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Aims: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated neuropathy with a highly variable clinical presentation. Hence, diagnosis is challenging and mostly relies on clinical suspicion. Various treatment approaches with different side effects and economical burden are available (steroids, IVIG, plasmapheresis or combined modality treatment) and their usage depends on the severity of the disease. Retrospectively, we investigated a cohort of CIDP patients with the aim to disclose diagnostic and therapeutic challenges which may have an impact in daily neurological practise.

Methods: We analysed clinical data, diagnostic workup and treatment of 18 adult patients with CIDP under intravenous immunoglobulin therapy (IVIG) from 2003-2014.

Results: We identified 15 men and 3 women with a mean symptom duration of 32 months before the diagnosis was made. CIDP was diagnosed in all ages (mean 59 years; range 18-78). Based on EFNS criteria clinical presentation was typical in 7 and atypical in 11 patients. Predominantly distal manifestation (DADS) was found in 1 patient, pure sensory symptoms in 9 patients and focal involvement of the brachial plexus in 1 patient. Just half of all patients fulfilled the electrophysiological criteria of prominent demyelinating features (EFNS/ INCAT criteria for CIDP). Supportive laboratory features such as elevated CSF protein were found in 15/18 patients. Nerve biopsy was performed in selected patients and showed demyelination features in 3 out of 7 patients. In 1 patient MRI abnormalities of proximal nerve fascicles at the cervicobrachial plexus could be detected. Ten patients received additional immunosuppressive therapy beside immunoglobulins, most often prednisone (n=9), followed by azathioprine (n=3), mycophenolat (n=3) and methotrexate (n=1). Interestingly, the IVIG-dose (between 0.4-1mg/kg/KG) and therapeutic interval (between 3-12 weeks) were highly variable. In 2 patients IVIG was stopped because of inefficacy or complete remissions.

Conclusions: In this retrospective cohort we illustrate the clinical, diagnostic and therapeutic variability in CIDP patients. Therefore, strict clinical and electrophysiological criteria of CIDP are less sensitive for daily routine, especially for atypical forms. Supportive criteria, especially elevated CSF protein, could be helpful to define the diagnosis. Of note, due to a mild initial presentation and atypical presentation the time from first symptoms to definite diagnosis was rather long. Difference in IVIG-dose and interval as well as the use of concomitant immunosuppression were also highly variable which calls for individual treatment approaches rather than a standard treatment.