Clinical Characteristics, Electrodiagnostic, and Imaging Findings of Atypical Forms of Motor Neuron Disease

OBJECTIVE: To describe the clinical characteristics, electrodiagnostic, and imaging findings of Hirayama disease (HD), late onset monomelic amyotrophy (LMA), and brachial amyotrophic diplegia (BAD). METHOD: A retrospective analysis of the medical records, electrodiagnostic, and imaging findings of 12 patients (4 HD, 2 LMA, 6 BAD) was done. For patients whose last clinic follow-up exceeded 6 months a telephone survey was done to see if there were any symptom changes. RESULTS: The clinical, electrodiagnostic, and imaging findings of the HD and BAD patients were similar to previous studies. Except for a later onset, age disease duration was too short to distinguish LMA from HD or other motor neuron diseases. One patient in the BAD group progressed to amyotrophic lateral sclerosis (ALS) and another died due to undetermined respiratory failure. These two patients showed abnormalities in their lower extremities, thoracic paraspinal, and craniocervical muscles on needle electromyography. Except for another patient, none of the other three patients showed abnormalities in their lower extremities, thoracic paraspinals, or craniocervical muscles on needle electromyography. CONCLUSION: HD and BAD can be considered as separate disease entities. However, a longer follow-up period than previously recommended is necessary to differentiate BAD from ALS. Follow-up period was too short to determine whether LMA can also be considered as a separate disease entity.

The Prevalence and Clinical Features of Diffuse Idiopathic Skeletal Hyperostosis in the Patients with Dorsal Back Pain

OBJECTIVE: To investigate the prevalence and clinical features of diffuse idiopathic skeletal hyperostosis (DISH) in patients with dorsal back pain. METHOD: A total of 229 patients (119 men and 110 women) with dorsal back pain were included. Medical records including age, sex, chief complaints, medical history, physical findings, laboratory data, and musculoskeletal radiographs were reviewed retrospectively. RESULTS: Twenty-seven (11.8%, by Resnick's criteria) and thirty-eight (16.6%, by Julkunen's criteria) of the 229 patients were diagnosed with DISH by thoracic spine radiographs. A significant increase of risk with age was observed (Resnick's criteria: odds ratio 1.10, 95% CI 1.05~1.16, p< 0.001, Julkunen's criteria: odds ratio 1.08, 95% CI 1.04~1.12, p<0.001). Male sex (Resnick's criteria: odds ratio 2.65, 95% CI 1.01~6.95, p=0.048, Julkunen's criteria: odds ratio 2.87, 95% CI 1.27~6.59, p=0.011) and inflammatory pain (Resnick's criteria: odds ratio 7.76, 95% CI 2.69~22.33, p<0.001, Julkunen's criteria: odds ratio 3.84, 95% CI 1.55~9.47, p=0.004) showed higher risk for development of DISH. Mean body mass index of patients with DISH were higher in all age groups, and they showed higher prevalence of DM and hyperlipidemia than general population. CONCLUSION: Patients with dorsal back pain showed higher prevalence of DISH than previous results with random samples. Aging, male sex and inflammatory pain might be a risk factor for development of DISH. Also, obesity, DM and hyperlipidemia might be strongly related with DISH.

Neuroplasticity Induced by Robot-assisted Gait Training in a Stroke Patient: A case report

In this case study, we investigated the effects of robot-assisted gait training on the plasticity of motor system in a stroke patient using functional MRI. A patient who suffered from the left hemiparesis following the right MCA infarction performed gait training with a robot-assisted gait orthosis. Before and after gait training, motor performances were assessed and functional MRIs were acquired with motor activation task of affected lower limb. After gait training with a robot-assisted orthosis, the patient's motor performances were improved and cortical activities were changed. Activation in the ipsilesional primary sensorimotor cortex was increased and cortical reorganization was induced in a way that nearby regions were recruited for the movement of affected lower limb. The results of this study showed that gait training with a robot-assisted orthosis induced cortical reorganization of the motor network that resulted in enhancement of motor performance of the lower limb.