Narcolepsy as an initial manifestation of neuromyelitis optica with antiaquaporin-4 antibody

Baba, Toru; Nakashima, Ichiro; Kanbayashi, Takashi; Konno, Masatoshi; Takahashi, Toshiyuki; Fujihara, Kazuo; Misu, Tatsuro; Takeda, Atsushi; Shiga, Yusei; Ogawa, Hiroyasu; Itoyama, Yasuto

doi:10.1007/s00415-009-0139-4

Cited by 84 publications

(58 citation statements)

References 11 publications

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“…A number of hypothalamic symptoms, such as narcolepsy with decreased hypocretin-1 CSF level, anorexia and weight loss, hyperphagia and obesity, hypothermia or fever of unknown etiology, syndrome of inappropriate secretion of antidiuretic hormone, diffuse anhydrosis, bradycardia and hypotension, as well as recurring episodes of coma, have all been described in association with NMOSD 32,37-41 . Interestingly, enough in some patients, these symptoms have been observed preceding optic neuritis or myelitis, whereas in others they occurred in association with both AQP4-IgG seropositivity and hypothalamic abnormalities on brain MRI, but with no subsequent development of optic neuritis or myelitis [40][41][42][43] . Symptoms of encephalopathy may also occur in patients with established diagnosis of NMO or may in some patients precede the onset of optic neuritis or myelitis.…”

Section: Requirement Of Optic Neuritis or Myelitis Presence As A Pitfmentioning

confidence: 99%

The expanded spectrum of neuromyelitis optica: evidences for a new definition

Lana-Peixoto

Callegaro

2012

Arq. Neuro-Psiquiatr.

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Neuromyelitis optica (NMO) has been traditionally described as the association of recurrent or bilateral optic neuritis and longitudinally extensive transverse myelitis (LETM). Identification of aquaporin-4 antibody (AQP4-IgG) has deeply changed the concept of NMO. A spectrum of NMO disorders (NMOSD) has been formulated comprising conditions which include both AQP4-IgG seropositivity and one of the index events of the disease (recurrent or bilateral optic neuritis and LETM). Most NMO patients harbor asymptomatic brain MRI lesions, some of them considered as typical of NMO. Some patients with aquaporin-4 autoimmunity present brainstem, hypothalamic or encephalopathy symptoms either preceding an index event or occurring isolatedly with no evidence of optic nerve or spinal involvement. On the opposite way, other patients have optic neuritis or LETM in association with typical lesions of NMO on brain MRI and yet are AQP4-IgG seronegative. An expanded spectrum of NMO disorders is proposed to include these cases.

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Section: Requirement Of Optic Neuritis or Myelitis Presence As A Pitfmentioning

confidence: 99%

The expanded spectrum of neuromyelitis optica: evidences for a new definition

Lana-Peixoto

Callegaro

2012

Arq. Neuro-Psiquiatr.

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“…Hypersomnia -Excessive daytime sleepiness has been reported as a symptom of NMO spectrum disorder, associated with low levels of hypocretin-1, a hypothalamic neuropeptide for sleep-wake regulation, in the cerebrospinal fluids as seen in narcolepsy 22,23 . Nocturnal polysomnography and multiple sleep latency test revealed that our patient's sleep latency was less than ten minutes with sleep onset REM periods and the total sleep time was over nine hours.…”

Section: Uncommon Clinical Presentations In Nmomentioning

confidence: 99%

Atypical presentations of neuromyelitis optica

Sato

Fujihara

2011

Arq. Neuro-Psiquiatr.

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Neuromyelitis optica (NMO) is an inflammatory disease of central nervous system classically characterized by acute, severe episodes of optic neuritis and longitudinally extensive transverse myelitis, usually with a relapsing course. The identification of an autoantibody exclusively detected in NMO patients against aquaporin-4 (AQP-4) has allowed identification of cases beyond the classical phenotype. Brain lesions, once thought as infrequent, can be observed in NMO patients, but lesions have different characteristics from the ones seen in multiple sclerosis. Additionally, some AQP-4 antibody positive patients may present with a variety of symptoms not being restricted to optic neuritis and acute myelitis during the first attack or in a relapse. Examples are not limited to, but may include patients only with brain and/or brainstem lesions, narcolepsy with hypothalamic lesions or patients with intractable hiccups, nausea and vomiting. The prompt identification of NMO patients with atypical presentations may benefit these patients with institution of early treatment to reduce disability and prevent further attacks. Key words: neuromyelitis optica, aquaporin 4, myelitis, optic neuritis, diagnosis, differential, nausea, vomiting, hiccup, brain diseases.Apresentações atípicas da neuromielite óptica RESUMO Neuromielite óptica (NMO) é uma doença inflamatória do sistema nervoso central caracterizada classicamente por neurite óptica grave e mielite transversa longitudinalmente extensa, com um curso usualmente recorrente. A identificação do anticorpo detectado exclusivamente nos pacientes com NMO contra a aquaporina-4 (AQP-4) permitiu a identificação de casos além do fenótipo clássico. Lesões cerebrais, que antes eram descritas como infrequentes, podem ser observadas em pacientes com NMO, mas as lesões possuem características diferentes das lesões observadas na esclerose múltipla. Além disso, alguns pacientes positivos para o anticorpo contra a AQP-4 podem apresentar uma variedade de sintomas não restritos à neurite óptica e mielite aguda, seja durante o primeiro ataque seja em uma recorrência. Exemplos não estão limitados aos descritos a seguir, mas incluem pacientes com lesões cerebrais e/ou tronco cerebral, narcolepsia com lesões hipotalâmicas ou pacientes com quadros intratáveis de soluços, náusea e vômitos. A identificação rápida dos pacientes com NMO com apresentações atípicas pode beneficiar estes pacientes com a instituição precoce do tratamento a fim de reduzir a incapacidade e prevenir ataques subsequentes. Palavras-Chave: neuromielite óptica, aquaporina 4, mielite, neurite óptica, diagnóstico diferencial, náusea, vômito, soluço, encefalopatias.

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“…35,36,38,41 Lesions within the thalamus and hypothalamus are usually of this type and may be extensive. 36,42 While often asymptomatic, hypothalamic lesions have been described in patients with NMO with the syndrome of inappropriate antidiuretic hormone secretion 43 ; narcolepsy 44 ; behavioral change 42 ; and hypothermia, hypotension, hypersomnia, and obesity. 45 Dorsal brain stem lesions adjacent to the fourth ventricle, which may be contiguous with cervical spinal cord lesions, can result in a variety of bulbar symptoms, among which intractable hiccups, nausea, and vomiting are relatively specific for NMO and attributable to involvement of the area postrema and the nucleus tractus solitarius.…”

Section: Mr Imaging Lesion Distributionmentioning

confidence: 99%

Conventional and Advanced Imaging in Neuromyelitis Optica

Barnett

Sutton

Ghadiri

et al. 2013

American Journal of Neuroradiology

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SUMMARY:Myelitis and optic neuritis are prototypic clinical presentations of both multiple sclerosis and neuromyelitis optica. Once considered a subtype of multiple sclerosis, neuromyelitis optica, is now known to have a discrete pathogenesis in which antibodies to the water channel, aquaporin 4, play a critical role. Timely differentiation of neuromyelitis optica from MS is imperative, determining both prognosis and treatment strategy. Early, aggressive immunosuppression is required to prevent the accrual of severe disability in neuromyelitis optica; conversely, MS-specific therapies may exacerbate the disease. The diagnosis of neuromyelitis optica requires the integration of clinical, MR imaging, and laboratory data, but current criteria are insensitive and exclude patients with limited clinical syndromes. Failure to recognize the expanding spectrum of cerebral MR imaging patterns associated with aquaporin 4 antibody seropositivity adds to diagnostic uncertainty in some patients. We present the state of the art in conventional and nonconventional MR imaging in neuromyelitis optica and review the place of neuroimaging in the diagnosis, management, and research of the condition. ABBREVIATIONS: AQP4 ϭ aquaporin 4; LESCL ϭ longitudinally extensive spinal cord lesion; MT ϭ magnetization transfer; NMO ϭ neuromyelitis optica

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Narcolepsy as an initial manifestation of neuromyelitis optica with antiaquaporin-4 antibody

Cited by 84 publications

References 11 publications

The expanded spectrum of neuromyelitis optica: evidences for a new definition

The expanded spectrum of neuromyelitis optica: evidences for a new definition

Atypical presentations of neuromyelitis optica

Conventional and Advanced Imaging in Neuromyelitis Optica

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