Abstract
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a treatable chronic disorder of the peripheral nervous system. We retrospectively studied 30 children with a suspected diagnosis of CIDP. The diagnosis of CIDP was compared against the childhood CIDP revised diagnostic criteria 2000. Of the 30 children, five did not meet the criteria and four others met the criteria but subsequently had alternative diagnosis, leaving a total of 21 children (12 male) with CIDP as the final diagnosis. Thirteen children presented with chronic symptom-onset (>8 weeks). The majority presented with gait difficulties or pain in legs (n = 16). 12 children (57%) met the neurophysiological criteria and 18/19 (94%) met the cerebrospinal fluid criteria. Nerve biopsy was suggestive in 3/9 (33%), with magnetic resonance imaging supportive in 9/20 (45%). Twenty-one children received immuno-modulatory treatment at first presentation, of which majority (n = 19, 90%) received IVIG (immunoglobulin) monotherapy with 13 (68%) showing a good response. 8 children received second line treatment with either IVIG or steroids or plasmapharesis (PE) and 4 needed other immune-modulatory agents. During a median follow-up of 3.6 years, 9 (43%) had a monophasic course and 12 (57%) had a relapsing–remitting course. At last paediatric follow up 7 (33%) were off all treatment, 9 (43%) left with no or minimal residual disability and 6 (28%) children were walking with assistance (n = 3) or were non-ambulant (n = 3). Our review highlights challenges in the diagnosis and management of paediatric CIDP. It also confirms that certain metabolic disorders may mimic CIDP.
Highlights
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune mediated treatable disorder of the peripheral nerves, with predominant motor involvement and an insidious onset over months, or recurrent episodes [1]
Revised diagnostic criteria for childhood CIDP proposed by Nevo et al [1] (Table 1) were used to define criteria for diagnosing children with CIDP
Thirty children with CIDP suspicion were identified (Fig. 1) and their characteristics were compared with the re
Summary
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune mediated treatable disorder of the peripheral nerves, with predominant motor involvement and an insidious onset over months, or recurrent episodes [1]. Children present with slowly progressive or relapsing episodes of gait difficulty, distal symmetric weakness and sometimes paraesthesiae [2]. Laboratory findings include elevated cerebrospinal fluid (CSF) pro-. Electrophysiological and pathological studies show evidence of demyelination. The diagnosis of CIDP is usually straightforward, but atypical presentations can represent a significant diagnostic challenge [3]. Differential diagnoses include its acute counterpart, Guillain–Barré syndrome (GBS), as well as hereditary and metabolic causes of childhood polyneuropathy [2]
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