Sensitivity of Pyramidal Signs in Patients with Cervical Spondylotic Myelopathy

STUDY DESIGN: This was a retrospective study. PURPOSE: The purpose of this study was to study the relationship between prevalence of pyramidal signs and the severity of cervical myelopathy. The study is focused on patients having increased signal intensity in T2-weighted magnetic resonance imaging. OVERVIEW OF LITERATURE: Cervical spondylotic myelopathy is the most common cause of spinal cord dysfunction in elderly population. It is the consequence of spondylotic changes leading to cervical cord injury with resulting clinical deficits. Diagnosis in such patients is made based on clinical and radiographic features. A patient must have both symptoms and signs consistent with cervical cord injury as well as radiographic evidence of damage to spondylotic cord. METHODS: Forty-six patients with complaint of cervical spondylotic myelopathy with increased signal intensity in T2-weighted magnetic resonance imaging were included in the study. The neurological finding of the patients was reviewed for the presence of pyramidal signs. The prevalence of each pyramidal sign was calculated and correlated to severity of cervical myelopathy. The motor function scores of the upper and lower extremities for cervical myelopathy set by the Japanese Orthopedic Association (motor Japanese Orthopaedic Association score, m-JOA) scores were used to assess severity of myelopathy. RESULTS: The most prevalent signs were hyperreflexia (89.1%), Hoffmann reflex (80.4%), Babiniski sign (56.5%), and ankle clonus (39.1%). Babiniski sign, ankle clonus, and Hoffmann reflex showed significant association with the lower m-JOA score. CONCLUSIONS: In patients with cervical myelopathy, hyperreflexia exhibited highest sensitivity whereas ankle clonus demonstrated lowest sensitivity. The prevalence of the pyramidal signs is correlated with increasing severity of myelopathy.

Monomelic Amyotrophy (Hirayama Disease) With Upper Motor Neuron Signs: A Case Report

Monomelic amyotrophy (MMA), also known as Hirayama disease, is a sporadic juvenile muscular atrophy in the distal upper extremities. This disorder rarely involves proximal upper extremities and presents minimal sensory symptoms with no upper motor neuron (UMN) signs. It is caused by anterior displacement of the posterior dural sac and compression of the cervical cord during neck flexion. An 18-year-old boy visited our clinic with a 5-year history of left upper extremity pain and slowly progressive weakness affecting the left shoulder. Atrophy was present in the left supraspinatus and infraspinatus. On neurological examination, positive UMN signs were evident in both upper and lower extremities. Electrodiagnostic study showed root lesion involving the fifth to seventh cervical segment of the cord with chronic and ongoing denervation in the fifth and sixth cervical segment innervated muscles. Cervical magnetic resonance imaging (MRI) showed asymmetric cord atrophy apparent in the left side and intramedullary high signal intensity along the fourth to sixth cervical vertebral levels. With neck flexion, cervical MRI revealed anterior displacement of posterior dural sac, which results in the cord compression of those segments. The mechanisms of myelopathy in our patient seem to be same as that of MMA. We report a MMA patient involving proximal limb with UMN signs in biomechanical concerns and discuss clinical importance of cervical MRI with neck flexion. The case highlights that clinical variation might cause misdiagnosis.