Symptomatic narcolepsy as a presenting feature of neuromyelitis optica

Kallollimath, Pradeep; Gujjar, Amruth; Patil, Megharanjini

doi:10.4103/aian.aian_74_18

Cited by 9 publications

(6 citation statements)

References 11 publications

(11 reference statements)

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“…Most are in the Japanese population, which has a higher prevalence of NMO-SD [120][121][122][123]. Additional cases have been identified outside of Japan [119,125,126,127]. Additional hypothalamic symptoms usually co-exist with hypersomnia, but cataplexy, hypnagogic hallucinations, and sleep paralysis have not been reported.…”

Section: Aquaporin-4 Antibody-associated Neuromyelitis Optica Spectru...mentioning

confidence: 99%

Sleep Disturbances Associated with Neurological Autoimmunity

Devine

Louis

2021

Neurotherapeutics

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Associations between sleep disorders and neurological autoimmunity have been notably expanding recently. Potential immune-mediated etiopathogenesis has been proposed for various sleep disorders including narcolepsy, Kleine-Levin syndrome, and Morvan syndrome. Sleep manifestations are also common in various autoimmune neurological syndromes, but may be underestimated as overriding presenting (and potentially dangerous) neurological symptoms often require more urgent attention. Even so, sleep dysfunction has been described with various neural-specific antibody biomarkers, including IgLON5; leucine-rich, glioma-inactivated protein 1 (LGI1); contactin-associated protein 2 (CASPR2); N-methyl-D-aspartate (NMDA)-receptor; Ma2; dipeptidyl-peptidase-like protein-6 (DPPX); alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPA-R); anti-neuronal nuclear antibody type-1 (ANNA-1, i.e., Hu); anti-neuronal nuclear antibody type-2 (ANNA-2, i.e., Ri); gamma-aminobutyric acid (GABA)-B-receptor (GABA-B-R); metabotropic glutamate receptor 5 (mGluR5); and aquaporin-4 (AQP-4). Given potentially distinctive findings, it is possible that sleep testing could potentially provide objective biomarkers (polysomnography, quantitative muscle activity during REM sleep, cerebrospinal fluid hypocretin-1) to support an autoimmune diagnosis, monitor therapeutic response, or disease progression/relapse. However, more comprehensive characterization of sleep manifestations is needed to better understand the underlying sleep disruption with neurological autoimmunity.

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Section: Aquaporin-4 Antibody-associated Neuromyelitis Optica Spectru...mentioning

confidence: 99%

Sleep Disturbances Associated with Neurological Autoimmunity

Devine

Louis

2021

Neurotherapeutics

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“…The myriad of neuropsychiatric presentations of NMOSD can mimic all those encountered clinically in lupus, and we suggest after ruling infections in lupus, it's worthwhile testing for AQP4 positivity, as it portends a relapsing course, culminating in damage accrual, and a progressive neurological disability. Unusual presentations for NMOSD including psychosis, altered sensorium, cognitive disturbances, apraxia, catatonia, autonomic disturbances, syndrome of inappropriate diuretic secretion (SIADH), and symptomatic narcolepsy [25][26][27][28] can also overlap with neuropsychiatric manifestations of lupus. 4,29 Testing for AQP4 becomes imperative in any neuropsychiatric manifestations of lupus, especially the refractory or relapsing subset, to rule out concomitant NMOSD.…”

Section: Discussionmentioning

confidence: 99%

Overlap syndrome of anti-aquaporin-4 positive neuromyelitis optica spectrum disorder and systemic lupus erythematosus: A systematic review of individual patient data

Kopp

Prasad

Naidu

et al. 2023

Lupus

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Background Neurological involvement can occur in systemic lupus erythematosus (SLE) due to co-existing neuromyelitis optica spectrum disorder (NMOSD). The symptoms can mimic those of neuropsychiatric manifestations of SLE. Pathogenic anti-aquaporin-4 (AQP4) antibodies, commonly found in NMOSD, are responsible for the neuroinflammatory response and secondary demyelinating lesions. These anti-AQP4 antibodies can be the drivers of neuroinflammatory process in SLE patients, which is distinct from the immunopathogenesis seen in traditional neuropsychiatric SLE. The clinical course is often a relapsing one and is managed differently. In this review, we describe and outline the clinical course and outcomes of AQP4+ NMOSD/SLE overlap cases. Methods To investigate the co-existence of SLE with AQP4+NMOSD, we conducted a systematic review of individual patient data from case reports and case series reported in major databases. The study extracted clinic-demographic features, imaging and laboratory profiles, treatment approaches, and outcomes of these patients. Inclusion criteria for the review required patients to have positivity for AQP4 or NMO in the blood and/or cerebrospinal fluid (CSF) and exhibit at least one manifestation of both NMOSD and SLE. Results In this overlap between SLE and AQP4+NMOSD, a high female preponderance was observed, with 42 out of 46 patients (91.3%) being female. Nearly half of the NMOSD cases (47.8%) had onset after lupus, with a median of 5 years between the two diagnoses. Hematological manifestations were seen in the majority of patients (63%), as well as longitudinally extensive transverse myelitis (87%), and brainstem involvement on imaging (29.6%). Cerebrospinal fluid analysis showed a dominantly lymphocytic pleocytosis, with oligoclonal bands being reported scarcely. Although cyclophosphamide was the most common steroid sparing agent used for maintenance, robust evidence for both efficacy and safety in AQP4+NMOSD is available for mycophenolate mofetil, azathioprine, and rituximab. The majority of reported cases showed a relapsing course, while one patient had a monophasic course. Conclusion AQP4+NMOSD in SLE patients is a relapsing and neurologically disabling disorder that can mimic neuropsychiatric manifestations, frequently occurs after the onset of lupus or may predate, responds to immunosuppressants, and necessitates indefinite treatment.

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“…52 The first case of narcolepsy type 1 due to a medical condition in NMOSD was reported from Japan and included in a series where the authors identified 4 MS and NMOSD patients with bilateral hypothalamic lesions and reduced CSF orexin levels. 53,54 Although reports of "symptomatic narcolepsy" 54 in NMOSD are increasing, there are only 17 reported cases to date 53,[55][56][57][58][59][60][61][62][63][64][65][66][67][68][69] ; (summarised in Table 5). It is noteworthy that narcolepsy is one of the core clinical characteristics included in the 2015 NMOSD IPND diagnostic criteria.…”

Section: Narcolepsy Typementioning

confidence: 99%

“…Narcolepsy was a forme fruste of NMOSD in 2 cases though more commonly occurred following other NMOSD manifestations. 53,67 Narcolepsy was also associated with autonomic and endocrine dysfunction 8/17 (47%) of cases. 55-57, 60, 61, 63, 64, 66 Most cases of hypersomnolence (12/17) resolved at least partially with immunotherapy though 4 cases had persistent somnolence.…”

Section: Narcolepsy Typementioning

confidence: 99%

The characterisation of sleep and circadian rhythm in neuromyelitis optica

Elhadd

Healy

Gibbons

et al. 2021

MRAJ

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Around a third of a human lifespan is spent in the essential life process of sleep. Disordered sleep is present in 15-35% of adults and is frequently underrecognized and underreported. Sleep disorders are associated with an elevated risk of hypertension, diabetes, obesity, depression, heart attack and stroke. The burden to health care associated with sleep disorders is significant and likely underestimated. Neuromyelitis optica spectrum disorder (NMOSD) is a relapsing autoimmune disorder of the central nervous system characterised by devastating relapses which can result in blindness, disability, and death. Symptomatic narcolepsy is a core feature of the disease and though sleep disruption is common, it has not been systemically studied in large NMOSD cohorts. We present a literature review concerning sleep and circadian rhythm in NMOSD and offer a conceptual model of possible underlying pathophysiological mechanisms with suggestions for future research.

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Symptomatic narcolepsy as a presenting feature of neuromyelitis optica

Cited by 9 publications

References 11 publications

Sleep Disturbances Associated with Neurological Autoimmunity

Sleep Disturbances Associated with Neurological Autoimmunity

Overlap syndrome of anti-aquaporin-4 positive neuromyelitis optica spectrum disorder and systemic lupus erythematosus: A systematic review of individual patient data

The characterisation of sleep and circadian rhythm in neuromyelitis optica

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