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Distinctive patterns of sonographic nerve enlargement in Charcot-Marie-Tooth type 1A and hereditary neuropathy with pressure palsies.
Clin Neurophysiol. 2015 Jul; 126(7):1413-20.CN

Abstract

OBJECTIVE

The extent of sonomorphologic differences of peripheral nerves between CMT and HNPP is unknown.

METHODS

We recruited 9 patients with CMT-1A and 9 with HNPP. Patients underwent a standardized sonographic protocol, which evaluated nerve size and vascularization. We quantitatively assessed fascicle size and echogenicity.

RESULTS

All 18 patients demonstrated nerve enlargement, but no increased vascularization. HNPP demonstrated larger nerves at sites of entrapment (median nerve at the carpal tunnel p=0.049, ulnar nerve at the sulcus p<0.001), greater swelling ratios of median (p<0.001), ulnar (p=0.017) and fibular nerve (p=0.005) than CMT-1A. CMT-1A revealed larger nerves proximal to sites of entrapment (median and fibular nerve, brachial plexus p<0.001). Nerve fascicles where larger (p<0.001) and more hypo-echogenic in CMT-1A. Nerve, fascicle size nor echogenicity correlated with age, gender or MRC sum-score.

CONCLUSIONS

Ultrasonography of nerves reveals specific phenotypes differentiating CMT-1A from HNPP. In CMT-1A enlargement of nerves and fascicles is multifocal among multiple nerves, whereas in HNPP nerve enlargement is restricted to sites of entrapment.

SIGNIFICANCE

Our findings of specific sonomorphological phenotypes, differentiating CMT-1A from HNPP, may help to improve our pathophysiological insights in CMT and HNPP.

Authors+Show Affiliations

Department of Neurology, UMC Utrecht, Utrecht, The Netherlands; Brain Center Rudolf Magnus, Department of Neuroscience, UMC Utrecht, Utrecht, The Netherlands. Electronic address: h.s.goedee-2@umcutrecht.nl.Department of Neurology, St. Elisabeth Hospital, Tilburg, The Netherlands; Department of Clinical Neurophysiology, St. Elisabeth Hospital, Tilburg, The Netherlands.Department of Neurology, UMC Utrecht, Utrecht, The Netherlands; Brain Center Rudolf Magnus, Department of Neuroscience, UMC Utrecht, Utrecht, The Netherlands.Department of Neurology, St. Elisabeth Hospital, Tilburg, The Netherlands; Department of Clinical Neurophysiology, St. Elisabeth Hospital, Tilburg, The Netherlands.

Pub Type(s)

Comparative Study
Journal Article

Language

eng

PubMed ID

25454274

Citation

Goedee, Stephan H., et al. "Distinctive Patterns of Sonographic Nerve Enlargement in Charcot-Marie-Tooth Type 1A and Hereditary Neuropathy With Pressure Palsies." Clinical Neurophysiology : Official Journal of the International Federation of Clinical Neurophysiology, vol. 126, no. 7, 2015, pp. 1413-20.
Goedee SH, Brekelmans GJ, van den Berg LH, et al. Distinctive patterns of sonographic nerve enlargement in Charcot-Marie-Tooth type 1A and hereditary neuropathy with pressure palsies. Clin Neurophysiol. 2015;126(7):1413-20.
Goedee, S. H., Brekelmans, G. J., van den Berg, L. H., & Visser, L. H. (2015). Distinctive patterns of sonographic nerve enlargement in Charcot-Marie-Tooth type 1A and hereditary neuropathy with pressure palsies. Clinical Neurophysiology : Official Journal of the International Federation of Clinical Neurophysiology, 126(7), 1413-20. https://doi.org/10.1016/j.clinph.2014.08.026
Goedee SH, et al. Distinctive Patterns of Sonographic Nerve Enlargement in Charcot-Marie-Tooth Type 1A and Hereditary Neuropathy With Pressure Palsies. Clin Neurophysiol. 2015;126(7):1413-20. PubMed PMID: 25454274.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Distinctive patterns of sonographic nerve enlargement in Charcot-Marie-Tooth type 1A and hereditary neuropathy with pressure palsies. AU - Goedee,Stephan H, AU - Brekelmans,Geert J F, AU - van den Berg,Leonard H, AU - Visser,Leo H, Y1 - 2014/10/12/ PY - 2014/05/23/received PY - 2014/07/06/revised PY - 2014/08/29/accepted PY - 2014/12/3/entrez PY - 2014/12/3/pubmed PY - 2015/9/2/medline KW - Charcot–Marie–Tooth (CMT) KW - Hereditary neuropathy with pressure palsies (HNPP) KW - Nerve hypertrophy KW - Polyneuropathy KW - Sonography SP - 1413 EP - 20 JF - Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology JO - Clin Neurophysiol VL - 126 IS - 7 N2 - OBJECTIVE: The extent of sonomorphologic differences of peripheral nerves between CMT and HNPP is unknown. METHODS: We recruited 9 patients with CMT-1A and 9 with HNPP. Patients underwent a standardized sonographic protocol, which evaluated nerve size and vascularization. We quantitatively assessed fascicle size and echogenicity. RESULTS: All 18 patients demonstrated nerve enlargement, but no increased vascularization. HNPP demonstrated larger nerves at sites of entrapment (median nerve at the carpal tunnel p=0.049, ulnar nerve at the sulcus p<0.001), greater swelling ratios of median (p<0.001), ulnar (p=0.017) and fibular nerve (p=0.005) than CMT-1A. CMT-1A revealed larger nerves proximal to sites of entrapment (median and fibular nerve, brachial plexus p<0.001). Nerve fascicles where larger (p<0.001) and more hypo-echogenic in CMT-1A. Nerve, fascicle size nor echogenicity correlated with age, gender or MRC sum-score. CONCLUSIONS: Ultrasonography of nerves reveals specific phenotypes differentiating CMT-1A from HNPP. In CMT-1A enlargement of nerves and fascicles is multifocal among multiple nerves, whereas in HNPP nerve enlargement is restricted to sites of entrapment. SIGNIFICANCE: Our findings of specific sonomorphological phenotypes, differentiating CMT-1A from HNPP, may help to improve our pathophysiological insights in CMT and HNPP. SN - 1872-8952 UR - https://www.unboundmedicine.com/medline/citation/25454274/Distinctive_patterns_of_sonographic_nerve_enlargement_in_Charcot_Marie_Tooth_type_1A_and_hereditary_neuropathy_with_pressure_palsies_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1388-2457(14)00530-6 DB - PRIME DP - Unbound Medicine ER -