Pediatric CIDP: Diagnosis and Management. A Single-Center Experience

Abstract

Background: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare acquired polyneuropathy that especially among youngest children should be differentiated with hereditary neuropathies. Even though upon diagnosis treatment options are similar in children and adults, diagnostic challenges are faced in the pediatric population.Methods: We conducted a retrospective analysis of clinical symptoms, nerve conduction study results, modes of treatment, and final outcome in 37 children aged 3.5–17 years with a final diagnosis of CIDP (18 girls, 19 boys). We established three groups of patients based on age at onset of CIDP: 0–4, 4–13, and 13–18 years. Follow-up ranged from 10 to 222 months.Results: In our analysis, 19/37 patients (51.4%) had an atypical presentation: distal variant of CIDP in 12/37 patients (32.4%) and pure motor variant of CIDP in 5/37 patients (13.5%), and one patient had a pure sensory variant (1/37, 2.7%). Furthermore, 3/37 patients (8.1%) had additional concurring symptoms, including involuntary movements of face muscles (1/37, 2.7%) or hand tremor (2/37, 5.4%). During the follow-up, 23/37 patients (62.2%) received intravenous immunoglobulin (IVIg); 22/37 patients (59.5%) received steroids, 6/37 patients (16.2%) received IVIg and steroids, and 12/37 patients (32.4%) received immunosuppressive drugs, mostly azathioprine, but also methotrexate and rituximab. One patient was treated with plasmapheresis. Complete remission was achieved in 19/37 patients (51.4%) with CIDP in its typical form. Remission with residual symptoms or minimal deficit was observed in 4/37 patients (10.8%), whereas 14/37 patients (37.8%) remain on treatment with gradual improvement.Conclusion: Childhood CIDP may occur in its typical form, but even ~50% of children can present as an atypical variant including distal, pure motor, or pure sensory. Most children have a good prognosis; however, many of them may require long-term treatment. This highlights the importance of an early diagnosis and treatment for childhood CIDP.

Immune‐mediated polyneuropathy in cats: Clinical description, electrodiagnostic assessment, and treatment

“…41 Finally, corticosteroids are superior to no treatment in CIDP patients, which also differs from our results. [35][36][37]40,41 Our study had some limitations based on its retrospective nature.…”

“…First, according to a previous study, juvenile CIDP differed from the adult form in the absence of sex predilection and motor predominance 34 . More recent studies challenged these findings with mainly sensorimotor or sensory variants (40/45 patients) and chronic onset (>8 weeks) in the juvenile CIDP population 35,36 . Purely motor CIDP (only 2% of CIDP patients) was associated with chronic onset and a median age at onset of 48 years 37 .…”

Unusual Diagnosis and Extended Management of Chronic Inflammatory Demyelinating Polyneuropathy in an Unimmunized Patient

“…Furthermore, they recommend adding immunosuppressants or immunomodulators if the maintenance dose is inadequate. The prognosis of CIDP in pediatric populations is generally good, with a remission rate of 51.4% and follow up period ranging from 10-222 months [4]. In our patient's case, steroids were avoided as maintenance therapy, and plasmapheresis was not started as an option since patient was responding to IVIG every 3 weeks.…”

“…She was started on an IVIG regiment, as described in Łukawska, et al 2021. The study indicated significant symptom remission with an initial IVIG dose of 2 g/kg over 5 days followed by a maintenance dose of 0.4-1.2 g/kg every 2-6 weeks along with a steroid treatment [4]. The patient was discharged from the hospital five days later, after the initial IVIG dose was completed, in February 2022.…”

What Is in the Literature

“…A review of 37 children from one institution believed to have CIDP indicates that ∼50% had an atypical presentation, which included the distal variant, pure motor variant, and rarely the sensory variant. 10 Sixty-eight percent tested negative for common genes (above). Nerve conduction studies can be difficult to perform in children, but 35 patients fulfilled EFNS/PNS criteria.…”