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
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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