Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) Diagnosis and Treatment

Clinical Presentation and Diagnosis

CIDP progresses over more than 2 months, distinguishing it from acute conditions like Guillain-Barré syndrome, which progresses over days to weeks 1
Cerebrospinal fluid analysis typically shows cytoalbuminologic dissociation (elevated protein with normal cell count) 1
Electrophysiological studies show evidence of demyelination and help distinguish CIDP from other neuropathies 1
MRI of the brachial or lumbosacral plexus can help identify focal or diffuse peripheral nerve abnormalities 1
Nerve biopsy may be useful in evaluating atypical forms of CIDP 1

Multifocal Acquired Demyelinating Sensory and Motor Neuropathy (MADSAM or Lewis-Sumner Syndrome) is characterized by asymmetric involvement with preserved reflexes in areas not affected by weakness 1
Chronic neurovisceral variants may present with slower progression of neurological symptoms 2
Guillain-Barré syndrome (acute onset with progression over days to weeks) should be considered in the differential diagnosis 1
Diabetic polyradiculoneuropathy, leptomeningeal metastases, infectious causes, and systemic inflammatory disorders like lupus should also be considered in the differential diagnosis 1
Other peripheral neuropathies including those associated with metabolic disorders, toxins, or nutritional deficiencies should be considered in the differential diagnosis 3, 4

For severe or progressing symptoms, consider pulse corticosteroids (methylprednisolone 1g IV daily for 3-5 days) plus IVIG 2g/kg over 5 days 5
Plasmapheresis may be used in severe cases not responding to other therapies 5
Rituximab may be considered in consultation for cases with limited improvement 5

Dysphagia may occur in some cases, according to the American Society of Clinical Oncology, in patients with CIDP 6