Congenital Guillain-Barré syndrome associated with maternal inflammatory bowel disease is responsive to intravenous immunoglobulin.
A 34-week floppy preterm infant born to a mother with acute ulcerative colitis presented with a progressive reduction in spontaneous limb movements, severe generalized hypotonia, areflexia, autonomic dysfunction and respiratory failure. Electromyography revealed pronounced denervation activity and markedly slow nerve conduction velocity (3 m/s) with evidence of conduction block. These findings indicated demyelination with additional axonal features. The infant was diagnosed with congenital Guillain-Barré syndrome, was treated with intravenous immunoglobulin and showed clinical improvement within 48 hours of treatment. The relationship between inflammatory bowel syndrome and inflammatory demyelinating polyneuropathy is discussed.
Bamford
Nigel S
NS
Department of Neurology, Columbia University College of Physicians and Surgeons, Neurological Institute, New York, NY 10032, USA. nsb4@columbia.edu
Trojaborg
Werner
W
Sherbany
Ariel A
AA
De Vivo
Darryl C
DC
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D003093:Colitis, Ulcerative / Q000175:diagnosis* / Q000188:drug therapy; D004576:Electromyography / Q000187:drug effects; D005260:Female; D005500:Follow-Up Studies; D020275:Guillain-Barre Syndrome / Q000151:congenital* / Q000188:drug therapy; D006801:Humans; D007116:Immunization, Passive; D007223:Infant; D007231:Infant, Newborn; D007235:Infant, Premature, Diseases / Q000188:drug therapy*; D008297:Male; D009046:Motor Neurons / Q000187:drug effects / Q000502:physiology; D018482:Muscle, Skeletal / Q000294:innervation; D009460:Neurologic Examination / Q000187:drug effects; D011247:Pregnancy; D011248:Pregnancy Complications / Q000175:diagnosis* / Q000188:drug therapy
D000305:Adrenal Cortex Hormones