Unlabelled: Recent studies suggest that sleep disorders are present in two-thirds of patients with autoimmune encephalitis. In anti-Ma2 encephalitis, hypersomnia appears to be frequent. However, only few cases of type 1 narcolepsy have been reported to date with anti-Ma2 encephalitis. We report 2 new cases of patients with narcolepsy secondary to anti-Ma2 encephalitis. Patient 1, a 68-year-old man, had narcolepsy type 1, including sleep attacks, cataplexy, abnormal Multiple Sleep Latency Tests and hypocretin-1 deficiency (< 50 ng/L) in the cerebrospinal fluid (CSF), associated with a cerebellar syndrome. Anti-Ma2 antibodies were present in the serum and CSF and antivoltage-gated potassium channel antibodies in the serum. He benefited from a treatment with pitolisant. Patient 2, a 42-year-old man, had narcolepsy type 2, including hypersomnolence, no cataplexy, intermediate CSF levels of hypocretin-1 (138 ng/L), abnormal Multiple Sleep Latency Tests, and a limbic encephalitis presentation. Anti-Ma2 antibodies were present in the serum and CSF, and anti-Ma1 antibodies were in the CSF. For both, repeated polysomnographies were necessary to establish the precise diagnosis of central hypersomnia, emphasizing the importance of carrying out sleep investigations in a tertiary neurology center with sleep medicine expertise in patients with anti-Ma2 encephalitis.
Citation: Brunet de Courssou J-B, Testard P, Sallansonnet-Froment M, et al. Narcolepsy secondary to anti-Ma2 encephalitis: two case reports. . 2023;19(4):837-841.
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http://dx.doi.org/10.5664/jcsm.10448 | DOI Listing |
Curr Opin Neurol
June 2024
Reference Centre for Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis, Hospices Civils de Lyon, Neurological Hospital, Bron.
Purpose Of Review: Immune checkpoint inhibitors (ICI) may trigger immune-related adverse events which rarely affect the central nervous system (CNS-irAEs). Over the past few years, cumulative data have led to the characterization of well defined syndromes with distinct cancer and antibody associations as well as different outcomes.
Recent Findings: The most frequent CNS-irAE is encephalitis, which includes three main groups: meningoencephalitis, a nonfocal syndrome usually responsive to corticosteroids; limbic encephalitis, associated with high-risk paraneoplastic neurological syndromes (PNS) antibodies (e.
Cureus
January 2024
Medicine and Surgery, Taibah University, Medina, SAU.
Cognitive dysfunction and behavioral change can be some of the manifestations of cancer, occurring as a part of paraneoplastic neurological syndrome, most commonly in small cell lung cancer. Paraneoplastic limbic encephalitis is the leading cause of cognitive disturbance and abnormal behavior in paraneoplastic syndromes, which is usually autoantibody-mediated. Autoantibodies are the main contributors to the development of cognitive dysfunction and behavioral change in cancer patients, with studies suggesting a higher liability for antibody-positive cancer patients to be affected.
View Article and Find Full Text PDFNeuroradiol J
April 2024
Department of Neuroradiology, Clinical Neuroscience Center, University Hospital Zurich, University of Zurich, Switzerland.
Introduction: MRI is the imaging modality of choice for assessing patients with encephalopathy. In this context, we discuss a novel biomarker, the "split ADC sign," where the cerebral cortex demonstrates restricted diffusion (high DWI signal and low ADC) and the underlying white matter demonstrates facilitated diffusion (high or low DWI signal and high ADC). We hypothesize that this sign can be used as a biomarker to suggest either acute encephalitis onset or to raise the possibility of an autoimmune etiology.
View Article and Find Full Text PDFNeurol Sci
May 2024
The "Double-First Class" Application Characteristic Discipline of Hunan Province (Clinical Medicine), Changsha Medical University, Changsha, Hunan, People's Republic of China.
Association between anti-GABAR encephalitis and myasthenia gravis is extremely rare with few reported cases. Herein, we report a case of a female patient diagnosed with anti-GABAR encephalitis and thymoma at the first admission. She was administered glucocorticoids for long-term immunotherapy, and thymectomy with biopsy demonstrated a type A thymoma.
View Article and Find Full Text PDFEpilepsy Behav Rep
October 2023
Rashid Hospital, Dubai, United Arab Emirates.
Anti-Ma2 encephalitis is an autoimmune disorder that typically involves the brainstem, limbic system, and diencephalon. It can be paraneoplastic and is more common in males. We describe an unusual presentation of anti-Ma2 encephalitis in a patient with an XY chromosome and a female phenotype.
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