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Abstract
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.
Results: Median age at symptom onset was 66 years. Of 38 patients, 34 were male. Median time to nadir of disease was 4 months (in 30% >1 year). The most frequent syndromes included limbic encephalitis (42%) and Morvan syndrome (29%). Seventy-seven percent of the patients had ≥3 of the following symptoms: encephalopathy (cognitive deficits/seizures), cerebellar dysfunction, peripheral nervous system hyperexcitability, dysautonomia, insomnia, neuropathic pain, or weight loss. A tumor, mostly thymoma, occurred in 19% of the patients. Immunoglobulin G4 subclass antibodies were present in all patients; 63% also had immunoglobulin G1 antibodies. Treatment response occurred in 93% of the patients and 25% had clinical relapses.
Conclusions: Caspr2 antibodies associate with a treatable disorder that predominantly affects elderly men. The resulting syndrome may vary among patients but it usually includes a set of well-established symptoms. Recognition of this spectrum of symptoms and consideration of the protracted clinical course are important for early diagnosis of this disorder. Prompt immunotherapy and tumor therapy (if needed) often result in improvement.
GLOSSARY
- Caspr2=
- contactin-associated protein-like 2;
- CBA=
- cell-based assay;
- FLAIR=
- fluid-attenuated inversion recovery;
- IgG=
- immunoglobulin G;
- IHC=
- immunohistochemistry;
- IVIg=
- IV immunoglobulin;
- LE=
- limbic encephalitis;
- LGI1=
- leucine-rich glioma-inactivated 1;
- MG=
- myasthenia gravis;
- NMDAR=
- NMDA receptor;
- PNH=
- peripheral nerve hyperexcitability;
- RIA=
- radioimmunoassay;
- VGKC=
- voltage-gated potassium channel
Footnotes
↵* These authors contributed equally to this work.
Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Supplemental data at Neurology.org
- Received December 4, 2015.
- Accepted in final form April 25, 2016.
- © 2016 American Academy of Neurology
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- Author response: Morvan syndrome and severe hyponatremia
- Morvan syndrome and severe hyponatremia
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