HyperCKemia Related to the Initial and Recurrent Attacks of Neuromyelitis Optica

Deguchi, Shoko; Deguchi, Kentaro; Sato, Kota; Yunoki, Taijun; Omote, Yoshio; Morimoto, Nobutoshi; Kurata, Tomoko; Ikeda, Masami; Takahashi, Toshiyuki; Akiyama, Masashi; Abe, Kōji

doi:10.2169/internalmedicine.51.7898

Cited by 32 publications

(29 citation statements)

References 8 publications

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“…Spinal cord lesions are regularly located in the white matter but involvement of grey matter has been shown before in MRI (Deguchi et al, 2012). However clinical and electrophysiological involvement of the second motor neuron have not been shown so far.…”

Section: Possible Second Motor Neuron Damage In Neuromyelitis Opticamentioning

confidence: 96%

Possible second motor neuron damage in neuromyelitis optica

Laemmer

Maihöfner

Gölitz

et al. 2014

Clinical Neurophysiology

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Section: Possible Second Motor Neuron Damage In Neuromyelitis Opticamentioning

confidence: 96%

Possible second motor neuron damage in neuromyelitis optica

Laemmer

Maihöfner

Gölitz

et al. 2014

Clinical Neurophysiology

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“…There are three case reports of AQP4 antibody seropositive NMO patients who developed hyperCKemia during the two weeks before the onset of CNS symptoms (i.e. hiccup, optic neuritis, or myelitis), and 2 of the three cases had interstitial pneumonia (17, 18, 34, 99, 116). The pathogenic role of AQP4 antibody outside the CNS however remains unclear.…”

Section: Nmo Pathology Outside Cnsmentioning

confidence: 99%

The Pathology of an Autoimmune Astrocytopathy: Lessons Learned from Neuromyelitis Optica

et al. 2013

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Neuromyelitis optica (NMO) is a disabling autoimmune astrocytopathy characterized by typically severe and recurrent attacks of optic neuritis and longitudinally-extensive myelitis. Until recently, NMO was considered an acute aggressive variant of multiple sclerosis (MS), despite the fact that early studies postulated that NMO and MS may be two distinct diseases with a common clinical picture. With the discovery of a highly specific serum autoantibody (NMO-IgG), Lennon and colleagues provided the first unequivocal evidence distinguishing NMO from MS and other CNS inflammatory demyelinating disorders. The target antigen of NMO-IgG was confirmed to be aquaporin-4 (AQP4), the most abundant water channel protein in the central nervous system (CNS), mainly expressed on astrocytic foot processes at the blood brain barrier, subpial and subependymal regions. Pathological studies demonstrated that astrocytes were selectively targeted in NMO as evidenced by the extensive loss of immunoreactivities for the astrocytic proteins, AQP4 and glial fibrillary acidic protein (GFAP), as well as perivascular deposition of immunoglobulins and activation of complement even within lesions with a relative preservation of myelin. In support of these pathological findings, GFAP levels in the cerebrospinal fluid (CSF) during acute NMO exacerbations were found to be remarkably elevated in contrast to MS where CSF-GFAP levels did not substantially differ from controls. Additionally, recent experimental studies showed that AQP4 antibody is pathogenic, resulting in selective astrocyte destruction and dysfunction in vitro, ex vivo, and in vivo. These findings strongly suggest that NMO is an autoimmune astrocytopathy where damage to astrocytes exceeds both myelin and neuronal damage. This chapter will review recent neuropathological studies that have provided novel insights into the pathogenic mechanisms, cellular targets, as well as the spectrum of tissue damage in NMO.

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“…Importantly, GABA A R are not only expressed in astroglial SEZ cells, but in general in adult astrocytes [25], the cell class most strongly expressing AQP4 in the CNS and the one mainly damaged by AQP4-IgG in NMO. We therefore recommend using GABA A R agonists in patients with NMO with caution, in particular during acute attacks, until an effect similar to that observed by Li et al can be excluded in the human brain (as well as in AQP4-expressing extra-CNS cells potentially targeted by AQP4-IgG [5,[26][27][28][29][30][31][32]). …”

Section: Hypothesismentioning

confidence: 91%