Journal ArticleDOI
Faciobrachial dystonic seizures precede Lgi1 antibody limbic encephalitis.
Sarosh R. Irani,Andrew W. Michell,Bethan Lang,Philippa Pettingill,Patrick Waters,Marvin Johnson,Jonathan M. Schott,Richard J. E. Armstrong,Alessandro S. Zagami,Andrew Bleasel,Ernest Somerville,Shelagh M. J. Smith,Angela Vincent,Angela Vincent +13 more
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TLDR
A distinctive seizure semiology is described that closely associates with voltage‐gated potassium channel (VGKC)‐complex/Lgi1 antibodies and commonly precedes the onset of limbic encephalitis (LE).Abstract:
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 ¼ 15) or referring clinicians (n ¼ 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 2010;000:000–000read more
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Journal ArticleDOI
A clinical approach to diagnosis of autoimmune encephalitis
Francesc Graus,Maarten J. Titulaer,Ramani Balu,Susanne M. Benseler,Christian G. Bien,Tania Cellucci,Irene Cortese,Russell C. Dale,Jeffrey M. Gelfand,Michael D. Geschwind,Carol A. Glaser,Jérôme Honnorat,Romana Höftberger,Takahiro Iizuka,Sarosh R. Irani,Eric Lancaster,Frank Leypoldt,Harald Prüss,Alexander Rae-Grant,Markus Reindl,Myrna R. Rosenfeld,Kevin Rostasy,Albert Saiz,Arun Venkatesan,Angela Vincent,Klaus Peter Wandinger,Patrick Waters,J. Dalmau,J. Dalmau +28 more
TL;DR: Through logical differential diagnosis, levels of evidence for autoimmune encephalitis (possible, probable, or definite) are achieved, which can lead to prompt immunotherapy.
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Autoantibodies associated with diseases of the CNS: new developments and future challenges
TL;DR: The new specialty of immunotherapy-responsive CNS disorders is likely to expand further as more antibody targets are discovered, and antibody assays can help with diagnosis.
Journal ArticleDOI
Encephalitis with refractory seizures, status epilepticus, and antibodies to the GABAA receptor: a case series, characterisation of the antigen, and analysis of the effects of antibodies
Mar Petit-Pedrol,Thaís Armangue,Thaís Armangue,Xiaoyu Peng,Luis Bataller,Tania Cellucci,Rebecca Davis,Lindsey McCracken,Eugenia Martinez-Hernandez,Warren P. Mason,Michael C. Kruer,David G. Ritacco,Wolfgang Grisold,Brandon F. Meaney,Carmen Alcalá,Peter A.E. Sillevis-Smitt,Maarten J. Titulaer,Rita J. Balice-Gordon,Francesc Graus,Josep Dalmau +19 more
TL;DR: High titres of serum and CSF GABAA receptor antibodies are associated with a severe form of encephalitis with seizures, refractory status epilepticus, or both and are potentially treatable.
Journal ArticleDOI
Neuronal autoantigens—pathogenesis, associated disorders and antibody testing
Eric Lancaster,Josep Dalmau +1 more
TL;DR: The various targets of neuronal antibodies are reviewed, focusing predominantly on autoantigens located on the cell surface or synapses, and an algorithm to identify and assess antibodies that bind to cell-surface and synaptic antigens is provided.
Journal ArticleDOI
Autoimmune encephalitis epidemiology and a comparison to infectious encephalitis.
Divyanshu Dubey,Sean J. Pittock,Cecilia Kelly,Andrew McKeon,Alfonso Sebastian Lopez-Chiriboga,Vanda A. Lennon,Avi Gadoth,Carin Y. Smith,Sandra C. Bryant,Christopher J. Klein,Allen J. Aksamit,Michel Toledano,Bradley F. Boeve,Jan Mendelt Tillema,Eoin P. Flanagan +14 more
TL;DR: To evaluate the incidence and prevalence of autoimmune encephalitis and compare it to that of infectiousEncephalitis, a large number of patients with known or suspected cases of the disease are referred to a single hospital for evaluation.
References
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Journal ArticleDOI
Antibodies to Kv1 potassium channel-complex proteins leucine-rich, glioma inactivated 1 protein and contactin-associated protein-2 in limbic encephalitis, Morvan's syndrome and acquired neuromyotonia.
Sarosh R. Irani,Sian K Alexander,Patrick Waters,Kleopas A. Kleopa,Philippa Pettingill,Luigi Zuliani,Elior Peles,Camilla Buckley,B Lang,Angela Vincent +9 more
TL;DR: The identification of leucine-rich, glioma inactivated 1 protein and contactin-associated protein-2 as the major targets of potassium channel antibodies, and their associations with different clinical features, begins to explain the diversity of these syndromes.
Journal ArticleDOI
Potassium channel antibody-associated encephalopathy: a potentially immunotherapy-responsive form of limbic encephalitis
Angela Vincent,Camilla Buckley,Jonathan M. Schott,Ian Baker,Bonnie‐Kate Dewar,Niels Detert,Linda Clover,Abigail Parkinson,Christian G. Bien,Salah Omer,Bethan Lang,Martin N. Rossor,Jackie Palace +12 more
TL;DR: VGKC-Ab-associated encephalopathy is a relatively common form of autoimmune, non-paraneoplastic, potentially treatable encephalitis that can be diagnosed by a serological test.
Journal ArticleDOI
Investigation of LGI1 as the antigen in limbic encephalitis previously attributed to potassium channels: a case series
Meizan Lai,Maartje G. Huijbers,Eric Lancaster,Francesc Graus,Luis Bataller,Rita J. Balice-Gordon,John K. Cowell,Josep Dalmau +7 more
TL;DR: The term limbic encephalitis associated with antibodies against voltage-gated potassium channels should be changed to limbicEncephalitisassociated with LGI1 antibodies, and this disorder should be classed as an autoimmune synaptic encephalopathy.
Journal ArticleDOI
Mutations in LGI1 cause autosomal-dominant partial epilepsy with auditory features.
Sergey Kalachikov,Oleg V. Evgrafov,Barbara Ross,Melodie R. Winawer,Christie Barker-Cummings,Filippo Martinelli Boneschi,Chang Choi,Pavel Morozov,Kamna Das,Elita Teplitskaya,Andrew Yu,Eftihia Cayanis,Penchaszadeh Gk,Andreas H. Kottmann,Timothy A. Pedley,W. Allen Hauser,Ruth Ottman,T. Conrad Gilliam +17 more
TL;DR: Discovery of LGI1 as a cause of ADPEAF suggests new avenues for research on pathogenic mechanisms of idiopathic epilepsies and shows that the expression pattern of mouse Lgi1 is predominantly neuronal and is consistent with the anatomic regions involved in temporal lobe epilepsy.
Journal ArticleDOI
Antibodies to glutamic acid decarboxylase define a form of limbic encephalitis
TL;DR: A cohort of patients with recent‐onset temporal lobe epilepsy caused by LE was studied to test for GAD antibody positivity and response to immunotherapies.
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