 |  | Rudolph's Pediatrics Section 29. Disorders of the Nervous System > Part 3. Acute Deterioration of Neurologic Function > Chapter 556. Immune- and Inflammatory-Mediated Central Nervous System SyndromesMark P. Gorman | |
Topics Discussed: central nervous system dysfunction; hashimoto's encephalitis; immune-mediated neuropathies; neurology; neuropsychiatric systemic lupus erythematosus; pediatric neurology; rheumatic disorders; vasculitis, central nervous system.
Sections: References.
Excerpt:"Inflammation of the CNS blood vessels can be restricted to the
CNS (primary) or associated with a number of systemic conditions
(secondary). Primary CNS vasculitis of childhood can be diagnosed
based on the following criteria: newly acquired neurologic deficit,
conventional or magnetic resonance (MR) angiographic or histologic
evidence of CNS vasculitis, and no identifiable associated systemic
condition.145 Secondary CNS vasculitis can be associated
with CNS infections or systemic autoimmune disorders. The differential
diagnosis also includes noninflammatory conditions such as moyamoya
disease, sickle cell disease, and arterial dissection. This section
will focus on primary CNS vasculitis of childhood. The incidence
of this disorder is unknown, but there are an estimated 150 to 200
new cases per year in North America with a median age of presentation
of 7 years.145,146Neurological symptoms frequently complicate systemic autoimmune
disorders, including systemic lupus erythematosus (SLE), sarcoidosis,
Sjogren syndrome, and antiphospholipid antibody syndrome, among
others.148 Among these, SLE is the most common
in children and will be the focus of this section. The pathogenesis
of neuropsychiatric SLE is uncertain, but likely involves vascular
abnormalities including vasculitis and thrombosis, as well as primary
parenchymal disease mediated by autoantibodies and cytokines. Antiphospholipid
antibodies play an important role and should be checked in all patients
with..."
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