Nosology of Juvenile Muscular Atrophy of Distal Upper Extremity: From Monomelic Amyotrophy to Hirayama Disease—Indian Perspective

Since its original description by Keizo Hirayama in 1959, “juvenile muscular atrophy of the unilateral upper extremity” has been described under many nomenclatures from the east. Hirayama disease (HD), also interchangeably referred to as monomelic amyotrophy, has been more frequently recognised in the west only in the last two decades. HD presents in adolescence and young adulthood with insidious onset unilateral or bilateral asymmetric atrophy of hand and forearm with sparing of brachioradialis giving the characteristic appearance of oblique amyotrophy. Symmetrically bilateral disease has also been recognized. Believed to be a cervical flexion myelopathy, HD differs from motor neuron diseases because of its nonprogressive course and pathologic findings of chronic microcirculatory changes in the lower cervical cord. Electromyography shows features of acute and/or chronic denervation in C7, C8, and T1 myotomes in clinically affected limb and sometimes also in clinically unaffected contralateral limb. Dynamic forward displacement of dura in flexion causes asymmetric flattening of lower cervical cord. While dynamic contrast magnetic resonance imaging is diagnostic, routine study has high predictive value. There is a need to lump all the nomenclatures under the rubric of HD as prognosis in this condition is benign and prompt diagnosis is important to institute early collar therapy.

Hirayama disease

Object Controversy exists over the choice of surgical candidates and prognosis of Hirayama disease. The purpose of this study was to examine the outcomes of patients with cervical flexion myelopathy who received surgical treatment. Methods A retrospective study was conducted. From May 2002 through December 2006, 6 young patients with cervical flexion myelopathy were seen in the Department of Neurosurgery at Chang Gung Memorial Hospital. The neurological and radiological findings in all 6 patients met the criteria for Hirayama disease. All patients had evidence of a tight dural canal or forward migration of the posterior wall of the dural canal in dynamic MR imaging studies. Five patients were treated with surgical decompressive procedures (4 anterior and 1 posterior) and 1 patient received conservative treatment. Duration of follow-up ranged from 13 months to 4 years. Results Motor function improved in 3 of 5 surgically treated patients and sensory function improved in 2. Neurological symptoms were unchanged in the conservatively treated patient. During follow-up MR imaging in the surgical group, anterior effacement during neck flexion was noted in 1 patient treated with a posterior approach. Conclusions Hirayama disease is so rare that it is easily misdiagnosed. Diagnosis is achieved via clinical presentation, neurophysiological examination, and neuroradiological imaging studies (dynamic MR imaging). The anterior decompressive approach may be better for patients showing anterior effacement and severe cervical kyphosis during neck flexion in MR imaging.

Dynamic electrophysiological examination of cervical flexion myelopathy

Cervical flexion myelopathy is thought to arise following compression of the spinal cord by vertebrae or intervertebral discs and dura mater, or from overstretching of the spinal cord induced by cervical spinal flexion. However, the influence of spinal flexion on the spinal cord and the detailed origins of this disease are unknown. In this article the authors report a case of cervical flexion myelopathy in which dynamic electrophysiological examination was performed using an epidural electrode. This investigation showed the real-time influence of flexion of the cervical spine on spinal cord function. This technique was considered to be useful for diagnosis and in decision making for treatment. Anterior fusion was the optimal surgical method for treating this disease.

Biomechanical study of cervical flexion myelopathy using a three-dimensional finite element method

Object The goal of this study was to perform a biomechanical study of cervical flexion myelopathy (CFM) using a finite element method. Methods A 3D finite element model of the spinal cord was established consisting of gray matter, white matter, and pia mater. After the application of semi-static compression, the model underwent anterior flexion to simulate CFM. The flexion angles used were 5° and 10°, and stress distributions inside the spinal cord were then evaluated. Results Stresses on the spinal cord were very low under semi-static compression but increased after 5° of flexion was applied. Stresses were concentrated in the gray matter, especially the anterior and posterior horns. The stresses became much higher after application of 10° of flexion and were observed in the gray matter, posterior funiculus, and a portion of the lateral funiculus. Conclusions The 5° model was considered to represent the mild type of CFM. This type corresponds to the cases described in the original report by Hirayama and colleagues. The main symptom of this type of CFM is muscle atrophy and weakness caused by the lesion of the anterior horn. The 10° model was considered to represent a severe type of CFM and was associated with lesions in the posterior fand lateral funiculi. This type of CFM corresponds to the more recently reported clinical cases with combined long tract signs and sensory disturbance.