Assessment of serum-mediated neurotoxicity in Navajo neuropathy. Electromyogr Clin Neurophysiol 2000 Jun;40(4):211-4
Date
07/25/2000Pubmed ID
10907598Scopus ID
2-s2.0-0033932019 (requires institutional sign-in at Scopus site) 4 CitationsAbstract
Navajo neuropathy is a unique sensorimotor neuropathy which is geographically restricted to Navajo children living on the Navajo Reservation. Affected patients present with weakness, loss of sensation in extremities, corneal ulcerations, and a high incidence of childhood infections. Metabolic complications, such as severe liver disease, may further contribute to peripheral nerve injury in affected patients. In this study, serum-mediated injury to rat peripheral nerve was critically assessed. Serum samples from affected Navajo patients were tested in vivo for effects on peripheral nerve function. Injection of serum from affected Navajo patients into rat sciatic nerve produced a modest slowing of nerve conduction velocity without effecting evoked-compound muscle action potential (CMAP) amplitudes. By comparison, injection of serum from patients with MGUS neuropathy, an immune-mediated disorder, diminished evoked-CMAP amplitudes by approximately 70%. Navajo neuropathy sera had no effect in vitro on the neurite outgrowth of developing dorsal root ganglia neurons. The results argue against serum-mediated toxic injury to peripheral nerves in Navajo neuropathy.
Author List
Lawlor MW, Holve S, Stubbs EB JrAuthor
Michael W. Lawlor MD, PhD Adjunct Professor in the Pathology department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
AnimalsArizona
Child
Corneal Ulcer
Electromyography
Evoked Potentials, Motor
Ganglia, Spinal
Humans
Indians, North American
Liver Diseases
Motor Neuron Disease
Muscle Weakness
Neural Conduction
Neurites
Neurons, Afferent
Paraproteinemias
Peripheral Nervous System Diseases
Rats
Sciatic Neuropathy
Sensation Disorders
