Acute, severe, central and peripheral nervous system combined demyelination

doi:10.1016/0887-8994(86)90040-8

Pediatric Neurology

Volume 2, Issue 1, January–February 1986, Pages 47-50

https://doi.org/10.1016/0887-8994(86)90040-8 Get rights and content

Abstract

Acute disseminated encephalomyelopathy and Guillain-Barré syndrome are both immunologically mediated para-infectious demyelinating disorders, the former affecting the central nervous system and the latter affecting the peripheral nervous system. The term encephalo-myelo-radiculo-neuropathy was introduced to describe cases in which major involvement of one system, most commonly the peripheral, was associated with mild involvement of the other. We present a case of acute severe demyelination simultaneously affecting both the central and the peripheral nervous systems in a 10-year-old female. This clinical picture combines acute disseminated encephalomyelopathy and Guillain-Barré syndrome, both of which are extremely severe.

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Cited by (41)

Combined central and peripheral demyelination: A case description
2017, Neurologia
A 3-year-old boy with GuillaineBarré syndrome and encephalitis associated with Mycoplasma pneumoniae infection
2014, Journal of Infection and Chemotherapy
Citation Excerpt :
Conduction velocity of peripheral motor nerves was abnormally low 6 weeks after the onset of neurological symptoms and was barely within normal limits 1 year later. Amit et al. described a 10-year-old girl with GBS associated with acute disseminated encephalo-myelopathy [8]. She had a deep coma, and her contrast-enhanced CT revealed diffuse multifocal enhancement of the white matter and an extensive hyperlucent area in the anterior part of the corpus callosum on acute episodes.
Mycoplasma pneumoniae is a common cause of respiratory tract illness in children. Among the most common extrapulmonary manifestations are disorders of the central nervous system, including meningitis, meningoencephalitis, cerebellitis, polyneuropathy, acute disseminated encephalomyelitis, and Guillain–Barré syndrome.
Guillain–Barré syndrome, also known as acute inflammatory demyelinating polyradiculoneuropathy, is an acute-onset, immune-mediated disorder of the peripheral nervous system. The central nervous system is usually intact in patients with Guillain–Barré syndrome. However, there have been some reports of an association of Guillain–Barré syndrome with central nervous system involvement in children.
We report a 3-year-old boy with M. pneumoniae infection associated with Guillain–Barré syndrome and encephalitis.
Both serum anti-GM1 ganglioside (IgG and IgM) and anti-galactocerebroside IgG antibodies were detected in our patient: the former in the earlier stage of the disease, and the latter in the later stage. We speculate that anti-GM1 ganglioside was associated more with encephalitis, and anti-galactocerebroside antibody was associated more with GBS in our case.
Our patient is the youngest report of Guillain–Barré syndrome with central nervous system involvement, and the first report of a pediatric patient with associated M. pneumoniae infection. Such cases are rarely reported, but highlight the need for awareness of the association of the infection with Guillain–Barré syndrome with central nervous system involvement.
Acute disseminated encephalomyelitis
2014, Handbook of Clinical Neurology
Citation Excerpt :
Symptoms can range from non-specific, such as malaise and fatigue, to fulminant, progressing rapidly to obtundation and coma. The clinical course of ADEM is classically monophasic, with most patients (70–77%) reporting an antecedent infection or vaccination (Amit et al., 1986; Hynson et al., 2001; Tenembaum et al., 2002). In children, a prodromal phase precedes the neurologic symptoms and consists of malaise, fever, headache, nausea, and vomiting, progressing within days to maximal neurologic symptoms.
Acute disseminated encephalomyelitis (ADEM) is an acute multifocal demyelinating disease of the central nervous system that typically follows an infectious illness. Its clinical course in most cases is monophasic; however, relapsing ADEM is rarely seen, which poses a diagnostic challenge for distinguishing this disease from multiple sclerosis (MS). Although typically encountered in children, it also occurs in adults with disease characteristics slightly different from the pediatric cases. Formerly, ADEM occurred particularly often in children with measles. However, the illness most often follows a non-descript viral or even bacterial infectious illness. ADEM occurs throughout the world, and may even be more common in less-developed countries, where MS is rare, than in developed ones, where MS is common. Children seldom get MS as opposed to adults, indicating that ADEM constitutes a distinct entity from MS. The prognosis of ADEM is generally good, but severe neurologic sequelae after ADEM are occasionally seen. In this chapter, the etiology, clinical/laboratory/radiologic characteristics, treatment options, and prognosis of ADEM are discussed.
The Magnetic Resonance Imaging Appearance of Monophasic Acute Disseminated Encephalomyelitis. An Update Post Application of the 2007 Consensus Criteria.
2013, Neuroimaging Clinics of North America
Citation Excerpt :
With that in mind, the neurologic features that have previously been noted in ADEM include: unilateral or bilateral pyramidal signs (60%–95%); cranial nerve palsies (22%–89%); hemiparesis (76%–79%); ataxia (18%–65%); hypotonia (34%–47%); seizures (10%–47%); visual loss due to optic neuritis (7%–23%); and speech impairment (5%–21%).3,7–9,11,13,14,16,17 Peripheral nervous system involvement has been reported in the adult cohort (with frequencies up to 43.6% of patients), but is considered rare in childhood ADEM patients.35–38 Only 2 studies have looked at presenting signs and symptoms of patients with ADEM according to the new consensus definitions.
Acute Disseminated Encephalomyelitis
2010, Blue Books of Neurology
This chapter describes the proposed clinical and pathologic definitions of acute disseminated encephalomyelitis (ADEM) and discusses the clinical utility and limitations of individual criteria for ADEM. A practical approach for evaluation and treatment of ADEM for clinicians are outlined. ADEM is traditionally considered an uncommon monophasic, acute or subacute, multifocal, idiopathic inflammatory demyelinating disease (IIDD) that predominantly involves the white matter of the central nervous system (CNS). The pathologic hallmark of ADEM is perivenular inflammation with limited sleeves of demyelination. Retrospective studies using various clinical criteria for ADEM led to several clinical, laboratory, and imaging observations that are associated with a final diagnosis of ADEM versus multiple sclerosis (MS). The utility and limitations of these observations for application in clinical practice are discussed. Multiple clinical factors should be considered in combination before arriving at a probable diagnosis of ADEM, including (1) presenting signs and symptoms; (2) conventional and advanced MRI abnormalities; (3) sufficient follow-up confirming a monophasic course based on clinical and/or neuroimaging criteria; (4) lack of alternative diagnosis; and (5) brain histopathology if available. It is unclear whether ADEM presents differently in adults than in children, specific criteria should be applied with caution in adults.
Demyelinating disease of central and peripheral nervous systems associated with a A8344G mutation in tRNALys
2009, Neuromuscular Disorders
We describe a patient with acute combined demyelinating disease of the central and peripheral nervous systems associated with the A8344G mutation in the mitochondrial tRNA lysine gene. A 7-year-old boy presented with acute onset of palpitations, tinnitus, ataxia, bilateral sixth nerve palsy, and flaccid quadriparesis. Serum creatine kinase and lactate were mildly increased. Electromyography showed demyelinating sensory and motor polyneuropathy. Brain magnetic resonance imaging demonstrated demyelination in the left thalamus and magnetic resonance spectroscopy revealed a lactate peak corresponding to this lesion. Histologic analysis of the muscle showed cytochrome c-oxidase-deficient fibers and ragged red fibers. Respiratory chain analyses revealed deficiencies of complexes I and IV. Molecular genetic analyses of the muscle showed an A8344G (MERRF) mutation in mitochondrial tRNA lysine. To the best of our knowledge, this is the first description of this mutation associated with acute combined demyelinating disease of the central and peripheral nervous systems.

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Case reportAcute, severe, central and peripheral nervous system combined demyelination

Abstract

J Neurol Sci

Papilledema and Landry-Guillain-Barré Syndrome. Case reports and review

Brain

Encephalo-Myelo-Radiculo-Neuropathy: Involvement of the CNS in children with Guillain-Barré-Strohl Syndrome

Dev Med Child Neurol

L'Encephalomyelo-radiculoneuropathy. La participation du systeme nerveux central dans la polyradiculite aigue de l'enfant

Arch Fr Pediatr

Guillain-Barré disease (Encephalo-myeloradiculitis): A review of 33 cases

J Lancet

Disseminated vasculomyelinopathy

Acta Neurol Scand

Case report
Acute, severe, central and peripheral nervous system combined demyelination