Diagnosis
Carol L. Koski, M.D. and Richard A. Lewis, M.D., Principal Authors
A diagnosis of CIDP can be difficult because:
- the clinical heterogenicity of the disease presentation
- its multifocality and predilection for proximal nerve segments,
- the limitations that exist in electrophysiologic and pathologic techniques, and
- the lack of a specific diagnostic test. (Latov N, 2002)
The patient should be evaluated for CIDP with electrodiagnostic studies, and possibly cerebrospinal fluid (CSF) testing and a nerve biopsy if the following exist:
- symmetrical proximal and distal motor and sensory loss
- loss of deep tendon reflexes (DTRs) in affected extremities
- clinical course progressing for more than 2 months.
Early diagnosis and treatment may yield better functional recovery, most likely by minimizing endoneurial damage, including ongoing demyelination and secondary axonal loss. (DeSousa, 2006)
In making a diagnosis of CIDP, it is reasonable to look for other disorders or diseases which may be associated with CIDP but can also cause other forms of peripheral nerve damage. These include:
- collagen vascular disease (especially systemic lupus erythematosus),
- HIV infection, and
- diabetes mellitus.
Approximately 50% of patients have atypical presentations, which probably reflect the distribution of lesions. Clinically, the patient may experience both motor weakness and sensory loss. The weakness in the limbs is usually symmetric, but in some cases, the presentation may be asymmetric and either become symmetric over time or, if not, the disorder is designated as either the Lewis-Sumner syndrome or multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy. Other patients may have only motor impairments, while a small proportion present with sensory ataxia.
