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연제번호 : P-225 북마크
제목 Diagnosis of motor neuron disease in patient with cerebellar lesion
소속 Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, South Korea, Department of Rehabilitation Medicine1
저자 Soyeon Jun1*, Youngjin Ko1†, Heeyoung Lim1, Inah Kim1
Introduction
Motor neuron disease (MND) may be difficult to diagnose cause the symptoms are mimic many other diseases. Physicians should consider clinical symptoms, specific findings in physical exams, and family history of the diseases. Also, electrodiagnostic exam can be helpful. Despite the difficulty of diagnosing MND, early diagnosis is important in the treatment of patients. Here, we introduce a case report of the middle-aged woman with a progressive gait disturbance. Furthermore, she was accompanied by a cerebellar lesion, making it difficult to find the cause of the gait disturbance.

Case report
A 59-year-old woman visited our clinic referred from the neurosurgery department for the electrodiagnostic exam for progressive gait disturbance. Five months before her visit to the hospital, she felt a gradual weakening of the muscle power in both legs and right hand. She first visited a local clinic to evaluate that symptoms and performed brain and cervical magnetic resonance images (MRI). There were nonspecific findings in cervical MRI images but there were cerebellar encephaloceles without any traumatic history (Figure 1). On physical examination, there was slight weakness of the right upper arm and bilateral knee extensor power to G grade. Muscle atrophy was observed in the bilateral thenar and first dorsal interosseous muscles. She showed normal sensory symptoms and mild increased deep tendon reflexed in bilateral biceps jerk, triceps jerk, and knee jerk. Also, there were some pathologic reflexes like Hoffmann sign and ankle clonus. On the nerve conduction study (NCS) there was non specific finding. On needle electromyography, abnormal spontaneous activities at rest in left (L4-L5) paraspinalis, bilateral upper and lower muscles (Figure 2). Also, we examed sensory evoked potential and normal latencies in both median, and both posterior tibial nerves were observed. With these findings, we considered the motor neuron disease, especially amyotrophic lateral sclerosis accompanied by bilateral L5-S1 radiculopathy. After then, we prescribed riluzole 100mg per day and physical therapy to the patient. She is continuing to follow up through the outpatient clinic.

Conclusion
Similar clinical features between the motor neuron disease and the other diseases make MND difficult to diagnose. Despite the possibility of walking disorders related to cerebellar lesions, we suspected the possibility of MND in the patient and could make an accurate diagnosis early through further examination such as electromyography. Therefore, we should not overlook the possibility of MND when examining patients with gait disturbance and muscle weakness.
File.1: Fig1.jpg
T2 weighted axonal images showed multifocal encephalomalatic changes at the posterior surface of bilateral cerebellum with adjacent multiple small CSF-filling defects with suspicion of containing brain tissue in the occipital bone. These findings were suggestive of occipital intradiploic encephaloceles.
File.2: Fig2.jpg
Needle Electromyography