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

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.

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MORVAN SYNDROME: A RARE CAUSE OF SYNDROME OF INAPPROPRIATE ANTIDIURETIC HORMONE SECRETION

Medicine and Pharmacy Reports ◽

10.15386/cjmed-755 ◽

2017 ◽

Vol 90 (3) ◽

pp. 353-355 ◽

Cited By ~ 1

Author(s):

Seref Demirbas ◽

Musa Baris Aykan ◽

Haydar Zengin ◽

Semir Mazman ◽

Kenan Saglam

Keyword(s):

Peripheral Nerve ◽

Antidiuretic Hormone ◽

Neuropsychiatric Symptoms ◽

Hormone Secretion ◽

Inappropriate Antidiuretic Hormone Secretion ◽

Voltage Gated ◽

Antidiuretic Hormone Secretion ◽

Potential Association ◽

Peripheral Nerve Hyperexcitability ◽

Autonomic Instability

The syndrome of inappropriate antidiuretic hormone secretion (SIADH) accounts for an important part of hyponatremia cases. The causes of SIADH can be detected almost always. As a rare disorder, Morvan Syndrome can be defined by the sum of peripheral nerve hyperexcitability, autonomic instability and neuropsychiatric features. Antibodies to voltage-gated potassium channels (Anti – VGKC-Ab) including contactin associated protein-like 2 antibodies (CASPR2-Ab) and leucine-rich glioma inactivated protein 1 antibodies (LGI1-Ab) were previously known for the potential association with this condition. We present a Morvan Syndrome in a patient who presented with various neuropsychiatric symptoms and SIADH.

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Small cell carcinoma of the cervix complicated by syndrome of inappropriate antidiuretic hormone secretion: a case report

Journal of International Medical Research ◽

10.1177/0300060520985657 ◽

2021 ◽

Vol 49 (2) ◽

pp. 030006052098565

Author(s):

Cai-Fu Zhao ◽

Su-Fen Zhao ◽

Ze-Qing Du

Keyword(s):

Cell Carcinoma ◽

Antidiuretic Hormone ◽

Small Cell Carcinoma ◽

Poor Prognosis ◽

Hormone Secretion ◽

Clinical Manifestations ◽

Small Cell ◽

Laboratory Examination ◽

Inappropriate Antidiuretic Hormone Secretion ◽

Antidiuretic Hormone Secretion

Small cell carcinoma of the cervix is a rare malignant tumor in the clinical setting. Clinical manifestations of this tumor are mostly similar to those of normal types of cervical cancer. Small cell carcinoma of the cervix only shows symptoms of neuroendocrine tumors, such as syndrome of inappropriate antidiuretic hormone secretion (SIADH). Most of the hyponatremia caused by SIADH can be managed after removal of the cause. Hyponatremia is a predictor of poor prognosis and can be used as an indicator of partial recurrence. We report a case of small cell carcinoma of the cervix complicated by SIADH. Our patient presented with irregular vaginal bleeding after menopause. After one cycle of chemotherapy, there was trembling of the limbs, and a laboratory examination showed low Na+ and low Cl− levels. After limited water intake, intravenous hypertonic saline, and intermittent diuretic treatment, the patient’s blood Na+ levels returned to normal. After a radical operation, the above-mentioned symptoms disappeared.

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CLINICAL SPECTRUM OF MORVAN SYNDROME: A SINGLE CENTRE STUDY

10.36106/ijar/1600858 ◽

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.

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THUR 153 Antibodies against the voltage-gated potassium channel complex

Journal of Neurology Neurosurgery & Psychiatry ◽

10.1136/jnnp-2018-abn.65 ◽

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.

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