Chronic Inflammatory Demyelinating Polyradiculoneuropathy in a 26 Month Old Boy

Introduction: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune mediated treatable disorder of the peripheral nerves, with predominant motor involvement and an insidious onset, or recurrent episodes. Children present slowly progressive or relapsing episodes of gait difficulty, distal symmetric weakness and sometimes paraesthesiae. Reflexes are absent or depressed. Laboratory findings include elevated cerebrospinal fluid (CSF) protein with no increase in cells. Electromyography shows demyelination.

Case Presentation: A previously healthy 26-month-old boy, brought to the emergency department due to imbalanced gait and muscle strength decrease, with two days of evolution. Neurological examination revealed unstable gait, without preferential side, and decrease in distal muscle strength. Reflexes were absent in the lower limbs, and decreased in the uppers. He also presented altered sensitivity.

From the study, we highlight, elevated CSF protein with no increase in cells, and negative microbiological and virological study. Electromyography evidenced severe demyelinating neuropathy. Immunoglobulin and carbamazepine was started. He was oriented to physiotherapy, with partial recovery of limbs strength.

One year later, presented a similar clinical episode, with overlapping study, namely the electromyography. Immunoglobulin was restarted, having performed on a monthly basis for 8 months.

At 9-years-old, there was a new recurrence, in post-infectious context, with affection of upper and lower limbs. Electromyography revealed severe demyelinating neuropathy, without alterations suggestive of acute lesion.

Nowadays, at 12-years-old, he has autonomous gait, with a marked distal motor deficit at lower limbs and slight decrease in upper limbs strength. He has patellar reflexes, but not aquilians.

Conclusion: Authors pretend to emphasize the challenges in the diagnosis and management of paediatric CIDP. Despite being a rare diagnosis, clinical suspicion, early recognition and investigation with prompt management can result in better outcomes of this potentially treatable neuropathy. We also highlight the importance of corticosteroids in this pathology, contrary to it role in Guillain-Barré syndrome.