Background: Chronic inflammatory demyelinating polyneuropathy (CIDP) represents a spectrum of treatable chronic immune-mediated disorders affecting peripheral nerves and nerve roots. CIDP typically presents with symmetric motor deficits, including proximal and distal weakness and areflexia. Sensory symptoms such as paresthesia and hypoesthesia are often more pronounced than pain and temperature alterations. The disease course can be progressive or marked by remitting-relapsing patterns. Treatment generally involves immunosuppressants or immunomodulators such as glucocorticoids, intravenous immunoglobulin, or plasma exchange. Diagnosis relies on electrodiagnostic evidence of peripheral demyelination, supplemented by laboratory tests, cerebrospinal fluid analysis, neuroimaging, and occasionally nerve biopsy. Distinguishing CIDP from other chronic demyelinating neuropathies is critical due to differing treatment approaches that significantly impact patient outcomes. Objective: To assess clinical features, electroneuromyographic findings, cerebrospinal fluid analysis, and imaging results in patients undergoing follow-up at our institution. Methods: A retrospective review of medical records, electroneuromyography, laboratory tests, and imaging studies was conducted. Results: Seventeen patients meeting CIDP diagnostic criteria were identified from our neuromuscular diseases out patient clinic. Coexisting conditions included well-controlled diabetes mellitus in two patients, Waldenströmma croglobulinemia in one, neurofibromatosis in another, and exacerbation post-influenza vaccination in one case. Cerebrospinal fluid analysis indicated elevated protein levels in all but one patient, averaging 326.37mg/dL (median: 345mg/dL, range: 36 – 964.3mg/dL). Electroneuromyography demonstrated demyelinating and axonal polyneuropathy in 11 patients, with demyelinating patterns in five and axonal patterns in one. Magnetic resonance imaging revealed root thickening in three out of tem patients tested. Genetic testing for hereditary neuropathies or the TTR gene panel was negative in all seven patients screened. Three patients showed poor response to treatment, including one with CIDP associated with Waldenström macroglobulinemia. Conclusion: This case series confirms the presenceof CIDP in all patients based on diagnostic criteria and consistent electroneuromyographic findings. Most patients exhibited elevated cerebrospinal fluid protein levels, and imaging studies were crucial in diagnosing and managing uncertain cases. Treatment out comes were generally favorable, underscoring the efficacy of current therapeutic modalities.
Panka et al. (Mon,) studied this question.