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Single-Fiber Recordings of Nociceptive Fibers in Patients With HSAN Type V With Congenital Insensitivity to Pain
Natl Hosp Norway, Oslo Univ Hosp, Clin Neurophysiol Sect, Dept Neurol, Pb 4950 Nydalen, N-0424 Oslo, Norway..
Natl Hosp Norway, Oslo Univ Hosp, Clin Neurophysiol Sect, Dept Neurol, Pb 4950 Nydalen, N-0424 Oslo, Norway..
Natl Hosp Norway, Oslo Univ Hosp, Clin Neurophysiol Sect, Dept Neurol, Pb 4950 Nydalen, N-0424 Oslo, Norway..
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
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2016 (English)In: The Clinical Journal of Pain, ISSN 0749-8047, E-ISSN 1536-5409, Vol. 32, no 7, p. 636-642Article in journal (Refereed) Published
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Abstract [en]

Objectives: Nerve growth factor (NGF) is a protein important for growth and survival, but also for modulation of sensitivity of nociceptors and sympathetic neurons. The purpose of the present study was to investigate the effects of reduced NGF signaling in patients with hereditary sensory and autonomic neuropathies type V, congenital insensitivity to pain, caused by a mutation of the NGF beta gene, including a characterization of single nociceptive fibers using microneurography (MNG). Materials and Methods: One homozygote and 2 heterozygote patients with this mutation were examined with electromyography/neurography, thermal testing, quantitative sudomotor axon reflex test, and electrically induced axon reflex erythema in addition to MNG. Results: Low quantitative sudomotor axon reflex test measurements of 0.02 (left foot) and 0.03 (right foot) mL/cm(2) and elevated thermal thresholds for warmth and cold detection testing showed clear impairment of small nerve fibers, both sudomotor efferent and somatic afferent fibers, in the patient homozygote for the mutation. MNG from one of the heterozygote patients revealed changes in the small nociceptive fibers in skin, including abnormally low conduction velocity, spontaneous activity in A-delta fibers and C-nociceptors and abnormal or lacking response to heat. Discussion: The findings of grossly intact pain thresholds compared with anamnestic insensitivity of pain in deep somatic tissue such as bone suggest a gradient of impairment dependent on different NGF availability in various tissues. Even though these patients in some aspects report insensitivity to pain, they also report chronic spontaneous pain as their main symptom, strikingly highlighting differential mechanisms of insensitivity to evoked pain versus spontaneous pain.

Place, publisher, year, edition, pages
2016. Vol. 32, no 7, p. 636-642
Keywords [en]
NGF, pain, insensitivity, microneurography
National Category
Neurology
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URN: urn:nbn:se:uu:diva-299835DOI: 10.1097/AJP.0000000000000303ISI: 000378712600012PubMedID: 27270876OAI: oai:DiVA.org:uu-299835DiVA, id: diva2:950289
Funder
German Research Foundation (DFG), NA 970 1/1Available from: 2016-07-29 Created: 2016-07-28 Last updated: 2017-11-28Bibliographically approved

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