Section 29. Disorders of the Nervous System >
Part 3. Acute Deterioration of Neurologic Function >
Chapter 556. Immune- and Inflammatory-Mediated Central Nervous System Syndromes
CNS Immune-Mediated Demyelinating DisordersMark P. Gorman
Topics Discussed: central nervous system dysfunction; cerebellar lesion; demyelinating autoimmune diseases, cns; demyelinating disease; demyelinating disease of central nervous system; encephalomyelitis, acute disseminated; immune-mediated neuropathies; multiple sclerosis; multiple sclerosis relapse; neurology; optic neuritis; pediatric neurology; transverse myelitis.
Sections: Immune-Mediated Cerebellar Syndromes, CNS Vasculitis, References.
Excerpt:"Demyelinating disorders comprise the largest subgroup within
CNS immune-mediated disorders. Myelin is composed of a lipid bilayer
of cholesterol, phospholipids, and glycolipids along with membrane-associated
proteins, such as proteolipid protein and myelin basic protein.1,2 In
the CNS, oligodendrocytes produce myelin, which surrounds axons
with periodic interruptions at nodes of Ranvier. The main functions
of myelin are to speed the conduction of action potentials along
axons and to support the development and maintenance of axons. In the past, variable terminology and lack of consistent definitions
in the literature hampered our understanding of pediatric CNS demyelinating
disorders. In April 2007, diagnostic definitions were proposed by
the International Pediatric Multiple Sclerosis (MS) Study Group.5 Although
they have not yet been prospectively validated, these definitions
provide a very useful framework both for clinical and research purposes. Acute disseminated encephalomyelitis (ADEM) is defined as an acute
or subacute inflammatory demyelinating event affecting multifocal
areas of the CNS with multiple accompanying symptoms, which must
include encephalopathy.5 The incidence of ADEM
is approximately 4 cases per 1 million persons under the age 20
per year.18 It is more common in children than
adolescents and adults, with a mean age of onset between 5 and 8 years
of age. Idiopathic, complete acute transverse..."
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