作者
Sarosh R Irani, Andrew W Michell, Bethan Lang, Philippa Pettingill, Patrick Waters, Michael R Johnson, Jonathan M Schott, Richard JE Armstrong, Alessandro S. Zagami, Andrew Bleasel, Ernest R Somerville, Shelagh MJ Smith, Angela Vincent
发表日期
2011/5
期刊
Annals of neurology
卷号
69
期号
5
页码范围
892-900
出版商
Wiley Subscription Services, Inc., A Wiley Company
简介
Objective
To describe a distinctive seizure semiology that closely associates with voltage‐gated potassium channel (VGKC)‐complex/Lgi1 antibodies and commonly precedes the onset of limbic encephalitis (LE).
Methods
Twenty‐nine patients were identified by the authors (n = 15) or referring clinicians (n = 14). The temporal progression of clinical features and serum sodium, brain magnetic resonance imaging (MRI), positron emission tomography/single photon emission computed tomography, and VGKC‐complex antibodies was studied.
Results
Videos and still images showed a distinctive adult‐onset, frequent, brief dystonic seizure semiology that predominantly affected the arm and ipsilateral face. We have termed these faciobrachial dystonic seizures (FBDS). All patients tested during their illness had antibodies to VGKC complexes; the specific antigenic target was Lgi1 in 89%. Whereas 3 patients never …
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SR Irani, AW Michell, B Lang, P Pettingill, P Waters… - Annals of neurology, 2011