Chronic Inflammatory Demyelinating Polyradiculoneuropathy

CNS/PNS Boundary Transgression by Central Glia in the Absence of Schwann Cells or Krox20/Egr2 Function

“…In this respect, the experiments discussed above and designed to elucidate the basis of the segregation between Schwann cells and central glia will be of particular interest, because their translation to humans might provide the basis for future applications in human pathology. This might be the case for PNS disorders such as chronic inflammatory demyelinating polyradiculoneuropathy (Burns, 2004). This potentially disabling immune-mediated disease results in multifocal inflammation and demyelination of peripheral nerves predominating in nerve roots.…”

Chronic Inflammatory Demyelinating Polyneuropathy in Children: A Review of Clinical Characteristics and Recommendations for Treatment

“…CIDP is an autoimmune disorder and demyelination is segmental and occurs everywhere from the nerve roots to the distal portion of nerves. In addition to demyelination, biopsy of nerve reveals inflammatory infiltrates and subperineural edema (4,5). The diagnosis of CIDP is based on electrodiagnosis that shows the severity of the disease, lumbar puncture and spine magnetic resonance imaging (MRI).…”

“…Upper extremity weakness, hand tremor (19), and ataxia (23) are also seen in some cases. There is also reduced or absence of deep tendon reflexes (24). Some studies showed that at least one-third of pediatric patients may have sensory symptoms such as paresthesias, dysesthesias and large fiber sensory loss (25).…”

“…These contain infectious diseases such as HIV and Lyme disease; metabolic disorders such as uremia, hypothyroidism or vitamin B12 deficiency; inflammatory origins such as vasculitis; exposure to toxins such as Glue sniffing, lead or heavy metals and also diphtheria, lymphoma and porphyria (4,25,44). In patient with chronic course, inherited neuropathies would be more noticeable in differential diagnosis, particularly since the onset is usually in childhood.…”

“…These include metachromatic leukodystrophy, Krabbe's disease, hereditary motor sensory neuropathies (HMSN) (Charcot-Marie-tooth disease) with types 1a, 1b, X, and hereditary liability to pressure palsies. Distinguishing between hereditary and acquired neuropathies includes long-standing weakness without relapses, distal weakness more than proximal weakness, typically more severe sensory loss, and uniform rather than patchy involvement of nerves on electrophysiologic testing (4). The sensory loss may be more prominent than CIDP.…”