Monomelic amyotrophy: a rare variant of lower motor neuron disorder (2 clinical cases)

Cover Page

Abstract

Monomelic amyotrophy (MMA) is a rare variant of lower motor neuron disease with benign progression. The disease is characterized by muscular weakness and atrophy in the hand and forearm on one side or asymmetrical lesion of both upper limbs with the presence of cold paresis, fasciculations, and fine tremor of the fingers. Sensory disorders, pyramidal symptoms, and involvement of the leg muscles are not typical of it. The final diagnosis of MMA can be made only on the basis of clinical neurological and electrophysiological tests as well as long-term follow-up of the patient. Timely recommendations for correction of the movement pattern and prevention of flexion compression may slow down the disease progression in some cases and, thereby, improve the quality of life of patients. The article describes two clinical cases with a probable diagnosis of MMA.

About the authors

T. M. Alekseeva

I.I. Mechnikov Northwestern State Medical University, Ministry of Health of the Russian Federation, St. Petersburg

Author for correspondence.
Email: platonova@neurology.ru
Russian Federation

V. S. Demeshonok

I.I. Mechnikov Northwestern State Medical University, Ministry of Health of the Russian Federation, St. Petersburg

Email: platonova@neurology.ru
Russian Federation

N. Yu. Aleksandrov

I.I. Mechnikov Northwestern State Medical University, Ministry of Health of the Russian Federation, St. Petersburg

Email: platonova@neurology.ru
Russian Federation

A. D. Khalikov

I.I. Mechnikov Northwestern State Medical University, Ministry of Health of the Russian Federation, St. Petersburg

Email: platonova@neurology.ru
Russian Federation

M. G. Sokolova

I.I. Mechnikov Northwestern State Medical University, Ministry of Health of the Russian Federation, St. Petersburg

Email: platonova@neurology.ru
Russian Federation

References

  1. Богданов Э.И., Нурмиева С.Р. Синдромы изолированной амиотрофии плечевого пояса и рук. Неврол. журн. 2010; 5: 4–9.
  2. Дамулин И.В., Чуркина И.Г., Яворская С.А. и др. Мономелическая амиотрофия. Неврол. журн. 2005; 10 (3): 35–40.
  3. Стучевская Т.Р., Казаков В.М., Руденко Д.И. и др. Болезни нижнего двигательного нейрона с преимущественным поражением верхних конечностей: это самостоятельные формы или атипичные варианты бокового амиотрофического склероза? Журн. неврол. и психиатр. 2006;1:14–20.
  4. Bo Zhou, Lei Chen, Dongsheng Fan et al. Clinical features of Hirayama disease in mainland China. Amyotrophic Lateral Sclerosis 2010; 11:133–139.
  5. Hassan K.M., Hirdesh Sahni1, Atul Jha. Clinical and radiological profile of Hirayama disease: A flexion myelopathy due to tight cervical dural canal amenable to collar therapy. Annals of Indian Academy of Neurology 2012; 15: 106–112.
  6. Hirayama K., Toyokura Y., Tsubaki T. Juvenile muscular atrophy of unilateral upper extremity: a new clinical entity. Psychiatria et Neurologia Japonica 1959; 61: 2190–2197.
  7. Hirayama K. Non-progressive juvenile spinal muscular atrophy of the distal upper limb (Hirayama’s disease). Handbook of Clinical Neurology, J. M. De Jong, Ed., 1991; 15: 107–120.
  8. Kohno M, Takahashi H, Yagishita A, Tanabe H. „Disproportion theory” of the cervical spine and spinal cord in patients with juvenile cervical fl exion myelopathy: A study comparing cervical magnetic resonance images with those of the normal controls. Surg Neurol 1998; 50:421–430.
  9. Nalini Atchayaram, Vasudev M.K., Gaurav Goel. Familial monomelic amyotrophy (Hirayama disease): Two brothers with classical flexion induced dynamic changes of the cervical dural sac. Neurology India 2009;57 (6): 810–811.
  10. Tashiro K., Kikuchi S., Itoyama Y. et al. Nationwide survey of juvenile muscular atrophy of distal upper extremity (Hirayama disease) in Japan. Amyotrophic Lateral Sclerosis 2006; 7: 38–45.
  11. Toma S., Shiozawa Z. Amyotrophic cervical myelopathy in adolescence. J Neurol Neurosurg Psychiatry 1995; 58: 56–64.

Statistics

Views

Abstract: 912

PDF (Russian): 863

Article Metrics

Metrics Loading ...

Dimensions

PlumX


Copyright (c) 2015 Alekseeva T.M., Demeshonok V.S., Aleksandrov N.Y., Khalikov A.D., Sokolova M.G.

Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 International License.

This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies