NON PROGRESSIVE JUVENILE SPINAL MUSCULAR ATROPHY (HIRAYA-MA DISEASE): CLINICAL AND RADIOLOGICAL CORRELATIONS
Abstract
Non-progressive juvenile spinal muscular atrophy, also known as Hirayama disease, is a rare form of non-progressive motor neuron disease caused by necrosis of the anterior horns of the lower cervical cord. Magnetic resonance study is able to show forward displacement of the posterior wall of the lower cervical dural canal in neck flexion, which is presumed to be pathognomonic sign of Hirayama disease. Flexion MRI of the cervical spine can help to diagnosis HD in the early stages. We report two cases of Hirayama disease with different disease duration and different clinical and radiological presentation.
About the Authors
Zh. I. Savintceva
N. P Bechtereva Institute of the human brain of Russian academy of sciences
Russian Federation
Russian Federation
N. A. Totolyan
N. P Bechtereva Institute of the human brain of Russian academy of sciences
Russian Federation
Russian Federation
T. N. Trofimova
N. P Bechtereva Institute of the human brain of Russian academy of sciences; «Scandinavia» clinic, «AVA-PETER»
Russian Federation
Russian Federation
T. Yu. Skvortsova
N. P Bechtereva Institute of the human brain of Russian academy of sciences
Russian Federation
Russian Federation
Лидия Прахова
Институт мозга человека им. Н. П. Бехтеревой Российской академии наук
Russian Federation
Russian Federation
Александр Ильвес
Институт мозга человека им. Н. П. Бехтеревой Российской академии наук
Russian Federation
Russian Federation
References
1. Kira J., Ochi H. Juvenile muscular atrophy of the distal upper limb (Hirayama disease) associated with atrophy // J. Neurol. Neurosurg. Psychiatry.- 2001.- Vol. 70.- P. 789-801.
2. Кременева Е. И., Воробьева А. А., Адарчева Л. С., Коновалов Р. Н., Суслин А. С., Кротенкова М. В., Захарова М. Н., Гришина Д. А., Антелава А. Л., Брюхов В. В., Терехов А. В. Предупрежден - значит вооружен: особенности МРТ-исследования при болезни Хираяма // Лучевая диагностика и терапия.- 2015.- Т. 3, № 6.- С. 35-43.
3. Менделевич Е. Г., Валиева Л. К., Богданов Э. И., Анисимов В. И. Болезнь Хираямы, или дистальная цервикальная миелопатия: новые варианты клинических и МРТ параметров // Неврологический вестник.- 2015.- Т. XLVII, № 3.- С. 106-110.
4. Raval M., Kumari R., Dung A. A. MRI findings in Hirayama disease // Indian J. Radiol Imaging.- 2010.- Vol. 20, № 4.- P 245-299.
5. Chi-Jen Chen, Hui-Ling Hsu, Ying-Chi Tseng, Rong-Kuo Lyu, Chiung-Mei Chen, Ying-Chih Huang, Li-Jen Wang, Yon-Cheong Wong, Lai-Chu See. Hirayama Flexion Myelopathy: NeutralPosition MR Imaging Findings - Importance of Loss of Attachment // RSBA.- 2004.- Vol. 231, № 1.
6. Kikuchi S., Tashiro K., Kitagawa K., Iwasaki Y., Abe H. A mechanism of juvenile muscular atrophy localized in the hand and forearm (Hirayama's disease): flexion myelopathy with tight dural canal in flexion [in Japanese] // Clin. Neurol (Tokyo).- 1987.- Vol. 27.- P 412-419.
7. Tokumaru Y., Hirayama K. Anterior shift of posterior lower cervical dura mater in patients with juvenile muscular atrophy of unilateral upper extremity // Clin. Neurol. (Tokyo).- 1989.- Vol. 29.- P 1237-1243.
8. Mukai E., Matsuo T., Muto T., Takahashi A., Sobue I. Magnetic resonance imaging of juvenile-type distal and segmental muscular atrophy of upper extremities // Clin. Neurol. (Tokyo).- 1987.- Vol. 27.- P 99-107.
9. Mukai E., Sobue I., Muto T., Takahashi A., Goto S. Abnormal radiological findings on juvenile-type distal and segmental muscular atrophy of upper extremities // Clin. Neurol. (Tokyo).- 1985.- Vol. 25.- P. 620- 626.
10. Hirayama K., Toyokura Y., Tsubaki T. Juvenile muscular atrophy of unilateral upper extremity: a new clinical entity // Psychiatr Neurol. Jаp.- 1959.- Vol. 61.- P 2190-2197.
11. Hirayama K., Tomonaga M., Kitano K., Yamada T., Kojima S., Arai K. Focal cervical poliopathy causing juvenile muscular atrophy of distal upper extremity: a pathological study // J. Neurol. Neurosurg. Psychiatry.- 1987.- Vol. 50.- P 285-290.
12. Pradhan S., Gupta R. K. Magnetic resonance imaging in juvenile asymmetric segmental spinal muscular atrophy // J. Neurol. Sci.- 1997.- Vol. 146.- P 133-138.
13. Hirayama K., Tokumaru Y. Cervical dural sac and spinal cord in juvenile muscular atrophy of distal upper extremity // Neurology. - 2000.- Vol. 54.- P 1922-1926.
14. Shinomiya K., Dawson J., Spengler D. M., Konrad P., Blumenkopf B. An analysis of the posterior epidural ligament role on the cervical spinal cord // Spine.- 1996.- Vol. 21.- P 2081-2088.
15. Schröder R., Keller E., Flacke S., Schmidt S., Pohl C., Klockgether T. MRI findings in Hirayama’s disease: flexion-induced cervical myelopathy or intrinsic motor neuron disease? // J. Neurol.- 1999.- Vol. 246.- P 1069-1074.
16. Willeit J., Kiechl S., Kiechl-Kohlendorfer U., Golaszewski S., Peer S., Poewe W. Juvenile asymmetric segmental spinal muscular atrophy (Hirayama’s disease): three cases without evidence of «flexion myelopathy» // Acta Neurol. Scand.- 2001.- Vol. 104.- P 320-322.
17. Lai V., Wong Y. C., Poon W. L., Yuen M. K., Fu Y. P., Wong O. W. Forward shifting of posterior dural sac during flexion cervical magnetic resonance imaging in Hirayama disease: an initial study on normal subjects compared to patients with Hirayama disease // Eur. J. Radiol.- 2011.- Vol. 80, №3.- P 724-728.
18. Nalini A., Praveen-Kumar S., Ebenezer B., Ravishankar S., Subbakrishna D. K. Multichannel somato sensory evoked potential study demonstrated abnormalities in cervical cord function in brachial monomelic amyotrophy // Neurol. India.- 2008.- Vol. 56.- P. 368-373.
19. Restuccia D., Rubino M., Valeriani M., Mirabella M., Sabatelli M., Tonali P. Cervical cord dysfunction during neck flexion in Hirayama’s disease // Neurology.- 2003.- Vol. 60.- P 1980-1983.
20. Ammendola A., Gallo А., Iannaccone T., Tedeschi G. Hirayama disease: three cases assessed by F wave, somatosensory and motor evoked potentials and magnetic resonance imaging not supporting flexion myelopathy // Neurol Sci.- 2008.- Vol. 29.- P 303-311.
21. Boelmans K., Kaufmann J., Schmelzer S., Vielhaber S., Kornhuber M., Münchau A., Zierz S., Gaul C. Hirayama disease is a pure spinal motor neuron disorder-a combined DTI and transcranial magnetic stimulation study // J. Neurol.- 2013.- Vol. 260, № 2.- P 540-548.
22. Chen C. J., Chen C. M., Wu C. L., Ro L. S., Chen S. T., Lee T. H. Hirayama Disease: MR Diagnosis // AJNR Am. J. Neuroradiol.- 1998.- Vol. 19.- P. 365-368.
23. Hirayama K. Juvenile muscular atrophy of unilateral upper extremity (Hirayama disease) - half-century progress and establishment since its discovery // Brain Nerve.- 2008.- Vol. 60.- P. 17-29.
24. Tokumaru Y., Hirayama K. Cervical collar therapy for juvenile muscular atrophy of distal upper extremity (Hirayama disease): Results from 38 cases // Rinsho Shinkeigaku.- 2001.- Vol. 41.- P 173-178.
25. Ciceri E. F., Chiapparini L., Erbetta A., Longhi L., Cicardi B., Milani N. et al. Angiographically proven cervical venous engorgement: A possible concurrent cause in the pathophysiology of Hirayama’s myelopathy // Neurol. Sci.- 2010.- Vol. 31.- P 845-848.
Review
For citations:
Savintceva Zh.I., Totolyan N.A., Trofimova T.N., Skvortsova T.Yu., , NON PROGRESSIVE JUVENILE SPINAL MUSCULAR ATROPHY (HIRAYA-MA DISEASE): CLINICAL AND RADIOLOGICAL CORRELATIONS. Diagnostic radiology and radiotherapy. 2016;(2):58-66. (In Russ.)
Views:
8336
Email this article 