Response of Hirayama disease to surgical intervention: case report
Keywords:
Hirayama disease, Monomelic amyotrophy, Juvenile muscular atrophyAbstract
Hirayama disease also known as monomelic amyotrophy, primarily involves distal upper limb extremities.It differs from the known types of motor neuron diseases because of its nonprogressive behavior and pathologic findings of focal ischemic changes in the anterior horn of the lower cervical cord. We present a young male with Hirayama disease who had a left upper extremity involvement which was progressive in nature. He didn’t respond with initial treatment of cervical collar. Consequently surgical intervention improves muscle weakness and decrease the neurological deficit.
References
Hirayama K, Tokumaru Y. Cervical Dural sac and spinal cord in juvenile muscular atrophy of distal upper extremity. Neurology. 2000;54:1922-6.
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;27:412-9.
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. Rinsho Shinkeigaku. 1987;27:412-9.
Ando T, Fukatsu H, Kameyama T, Takahashi A, Yamada H. A case of flexion myelopathy presenting with reversible muscular weakness and atrophy of the unilateral proximal upper limb Rinsho Shinkeigaku. 1993 May;33(5):575-8.
Tokumaru Y, Hirayama K. Cervical collar therapy for juvenile muscular atrophy of distal upper extremity (Hirayama disease): results from 38 cases. Rinsho Shinkeigaku. 2001;41:173-8.
Tokumaru Y, Hirayama K. A cervical collar therapy for nonprogressive juvenile spinal muscular atrophy of the distal upper limb (Hirayama's disease). Clin Neurol (Tokyo). 1992;32:1102-6.
H. Imamura, S. Matsumoto, M. Hayase, Y. Oda, H. Kikuchi, M. Takano. A case of Hirayama's disease successfully treated by anterior cervical decompression and fusion. Brain Nerve. 2001;53(11):1033-8.
Masaki T, Hashida H, Sakuta M, Kunogi J. A case of flexion myelopathy presenting juvenile segmental muscular atrophy of upper extremities: a successful treatment by cervical spine immobilization. Rinsho Shinkeigaku. 1990 Jun;30(6):625-9.
S. Konno, S. Goto, M. Murakami, M. Mochizuki, H. Motegi, H. Moriya. Juvenile amyotrophy of the distal upper extremity: pathologic findings of the Dura mater and surgical management. Spine. 1997;22(5):486-92.