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Efficacy of transcutaneous electrical nerve stimulation in the treatment of a patient with amyotrophic lateral sclerosis due to syringomyelia
- Authors: Al-Zamil M.K.1, Kulikova N.G.1,2, Vasilieva E.S.3
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Affiliations:
- Peoples' Friendship University of Russia
- National Medical Research Center for Rehabilitation and Balneology
- Moscow State University of Medicine and Dentistry named after A.I. Evdokimova
- Issue: Vol 21, No 2 (2022)
- Pages: 143-158
- Section: Clinical notes and case reports
- URL: https://rjpbr.com/1681-3456/article/view/111885
- DOI: https://doi.org/10.17816/rjpbr111885
- ID: 111885
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Abstract
This paper demonstrates a clinical case of the development of amyotrophic lateral sclerosis with lesions of the anterior horns of the spinal cord in the cervicothoracic region against the background of syringomyelia.
The main complaints of the patient at the initial request for medical care were associated with weakness and hypotrophy of the right hand. Electrokymography revealed signs of damage to the anterior horns of the spinal cord at the C5–Th1 level with a predominant lesion of the motor fibers of the right median and ulnar nerves. As a result, a diagnosis of amyotrophic lateral sclerosis was made. Magnetic resonance imaging revealed the presence of Chiari I malformation and severe syringomyelia of the cervical and thoracic calving of the spinal cord.
The patient underwent surgical posterior decompression of the foramen magnum. However, the treatment was not effective with the worsening of the neurological deficit in the right hand and the spread of motor deficit and hypotrophy to the left hand.
After the use of transcutaneous electrical neurostimulation of the median and ulnar nerves, there was a significant regression of motor deficit with a decrease in the severity of malnutrition without significant changes in the electromyography parameters of the median and ulnar nerves.
The absence of neurological deficit in the lower extremities, with the preservation of tendon reflexes in the norm, and the absence of pathological reflexes, indicates a high plasticity of the pyramidal tracts at the level of the spinal cord.
Regression of motor deficit after the use of transcutaneous electrical nerve stimulation is due to improvement in the state of altered motor units with hypertrophy of muscle fibers and acceleration of reinnervation processes.
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About the authors
Mustafa Kh. Al-Zamil
Peoples' Friendship University of Russia
Author for correspondence.
Email: alzamil@mail.ru
ORCID iD: 0000-0002-3643-982X
SPIN-code: 3434-9150
MD, Dr. Sci. (Med.), Professor
Russian Federation, MoscowNatalia G. Kulikova
Peoples' Friendship University of Russia; National Medical Research Center for Rehabilitation and Balneology
Email: fbrmed@mail.ru
ORCID iD: 0000-0002-6895-0681
SPIN-code: 1827-7880
MD, Dr. Sci. (Med.), Professor
Congo, Moscow; MoscowEkaterina S. Vasilieva
Moscow State University of Medicine and Dentistry named after A.I. Evdokimova
Email: alzamil@mail.ru
ORCID iD: 0000-0003-3087-3067
SPIN-code: 5423-8408
MD, Dr. Sci. (Med.), Professor
Russian Federation, MoscowReferences
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Supplementary files
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Fig. 1. Increase in the incidence of syringomyelia between 1962 and 2003 in New Zealand, % [1].
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Fig. 2. Classification of syringomyelia T.H. Milhorat (2000).
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Fig. 3. Magnetic resonance imaging of the thoracic spine: Extramedullary extramedullary compression of the spinal cord by herniated intervertebral disc between the VII and VIII thoracic vertebrae with the formation of syringomyelic cyst brush above the compression level with upward propagation to the cervical calving. (Own observation).
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Fig. 4. Magnetic resonance imaging of the lumbosacral spine: fixation of the spinal cord due to a rigid end thread (arrow).
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Fig. 5. Illustration of the alleged mechanism of formation and progression of syringomyelia: a — brain expansion during cardiac systole creates a pressure wave of cerebrospinal fluid (thick arrows). Obstruction of the spinal canal (for example, with an extramedullary tumor) prevents the process of normal attenuation of pulsational pressure of the cerebrospinal fluid, as a result of which the cerebrospinal fluid is directed to the Virchow–Robin spaces of the spinal cord due to excessive pulsation. After the formation of the syrinx, pulsating waves of cerebrospinal fluid (thin arrows) affect the spinal cord, as a result, the pressure inside the syrinx rises, which leads to the movement of fluid inside the syrinx caudally and, ultimately, to an increase in its size; b — it is shown how, over time, due to an increase in pulsation pressure directed from the spinal canal to the parenchyma of the spinal cord (arrows), microcysts are formed in the substance of the spinal cord (circles), which gradually merge into the syrinx.
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Fig. 6. The distribution of patients into groups depending on etiological factors in the study of 1243 patients with syringomyelia in Japan in 1995 [10].
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Fig. 7. Signs of hypotrophy of the muscles of the tenar, hypotenar and the back interosseous muscles of the right hand; claw-like deformity.
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Fig. 8. Magnetic resonance imaging of the brain: signs of omission of the tonsils of the cerebellar pedicle into the large occipital foramen. Arnold–Chiari anomaly type 1.
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Fig. 9. Magnetic resonance imaging of the cervical spine: axial (left) and coronal (right) sections. A huge syringomyelic cyst is visible, which begins from the II cervical vertebra and passes into the thoracic region, reaching a maximum at the level of the VI cervical vertebra and below.
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Fig. 10. Magnetic resonance imaging of the thoracic spine: axial section. A huge syringomyelic cyst is visible, which continues from the cervical spine and ends at the level of the XII thoracic vertebra, reaching a maximum between the II and VI thoracic vertebrae.
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Fig. 11. Magnetic resonance imaging of the cervical spine: cross-section at the level of the VI cervical vertebra. Two drawings of the same slice are shown: on the left — an MRI image, on the right — an MRI with illustrations, where the spinal cord tissue is isolated (ring), and the arrows inside indicate the border of the syringomyelic cyst.
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Fig. 12. Magnetic resonance imaging of the thoracic spine: a cross section at the level of the V thoracic vertebra. Two drawings of the same slice are shown: on the left — an MRI image, on the right — an MRI with illustrations, where the spinal cord tissue (a thin ring) is highlighted in red, and the arrows inside indicate the border of the syringomyelic cyst.
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Fig. 13. Results of the study of motor fibers of median nerves on the left (1) and right (2), as well as ulnar nerves on the left (4) and right (5). The results of the study of sensory fibers of median (3) and ulnar (6) nerves are indicated: the upper line is the result of the study of the left nerve, the lower line — the right nerve. The figures show a marked decrease in the amplitude of the M-response in the study of the motor fibers of the right median and ulnar nerves and a moderate decrease in the amplitude of the M-response in the study of the left ulnar nerve, while the pulse rate and the amplitude of the sensory fibers are within normal limits.
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Fig. 14. The results of the study of the motor fibers of the peroneal nerves on the left (1) and right (2). The results of the study of the sensory fibers of the peroneal nerves (5) are indicated: the upper line is left, the lower one is right. The results of the study of the F-response during stimulation of the tibial nerves on the left (3) and right (4) are indicated. From the figure, it can be noted that the pulse rate and the amplitude of the M-response during stimulation of the motor fibers of the peroneal nerves are within the normal range. The speed of the impulses and the amplitude of the action potentials in the study of the sensory fibers of the peroneal nerves are normal. It is possible to note the asymmetry of the amplitudes of F-responses S>D.
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Fig. 15. Results of needle electromyography of the short abductor muscle of the thumb on the left (m. abductor pollicis brevis): action potentials with a large amplitude reaching 9196 mv (normally up to 2000 mv) are visible, lengthening the duration in some action potentials to 14.7 ms and decreasing the duration in others to 5.7 ms (norm from 7.4 up to 11.2).
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Fig. 16. Registration of spontaneous activity in the study of the short abductor muscle of the thumb on the left (m.abductor pollicis brevis): а — fasciculation potentials; b — fibrillation potentials and frequent high-amplitude positive acute waves.
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Fig. 17. Technique of percutaneous electroneurostimulation of the left median nerve.
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Fig. 18. After treatment, there is a decrease in tenar and hypotenar hypotrophy and the severity of claw-like deformity of the hands.
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