chronische inflammatorische demyelinisierende Polyneuropathie
Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired demyelinating disease involving peripheral nerves, and is generally considered the chronic counterpart to Guillain-Barré syndrome (GBS).
Clinical presentation
Patients typically present with a gradual and protracted (>2 months) weakness of both proximal and distal musculature associated with areflexia and sensory changes .
In some patients, presentation can be acute and closely mimic Guillain-Barré syndrome .
Pathology
Chronic inflammatory demyelinating polyneuropathy is an acquired demyelinating disease of the peripheral nervous system. Affected nerves demonstrate segmental infiltration with inflammatory cells (lymphocytes) and demyelination . Over time there is proliferation of Schwann cells and deposition of collagen resulting in thickening of the nerve and the characteristic onion bulb appearance . Involvement of the nerves results in changes of denervation in the supplied muscles .
Radiographic features
MRI
MRI is the modality of choice for imaging the changes of chronic inflammatory demyelinating polyneuropathy, the hallmark of which is thickening and enhancement of peripheral nerves, brachial and lumbosacral plexus and nerve roots. In many cases, the nerves become so thickened that they resemble onion bulbs. Approximately one-third of patients have cranial nerve involvement .
In addition, the supplied muscles will demonstrate changes of denervation :
- acute to subacute
- diffuse increase in T2 signal
- diffuse increased enhancement (seen best on fat suppressed T1 C+)
- chronic
- fatty atrophy
Treatment and prognosis
Chronic inflammatory demyelinating polyneuropathy can be treated with steroid, intravenous immunoglobulin, plasmapheresis and immunosuppression .
Differential diagnosis
- Guillain-Barré syndrome (GBS)
- acute presentation of CIDP can be similar
- difficult to differentiate in the first 6 weeks
- after 6-8 weeks GBS should be improving whereas CIDP will demonstrate chronic inflammation
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Assoziationen und Differentialdiagnosen zu chronische inflammatorische demyelinisierende Polyneuropathie: