Journal of
J. Neurol. 214, 305--312 (1977)
Neurology © by Springer-Verlag 1977
Motor and Sensory Conduction Velocity in Charcot-Marie-Tooth Disease C. Vasilescu and A. Florescu Institute of Neurology and Psychiatry, Str. Povernei No. 42, Bucharest I, Romania
Zusammenfassung. Leitgeschwindigkeit in den peripheren Nerven (sensiblen, motorischen und gemischten Fasern) wurde bei 23 Patienten yon CharcotMarie-Tooth-Krankheit durchgeftihrt. Es wurde in den meisten F~illen eine starke Verminderung der Leitgeschwindigkeit, in allen 3 Gruppen von Fasern, insbesondere in den distalen Segmenten (Finger und Zehen) der sensiblen Fasern beobachtet. Trotzdem waren die Mittelwerte der sensiblen, motorischen und gemischten Fasern nicht sehr verschieden. Diese Befunde weisen auf eine segmentale Demyelinisierung hin, in den meisten F~illen. Key words: M o t o r conduction - Sensory conduction
Charcot-Marie-Tooth
disease. Electromyographic studies in Charcot-Marie-Tooth disease (Gilliatt and Thomas, Lambert and Dyck, Dyck and Lambert, Vasilescu, Kaeser, Dyck, among others) have shown that conduction velocity (CV) is markedly slowed in most of the cases while normal in a few. In a study which also included ultrastructural examinations, Dyck and Lambert identified two types of the disease: the neuritic form ("hypertrophic neuropathy of Charcot-Marie-Tooth type") with markedly diminished CV in the sensory fibres of peripheral nerves and extensive segmental demyelination accompanied by hypertrophic changes in the biopsies, and the neuronal form with normal or slightly diminished CV and presumptive neuronal degeneration as pathological substrate. Thomas and Calne have declared that the two types of the disease are also heterogeneous from the genetic point of view. In the present work we measure systematically the motor, sensory and mixed, sensorimotor potentials in distal and proximal segments of peripheral nerves of patients with Charcot-Marie-Tooth disease and discuss the value of such systematic measurements, taking into account the biopsy data in the literature.
Material and Methods The study was performed on 23 subjects with objective signs of the disease, whose age ranged from 4 to 68 years (mean age 33.4 years). The pedigree suggested that the transmission was autosomal dominant in ten and autosomal recessive in one of the 11 families to which the 23
306
C. Vasilescu and A. Florescu
patients belonged. CV measurements were carried out in the fastest conducting motor fibres of the median, ulnar and peroneal nerves, and the distal latency was recorded. Concomitantly, the CV in the sensory fibres of the same nerves was measured. All the recordings were performed by means of a 2-channel 14A21 DISA electromyograph. Stimulation of digital sensory fibres was performed by type 13K69 surface ring electrodes with the cathode oriented proximally. The EPs were recorded by Disa insulated, monopolar needle electrodes with 2--3 mm bare tips. A number of 128--1024 potentials were totaled by a type 14G01 Disa Averager, the automatic reading of latency (at the peak of the first positive deflection of the sensory action potential evoked) and of EP duration being thus obtained. Further technical details are given in a previous work (Vasilescu).
Results
The 23 patients were characterized clinically by d i m i n u t i o n of superficial a n d / o r deep sensation distally in the extremities, w e a k e n i n g of distal m u s c u l a t u r e in the limbs, absence or decrease of t e n d o n reflexes in the lower a n d u p p e r limbs, foot deformities, i m p a i r e d gait, m a r k e d a t r o p h y of the peronei a n d small distal muscles symmetrically in lower a n d u p p e r extremities. The results o b t a i n e d on the 23 patients by the study of CV a n d T L in the m o t o r fibres of the three (ulnar, m e d i a n a n d peroneal) nerves are illustrated in
MOTOR CONDUCTION VELOCITY Peroneal Median Ulnar /0
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Fig. 1. Motor CV in the peroneal, median and ulnar nerves. Distribution pattern of individual values in patients (dots). Mean values in each nerve in patients ( - - - - - - ) and controls ( - - - - - - )
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J. Neurol. 214, 305--312 (1977)
Neurology © by Springer-Verlag 1977
Motor and Sensory Conduction Velocity in Charcot-Marie-Tooth Disease C. Vasilescu and A. Florescu Institute of Neurology and Psychiatry, Str. Povernei No. 42, Bucharest I, Romania
Zusammenfassung. Leitgeschwindigkeit in den peripheren Nerven (sensiblen, motorischen und gemischten Fasern) wurde bei 23 Patienten yon CharcotMarie-Tooth-Krankheit durchgeftihrt. Es wurde in den meisten F~illen eine starke Verminderung der Leitgeschwindigkeit, in allen 3 Gruppen von Fasern, insbesondere in den distalen Segmenten (Finger und Zehen) der sensiblen Fasern beobachtet. Trotzdem waren die Mittelwerte der sensiblen, motorischen und gemischten Fasern nicht sehr verschieden. Diese Befunde weisen auf eine segmentale Demyelinisierung hin, in den meisten F~illen. Key words: M o t o r conduction - Sensory conduction
Charcot-Marie-Tooth
disease. Electromyographic studies in Charcot-Marie-Tooth disease (Gilliatt and Thomas, Lambert and Dyck, Dyck and Lambert, Vasilescu, Kaeser, Dyck, among others) have shown that conduction velocity (CV) is markedly slowed in most of the cases while normal in a few. In a study which also included ultrastructural examinations, Dyck and Lambert identified two types of the disease: the neuritic form ("hypertrophic neuropathy of Charcot-Marie-Tooth type") with markedly diminished CV in the sensory fibres of peripheral nerves and extensive segmental demyelination accompanied by hypertrophic changes in the biopsies, and the neuronal form with normal or slightly diminished CV and presumptive neuronal degeneration as pathological substrate. Thomas and Calne have declared that the two types of the disease are also heterogeneous from the genetic point of view. In the present work we measure systematically the motor, sensory and mixed, sensorimotor potentials in distal and proximal segments of peripheral nerves of patients with Charcot-Marie-Tooth disease and discuss the value of such systematic measurements, taking into account the biopsy data in the literature.
Material and Methods The study was performed on 23 subjects with objective signs of the disease, whose age ranged from 4 to 68 years (mean age 33.4 years). The pedigree suggested that the transmission was autosomal dominant in ten and autosomal recessive in one of the 11 families to which the 23
306
C. Vasilescu and A. Florescu
patients belonged. CV measurements were carried out in the fastest conducting motor fibres of the median, ulnar and peroneal nerves, and the distal latency was recorded. Concomitantly, the CV in the sensory fibres of the same nerves was measured. All the recordings were performed by means of a 2-channel 14A21 DISA electromyograph. Stimulation of digital sensory fibres was performed by type 13K69 surface ring electrodes with the cathode oriented proximally. The EPs were recorded by Disa insulated, monopolar needle electrodes with 2--3 mm bare tips. A number of 128--1024 potentials were totaled by a type 14G01 Disa Averager, the automatic reading of latency (at the peak of the first positive deflection of the sensory action potential evoked) and of EP duration being thus obtained. Further technical details are given in a previous work (Vasilescu).
Results
The 23 patients were characterized clinically by d i m i n u t i o n of superficial a n d / o r deep sensation distally in the extremities, w e a k e n i n g of distal m u s c u l a t u r e in the limbs, absence or decrease of t e n d o n reflexes in the lower a n d u p p e r limbs, foot deformities, i m p a i r e d gait, m a r k e d a t r o p h y of the peronei a n d small distal muscles symmetrically in lower a n d u p p e r extremities. The results o b t a i n e d on the 23 patients by the study of CV a n d T L in the m o t o r fibres of the three (ulnar, m e d i a n a n d peroneal) nerves are illustrated in
MOTOR CONDUCTION VELOCITY Peroneal Median Ulnar /0
609
50-
,9
|
40U
_0 3 0
Z 0
D 211.
•
r,,, z @ u I11.
Q
Q
•
Fig. 1. Motor CV in the peroneal, median and ulnar nerves. Distribution pattern of individual values in patients (dots). Mean values in each nerve in patients ( - - - - - - ) and controls ( - - - - - - )
TERMINAL LATENCY Peroneal Median Ulnar 201816. 1412E )L.J
ZLU
10-
~0
8-
•
"1"-I
I'--
