Cranial nerves are infrequently involved in typical chronic inflammatory demyelinating polyneuropathy, but involvement is more common in multifocal acquired demyelinating sensory and motor neuropathy (about 50%). The facial nerve is the most commonly affected cranial nerve, often bilateral.
In a large Italian database-derived, case-control study of dietary and environmental factors, antecedent infections and diet appeared to influence the onset and course of chronic inflammatory demyelinating polyradiculoneuropathy. The diagnosis and treatment of this disorder remain a challenge.
MRI of the brachial plexus and/or lumbar plexus may be helpful in making a diagnosis of chronic inflammatory demyelinating polyradiculopathy in patients who do not meet the standard criteria. Imaging findings include increased signal intensity, nerve hypertrophy, and nerve contrast enhancement.
Rituximab has been used “off label” for the treatment of primary chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with variable reports of success. These authors reported a high rate of response in patients who developed CIDP in the setting of other systemic immune-mediated disorders.
Acute inflammatory demyelinating polyneuropathy and acute-onset chronic inflammatory demyelinating polyneuropathy (CIDP) may present with identical clinical pictures and can be differentiated only with the passage of time. CIDP will have a slower course of progression and may involve relapses.
In the United States, first-line treatment for chronic inflammatory demyelinating polyneuropathy (CIDP) is intravenous immunoglobulin (IVIG). Authors of this retrospective survey from Europe found that corticosteroid treatment had similar efficacy as IVIG and should be considered as first-line therapy for CIDP.