Plasmapheresis therapy in children with inflammatory demyelinating disorders of the CNS

Inflammatory demyelinating disorders of the CNS in children are treated with high dose steroids. If symptoms persist, more invasive treatment options are recommended. We discuss two patients: one with acute transverse myelitis and one with chronic relapsing remitting Multiple Sclerosis. A 9 year old boy presented with monoparesis of the left leg after influenza vaccination. Despite pulse steroid therapy clinical symptoms progressed to tetraparesis and affection of the respiratory function. MRI showed first extensive, then complete myelitis of the spinal cord without brain involvement. Cerebrospinal fluid showed disturbed blood-CSF barrier without pleocytosis or intrathecal antibody production. Detailed serological and immunological investigations (antinuclear and aquaporin 4 antibodies included) revealed no abnormal results. Therapy was switched to plasmapheresis (8 cycles alltogether) and a single cyclophosphamide pulse. To date, progression could be stopped and clinical symptoms are resolving slowly. A girl was diagnosed with relapsing remitting Multiple Sclerosis at the age of 9 years according to the Mc Donald criteria. Symptoms at two relapses at the age of 8 and 9 years consisted of facial palsy and facial paresthesias. Complete remission always followed steroid therapy. MRI showed scattered white matter lesions and OCBS in CSF. She was started on IVIG and then IFN-beta 1a subcutaneously. At the age of 13 years she acutely developed generalised ataxia, dysarthria, spasticity, bladder incontinence and paresthesias (EDSS 5.0). MRI showed a high lesion load with contrast enhancement. Steroids were only partially effective. Plasmapheresis was effectuated for a total of 7 cycles. After 2 weeks the patient showed minor truncal ataxia and minor fine motor skills disturbances (EDSS 2.5). MRI was free of contrast enhancing lesions. We demonstrate in two different cases that plasmapheresis therapy in patients with inflammatory demyelinating disorders of the CNS was safe and improved neurological symptoms.

multiple sclerosis - plasmapheresis therapy - transverse myelitis