Chronic inflammatory demyelinating polyneuropathy
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).
Patients typically present with a gradual and protracted (> 2 month) weakness of both proximal and distal musculature associated with areflexia and sensory changes 3.
In some patients, presentation can be acute and closely mimic Guillain-Barré syndrome (GBS) 2,4.
CIPD is an acquired demyelinating disease of the peripheral nervous system. Affected nerves demonstrate segmental infiltration with inflammatory cells (lymphocytes) and demyelination 4. Over time there is proliferation of Schwann cells and deposition of collagen resulting in thickening of the nerve and the characteristic onion bulb appearance 4. Involvement of the nerves results in changes of denervation in the supplied muscles 1.
MRI is the modality of choice for imaging the changes of CIDP, 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 5.
In addition, the supplied muscles will demonstrate changes of denervation 1:
- acute to subacute
- diffuse increase in T2 signal
- diffuse increased enhancement (seen best on fat suppressed T1 C+)
- fatty atrophy
Treatment and prognosis
CIDP can be treated with steroid, intravenous immunoglobulin (IVIG), plasmapheresis and immunosuppression 4.
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 2
- 1. Schulze M, Kötter I, Ernemann U et-al. MRI findings in inflammatory muscle diseases and their noninflammatory mimics. AJR Am J Roentgenol. 2009;192 (6): 1708-16. doi:10.2214/AJR.08.1764 - Pubmed citation
- 2. Li HF, Ji XJ. The Diagnostic, Prognostic, and differential value of enhanced MR imaging in Guillain-Barre syndrome. AJNR Am J Neuroradiol. 2011;32 (7): E140. doi:10.3174/ajnr.A2620 - Pubmed citation
- 3. Thawait SK, Chaudhry V, Thawait GK et-al. High-resolution MR neurography of diffuse peripheral nerve lesions. AJNR Am J Neuroradiol. 2011;32 (8): 1365-72. doi:10.3174/ajnr.A2257 - Pubmed citation
- 4. Merritt's Neurology. Lippincott Williams & Wilkins. (2010) ISBN:0781791863. Read it at Google Books - Find it at Amazon
- 5. Rentzos M, Anyfanti C, Kaponi A, Pandis D, Ioannou M, Vassilopoulos D. Chronic inflammatory demyelinating polyneuropathy: a 6-year retrospective clinical study of a hospital-based population. J Clin Neurosci. 2007 Mar;14(3):229-35. doi:10.1016/j.jocn.2006.11.001 - Pubmed citation
- primary demyelinating disorders
- clinically isolated syndrome (CIS)
- radiologically isolated syndrome (RIS)
multiple sclerosis (MS)
- McDonald diagnostic criteria for MS (current 2017 revision)
- neuromyelitis optica (NMO) (Devic disease)
- acute disseminated encephalomyelitis (ADEM) and acute haemorrhagic encephalomyelitis (AHEM)
- tumefactive demyelinating lesions
- transverse myelitis
- chronic inflammatory demyelinating polyneuropathy (CIDP)
- Guillain-Barre Syndrome (GBS)
- anti-MOG associated encephalomyelitis
- primary demyelinating disorders