Brain Lesions May Serve as Diagnostic Criteria for Neuromyelitis Optica 2011;19(8):13. A majority of patients with neuromyelitis optica also experience disease-specific brain lesions that could help distinguish neuromyelitis optica from multiple sclerosis. MONTREAL—Imaging studies of patients with neuromyelitis optica (NMO)–spectrum disorders reveal that effects of the aquaporin-4 (AQP4) autoantibody, a marker of the disease, may extend beyond the spinal cord and optic nerve, according to researchers
Introduction and Pathophysiology Introduction Spasticity has been defined as an increase in muscle tone due to hyperexcitability of the stretch reflex and is characterized by a velocity-dependent increase in tonic stretch reflexes. [1] Spasticity usually is accompanied by paresis and other signs, such as increased stretch reflexes, collectively called the upper motor neuron syndrome. Paresis particularly affects distal muscles, with loss of the ability to perform fractionated movements of the digits
Pathogenic T cell responses against aquaporin 4. Pohl M , Fischer MT , Mader S , Schanda K , Kitic M , Sharma R , Wimmer I , Misu T , Fujihara K , Reindl M , Lassmann H , Bradl M . Source Department of Neuroimmunology, Center for Brain Research, Medical University Vienna, Spitalgasse 4, 1090, Vienna, Austria.
Koji Shinoda Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Japan Takuya Matsushita Department of Clinical Neuroimmunology, Graduate School of Medical Sciences, Kyushu University, Japan Konosuke Furuta Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Japan Noriko Isobe Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Japan Tomomi Yonekawa Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Japan Yasumasa Ohyagi Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Japan Jun-ichi Kira kira@neuro.med.kyushu-u.ac.jp Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Japan Abstract This report describes, for the first time, an occurrence of wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) in a 19-year-old female with neuromyelitis optica (NMO) spectrum disorder, who had anti-aquaporin-4 (AQP4) antibody. A high signal intensity lesion on T2-weighted MRI was detected in the midbrain tegmentum adjacent to the aqueduct, and presumably involved the medial longitudinal fasciculus bilaterally at the caudal levels. Plasma exchange resolved both WEBINO syndrome and the midbrain lesion
Inflammatory lesions in the central nervous system of patients with neuromyelitis optica are characterized by infiltration of T cells and deposition of aquaporin-4-specific antibodies and complement on astrocytes at the glia limitans. Although the contribution of aquaporin-4-specific autoantibodies to the disease process has been recently elucidated, a potential role of aquaporin- 4-specific T cells in lesion formation is unresolved. To address this issue, we raised aquaporin-4-specific T cell lines in Lewis rats and characterized their pathogenic potential in the presence and absence of aquaporin-4-specific autoantibodies of neuromyelitis optica patients
Background: Longitudinally extensive transverse myelitis (LETM) is defined clinically by acute transverse myelitis (ATM) and radiologically by extensive spinal cord (SC) lesions spanning three or more vertebral segments on Magnetic Ressonance Image (MRI). Even that LETM carries a considerable diagnostic challenge, has been regarded as a spectrum of Neuromyelitis optica (NMO). The seropositivity for NMO IgG, a specific biomarker of NMO, is variable in LETM patients around the world and reach 30-35% in brazilian series.
Background and Objective: Neuromyelitis optica (NMO) shows various brain MRI abnormalities with recurrent CNS attacks, although NMO predominantly affects the spinal cord and optic nerve. The features and pathomechanisms of acute brain lesions associated with edema have not been clarified in NMO. We investigated diffusion weighted imaging (DWI) of brain MRI lesions with the enhancement patterns in patients with neuromyelitis optica spectrum disorder (NMOSD).
We retrospectively analyzed and compared patterns of anti-aquaporin-4 (AQP4) immunoreactivity of autopsied brains of 2 patients with classical multiple sclerosis (MS) and 2 patients with neuromyelitis optica (NMO). Serological examination for NMO-IgG was not performed in all the cases. We confirmed that the expression of AQP4 is strongly inhibited in demyelinating lesions of NMO, accompanied by the loss of grial fibrillary acidic protein (GFAP) expression
Primary loss and dysfunction of astrocytes may trigger demyelination, as seen in neuromyelitis optica, an inflammatory disease of the central nervous system. In most patients affected by this disease, injury to astrocytes is initiated by the action of autoantibodies targeting aquaporin 4 (AQP-4), a water channel on astrocytes.
Neuromyelitis optica has not been thoroughly studied in Brazilian patients following the discovery of NMOIgG and its specific antigen aquaporin-4.
BACKGROUND: Neuromyelitis optica (NMO) is a neurological inflammatory disease associated with autoimmunity to aquaporin 4, predominantly localised in astrocytic foot processes. Recent studies have revealed that loss of aquaporin 4 and glial fibrillar acidic protein (GFAP) is a prominent feature of NMO lesions, suggesting astrocytic impairment. OBJECTIVE: To reveal a useful clinical biomarker of NMO.
We report the case of a 60-year-old woman with myasthenia gravis (MG) and Basedow’s disease who seven years after thymectomy developed subacute myelitis, a limited form of neuromyelitis optica (NMO). The patient presented with a centrally located long spinal cord lesion (LCL) on cervical cord MRI, anti-aquaporin 4 (AQP4) antibody in serum, and HLA DPB1*0501