An Atypical Pediatric Presentation of a Chronic Polyradiculoneuropathy

慢性多发性神经根神经病在儿科的非典型表现

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Abstract

Here, we present a case of a 15-year-old male with polyradiculoneuropathy, which was diagnosed as chronic inflammatory demyelinating polyneuropathy (CIDP), who was refractory to initial treatment. The patient presented with a one-and-a-half-month history of decreased strength, most notable in the bilateral hip flexors and finger flexors/extensors, and areflexia. Electromyography and nerve conduction studies did not fulfill diagnostic criteria for a demyelinating polyneuropathy; however, the cerebrospinal fluid analysis demonstrated albuminocytologic dissociation and his physical exam was otherwise consistent with the diagnosis. He was treated with IV immunoglobulin (IVIg). He relapsed less than one month later with worsening weakness. Imaging revealed increased cauda equina enhancement when compared to the MRI from the previous admission, and labs were otherwise similar to the initial presentation. He was treated with a second course of IVIg in addition to high-dose IV methylprednisolone. Upon his second discharge, he was transitioned to oral corticosteroids, and at a follow-up visit one month later, he had fully regained his strength and demonstrated normal reflexes. This case highlights the variable nature of CIDP in its initial presentation, its course, and its response to treatment, particularly in young patients. Additionally, we would like to emphasize that this case of CIDP was in the context of chronic malnutrition and significant weight loss, which made the diagnostic picture more complex.

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