Unusual features in chronic inflammatory demyelinating polyneuropathy: Good outcome after prolonged ventilatory support

Abstract

Severe respiratory muscle paralysis and ventilatory failure is rare in chronic inflammatory demyelinating polyneuropathy (CIDP). We report a 14 year child who presented with respiratory failure, bulbar and multiple cranial nerves involvement along with bilateral phrenic nerve paralysis. He was diagnosed with CIDP after electrophysiological evaluation. He required AMBU ventilation for about 4 months (including domiciliary use), after which he recovered significantly. Along with several unusual features of CIDP, this report highlights good example of steady basic intensive care to save lives and rewarding outcome of prolonged respiratory support, provided by AMBU ventilation which is a rather primitive, but inexpensive device.

Unusual presentation of pediatric chronic inflammatory demyelinating polyneuropathy

“…However, she developed respiratory insufficiency due to phrenic nerve palsy in the fifth attack and was lost due to sepsis [12]. Jha et al [5] reported a case of a 14-yearold boy who developed bulbar and phrenic nerve palsy following insidiously progressive quadriparesis over 1 yr. The outcome was good after prolonged artificial manual breathing unit ventilation for 4 mo, including domiciliary use [5].…”

“…As mentioned above, few such reported cases were associated with phrenic nerve palsy. Lower cranial nerve palsy is also uncommon (5%) in CIDP [5]. The basis of the flaccid paralysis is conduction block due to demyelination; but axonal connections usually remain intact.…”

“…About 50% of patients of CIDP remain in remission after stoppage of steroids. Few patients may become refractory both to steroids and IVIG and may require plasmapheresis or other immune-modulators like cyclophosphamide, cyclosporine, azathioprine, interferon-α to maintain the patient in remission [5,15].…”

“…Symmetric motor weakness of the limbs accompanied with sensory symptoms is present in most cases, the initial attack sometimes being indistinguishable from GBS. Few patients may have cranial neuropathy, cerebellar ataxia and tremor, but respiratory failure is extremely rare [1,4,5]. Dalakas [6] reported the incidence of CIDP in general population to be 0.5-1.6/100,000 persons/ ___________________________________________ year and the prevalence to be 1-9/100,000 persons.…”

Chronic Inflammatory Demyelinating Polyneuropathy in Children: A Review of Clinical Characteristics and Recommendations for Treatment

“…Azathioprine is commonly administered when first-line therapy is unsuccessful or when a dose decline of corticosteroids is predicted. The doses of azathioprine consumption in childhood CIDP were 1 mg/ kg/day (in mixture with corticosteroids and IVIG), and 2 -3 mg/kg/day up to 50 -150 mg/day (28,48,58,65). Cyclosporine A inhibited the activation and proliferation of T cell.…”