Wednesday, May 28, 2014

Faciobrachial systolic seizures , correlate with anti VGKC and not CJD

Faciobrachial systolic seizures , correlate with anti VGKC and not CJDYoo J and Hirsch LJ. Limbic encephalitis associated with anti voltage gated potassium channel complex antibodies mimicking CJD. Jama Neurol 2014;71:79-82.

Case with following take home points:
FBDS are highly associated with ant VGKCC and not CJD and are treated with normal AED's

The two disorders otherwise may mimic each other

Anti VGKCC responds robustly to steroids

Both disorders can have elevated 14,3,3 in CSF

Absent restricted diffusion is a "red flag" against CJD

MRI of anti VGKCC is usually high T2 signal in uni or bilateral temporal lobes but they can also show cortical ribbon and basal ganglia changes mimicking CJD

FBDS mimic myoclonus but EEG confirms seizures

Spect scans show abnormal basal ganglia metabolism

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