CLINICAL SPECTRUM OF MORVAN SYNDROME: A SINGLE CENTRE STUDY

2021 ◽  
pp. 22-23
Author(s):  
Jagiasi Kamlesh A ◽  
Bagul Abhay S
Keyword(s):  
Central Nervous System ◽  
Potassium Channel ◽  
Strong Association ◽  
Autoimmune Disorder ◽  
Response To Therapy ◽  
Review Of Literature ◽  
Single Centre ◽  
Voltage Gated ◽  
Single Centre Study ◽  
Peripheral Nerve Hyperexcitability

INTRODUCTION: Morvan syndrome (MoS) is a rare autoimmune disorder characterised by peripheral nerve hyperexcitability, autonomic dysfunction and central nervous system symptoms. It has strong association with autoantibodies to voltage gated potassium channel complex. This was a prospective observationa METHODS: l study. We reported 6 cases of Morvan syndrome. Clinical features, investigations, treatment and outcome were described and review of literature was done. All RESULT: subjects were male. Myokymia and insomnia were present in all patients and no patient had seizure. Voltage gated potassium channel (VGKC) antibody was positive in 66% (n=4) of subjects, all four were positive for CASPER2 and 2 were also weakly positive for LGI1. All patients were treated with immunomodulation and we found good response to therapy. Pain was treated with carbamazepine and phenytoin. CONCLUSION: Morvan syndrome, a VGKC antibody spectrum disorder, is a clinical diagnosis. We found myokymia and insomnia in all patients and no patient had seizure. In our study, response to immunotherapy was good. Awareness about MoS is necessary, as it can be treated successfully.

scholarly journals Morvan’s syndrome with anti contactin associated protein like 2 – voltage gated potassium channel antibody presenting with syndrome of inappropriate antidiuretic hormone secretion

2016 ◽  
Vol 7 (04) ◽  
pp. 577-579 ◽  
Author(s):  
Anjani Kumar Sharma ◽  
Manminder Kaur ◽  
Madhuparna Paul
Keyword(s):  
Potassium Channel ◽  
Antidiuretic Hormone ◽  
Hormone Secretion ◽  
Significant Proportion ◽  
Clinical Manifestations ◽  
Autoimmune Disorder ◽  
Inappropriate Antidiuretic Hormone Secretion ◽  
Voltage Gated ◽  
Morvan’S Syndrome ◽  
Peripheral Nerve Hyperexcitability

ABSTRACTMorvan’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.

THUR 153 Antibodies against the voltage-gated potassium channel complex

2018 ◽  
Vol 89 (10) ◽  
pp. A18.2-A18
Author(s):  
Joyutpal Das ◽  
Vanisha Chauhan ◽  
Ryan Keh ◽  
Daniel Mills ◽  
Johal Nicholas ◽  
...  
Keyword(s):  
Potassium Channel ◽  
Intravenous Immunoglobulins ◽  
Voltage Gated ◽  
Morvan’S Syndrome ◽  
Classic Phenotype ◽  
Antibody Titres ◽  
Peripheral Nerve Hyperexcitability ◽  
Clinical Phenotyping ◽  
Positive Results ◽  
Autoimmune Encephalopathy

Voltage-gated potassium channel (VGKC) complex antibodies have been associated with a spectrum of presentations including peripheral nerve hyperexcitability (PNH), Morvan’s syndrome, autoimmune encephalopathy, epilepsy and recently psychosis.We retrospectively reviewed the medical records of 70 patients from the Greater Manchester Neuroscience Centre, who had tested positive for VGKC-complex antibodies between 2012 and 2015 to identify the clinical relevance of positive results.The majority were diagnosed with autoimmune encephalopathy(19) followed by epilepsy(14), psychosis(10) and PNH(6). The remaining fifteen had other neurological presentations and six had no primary neurological disorder. 39/70 patients who had antibody titres>400 pM, were diagnosed with autoimmune encephalopathy(19), epilepsy(9), psychosis(4), PNH(3) and other disorders(4). 24/39 patients, who received treatment with one or a combination of corticosteroids, intravenous immunoglobulins, cyclophosphamide, plasma exchange, azathioprine or rituximab, had a diagnosis of autoimmune encephalopathy(18), epilepsy(2), psychosis(2) and malignancy(2). 16/24 were treatment responsive. 3/31 patients with lower titres were also treated, but only one with the classic phenotype (PNH) responded to treatment.The classic phenotype often had a titre >400 pM. PNH may have a titre ≤400 pM. The patients without classic presentations typically had titres≤400 pM. Consistent with previous studies, clinical phenotyping and antibody titre helped to determine the relevance of VGKC-complex antibodies.

Mutations in voltage-gated potassium channel KCNC3 cause degenerative and developmental central nervous system phenotypes

2006 ◽  
Vol 38 (4) ◽  
pp. 447-451 ◽  
Author(s):  
Michael F Waters ◽  
Natali A Minassian ◽  
Giovanni Stevanin ◽  
Karla P Figueroa ◽  
John P A Bannister ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Potassium Channel ◽  
Voltage Gated

Immunohistochemical localization of the voltage-gated potassium channel subunit Kv1.4 in the central nervous system of the adult rat

2003 ◽  
Vol 26 (3) ◽  
pp. 209-224 ◽  
Author(s):  
Rafael Luján ◽  
Carlos de Cabo de la Vega ◽  
Eduardo Dominguez del Toro ◽  
Juan J Ballesta ◽  
Manuel Criado ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Potassium Channel ◽  
Immunohistochemical Localization ◽  
Voltage Gated ◽  
Adult Rat ◽  
The Central Nervous System

The Microbiome in Paediatric Crohn’s Disease—A Longitudinal, Prospective, 
Single-Centre Study

2019 ◽  
Vol 13 (8) ◽  
pp. 1044-1054 ◽  
Author(s):  
Shivani Kansal ◽  
Anthony G Catto-Smith ◽  
Karen Boniface ◽  
Sarah Thomas ◽  
Donald J Cameron ◽  
...  
Keyword(s):  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Statistical Significance ◽  
Strong Association ◽  
Limited Information ◽  
Single Centre ◽  
Single Centre Study ◽  
Healthy Control ◽  
Gut Mucosa ◽  
And Control

AbstractBackground and AimsThe gut mucosa is the principal site where Crohn’s disease [CD] inflammation occurs. Limited information is available about the gut mucosal microbiome during CD relapse and remission. The aim of our study was to characterize specific changes in the gut microbiome during relapse and remission in a large single-centre paediatric CD cohort.MethodsWe analysed the microbiome of 345 biopsies from 204 patients, including 88 CD first diagnosis [CDFD] patients, 38 relapse [CDRL] patients, 12 remission [CDRM] patients, and 66 controls. Species identification was conducted using oligotyping in combination with ARB/SILVA taxonomic annotation.ResultsWe observed 45 bacteria to differ between CDFD samples and controls with statistical significance, with Fusobacterium being the most implicated species in CDFD patients. We also identified gender-specific differences in CD. Five species showed a strong association with CDRL patients and 10 species with CDRM patients. Three taxa showed a positive co-occurrence across the two groups. Hespellia porcina [closest taxonomic neighbour to Clostridium oroticum] was the most strongly associated with CDRL samples. Interestingly, Fusobacterium was not part of the CDRL-associated taxa group. Faecalibacterium prausnitzii was equally present in CDFD and control samples.ConclusionThis is the first study that has investigated the gut mucosal microbiome in a paediatric CD cohort with longitudinal sampling. Importantly, the microbiome of patients in CDRM did not return to a healthy control state. Neither did the microbiome of patients with CDRL return to the profile seen at CDFD.

scholarly journals Therapeutic outcome of diverticular associated colitis – a retrospective single centre experience

2021 ◽  
Vol 84 (2) ◽  
pp. 275-281
Author(s):  
F Vulsteke ◽  
G De Hertogh ◽  
S Vermeire
Keyword(s):  
Ulcerative Colitis ◽  
Response To Therapy ◽  
Review Of Literature ◽  
Right Colon ◽  
Single Centre ◽  
Retrospective Case ◽  
Benign Condition ◽  
Case Identification ◽  
Single Centre Experience ◽  
Pathology Reports

Background: Diverticular associated colitis (DAC) has become increasingly appreciated as a form of inflammatory disease, localized mainly in the sigmoid, and defined as chronic inflammation of the interdiverticular mucosa with sparing of rectum, right colon and diverticula themselves. Methods: A retrospective case identification from January 2005 to December 2016 was performed. Patients with a diagnosis of DAC based on clinical, endoscopic and histological findings were enrolled. We analyzed their characteristics and response to therapy, and performed a review of literature. Results: Out of 377 pathology reports, 37 cases of DAC were identified, with a median age of 73 years and followed during 1-13 years. Six patients (16.22%) were refractory to conservative treatment and required surgery. In three patients (8.11%) evolution to ulcerative colitis (UC) was observed. Patients were divided into four endoscopic patterns, with a more benign course for type A “crescentic fold disease” compared to the other subtypes. Patients with type B “mild to moderate ulcerative colitis-like” were at significantly higher risk of persistent disease activity or relapse (p < 0.01). Conclusion: DAC is a multifaceted disease and considered to be a relatively benign condition. However, a subset of patients requires surgery and/or may progress to develop UC.

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