MOG‐antibody‐associated disorder with hypothalamic lesions associated with hypersomnia and decrease of orexin in CSF: A case report

Abstract

Background Myelin oligodendrocyte glycoprotein antibody‐associated disease (MOGAD) is an inflammatory disorder of the central nervous system. Here, we are the first to report a MOGAD patient with hypersomnolence caused by bilateral hypothalamic lesions. Case presentation The case involved a 51‐year‐old female patient with MOGAD who showed hypersomnolence. She had been diagnosed with MOGAD at 50 years old and admitted due to the development of optic neuritis and encephalitis while under corticosteroid and immunosuppressant treatment. These were improved after the introduction of intravenous methylprednisolone and plasma exchange. The orexin level in the cerebrospinal fluid (CSF) was decreased to 142.2 pg/mL (<200 pg/mL) upon admission and improved to a normal level (298.3 pg/mL) after immunotherapy. Bilateral hypothalamic lesions and reduction of the orexin level in the CSF were considered associated with hypersomnia. Conclusion The orexin level in CSF was useful to monitor hypothalamic dysfunction in a patient with MOGAD.

A comparative study of hypothalamic involvement in patients with myelin oligodendrocyte glycoprotein antibody‐associated disease, neuromyelitis optica spectrum disorder, and multiple sclerosis

“…In our case series, a greater percentage of MOGAD patients than NMOSD and MS patients received IVMP for treatment of hypothalamic lesions. In our study and previous case reports, immunotherapy, such as IVMP [18,19] or plasma exchange [19], resulted in neurological recovery and lesion resolution, suggesting a generally favourable outcome of hypothalamic involvement in MOGAD.…”

“…In addition to the previously reported hypersomnia [18,19], we also observed HPA axis dysfunction, including irregular menstruation and abnormal luteinizing hormone production, which has not been described before. Therefore, MOGAD patients should be aware of the potential for hypothalamic lesions if they have any of these clinical symptoms.…”

“…Previous articles that reported hypothalamic lesions in MOGAD patients were mostly reviews or small cohorts that summarized the radiological characteristics of MOGAD [ 23 , 24 , 25 , 26 , 27 ] and did not provide detailed information on the frequency, pathophysiology, or treatment of hypothalamic lesions. Only two recent case reports have described MOGAD patients with hypersomnia caused by hypothalamic lesions [ 18 , 19 ] and a reduced CSF orexin (hypocretin) level [ 19 ].…”

“…Notably, there is scant information regarding the prevalence and specific features of hypothalamic involvement in MOGAD and how it diverges from NMOSD and MS. Furthermore, reports on hypothalamic dysfunction in MOGAD are scarce [ 18 , 19 ]. Thus, hypothalamic involvement in MOGAD may be underrecognized and inadequately understood.…”

Sleep and neuroimmunology: a narrative review

“…Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), for which diagnostic criteria have only recently been established [ 8 ], has yet to be extensively studied in terms of sleep. Hypersomnia with hypothalamic lesions was reported in a MOGAD patient [ 53 ].…”