Back to Search
Start Over
Morvan syndrome: clinical and serological observations in 29 cases
- Source :
- Annals of Neurology
- Publication Year :
- 2016
-
Abstract
- Objective: A study was undertaken to describe the clinical spectrum, voltage-gated potassium channel (VGKC) complex antibody specificities, and central nervous system localization of antibody binding in 29 patients diagnosed with Morvan syndrome (MoS). Methods: Clinical data were collected using questionnaires. Radioimmunoassay, cell-based assays, and mouse brain immunohistochemistry were used to characterize the serum antibodies. Results: Neuromyotonia (100%), neuropsychiatric features (insomnia 89.7%, confusion 65.5%, amnesia 55.6%, hallucinations 51.9%), dysautonomia (hyperhidrosis 86.2%, cardiovascular 48.3%), and neuropathic pain (62.1%) were the most common manifestations. A total of 93.1% of MoS patients were male. VGKC-complex antibodies were present in 23 of 29 (79%) MoS patients at referral; 24 of 27 available sera had CASPR2, LGI1, or both CASPR2 and LGI1 antibodies (3 also with contactin-2 antibodies). CASPR2 antibodies were generally higher titer than LGI1 antibodies. Tumors (41.4%), mainly thymomas, were associated with CASPR2 antibodies and a poor prognosis, whereas LGI1 antibodies were associated with serum hyponatremia. In brain tissue regions including the hypothalamus, raphe, and locus coeruleus, commercial antibodies to LGI1 bound to neuronal cell bodies including the antidiuretic hormone-secreting and orexin-secreting hypothalamic neurons, whereas CASPR2 commercial antibodies bound more often to the neuropil. MoS antibodies bound similarly, but there was evidence of additional antibodies in some sera that were not adsorbed by LGI1- or CASPR2-expressing cells and bound to mouse Caspr2−/− tissue. Interpretation: MoS is clinically distinct from other VGKC-complex antibody-associated conditions, and usually is associated with high-titer CASPR2 antibodies, often accompanied by lower-titer LGI1 antibodies. CASPR2 and LGI1 antibodies bind to multiple brain regions, which helps to explain the multifocal clinical features of this disease, but other antibodies are likely to play a role in some patients and need to be characterized in future studies. ANN NEUROL 2012
- Subjects :
- Male
Serum
Pathology
Neuromyotonia
International Cooperation
Serology
Mice
0302 clinical medicine
Surveys and Questionnaires
Aged, 80 and over
Neurons
0303 health sciences
biology
Intracellular Signaling Peptides and Proteins
Brain
Radioimmunoassay
Middle Aged
3. Good health
Potassium channel complex
Treatment Outcome
Neurology
Potassium Channels, Voltage-Gated
Immunohistochemistry
Female
Antibody
medicine.symptom
Protein Binding
Adult
medicine.medical_specialty
Pain
Nerve Tissue Proteins
Antibodies
Morvan's syndrome
Young Adult
03 medical and health sciences
Contactin 2
medicine
Animals
Humans
Aged
Retrospective Studies
030304 developmental biology
Orexins
business.industry
Neuropeptides
Membrane Proteins
Proteins
Dysautonomia
medicine.disease
Syringomyelia
Immunology
biology.protein
Neurology (clinical)
business
030217 neurology & neurosurgery
Subjects
Details
- Language :
- English
- Database :
- OpenAIRE
- Journal :
- Annals of Neurology
- Accession number :
- edsair.doi.dedup.....4d36746cfbe81754ced1f241a9127291