Morvan syndrome with positive anti LGI1/CASPR2 antibodies in serum/cerebrospinal fluid:a case report and literature review
10.3760/cma.j.cn112138-20211014-00705
- VernacularTitle:血清/脑脊液中抗LGI1/CASPR2抗体均阳性Morvan综合征1例报道并文献复习
- Author:
Litao ZHAO
1
;
Yingxin YU
;
Hairong QIAN
;
Sheng YAO
;
Xiaochen HAN
;
Jianguo LIU
;
Xiaokun QI
Author Information
1. 解放军总医院第六医学中心神经内科, 北京 100048
- Keywords:
Morvan syndrome;
Encephalitis, autoimmune;
Leucine rich glioma-inactivated 1;
Contactin-associated protein 2
- From:
Chinese Journal of Internal Medicine
2022;61(6):678-681
- CountryChina
- Language:Chinese
-
Abstract:
To report a typical case of Morvan syndrome with positive anti-leucine rich glioma-inactivated 1(LGI1) and contactin-associated protein 2 (CASPR2) antibodies in serum and cerebrospinal fluid. A 39-years-old female initially presented weakness of extremeties. The main symptoms included paroxysmal limb pain, wheezing, itching, muscle twitching, epilepsy, hypomnesia, dysphoria, apathy, intractable insomnia, salivation and sweating. Tests of electrolytes found hypokalemia (2.7-3.1 mmol/L) and hyponatremia (130-136 mmol/L). Arterial blood gas analysis showed hypoxemia (oxygen saturation 50%-70%). Total thyroxine (TT4) was elevated to 207 nmol/L with positive thyroid peroxidase antibody (TPO-Ab) and thyroglobulin antibody (TG-Ab). LGI1and CASPR2 antibodies (CBA method) were positive in both serum and cerebrospinal fluid, and the remaining antibodies related to autoimmune encephalitis and paraneoplastic syndrome were negative. Head MRI was almost normal, while mild abnormalities were found in electroencephalogram. Electromyography showed slightly increased voltage of left quadriceps motor unit potential. After treated with corticosteroids, IVIG and mycophenolate mofetil, the patient completely improved. Cognitive function scores recovered from MoCA/MMSE (16/24) to MoCA/MMSE (26/29). Positivity of LGI1/CASPR2 antibodies both in serum/cerebrospinal fluid are rarely seen in patients with Morvan syndrome. Steroids and immunosuppressants are suggested for treatment as early as possible.
