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Neuromyelitis optica spectrum disorder is uncommon in children, and often seronegative for aquaporin-4 immunoglobulin G (AQP4-IgG). We conducted a retrospective study of 67 children presenting to a single Australian center with acquired demyelinating syndromes over a 7-year period. All patients were tested for AQP4-IgG. Five children (7.5%) had neuromyelitis optica spectrum disorder. One child was seropositive for AQP4-IgG (1.5%) and had a relapsing disease course with mild residual deficits. She also had a concomitant motor axonal neuropathy that improved with immunosuppressive therapy. Of the remaining 4 children, 3 had a monophasic course and 1 a relapsing course. Two were tested for anti–myelin oligodendrocyte glycoprotein (anti-MOG) antibody and both were seropositive. This study confirms that neuromyelitis optica spectrum disorder is uncommon in children, and that AQP4-IgG seropositivity is rare. Anti-MOG antibodies should be tested in children with neuromyelitis optica spectrum disorder. [ABSTRACT FROM AUTHOR]

Duchenne muscular dystrophy, an X-linked disorder, has an incidence of one in 5000 boys and presents in early childhood with proximal muscle weakness. Untreated boys become wheelchair bound by the age of 12 years and die of cardiorespiratory complications in their late teens to early 20s. The use of corticosteroids, non-invasive respiratory support, and active surveillance and management of associated complications have improved ambulation, function, quality of life and life expectancy. The clinical features, investigations and management of Duchenne muscular dystrophy are reviewed, as well as the latest in some of the novel therapies. [ABSTRACT FROM AUTHOR]

The clinical and radiological features of childhood acute transverse myelitis are compared to those of acute disseminated encephalomyelitis with spinal cord involvement in 22 children with acute transverse myelitis and 12 children with acute disseminated encephalomyelitis with spinal cord involvement. Children with acute transverse myelitis were more likely to have a sensory level (55%) and areflexia. Sixty-eight percent of the children with acute transverse myelitis, and 92% of children with acute disseminated encephalomyelitis had longitudinally extensive transverse myelitis. Demyelination was more extensive in acute disseminated encephalomyelitis (mean 15.6 vertebral segments) than in acute transverse myelitis (mean 8.0 vertebral segments). The outcome was normal to good in 82% with acute transverse myelitis and in 100% with acute disseminated encephalomyelitis. Persistent bladder dysfunction was uncommon in both. Poor prognostic factors in acute transverse myelitis are flaccid paraparesis, respiratory failure, and age less than 6 months. These clinical and radiological differences suggest acute transverse myelitis and acute disseminated encephalomyelitis are separate entities. [ABSTRACT FROM AUTHOR]

We analyzed the medical records and cerebral imaging of 30 children with early onset multiple sclerosis to compare the clinical and neuro-radiological features with acute demyelinating encephalomyelitis and adult multiple sclerosis. The female:male ratio was 1.3 before the age of 12 years and increased to 4.3 after age 12 years. Optic neuritis and pyramidal dysfunction were the most common presenting clinical features. The most common radiological features at first imaging were periventricular (77.2%), corpus callosal demyelination (63.6%), gadolinium enhancement (62.5%), and Dawson's fingers (40.9%). The median relapse rate per patient year was 1.2 ([IQR] 0.6-2.0). Seven patients reached a moderate Expanded-Disability-Status-Scale score of 3 and 3 patients were more severely affected (score of 6). Expanded-Disability-Status-Scale score increased by 0.7 for every relapse. Ten patients were commenced on immunomodulatory therapy (Interferon beta 1a, n = 9; Interferon beta 1b, n = 3; or Glatiramer acetate, n = 1), the majority of who had minimal side effects and a reduction in relapse rate. [ABSTRACT FROM AUTHOR]