty forming new memories, acalculia and finger agnosia.

Patient Data

Age: 60 years
Gender: Male
CT

CT brain

Non-contrast CT of the brain demonstrates no focal abnormality. No evidence of a stroke or tumour. No haemorrhage. 

MRI

MRI brain

Bilateral mesial temporal lobe increased T2 signal is demonstrated, more marked on the right, and seen extending in along the hippocampal head, body and tail, but also involving the fornicies, amygdala, and parahippocampal gyrus. There is no convincing contrast enhancement. No evidence of haemorrhage. MR spectroscopy demonstrates minor elevation of the choline peak (not shown). MR perfusion demonstrates and some apparent reduction in the right mesial temporal (not shown).

CSF was sent for extensive analysis. 

  1. Viral PCR: negative
  2. NMDA Receptor Antibodies: negative
  3. Antinuclear Antibodie: Negative
  4. Extractable Nuclear Antigens: negative
  5. Anti - Sm: Negative
  6. Anti - RNP :  Negative
  7. Anti - Ro : Negative
  8. Anti - La : Negative
  9. Anti - Topo1: Negative
  10. Anti - Jo1: Negative
  11. Anti-Endomysium: Negative
  12. Transglutaminase IgA:  Negative
  13. Anti Hu: Negative
  14. Anti Ri: Negative
  15. Anti Yo: Negative
  16. Anti VGKC Antibody: detected and elevated

Case Discussion

This is a case of autoimmune limbic encephalitis. When an auto-antibody is found it is usually to either VGKC or NMDAR.

Antibodies to the voltage gated potassium (VGKC) channel interfere with peripheral neuromuscular and ganglionic transmission and may be associated with a number of neurological syndromes including limbic encephalitis, neuromyotonia and seizures. Anti VGKC antibodies are tested by radioimmunoassay.

NMDAR antibody (negative in this case) was first found in young female patients with ovarian tumours and prominent psychiatric symptoms, amnesia, seizures, dyskinesias, autonomic dysfunction and decreased levels of consciousness. The antibodies are now also found in males and females with no known tumour and in children. Their presence suggests an immunotherapy-responsive condition 1

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Case information

rID: 15727
Case created: 3rd Nov 2011
Last edited: 11th Jun 2016
Inclusion in quiz mode: Included

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