Spectrum disorder of neuromyelitis optica in a patient presenting with intractable vomiting and hiccups, transverse myelitis and acute encephalopathy

Department of Neurophysiology, Liverpool Hospital, Elizabeth Drive, Liverpool 2170, NSW, Australia.
Journal of Clinical Neuroscience (Impact Factor: 1.38). 08/2012; 19(11):1576-8. DOI: 10.1016/j.jocn.2012.03.007
Source: PubMed


Optic neuropathy and transverse myelitis (TM) are common symptoms of multiple sclerosis (MS) but may also be seen in association with the antibody-mediated autoimmune disorder, neuromyelitis optica (NMO). We report a female patient presenting with intractable vomiting and hiccups and TM shortly followed by an acute encephalopathy, most likely due to NMO spectrum disorder. Serum and cerebrospinal fluid NMO antibodies were negative. Serial MRI abnormalities included longitudinally extensive TM of the cervical cord, focal T2-weighted hyperintensity of the area postrema and lesions in both thalami and the hypothalamus. Clinical and MRI involvement of these brain regions, which have high aquaporin expression, in conjunction with a spinal lesion extending over three vertebral segments strongly favoured a diagnosis of NMO. She required several courses of intravenous methylprednisolone and plasmapheresis before receiving intravenous rituximab therapy. NMO spectrum disorder should be considered in the differential diagnosis of atypical central nervous system presentations such as intractable vomiting and hiccups and acute encephalopathy. Recognition of this syndrome has significant implications as its treatment and prognosis differs from MS.

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    ABSTRACT: Neuromyelitis optica (NMO) is a severe autoimmune disease of the central nervous system characterized by spinal cord and optic nerve involvement. Brainstem manifestations have recently been described. To evaluate the time of occurrence, the frequency and the characteristics of brainstem symptoms in a cohort of patients with NMO according to the ethnic background and the serologic status for anti-aquaporin-4 antibodies (AQP4-abs). We performed a multicenter study of 258 patients with NMO according to the 2006 Wingerchuk criteria and we evaluated prospectively the frequency, the date of onset and the duration of various brainstem signs in this population. Brainstem signs were observed in 81 patients (31.4%). The most frequently observed signs were vomiting (33.1%), hiccups (22.3%), oculomotor dysfunction (19.8%), pruritus (12.4%), followed by hearing loss (2.5%), facial palsy (2.5%), vertigo or vestibular ataxia (1.7%), trigeminal neuralgia (2.5%) and other cranial nerve signs (3.3%). They were inaugural in 44 patients (54.3%). The prevalence was higher in the non-Caucasian population (36.6%) than in the Caucasian population (26%) (p<0.05) and was higher in AQP4-ab-seropositive patients (32.7%) than in seronegative patients (26%) (not significant). This study confirms the high frequency of brainstem symptoms in NMO with a majority of vomiting and hiccups. The prevalence of these manifestations was higher in the non Caucasian population.
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    ABSTRACT: Neuromyelitis optica (NMO, Devic's syndrome) is a clinical syndrome characterized by optic neuritis and (mostly longitudinally extensive) myelitis. If untreated, NMO usually takes a relapsing course and often results in blindness and tetra- or paraparesis. The discovery of autoantibodies to aquaporin-4, the most abundant water channel in the CNS, in 70-80% of patients with NMO (termed NMO-IgG or AQP4-Ab) and subsequent investigations into the pathogenic impact of this new reactivity have led to the recognition of NMO as an autoimmune condition and as a disease entity in its own right, distinct from classic multiple sclerosis. Here, we comprehensively review the current knowledge on the role of NMO-IgG/AQP4-Ab, B cells, T cells, and the innate immune system in the pathogenesis of NMO.
    Advances in Immunology 01/2014; 121:213-42. DOI:10.1016/B978-0-12-800100-4.00006-4 · 5.96 Impact Factor
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