A patient with anti-aquaporin 4 antibody who presented with recurrent hypersomnia, reduced orexin (hypocretin) level, and symmetrical hypothalamic lesions

Nozaki, Hiroaki; Shimohata, Takayoshi; Kanbayashi, Takashi; Sagawa, Y; Katada, Shinichi; Satoh, Masahisa; Onodera, Osamu; Tanaka, Keiko; Nishizawa, Masatoyo

doi:10.1016/j.sleep.2007.11.022

Cited by 37 publications

(23 citation statements)

References 11 publications

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“…Medullary involvement caused vomiting and hiccupping; more extensive brainstem disease caused the eye-movement disorder, bulbar dysfunction and disordered control of respiration. Of particular interest are a group of patients with lesions in the hypothalamus and around the third and fourth ventricles;9 10 13 the regions of the brain richest in aquaporin 4 12. Disordered regulation of arousal9 13 and temperature9 secondary to hypothalamic lesions has been described in NMO, although such remarkable weight loss as seen in our case has not being reported.…”

Section: Discussionmentioning

confidence: 57%

Symptomatic, radiological and pathological involvement of the hypothalamus in neuromyelitis optica

Viegas

Weir

et al. 2009

Journal of Neurology, Neurosurgery & Psychiatry

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This study describes a young girl who presented with involuntary weight loss, spontaneous vomiting and behavioural change. Imaging confirmed hypothalamic and brainstem involvement. Routine investigations (including cerebrospinal fluid analysis and neuromyelitis optica IgG) were unhelpful. Biopsy of the hypothalamic lesion implicated an aggressive inflammatory aetiology. There was a response to conventional immunosuppression, while a further relapse responded to plasma exchange. She died 21 months after presentation. Postmortem examination was highly suggestive of neuromyelitis optica, which was subsequently confirmed following the identification of aquaporin 4 antibodies.

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Section: Discussionmentioning

confidence: 57%

Symptomatic, radiological and pathological involvement of the hypothalamus in neuromyelitis optica

Viegas

Weir

et al. 2009

Journal of Neurology, Neurosurgery & Psychiatry

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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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“…Recently, there have been several reports of NMO spectrum disorders that developed hypersomnia due to symmetrical hypothalamic lesions and a mild decrease of the hypocretin levels [1,4,8]. However, it was uncertain whether these patients had REM sleep abnormalities or not.…”

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confidence: 99%

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

et al. 2009

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A patient with anti-aquaporin 4 antibody who presented with recurrent hypersomnia, reduced orexin (hypocretin) level, and symmetrical hypothalamic lesions

Cited by 37 publications

References 11 publications

Symptomatic, radiological and pathological involvement of the hypothalamus in neuromyelitis optica

Symptomatic, radiological and pathological involvement of the hypothalamus in neuromyelitis optica

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

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

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