P.216 Two interesting and unique cases of acquired neuropathies in pediatrics

Abstract

A 2-year-old boy known for an abdominal neuroblastoma was admitted after his fourth cycle of chemotherapy for generalized deterioration and pain when weight bearing. Multiple investigations and medication trials were made without finding the cause or relieving the patient's pain. While in hospital, the pain worsened and he developed gastroparesis, psychomotor regression and respiratory distress. A sensory polyneuropathy was confirmed by NCS, and the diagnosis of anti-Hu syndrome was subsequently made by CSF and serum paraneoplastic antibody panel. He received high-dose steroids, plasmapheresis, rituximab and cyclophosphamide, without clear improvement. After a few weeks, he underwent surgical resection of his neuroblastoma and was started on daratumumab bimonthly, mycophenolate mofetil and monthly IVIG after which he finally mildly improved. In summary, anti-Hu syndrome, while extremely rare in pediatrics, must be included in the differential diagnosis of refractory pain in children with a tumor diagnosis. A 15-year-old boy with no previous health issue presented with a subacute onset paraparesis. He had been recently diagnosed with diabetes of unknown type after a weight loss of 45 kg and started on insulin. He presented with progressive bilateral leg weakness, and the physical exam showed asymmetric weakness and decreased reflexes in the lower limbs, as well as sensory loss in a distal-to-proximal gradient. Investigations, such as a spine MRI and blood tests, showed no abnormality except for high HbA1c. The EMG and NCS confirmed involvement of multiple nerves and nerve roots with axonal loss. The final diagnosis was radiculo-plexo-neuropathy related to diabetes type II. The patient refused immunotherapy, and then completely recovered when his treatment was adjusted for diabetes type II.