Neuromyelitis optica spectrum disorders: beyond longitudinally extensive transverse myelitis

Painful tonic spasms and brainstem involvement in a patient with neuromyelitis optica spectrum disorder

“…In a recent study, brainstem involvement in NMOSD is present in almost 15% of cases, while other authors find it in up to 32% of the cases [6,7]. The most characteristic brainstem lesion involves aria postrema, but in almost 2% of the cases, the involvement of brainstem corticopinal tract may be distinctive for NMO [7]. Carbamazepine is recognized as the first choice for PTS, but other drugs can be considered, as levetiracetam and gabapentine.…”

MRI differential diagnosis of suspected multiple sclerosis

“…14 In particular, longitudinal signal abnormality in the area postrema (dorsal medulla oblongata) has been described as specific for NMO. 14 Unlike MS, NMO cerebral T2-weighted hyperintensities are distributed along the ependymal surfaces and therefore do not form Dawson's fingers at the callososeptal interface as seen in MS. 12 Oedema of the corpus callosum giving rise to a "marbled" pattern of signal intensity is an infrequent finding, but highly characteristic feature of NMO. 12 The presence of bilateral optic neuritis extending to the optic chiasm is another feature suggestive of NMO over MS. 12 Acute disseminated encephalomyelitis (ADEM) ADEM is an acute inflammatory demyelinating disease, commonly triggered by viral infection or vaccination.…”

“…13 MS lesions do not typically extend further than 1.5 vertebral levels and affect less than half of the cord, usually posteriorly or laterally in axial section, whereas NMO involves the entire cord cross sectional area (Fig 2b) and may eventually progress to atrophy with syrinx formation. 14 The initial brain MRI in NMO may be normal or demonstrate non-specific brain white matter lesions that do not fulfil the dissemination in space criteria for MS. MRI obtained in later course of NMO can demonstrate lesions in regions of high AQP4 antibody expression, specifically the peri-ependymal white matter around the third ventricle, hypothalamus, periaqueductal grey matter and dorsal aspect of the brainstem adjacent to the fourth ventricle. 14 In particular, longitudinal signal abnormality in the area postrema (dorsal medulla oblongata) has been described as specific for NMO.…”

Caracterização do perfil de síndromes dolorosas, psicofísica e medidas de excitabilidade cortical em doentes com neuromielite óptica controlada

“…In 1996, studies still considered that incomplete myelitis was not a part of the syndrome (O'Riordan et al, 1996). Although Abercrombie, Devic and Gault described brainstem symptoms in those patients with NMO, it was not until the 21 st century that they were definitely considered part of the disease (Samart and Phanthumchinda, 2010;Popescu, et al, 2011;Kremer, et al, 2014;Lana-Peixoto, et al, 2014;Lemos, et al, 2015). Interestingly, seminal papers that defined the disease as an entity apart fromMS) proposed diagnostic criteria that excluded patients with symptoms other than neuritis and myelitis (Wingerchuk et al, 1999).…”

“…Brain lesions were thought to be atypical in NMO. Nowadays, it is known that up to 60% of patients do have encephalic lesions, most of them asymptomatic Ito et al, 2009;Samart and Phanthumchinda, 2010;Collongues et al, 2010a;Kim et al, 2010;Popescu et al, 2010 and2011;Pires et al, 2012;Sato et al, 2013;Papadopoulos and Verkman, 2013;Kremer et al, 2014;Lana-Peixoto et al, 2014;Kimura et al, 2014;Wingerchuk et al, 2015;Song et al, 2015;Lemos et al, 2015;Kim et al, 2015). Even the classical criteria of 3-or-more-vertebral-segments-LETM to diagnose NMO was challenged recently, with the acknowledgement of shorter and discontinuous lesions in this syndrome (Flanagan et al, 2015).…”