Showing papers by "Natalio Fejerman published in 1993"

Neurofibromatosis Type One and West Syndrome: A Relatively Benign Association

Abstract: Summary: Fifteen patients with neurofibromatosis type one (NF1) and West syndrome (WS) were studied. The evolution of WS was clearly different from that seen in the other neurocutaneous syndromes, especially tuberous sclerosis. Steroids were efficacious and spasms disappeared. There was no relapse after discontinuation of steroids and antiepileptic drugs were succesfully stopped several years later. Mental outcome was also very good. The association of both conditions does not seem to be a coincidence. WS with NF1 seems remarkably benign and resembles idiopathic WS more than symptomatic WS.

Acute “axonal” Guillain–Barré syndrome in childhood

Abstract: We identified 5 of 44 consecutive children (11%) with Guillain–Barre syndrome who had electrophysiologic evidence of severe reduction of the mean amplitude of the compound motor action potentials (mean CMAP amplitude 10% of lower limit of normal. Children in the first group were more likely to require assisted ventilation (60% vs. 6.2%) and were more frequently quadriplegic at the peak of their disability (80% vs. 18.7%). They also required longer periods to improve one functional grade (mean 63.6 days vs. 16.6 days) and to become ambulatory (mean 156 days vs. 17.6 days). Moreover, only the children in the first group had distal atrophy of four limbs after 1 year of follow-up. Severe reduction of the mean amplitude of the CMAPs in children with GBS identifies a subgroup of patients with axonal damage that produces more severe weakness and delayed recovery. © 1993 John Wiley & Sons, Inc.