Childhood-onset chronic inflammatory demyelinating polyradiculoneuropathy with cranial nerve involvement.
A 17-year-old male presented with chronic diplopia and generalized motor weakness. He was previously diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy and acute disseminated encephalomyelitis in childhood. Cranial magnetic resonance imaging (MRI) revealed a rarely reported finding of thickening and enhancement of multiple cranial nerves. Nerve conduction studies and electromyography showed peripheral nerve demyelination with axonal involvement. There was improvement in the clinical examination, MRI, and electrophysiologic studies after combined corticosteroid and plasma exchange therapy. We review the clinical presentation, course, and response to therapy among children with chronic inflammatory demyelinating polyradiculoneuropathy, with specific emphasis on the frequency and pattern of cranial nerve involvement.
Costello
Fiona
F
Department of Ophthalmology, University of Iowa Hospitals and Clinics, Iowa City, Iowa 52242, USA.
Lee
Andrew G
AG
Afifi
Adel K
AK
Kelkar
Praful
P
Kardon
Randy H
RH
White
Matthew
M
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D003131:Combined Modality Therapy; D003389:Cranial Nerve Diseases / Q000150:complications* / Q000628:therapy; D003922:Diabetes Mellitus, Type 1 / Q000150:complications; D004172:Diplopia / Q000209:etiology; D004576:Electromyography; D004673:Encephalomyelitis, Acute Disseminated / Q000150:complications; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D018908:Muscle Weakness / Q000209:etiology; D010951:Plasma Exchange; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000150:complications*; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones