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Carles Gaig, Yaroslau Compta
PURPOSE OF REVIEW: Anti-IgLON5 disease is a novel entity characterized by a distinctive sleep disorder associated with a variety of neurological symptoms, antibodies against IgLON5, and pathological findings of neuronal tauopathy. The characteristic sleep disorder occurs in most patients, but other neurological symptoms are also important because they can be the presenting and most disabling problem and mimic other conditions. This review focuses on nonsleep neurological symptoms and presentations of anti-IgLON5 disease...
January 28, 2019: Current Opinion in Neurology
Ana Moreno-Estébanez, Milagros Garcia-Ormaechea, Beatriz Tijero, Tamara Fernández-Valle, Juan Carlos Gómez-Esteban, Koldo Berganzo
No abstract text is available yet for this article.
November 2018: Movement Disorders Clinical Practice
Elena Vetter, David G Olmes, Ralf Linker, Frank Seifert
No abstract text is available yet for this article.
October 23, 2018: Neurology
Stephan Wenninger, Benedikt Schoser
In 2014, antibodies against the cell surface protein IgLON5 were first described in patients with a complex neurological syndrome of sleep disturbances and movement disorders. Since then, the clinical spectrum has steadily expanded and now includes brainstem syndromes, autonomic and neuropsychiatric disorders and, more rarely, peripheral symptoms such as fasciculations and neuromyotonia. Anti-IgLON5 antibodies are thought to cause neurodegeneration in specific CNS regions with tau deposits ("tauopathy")...
September 2018: Fortschritte der Neurologie-Psychiatrie
Anna Heidbreder, Konstanze Philipp
PURPOSE OF REVIEW: This review aims to give an overview about the current knowledge of this novel neurological disorder associated to IgLON-5 antibodies and its treatment. RECENT FINDINGS: Anti-IgLON5 disease was first formally described in 2014. This newly discovered disorder recaps a complex neurological disorder with sleep, movement, and neuroimmunological and neurodegenerative aspects. The clinical manifestation of the anti-IgLON5 disease is very heterogeneous mostly including a sleep disorder with non-rapid eye movement (REM) sleep parasomnia and REM behavior disorder besides obstructive sleep apnea syndrome and stridor...
June 23, 2018: Current Treatment Options in Neurology
Massimiliano Montagna, Rizvana Amir, Ilse De Volder, Martin Lammens, Jef Huyskens, Barbara Willekens
IgLON5-associated encephalitis is a syndrome with different clinical presentations consisting of sleep dysfunction, bulbar dysfunction, chorea, and progressive supranuclear palsy-like symptoms whereas dysautonomy and cognitive decline usually appear in later stages of the disease. We report a case of a patient with IgLON5-associated encephalitis presenting with rapidly progressive cognitive decline and atypical inflammatory lesions on brain magnetic resonance imaging, oligoclonal bands on cerebrospinal fluid, anti-IgLON5 antibodies exclusively of the IgG1 class, and a fierce inflammatory reaction on brain biopsy, who responded favorably to immunotherapy...
2018: Frontiers in Neurology
Carles Gaig, Alex Iranzo, Joan Santamaria, Francesc Graus
PURPOSE OF REVIEW: To review the clinical and polysomnographic features of the sleep disorder occurring in the recently described anti-IgLON5 disease. The hallmark of the disease is the presence of antibodies against IgLON5, a neural cell adhesion molecule of unknown function. The disease presents a robust HLA association, and the neuropathological examination shows a novel neuronal tauopathy with predominant hypothalamic and brainstem involvement. RECENT FINDINGS: Most patients (> 80%) present sleep-related vocalizations with movements and behaviors and sleep-disordered breathing...
May 23, 2018: Current Neurology and Neuroscience Reports
David P Breen, Birgit Högl, Alfonso Fasano, Claudia Trenkwalder, Anthony E Lang
Patients with sleep-related motor and behavioral disorders present to a variety of subspecialty clinics (neurology, sleep medicine, respiratory medicine, psychiatry). Diagnosing these disorders can be difficult, and sometimes they have a significant impact on quality of life. Alongside a number of common and well-recognized conditions, several new disease entities have been described in recent years that present with abnormal nocturnal motor phenomena (such as ADCY5-associated disease and anti-IgLON5 disease)...
July 2018: Movement Disorders: Official Journal of the Movement Disorder Society
Alex Iranzo
Parasomnias and sleep-related movement disorders are important problems in older adults. Sleep paralysis is rare, but may occur in families. In a minority of patients with disorders of arousal, the episodes persist until the age of 70. Zolpidem and other medications may induce sleepwalking and sleep-related eating. Most patients with idiopathic rapid eye movement (REM) sleep behavior disorder eventually develop Parkinson's disease or dementia with Lewy bodies. Anti-IgLON5 disease includes abnormal behaviors in NREM and REM sleep...
March 2018: Sleep Medicine Clinics
Francesc Graus, Joan Santamaría
No abstract text is available yet for this article.
September 2017: Neurology® Neuroimmunology & Neuroinflammation
Ren Haitao, Liu Huiqin, Qu Tao, Yang Xunzhe, Shao Xiaoqiu, Li Wei, Zhang Jiewen, Cui Liying, Guan Hongzhi
The autoimmune encephalitis can develop with or without an underlying tumor. For tumor-negative autoimmune encephalitis, the causes are still largely unknown. Here we presented three patients with autoimmune encephalitis accompanied with vitiligo. Among them, two patients suffered from anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis and one patient suffered from anti-IgLON5 encephalopathy. All of them received intravenous immunoglobulin and steroids as immunotherapy. The two patients with anti-LGI1 encephalitis recovered and got a good prognosis...
September 15, 2017: Journal of Neuroimmunology
Annachiara Cagnin, Sara Mariotto, Michele Fiorini, Marina Gaule, Nicola Bonetto, Matteo Tagliapietra, Emanuele Buratti, Gianluigi Zanusso, Sergio Ferrari, Salvatore Monaco
A novel neuronal tauopathy, mainly confined to hypothalamus and brainstem tegmentum, has recently been reported in patients with autoantibodies to the neuronal cell-adhesion molecule IgLON5. We describe a patient with anti-IgLON5 syndrome, who presented with dysautonomia and sleep disorder, followed by subacute dementia. Postmortem brain examination disclosed neuronal tau pathology prevailing in the hippocampus, amygdala, and locus coeruleus, in addition to microglial/neuronal TDP-43 pathology, with overexpression of aberrantly phosphorylated forms and neurotoxic truncated fragments, in basal ganglia, nucleus basalis, thalamus, and midbrain...
2017: Journal of Alzheimer's Disease: JAD
Russell C Dale, Sudarshini Ramanathan
No abstract text is available yet for this article.
May 2, 2017: Neurology
Carles Gaig, Francesc Graus, Yarko Compta, Birgit Högl, Luis Bataller, Norbert Brüggemann, Caroline Giordana, Anna Heidbreder, Katya Kotschet, Jan Lewerenz, Stefan Macher, Maria J Martí, Teresa Montojo, Jesus Pérez-Pérez, Inmaculada Puertas, Caspar Seitz, Mateus Simabukuro, Nieves Téllez, Klaus-Peter Wandinger, Alex Iranzo, Guadalupe Ercilla, Lidia Sabater, Joan Santamaría, Josep Dalmau
OBJECTIVE: To report the presentation, main syndromes, human leukocyte antigen (HLA) association, and immunoglobulin G (IgG) subclass in the anti-IgLON5 disease: a disorder with parasomnias, sleep apnea, and IgLON5 antibodies. METHODS: This was a retrospective clinical analysis of 22 patients. The IgG subclass was determined using reported techniques. RESULTS: Patients' median age was 64 years (range 46-83). Symptoms that led to initial consultation included sleep problems (8 patients; 36%), gait abnormalities (8; 36%), bulbar dysfunction (3; 14%), chorea (2; 9%), and cognitive decline (1; 5%)...
May 2, 2017: Neurology
Jens Burchard Schröder, Nico Melzer, Tobias Ruck, Anna Heidbreder, Ilka Kleffner, Ralf Dittrich, Paul Muhle, Tobias Warnecke, Rainer Dziewas
OBJECTIVE: To report on dysphagia as initial sign in a case of anti-IgLON5 syndrome and provide an overview of the current literature. METHODS: The diagnostic workup included cerebral MRI, fiber optic endoscopic evaluation of swallowing (FEES) with the FEES tensilon test, a videofluoroscopic swallowing study, evoked potentials and peripheral nerve conduction studies, polysomnography, lumbar puncture, and screening for neural autoantibodies. A systematic review of all published cases of IgLON5 syndrome is provided...
January 2017: Neurology® Neuroimmunology & Neuroinflammation
Ren Haitao, Yang Yingmai, Huang Yan, Han Fei, Lv Xia, Hao Honglin, Liu Chaiyan, Winfried Stöcker, Cui Liying, Guan Hongzhi
Encephalopathy associated with autoantibodies to IgLON5 is a novel syndrome characterized by a distinct sleep disorder and brain-stem involvement. Since the initial description of this encephalopathy in 2014, only a few additional patients have been reported (Simabukuro et al., 2015). In this paper, we report a new case of anti-IgLON5 antibodies with major symptoms of chorea and parkinsonism, and responsive to immunotherapy.
November 15, 2016: Journal of Neuroimmunology
Lidia Sabater, Jesús Planagumà, Josep Dalmau, Francesc Graus
BACKGROUND: Antibodies against IgLON5, a neuronal adhesion protein of unknown function, are markers of a novel neurological disorder termed anti-IgLON5 syndrome. The disorder shows a remarkable association with the HLA-DQB1*0501 and HLA-DRB1*1001 alleles, and postmortem studies demonstrate a novel neuronal tauopathy predominantly involving the hypothalamus and tegmentum of the brainstem. The role of IgLON5 antibodies in the pathogenesis of the disease is currently unknown. Here, we have determined the target epitopes of IgLON5 antibodies, the effects of the IgLON5 antibodies in rat hippocampal neurons, and the IgG subclass responsible for these effects...
September 1, 2016: Journal of Neuroinflammation
Ellen Gelpi, Romana Höftberger, Francesc Graus, Helen Ling, Janice L Holton, Timothy Dawson, Mara Popovic, Janja Pretnar-Oblak, Birgit Högl, Erich Schmutzhard, Werner Poewe, Gerda Ricken, Joan Santamaria, Josep Dalmau, Herbert Budka, Tamas Revesz, Gabor G Kovacs
We recently reported a novel neurological syndrome characterized by a unique NREM and REM parasomnia with sleep apnea and stridor, accompanied by bulbar dysfunction and specific association with antibodies against the neuronal cell-adhesion protein IgLON5. All patients had the HLA-DRB1*1001 and HLA-DQB1*0501 alleles. Neuropathological findings in two patients revealed a novel tauopathy restricted to neurons and predominantly involving the hypothalamus and tegmentum of the brainstem. The aim of the current study is to describe the neuropathological features of the anti-IgLON5 syndrome and to provide diagnostic levels of certainty based on the presence of associated clinical and immunological data...
October 2016: Acta Neuropathologica
J F Holle, F Jessen, J Kuhn
Antibody-associated disorders of the central nervous system constitute a heterogeneous group of disorders that can be roughly divided into two categories: Classic paraneoplastic syndromes associated with so-called well-characterized antibodies (paraneoplastic neurological disorders, PND) and autoimmune disorders with antibodies to membrane-bound or synaptic antigens (autoimmune encephalitis, AE). The discovery of autoimmune encephalitis has led to a paradigm shift in diagnosis and therapy as well as a reclassification of some neuropsychiatric syndromes that were previously classified as idiopathic or simply covered with descriptive terms...
May 2016: Fortschritte der Neurologie-Psychiatrie
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