Faciobrachial Dystonic Seizures Precede Lgi1 Antibody Limbic Encephalitis

被引：645

作者：

Irani, Sarosh R. ^{[1
]}

Michell, Andrew W. ^{[2
]}

Lang, Bethan ^{[1
]}

Pettingill, Philippa ^{[1
]}

Waters, Patrick ^{[1
]}

Johnson, Michael R. ^{[3
]}

Schott, Jonathan M. ^{[4
]}

Armstrong, Richard J. E. ^{[1
,4
]}

Zagami, Alessandro S. ^{[5
,6
,7
]}

Bleasel, Andrew ^{[8
]}

Somerville, Ernest R. ^{[5
,6
,7
,9
]}

Smith, Shelagh M. J. ^{[10
]}

Vincent, Angela ^{[1
,11
]}

机构：

[1] Univ Oxford, Dept Clin Neurol, Oxford, England

[2] Univ Cambridge, Addenbrookes Hosp, Dept Clin Neurosci, Cambridge CB2 2QQ, England

[3] Univ London Imperial Coll Sci Technol & Med, Ctr Neurosci, London, England

[4] Natl Hosp Neurol & Neurosurg, Dementia Res Ctr, London WC1N 3BG, England

[5] Univ New S Wales, Sydney, NSW, Australia

[6] Prince Wales Hosp, Inst Neurol Sci, Sydney, NSW, Australia

[7] Prince Wales Hosp, Sch Clin, Sydney, NSW, Australia

[8] Univ Sydney, Westmead Hosp, Sydney, NSW 2006, Australia

[9] Prince Wales Hosp, Comprehens Epilepsy Serv, Sydney, NSW, Australia

[10] Natl Hosp Neurol & Neurosurg, Dept Clin Neurophysiol, London WC1N 3BG, England

[11] UCL, Inst Neurol, London, England

来源：

ANNALS OF NEUROLOGY | 2011年 / 69卷 / 05期

基金：

英国医学研究理事会;

关键词：

VOLTAGE-GATED POTASSIUM; TEMPORAL-LOBE EPILEPSY; LEUCINE-RICH; MUTATIONS; ONSET; FORM;

D O I：

10.1002/ana.22307

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

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 developed LE, 20 of the remaining 26 (77%) experienced FBDS prior to the development of the amnesia and confusion that characterize LE. During the prodrome of FBDS alone, patients had normal sodium and brain MRIs, but electroencephalography demonstrated ictal epileptiform activity in 7 patients (24%). Following development of LE, the patients often developed other seizure semiologies, including typical mesial temporal lobe seizures. At this stage, investigations commonly showed hyponatremia and MRI hippocampal high T2 signal; functional brain imaging showed evidence of basal ganglia involvement in 5/8. Antiepileptic drugs (AEDs) were generally ineffective and in 41% were associated with cutaneous reactions that were often severe. By contrast, immunotherapies produced a clear, and often dramatic, reduction in FBDS frequency. Interpretation: Recognition of FBDS should prompt testing for VGKC-complex/Lgi1 antibodies. AEDs often produce adverse effects; treatment with immunotherapies may prevent the development of LE with its potential for cerebral atrophy and cognitive impairment. ANN NEUROL 2011; 69: 892-900

引用

页码：892 / 900

页数：9

共 32 条

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