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

Viegas, Stuart; Weir, Andrew; Mm, Esiri; Kuker, Wilhelm; Waters, Patrick; Leite, M I; Vincent, Angela; Palace, Jacqueline

doi:10.1136/jnnp.2008.157693

Cited by 53 publications

(36 citation statements)

References 19 publications

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“…34 An initial episode at the level of the organum vasculosum of lama terminalis would present with fever episodes reflecting NMOIgG binding-induced cytokine action at the level of hypothalamus 35 or with hypothermia in the more advanced settings of destructive NMO hypothalamic lesions. 34,36 Febrile episodes have been described preceding NMO exacerbations and even as initial presenting NMO symptoms 11 but they have been attributed to viral infections. Third, autoantibodies must enter or be locally synthesized in sufficient concentrations to be pathogenic in the CNS.…”

Section: Resultsmentioning

confidence: 99%

Neuromyelitis optica unique area postrema lesions

Popescu

Lennon²,

Parisi³

et al. 2011

Neurology

265

218

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Objective: To characterize the neuropathologic features of neuromyelitis optica (NMO) at the medullary floor of the fourth ventricle and area postrema. Aquaporin-4 (AQP4) autoimmunity targets this region, resulting in intractable nausea associated with vomiting or hiccups in NMO.Methods: This neuropathologic study was performed on archival brainstem tissue from 15 patients with NMO, 5 patients with multiple sclerosis (MS), and 8 neurologically normal subjects. Logistic regression was used to evaluate whether the presence of lesions at this level increased the odds of a patient with NMO having an episode of nausea/vomiting.Results: Six patients with NMO (40%), but no patients with MS or normal controls, exhibited unilateral or bilateral lesions involving the area postrema and the medullary floor of the fourth ventricle. These lesions were characterized by tissue rarefaction, blood vessel thickening, no obvious neuronal or axonal pathology, and preservation of myelin in the subependymal medullary tegmentum. AQP4 immunoreactivity was lost or markedly reduced in all 6 cases, with moderate to marked perivascular and parenchymal lymphocytic inflammatory infiltrates, prominent microglial activation, and in 3 cases, eosinophils. Complement deposition in astrocytes, macrophages, and/or perivascularly, and a prominent astroglial reaction were also present. The odds of nausea/ vomiting being documented clinically was 16-fold greater in NMO cases with area postrema lesions (95% confidence interval 1.43-437, p ϭ 0.02).

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Section: Resultsmentioning

confidence: 99%

Neuromyelitis optica unique area postrema lesions

Popescu

Lennon²,

Parisi³

et al. 2011

Neurology

265

218

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“…In one case, the anorexia was associated with intractable nausea and vomiting and an inflammatory lesion involving the area postrema (4). In the other patient, the anorexia was accompanied by psychotic symptoms and was associated with an inflammatory lesion of the hypothalamus (5).…”

Section: Introductionmentioning

confidence: 91%

Anorexia Heralding the Onset of Neuromyelitis Optica

Iorio¹,

Plantone²,

Damato³

et al. 2013

Intern. Med.

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Neuromyelitis optica (NMO) is an inflammatory disease of the central nervous system (CNS) that preferentially affects the optic nerves and spinal cord. An autoantibody (NMO-IgG) targeting the aquaporin-4 water channel distinguishes NMO from other inflammatory disorders of the CNS. Recent studies have demonstrated that the area postrema and other circumventricular organs (CVOs) can be targeted in NMO.We herein report the case of a 12-year-old girl who experienced anorexia six months before the onset of NMO. Anorexia caused by hypothalamic or CVO dysfunction may herald the onset of NMO.

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“…However symptomatic lesions involving supratentorial white matter are exceptional and extensive (Pittock et al, 2006b). Even if a favourable outcome after PLEX has been reported in a few severe cases (Viegas et al, 2009;Watanabe et al, 2009ab), comparative data are still lacking.…”

Section: Brain Attacksmentioning

confidence: 99%