Treatment of progressive paralysis associated with cervical myelopathy and suspected amyotrophic lateral sclerosis: A case report

BACKGROUND: Amyotrophic lateral sclerosis (ALS) is an intractable progressive disease, with an incidence of 2.2– 2.3 per 100,000 individuals, which is not extremely low. ALS symptoms are accompanied by spinal myeloradicular motor deficit; its differential diagnosis is must because progressive paralysis needs emergency surgery. CASE DESCRIPTION: A 64-year-old man with suspected ALS showing progressive paralysis with cervical myelopathy was diagnosed as normal after performing a nerve conduction study preoperatively. Postoperative diffuse fasciculation after posterior decompression allowed the diagnosis of ALS through needle electromyography (EMG). Thereafter, the patient’s condition slowly deteriorated and he died after 16 months. CONCLUSION: Surgery might aggravate ALS symptoms; however, surgery for progressive paralysis in patients with suspected ALS is required for distinguishing patients with non-ALS paralysis. Approximately 70% of cases have spinal-onset ALS lacking typical cranial nerve symptoms; thus, to prevent unnecessary surgery, surgeons should at least know the characteristic features of ALS and should be aware that early diagnosis requires needle EMG for definitive diagnosis of ALS.