Neurology 2000;54:1922-1926
© 2000 American Academy of Neurology
Articles
Cervical dural sac and spinal cord in juvenile muscular atrophy of distal upper extremity
Keizo Hirayama, MD and
Yukio Tokumaru, MD
From the Department of Neurology, School of Medicine, Chiba University, Japan.
Address correspondence and reprint requests to Dr. Keizo Hirayama, Department of Neurology, School of Medicine, Chiba University, 1-8-1 Inohana Chuo-ku, Chiba, Japan 260-0856.
OBJECTIVE: To investigate specificity and significance of dynamic changes of the cervical dural sac and spinal cord during neck flexion in juvenile muscular atrophy of the distal upper extremity.
BACKGROUND: The disorder affects young people—predominantly men—and is progressive for several years. One autopsy case showed ischemic necrosis of the cervical anterior horn, suggesting that the disorder is a type of cervical myelopathy. Some authors classify it as monomelic amyotrophy, implying that it is a focal motor neuron disease.
METHODS: Neuroradiologic examinations including myelography, CT myelography, and MRI in a fully flexed neck position were performed on 73 patients with this disorder and on 20 disease control subjects.
RESULTS: A distinctive finding in the disorder was forward displacement of the cervical dural sac and compressive flattening of the lower cervical cord during neck flexion. The forward displacement was significantly greater in patients with disease duration less than 10 years than in age-matched control subjects and patients in a late, nonprogressive stage.
CONCLUSIONS: Radiologic abnormalities of the lower cervical dural sac and spinal cord support the hypothesis that this disorder is a type of cervical myelopathy.
Key words: Juvenile muscular atrophy—Spinal cord—Dura mater—Neuroradiology—Hirayama disease.
This article has been cited by other articles:
|
|
|
|
 
E. Sagui, E. Correa, D. Ricobono, M. Bregigeon, and C. Brosset
A woman with forearm amyotrophy
BMJ Case Reports,
December 14, 2009;
2009(dec14_1):
bcr0920092292 - bcr0920092292.
[Abstract]
[Full Text]
|
|
|
|
|
|
|
E. L. Dimberg and D. I. Rubin
Weak hands from a flexed neck
Neurology,
September 22, 2009;
73(12):
996 - 996.
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. Pradhan
Bilaterally symmetric form of Hirayama disease
Neurology,
June 16, 2009;
72(24):
2083 - 2089.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Gallo, M.A. Rocca, P. Tortorella, A. Ammendola, G. Tedeschi, and M. Filippi
A Multiparametric Brain and Cord MR Imaging Study of a Patient with Hirayama Disease
AJNR Am. J. Neuroradiol.,
November 1, 2006;
27(10):
2115 - 2117.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
U K Misra, J Kalita, V N Mishra, R V Phadke, and A Hadique
Effect of neck flexion on F wave, somatosensory evoked potentials, and magnetic resonance imaging in Hirayama disease
J. Neurol. Neurosurg. Psychiatry,
May 1, 2006;
77(5):
695 - 698.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
P. -Y. Jeannet, T. Kuntzer, T. Deonna, and E. Roulet-Perez
Hirayama disease associated with a severe rhythmic movement disorder involving neck flexions
Neurology,
April 26, 2005;
64(8):
1478 - 1479.
[Full Text]
[PDF]
|
|
|
|
|
|
|
S Ito, S Kuwabara, T Fukutake, Y Tokumaru, and T Hattori
HyperIgEaemia in patients with juvenile muscular atrophy of the distal upper extremity (Hirayama disease)
J. Neurol. Neurosurg. Psychiatry,
January 1, 2005;
76(1):
132 - 134.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
U. K. Misra, J. Kalita, V. N. Mishra, A. Kesari, and B. Mittal
A Clinical, Magnetic Resonance Imaging, and Survival Motor Neuron Gene Deletion Study of Hirayama Disease
Arch Neurol,
January 1, 2005;
62(1):
120 - 123.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
Y. Baba, M. Nakajima, H. Utsunomiya, Y. Tsuboi, F. Fujiki, T. Kusuhara, and T. Yamada
Magnetic resonance imaging of thoracic epidural venous dilation in Hirayama disease
Neurology,
April 27, 2004;
62(8):
1426 - 1428.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
C.-J. Chen, H.-L. Hsu, Y.-C. Tseng, R.-K. Lyu, C.-M. Chen, Y.-C. Huang, L.-J. Wang, Y.-C. Wong, and L.-C. See
Hirayama Flexion Myelopathy: Neutral-Position MR Imaging Findings--Importance of Loss of Attachment
Radiology,
April 1, 2004;
231(1):
39 - 44.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
D. Gandhi, M. Goyal, P. R. Bourque, and R. Jain
Case 68: Hirayama Disease
Radiology,
March 1, 2004;
230(3):
692 - 696.
[Full Text]
[PDF]
|
|
|
|
|
|
|
H. J. Schelhaas, B. P.C. van de Warrenburg, H. P.H. Kremer, and M. J. Zwarts
The "split hand" phenomenon: Evidence of a spinal origin
Neurology,
December 9, 2003;
61(11):
1619 - 1620.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
D. Restuccia, M. Rubino, M. Valeriani, M. Mirabella, M. Sabatelli, and P. Tonali
Cervical cord dysfunction during neck flexion in Hirayama's disease
Neurology,
June 24, 2003;
60(12):
1980 - 1983.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A Polo, M C. Dossi, A Fiaschi, G P Zanette, and N Rizzuto
Peripheral and segmental spinal abnormalities of median and ulnar somatosensory evoked potentials in Hirayama's disease
J. Neurol. Neurosurg. Psychiatry,
May 1, 2003;
74(5):
627 - 632.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. M. Van den Berg-Vos, J. Visser, H. Franssen, M. de Visser, J. M. B. V. de Jong, S. Kalmijn, J. H. J. Wokke, and L. H. Van den Berg
Sporadic lower motor neuron disease with adult onset: classification of subtypes
Brain,
May 1, 2003;
126(5):
1036 - 1047.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
J Kira and H Ochi
Juvenile muscular atrophy of the distal upper limb (Hirayama disease) associated with atopy
J. Neurol. Neurosurg. Psychiatry,
June 1, 2001;
70(6):
798 - 801.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. Pradhan, R. K. Gupta, K. Hirayama, and Y. Tokumaru
Cervical dural sac and spinal cord in juvenile muscular atrophy of distal upper extremity
Neurology,
February 27, 2001;
56(4):
575 - 575.
[Full Text]
[PDF]
|
|
|
|
