Multiphasic demyelinating disease involving central and peripheral nervous system in a child
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Studies on animal models show that both CNS and peripheral nervous system (PNS) axons may be demyelinated in experimental allergic encephalomyelitis (EAE).1 Conversely inflammatory demyelinating peripheral neuropathies are rarely associated with CNS demyelinating diseases in humans.2
We describe a 10-year-old child who developed a multiphasic CNS demyelinating disease and 18 months later an inflammatory demyelinating peripheral neuropathy. Both central and peripheral myelin involvement improved after IV immunoglobulin (IVIg) treatment.
Case report.
A 10-year-old child developed fever (37.5 °C), headache, neck pain, and left leg weakness (June 1999). Blood samples showed leucocytosis (12.8 103/L [normal 4.10 to 9.80]); EEG and brain and spinal cord MRI showed no abnormality; and CSF was clear with elevated cell count (179 n°/mm3 [normal < 3]). There was a normal protein level, and no oligoclonal bands were present. No viruses, bacteria, antibodies against neurotrophic virus, or Borrelia burgdorferi were found. In the following days the patient’s neurologic conditions worsened: he developed stupor, vomiting, meningism, visual loss, quadriplegia with increased deep tendon reflexes, and …
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