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Morvans syndrome

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https://www.readbyqxmd.com/read/27847683/clinical-utility-of-seropositive-voltage-gated-potassium-channel-complex-antibody
#1
Adham Jammoul, Luay Shayya, Karin Mente, Jianbo Li, Alexander Rae-Grant, Yuebing Li
BACKGROUND: Antibodies against voltage-gated potassium channel (VGKC)-complex are implicated in the pathogenesis of acquired neuromyotonia, limbic encephalitis, faciobrachial dystonic seizure, and Morvan syndrome. Outside these entities, the clinical value of VGKC-complex antibodies remains unclear. METHODS: We conducted a single-center review of patients positive for VGKC-complex antibodies over an 8-year period. RESULTS: Among 114 patients positive for VGKC-complex antibody, 11 (9...
October 2016: Neurology. Clinical Practice
https://www.readbyqxmd.com/read/27786401/anti-contactin-associated-protein-2-encephalitis-relevance-of-antibody-titres-presentation-and-outcome
#2
C G Bien, Z Mirzadjanova, C Baumgartner, M D Onugoren, T Grunwald, M Holtkamp, S Isenmann, P Kermer, N Melzer, M Naumann, M Riepe, W R Schäbitz, T J von Oertzen, F von Podewils, H Rauschka, T W May
BACKGROUND AND PURPOSE: To clarify the relevance of titres of IgG antibodies against contactin-associated protein-2 (CASPR2) in diagnosing anti-CASPR2 encephalitis and to describe features and outcomes. METHODS: This was a retrospective analysis of 64 patients with CASPR2 antibodies, categorized independently as 'autoimmune encephalitis' or 'other disease'. Logistic regression methods were performed to identify potential predictors of 'autoimmune encephalitis' in addition to CASPR2 antibodies...
October 27, 2016: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/27698847/paraneoplastic-morvan-s-syndrome-following-surgical-treatment-of-recurrent-thymoma-a-case-report
#3
Edvina Galié, Rosaria Renna, Domenico Plantone, Andrea Pace, Mirella Marino, Bruno Jandolo, Tatiana Koudriavtseva
Morvan's syndrome (MoS) is a rare, complex neurological disorder characterized by neuromyotonia, neuropsychiatric features, dysautonomia and neuropathic pain. The majority of MoS cases have a paraneoplastic aetiology, usually occurring prior to the diagnosis of the underlying tumour and showing improvement following its treatment. The present study reports the case of a 35-year-old Caucasian male patient who was diagnosed with stage IVA thymoma. Thymectomy, lung resection, diaphragmatic pleurectomy and pericardio-phrenectomy were performed 6 months after neoadjuvant chemotherapy...
October 2016: Oncology Letters
https://www.readbyqxmd.com/read/27695240/morvan-s-syndrome-with-anti-contactin-associated-protein-like-2-voltage-gated-potassium-channel-antibody-presenting-with-syndrome-of-inappropriate-antidiuretic-hormone-secretion
#4
Anjani Kumar Sharma, Manminder Kaur, Madhuparna Paul
Morvan's syndrome is a rare autoimmune disorder characterized by triad of peripheral nerve hyperexcitability, autonomic dysfunction, and central nervous system symptoms. Antibodies against contactin-associated protein-like 2 (CASPR2), a subtype of voltage-gated potassium channel (VGKC) complex, are found in a significant proportion of patients with Morvan's syndrome and are thought to play a key role in peripheral as well as central clinical manifestations. We report a patient of Morvan's syndrome with positive CASPR2-anti-VGKC antibody having syndrome of inappropriate antidiuretic hormone as a cause of persistent hyponatremia...
October 2016: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/27672870/poster-102-the-functional-impairments-in-a-patient-with-the-rare-morvan-s-syndrome-a-case-report
#5
Kim D Barker, Stephanie Tow
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
https://www.readbyqxmd.com/read/27667487/-voltage-gated-potassium-channel-complex-antibodies-associated-encephalopathy-and-related-diseases
#6
Osamu Watanabe
Voltage-gated potassium channel (VGKC) complex antibodies are auto-antibodies, initially identified in acquired neuromyotonia (aNMT; Isaacs' syndrome), which cause muscle cramps and difficulty in opening the palm of the hands. Subsequently, these antibodies were found in patients presenting with aNMT along with psychosis, insomnia, and dysautonomia, collectively termed Morvan's syndrome (MoS), and in a limbic encephalopathy (LE) patient with prominent amnesia and frequent seizures. Typical LE cases have a distinctive adult-onset, frequent, brief dystonic seizure semiology that predominantly affects the arms and ipsilateral face...
September 2016: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/27515199/morvan-s-syndrome-and-the-sustained-absence-of-all-sleep-rhythms-for-months-or-years-an-hypothesis
#7
Claude Touzet
Despite the predation costs, sleep is ubiquitous in the animal realm. Humans spend a third of their life sleeping, and the quality of sleep has been related to co-morbidity, Alzheimer disease, etc. Excessive wakefulness induces rapid changes in cognitive performances, and it is claimed that one could die of sleep deprivation as quickly as by absence of water. In this context, the fact that a few people are able to go without sleep for months, even years, without displaying any cognitive troubles requires explanations...
September 2016: Medical Hypotheses
https://www.readbyqxmd.com/read/27432809/encephalopathy-with-upper-body-hypertonia-and-myoclonus-in-patient-with-systemic-lupus-erythematosus-and-anti-caspr2
#8
A Castro, J C Romeu, R Geraldes, J A Pereira da Silva
Systemic lupus erythematosus (SLE) may involve the nervous system but there are no specific biomarkers of neuroSLE. Limbic encephalitis has been rarely associated with SLE. We present a case of a 22-year-old black woman where typical SLE psychosis evolved to an encephalopathy with atypical features, normal MRI, electroencephalogram slowing and frontal and occipito-temporal hypometabolism on fluorodeoxyglucose positron emission tomography (FDG PET).Memory deficits, bizarre behaviour, psychosis, neuromyotonia and movement disorders have been described in autoimmune central nervous system disorders and associated with specific antibodies...
July 17, 2016: Lupus
https://www.readbyqxmd.com/read/27428927/characterization-of-a-subtype-of-autoimmune-encephalitis-with-anti-contactin-associated-protein-like-2-antibodies-in-the-cerebrospinal-fluid-prominent-limbic-symptoms-and-seizures
#9
Bastien Joubert, Margaux Saint-Martin, Nelly Noraz, Géraldine Picard, Veronique Rogemond, François Ducray, Virginie Desestret, Dimitri Psimaras, Jean-Yves Delattre, Jean-Christophe Antoine, Jérôme Honnorat
IMPORTANCE: Autoantibodies against contactin-associated protein-like 2 (CASPR2) are observed in several neurological syndromes, including neuromyotonia (NMT), Morvan syndrome (MoS), and limbic encephalitis. OBJECTIVE: To characterize the clinical and biological presentations of patients with anti-CASPR2 antibodies in the cerebrospinal fluid (CSF). DESIGN, SETTING, AND PARTICIPANTS: We conducted a retrospective cohort analysis of 18 patients who had anti-CASPR2 antibodies in their CSF between March 2009 and November 2015 at the Centre National de Référence pour les Syndromes Neurologiques Paranéoplasiques in Lyon, France...
September 1, 2016: JAMA Neurology
https://www.readbyqxmd.com/read/27371488/the-clinical-spectrum-of-caspr2-antibody-associated-disease
#10
Agnes van Sonderen, Helena Ariño, Mar Petit-Pedrol, Frank Leypoldt, Peter Körtvélyessy, Klaus-Peter Wandinger, Eric Lancaster, Paul W Wirtz, Marco W J Schreurs, Peter A E Sillevis Smitt, Francesc Graus, Josep Dalmau, Maarten J Titulaer
OBJECTIVE: To report a large cohort of patients with antibodies against contactin-associated protein-like 2 (Caspr2) and provide the clinical spectrum of this disorder. METHODS: Serum and CSF samples were assessed at 2 neuroimmunology centers in Barcelona and Rotterdam. Patients were included if Caspr2 antibodies were confirmed with 2 independent techniques, including brain immunohistochemistry and cell-based assay. Clinical information was obtained by the authors or provided by treating physicians after patients' informed consent...
August 2, 2016: Neurology
https://www.readbyqxmd.com/read/27247812/morvan-syndrome-secondary-to-thymic-carcinoma-in-a-patient-with-systemic-lupus-erythematosus
#11
Gabrielle Macaron, Elie El Rassy, Salam Koussa
Morvan syndrome (MoS) is a rare paraneoplastic autoimmune disorder characterized by peripheral nerve hyperexcitability, autonomic dysfunction, and sleep disorders. Systemic lupus erythmatosus (SLE) cooccurs in 6-10% of patients with thymoma. It may occur before, concurrently with, or after thymoma diagnosis. This paper reports the first case of cooccurrence of SLE, thymic carcinoma, and MoS. The cooccurrence of SLE, thymoma, and MoS delineates the generalized autoimmunity process. Symptoms of both MoS and SLE abated upon tumor resection...
2016: Case Reports in Neurological Medicine
https://www.readbyqxmd.com/read/27224435/a-case-of-morvan-syndrome-mimicking-amyotrophic-lateral-sclerosis-with-frontotemporal-dementia
#12
Brin Freund, Manoj Maddali, Thomas E Lloyd
INTRODUCTION: Morvan syndrome is a rare autoimmune/paraneoplastic disorder involving antibodies to the voltage-gated potassium channel complex. It is defined by subacute encephalopathy, neuromuscular hyperexcitability, dysautonomia, and sleep disturbance. It may present a diagnostic dilemma when trying to differentiate from amyotrophic lateral sclerosis with frontotemporal dementia. METHODS: A 76-year-old man with a history of untreated prostate adenocarcinoma was evaluated for subacute cognitive decline, diffuse muscle cramps, and hyponatremia...
June 2016: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/27112685/autoimmune-sleep-disorders
#13
Michael H Silber
A number of autoantibodies, some paraneoplastic, are associated with sleep disorders. Morvan syndrome and limbic encephalitis, associated with voltage-gated potassium channel-complex antibodies, principally against CASPR2 and LGI1, can result in profound insomnia and rapid eye movement sleep behavior disorder (RBD). Patients with aquaporin-4 antibodies and neuromyelitis optica may develop narcolepsy in association with other evidence of hypothalamic dysfunction, sometimes as the initial presentation. Central sleep apnea and central neurogenic hypoventilation are found in patients with anti-N-methyl-d-aspartate receptor antibody encephalitis, and obstructive sleep apnea, stridor, and hypoventilation are prominent features of a novel tauopathy associated with IgLON5 antibodies...
2016: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/27112678/voltage-gated-potassium-channel-complex-autoimmunity-and-associated-clinical-syndromes
#14
Sarosh R Irani, Angela Vincent
Voltage-gated potassium channel (VGKC)-complex antibodies are defined by the radioimmunoprecipitation of Kv1 potassium channel subunits from brain tissue extracts and were initially discovered in patients with peripheral nerve hyperexcitability (PNH). Subsequently, they were found in patients with PNH plus psychosis, insomnia, and dysautonomia, collectively termed Morvan's syndrome (MoS), and in a limbic encephalopathy (LE) with prominent amnesia and frequent seizures. Most recently, they have been described in patients with pure epilepsies, especially in patients with the novel and distinctive semiology termed faciobrachial dystonic seizures (FBDS)...
2016: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/27026266/morvan-s-syndrome-and-myasthenia-gravis-related-to-familial-mediterranean-fever-gene-mutations
#15
Junpei Koge, Shintaro Hayashi, Hiroyuki Murai, Jun Yokoyama, Yuri Mizuno, Taira Uehara, Naoyasu Ueda, Osamu Watanabe, Hiroshi Takashima, Jun-ichi Kira
BACKGROUND: We present the first case of Morvan's syndrome (MoS) and myasthenia gravis (MG) related to familial Mediterranean fever (FMF) gene mutations. CASE PRESENTATION: A 40-year-old woman with a 1-year history of bilateral ptosis and limb muscle weakness presented to our hospital. She also had memory impairment, insomnia, hyperhidrosis, and muscle twitches. Electromyography confirmed widespread myokymia, and there was evidence of temporal region dysfunction on electroencephalography...
2016: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/26983964/a-chinese-female-morvan-patient-with-lgi1-and-caspr2-antibodies-a-case-report
#16
Li Zhang, Qiang Lu, Hong-Zhi Guan, Jun-Hua Mei, Hai-Tao Ren, Ming-Sheng Liu, Bin Peng, Li-Ying Cui
BACKGROUND: Morvan syndrome is a rare disorder characterized by the combination of peripheral nerve hyperexcitability, encephalopathy and dysautonomia with marked insomnia. It was reported to have association to antibodies to voltage-gated potassium channels including contactin associated protein-like 2 antibodies (CASPR2-Ab) and leucine-rich glioma inactivated protein 1 antibodies (LGI1-Ab). LGI1-Ab was reported to associate with seizures, amnesia, confusion, hyponatraemia and a good prognosis, while CASPR2-Ab with peripheral presentations, probable risk for tumor and a poor prognosis...
2016: BMC Neurology
https://www.readbyqxmd.com/read/26905913/a-case-series-of-patients-with-autoimmune-myasthenia-gravis-in-association-with-invasive-thymoma
#17
Pamela McCombe, Meenakshi Raj, Robert Henderson, Stefan Blum
OBJECTIVES: In some patients, autoimmune myasthenia gravis (MG) is associated with thymic hyperplasia or thymoma, and in some patients the thymoma is invasive. Little is known about the clinical course of subjects who present with MG and are found to have invasive thymoma. METHODS: We reviewed the patients at our clinic with MG and invasive thymoma, and have described their clinical features. RESULTS: Six MG patients were diagnosed with invasive thymoma...
March 2016: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/26639057/-intravenous-immunoglobulin-therapy-in-morvan-syndrome-secondary-to-recurrent-thymic-carcinoma
#18
Gabriel Horta Baas
Morvan's syndrome is a rare autoimmune channelopathy. A case of Morvan's syndrome is presented as a paraneoplastic syndrome associated to the recurrence of a well-differentiated thymic carcinoma, which showed a good clinical response to treatment with intravenous immunoglobulin.
2015: Medwave
https://www.readbyqxmd.com/read/26549678/atypical-case-of-morvan-s-syndrome
#19
Itay Lotan, Ruth Djaldetti, Mark A Hellman, Felix Benninger
Morvan's syndrome is a rare neurological condition characterized by the combination of neuromyotonia, autonomic instability and encephalopathy, associated with auto-antibodies against voltage-gated potassium channels. We report a patient with an initial presentation suggestive of typical Guillain-Barré syndrome (GBS), who later developed clinical and laboratory features compatible with Morvan's syndrome. Several months after resolution of the neurological symptoms, as well as disappearance of the characteristic anti-leucine-rich, glioma inactivated 1 (anti-LGI1) antibodies, the patient presented with episodes of fever of unknown origin, during which the antibodies became positive again, suggesting the possibility of a relapse...
March 2016: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/26480798/-morvan-s-syndrome-morvan-s-fibrillary-chorea
#20
Kimiyoshi Arimura, Osamu Watanabe
No abstract text is available yet for this article.
September 2015: Nihon Rinsho. Japanese Journal of Clinical Medicine
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