Sonography of the median nerve in CMT1A, CMT2A, CMTX, and HNPP

Autor: Schreiber, Stefanie, Oldag, Andreas, Tempelmann, Claus, Mawrin, Christian, Dengler, Reinhard, Schreiber, Frank, Goertler, Michael, Vielhaber, Stefan, Kornblum, Cornelia, Kollewe, Katja, Kropf, Siegfried, Schoenfeld, Ariel, Feistner, Helmut, Jakubiczka, Sibylle, Kunz, Wolfram S, Scherlach, Cordula
Rok vydání: 2012
Předmět:
Adult
Male
genetics [GTP Phosphohydrolases]
Adolescent
anatomy & histology [Forearm]
Neural Conduction
genetics [Myelin Proteins]
Connexins
physiology [Neural Conduction]
GTP Phosphohydrolases
Mitochondrial Proteins
Young Adult
MFN2 protein
human
Charcot-Marie-Tooth Disease
International Classification of Diseases
anatomy & histology [Wrist]
Humans
ddc:610
Aged
Ultrasonography
diagnostic imaging [Hereditary Sensory and Motor Neuropathy]
PMP22 protein
human
Electrodiagnosis
diagnostic imaging [Charcot-Marie-Tooth Disease]
genetics [Hereditary Sensory and Motor Neuropathy]
Middle Aged
Wrist
diagnostic imaging [Median Nerve]
innervation [Forearm]
connexin 32
Electrophysiological Phenomena
Median Nerve
Forearm
Phenotype
innervation [Wrist]
genetics [Mitochondrial Proteins]
Female
genetics [Charcot-Marie-Tooth Disease]
genetics [Connexins]
Hereditary Sensory and Motor Neuropathy
Myelin Proteins
Zdroj: Muscle & nerve 47(3), 385-395 (2013). doi:10.1002/mus.23681
ISSN: 1097-4598
DOI: 10.1002/mus.23681
Popis: In this study we compare the ultrasound features in the median nerve in patients with different types of Charcot-Marie-Tooth (CMT) disease and hereditary neuropathies with liability to pressure palsies (HNPP) as a typical entrapment neuropathy.Median nerve ultrasound and conduction studies were performed in patients with CMT1A (n = 12), MFN2-associated CMT2A (n = 7), CMTX (n = 5), and HNPP (n = 5), and in controls (n = 28).Median nerve cross-sectional area (CSA) was significantly increased in CMT1A, whereas, in axonal CMT2A, fascicle diameter (FD) was enlarged. CSA correlated with nerve conduction slowing in CMT1A and with axonal loss, as shown by motor and sensory nerve amplitudes in both CMT1A and CMT2A. A relatively low wrist-to-forearm-ratio (WFR0.8) or a relatively high WFR (1.8) appeared to be unlikely in MFN2 and Cx32 mutations of CMT2A and CMTX, respectively.Differences in CSA, FD, and WFR of the median nerve can be helpful in defining subtypes of hereditary neuropathies.
Databáze: OpenAIRE
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