Acute Combined Central and Peripheral Nervous System Demyelination in Children

Adamovic, Tanja; Riou, Émilie; Bernard, Geneviève; Vanasse, Michel; Décarie, Jean‐Claude; Poulin, Chantal; Gauvin, France

doi:10.1016/j.pediatrneurol.2008.07.022

Cited by 26 publications

(26 citation statements)

References 32 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…These findings were consistent with those of Adamovic et al ,17 who showed that among 13 paediatric patients with acute simultaneous inflammatory demyelination of the CNS and PNS, 6 were bedbound or wheelchair users and one remained on mechanical ventilation at discharge. In our series, however, the simultaneous onset group showed improvements similar to or better than the temporarily separated onset group after immunotherapy, suggesting a high efficacy of immunotherapy for simultaneous onset CCPD, despite severe manifestations.…”

Section: Discussionsupporting

confidence: 92%

“…This difference may be because of the classification criteria as well as the shorter observation period of the simultaneous onset group. However, Adamovic et al 17 reported that among 13 paediatric patients with acute simultaneous inflammatory demyelination of both the CNS and PNS, recurrence was seen only in 2 (15.4%) cases. Accordingly, as in our series, the simultaneous onset cases were followed-up nearly 4 years on average.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

A nationwide survey of combined central and peripheral demyelination in Japan

Ogata

Matsuse

Yamasaki

et al. 2015

J Neurol Neurosurg Psychiatry

View full text Add to dashboard Cite

show abstract

Section: Discussionsupporting

confidence: 92%

Section: Discussionmentioning

confidence: 99%

A nationwide survey of combined central and peripheral demyelination in Japan

Ogata

Matsuse

Yamasaki

et al. 2015

J Neurol Neurosurg Psychiatry

View full text Add to dashboard Cite

show abstract

“…20,26 Fever and seizures are described more frequently in ADEM compared to other acute demyelinating syndromes. Combined central and peripheral demyelination has been reported, [27][28][29][30] but should prompt diligent differential diagnostic workup including screening for other immune-mediated disorders, as well as for leukoencephalopathies of genetic/metabolic origin (e.g., mitochondriopathies, Krabbe disease, X-linked Charcot-Marie-Tooth disease). 31,32 NEUROIMAGING FEATURES MRI T2-weighted and fluid-attenuated inversion recovery images typically demonstrate multiple hyperintense bilateral, asymmetric patchy and poorly marginated lesions.…”

mentioning

confidence: 99%

Acute disseminated encephalomyelitis

et al. 2016

View full text Add to dashboard Cite

Acute disseminated encephalomyelitis (ADEM) is an immune-mediated demyelinating CNS disorder with predilection to early childhood. ADEM is generally considered a monophasic disease. However, recurrent ADEM has been described and defined as multiphasic disseminated encephalomyelitis. ADEM often occurs postinfectiously, although a causal relationship has never been established. ADEM and multiple sclerosis are currently viewed as distinct entities, generally distinguishable even at disease onset. However, pathologic studies have demonstrated transitional cases of yet unclear significance. ADEM is clinically defined by acute polyfocal neurologic deficits including encephalopathy. MRI typically demonstrates reversible, ill-defined white matter lesions of the brain and often also the spinal cord, along with frequent involvement of thalami and basal ganglia. CSF analysis may reveal a mild pleocytosis and elevated protein, but is generally negative for intrathecal oligoclonal immunoglobulin G synthesis. In the absence of a specific diagnostic test, ADEM is considered a diagnosis of exclusion, and ADEM mimics, especially those requiring a different treatment approach, have to be carefully ruled out. The role of biomarkers, including autoantibodies like antimyelin oligodendrocyte glycoprotein, in the pathogenesis and diagnosis of ADEM is currently under debate. Based on the presumed autoimmune etiology of ADEM, the current treatment approach consists of early immunotherapy. Outcome of ADEM in pediatric patients is generally favorable, but cognitive deficits have been reported even in the absence of other neurologic sequelae. This review summarizes the current knowledge on epidemiology, pathology, clinical presentation, neuroimaging features, CSF findings, differential diagnosis, therapy, and outcome, with a focus on recent advances and controversies. Neurology ® 2016;87 (Suppl 2):S38-S45 GLOSSARY ADEM 5 acute disseminated encephalomyelitis; ADEM-ON 5 acute disseminated encephalomyelitis followed by optic neuritis; AHL 5 acute hemorrhagic leukoencephalopathy; IgG 5 immunoglobulin G; IPMSSG 5 International Pediatric Multiple Sclerosis Study Group; MDEM 5 multiphasic disseminated encephalomyelitis; MOG 5 myelin oligodendrocyte glycoprotein; MS 5 multiple sclerosis; NMOSD 5 neuromyelitis optica spectrum disorders; OCB 5 oligoclonal band.Acute disseminated encephalomyelitis (ADEM) is an immune-mediated demyelinating CNS disorder, characterized clinically by new-onset polyfocal neurologic symptoms including encephalopathy, coupled with neuroimaging evidence of multifocal demyelination. ADEM is classically considered a monophasic illness, with highest incidence in early childhood. The first descriptions of an ADEM-like disorder with recognition of a temporal relationship to infections (especially smallpox and measles) date back to the 18th century.1 Over a century later, an association of ADEM with vaccines, notably rabies, was reported. Mortality rates were high (up to 30% for ADEM following measles infection), and neur...

show abstract

“…Despite the fact that in both series the peripheral nervous system involvement was characterized mainly by a demyelinating pattern in nerve conduction velocity studies, significant amount of patients presented principally neurophysiologic characteristics of axonal injury (34.6% in adult series 50 and 46% in pediatric series 51 ), unusual finding for the Guillain-Barré syndrome in Western countries. 52,53 An additional unexplained finding is poor evolution and long-term outcome of patients with combined central and peripheral nervous system demyelination compared with patients with isolated central nervous system demyelination, 50,51 or isolated Guillain-Barré syndrome, 51,54 despite the immunomodulatory treatment used. Nevertheless, in our patient, neurophysiologic studies did not permit to differentiate between Guillain-Barré syndrome and critical illness polyneuromyopathy.…”

Section: Discussionmentioning

confidence: 99%

Critical Illness Polyneuromyopathy in a Child with Severe Demyelinating Myelitis

et al. 2009

Self Cite

View full text Add to dashboard Cite

We report a child presenting with severe demyelinating myelitis complicated with critical illness polyneuropathy. This previously healthy 8-month-old boy presented with acute superior limb weakness, absent tendon reflexes, and respiratory failure. Spinal magnetic resonance imaging showed an extensive cervical demyelinating lesion. Spinal cord trauma was suspected and high doses of dexamethasone were administered. Electromyography and nerve conduction studies showed absence of compound muscle action potentials and sural nerve sensory action potential, which was suggestive of a severe Guillain-Barré syndrome. However, intravenous immunoglobulins did not induce any improvement. Afterward, sural nerve biopsy showed a mild neuropathy, but muscle biopsy revealed abnormalities compatible with severe critical illness myopathy. After 5 months of evolution without improvement, the patient died following withdrawal of life support therapy. This case highlights the possible occurrence of critical illness polyneuromyopathy when treatment with corticosteroids are used in patients with acute demyelinating myelitis.

show abstract

Acute Combined Central and Peripheral Nervous System Demyelination in Children

Cited by 26 publications

References 32 publications

A nationwide survey of combined central and peripheral demyelination in Japan

A nationwide survey of combined central and peripheral demyelination in Japan

Acute disseminated encephalomyelitis

Critical Illness Polyneuromyopathy in a Child with Severe Demyelinating Myelitis

Contact Info

Product

Resources

About