Survey on the Diagnostic Process of Amyotrophic Lateral Sclerosis
2
조회
0
다운로드
2
인용
0
참고문헌
키워드
초록
Objective To emphasize the need for precise diagnosis of amyotrophic lateral sclerosis (ALS), a progressive and degenerative disease of upper and lower motor neurons that often present initially with weakness at the upper or lower extremities, and frequently misdiagnosed as myelopathy, radiculopathy, peripheral neuropathy or arthropathy that may ultimately lead to unnecessary treatments including surgical procedures. Method We retrospectively reviewed medical records of 331 ALS patients who visited our hospital between 1998 and 2008. Symptoms at onset, progression of disease, radiologic findings, surgeries prior to diagnosis of ALS, outcome after surgery or conservative treatments, and electrodiagnostic study results were reviewed. Results Among the 331 patients with ALS, 34 (10.3%) had a history of surgical procedure and 37 (11.1%) underwent conservative treatment prior to diagnosis of ALS. 34 patients with a mean disease duration at diagnosis of 20.0±14.9 months, had surgery for symptoms that were later attributable to ALS. In 30 of the 34 patients, symptoms did not resolve after the intervention. 37 patients with a mean disease duration at diagnosis of 16.6±14.3 months, underwent conservative treatments such as physical therapy prior to diagnosis of ALS. Only in one patient (2.7%), symptoms improved after conservative treatment. Conclusion In the absence of a single confirmatory study for the diagnosis of ALS, clinical findings may be misinterpreted, leading to an erroneous diagnosis. Therefore, closer and more careful follow-up is necessary for patients with limb weakness in the absence of sensory symptoms, or bulbar abnormalities such as dysarthria and dysphagia.
Abstract
Objective To emphasize the need for precise diagnosis of amyotrophic lateral sclerosis (ALS), a progressive and degenerative disease of upper and lower motor neurons that often present initially with weakness at the upper or lower extremities, and frequently misdiagnosed as myelopathy, radiculopathy, peripheral neuropathy or arthropathy that may ultimately lead to unnecessary treatments including surgical procedures. Method We retrospectively reviewed medical records of 331 ALS patients who visited our hospital between 1998 and 2008. Symptoms at onset, progression of disease, radiologic findings, surgeries prior to diagnosis of ALS, outcome after surgery or conservative treatments, and electrodiagnostic study results were reviewed. Results Among the 331 patients with ALS, 34 (10.3%) had a history of surgical procedure and 37 (11.1%) underwent conservative treatment prior to diagnosis of ALS. 34 patients with a mean disease duration at diagnosis of 20.0±14.9 months, had surgery for symptoms that were later attributable to ALS. In 30 of the 34 patients, symptoms did not resolve after the intervention. 37 patients with a mean disease duration at diagnosis of 16.6±14.3 months, underwent conservative treatments such as physical therapy prior to diagnosis of ALS. Only in one patient (2.7%), symptoms improved after conservative treatment. Conclusion In the absence of a single confirmatory study for the diagnosis of ALS, clinical findings may be misinterpreted, leading to an erroneous diagnosis. Therefore, closer and more careful follow-up is necessary for patients with limb weakness in the absence of sensory symptoms, or bulbar abnormalities such as dysarthria and dysphagia.
참고문헌 (16)
- Norris F, "Onset, natural history and outcome in idiopathic adult motor neuron disease", J Neurol Sci, 118, pp.48~55 (1993)
- Belsh JM, "Misdiagnosis in patients with amyotrophic lateral sclerosis", Arch Intern Med, 150, pp.2301~2305 (1990)
- Househam E, "Diagnostic delay in amyotrophic lateral sclerosis: what scope for improvement?", J Neurol Sci, 180, pp.76~81 (2000)
- Sostarko M, "Severe progression of ALS", MND after intervertebral discectomy, 1998, pp.160(Suppl 1), S42~S46
- Bourke SC, "Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial", Lancet Neurol, 5, pp.140~147 (2006)
- Bourke SC, "Noninvasive ventilation in ALS: indications and effect on quality of life", Neurology, 61, pp.171~177 (2003)
- Belsh JM, "The amyotrophic lateral sclerosis (ALS) patient perspective on misdiagnosis and its repercussions", J Neurol Sci, 139, pp.110~116 (1996)
- Traynor BJ, "Amyotrophic lateral sclerosis mimic syndromes: a population-based study", Arch Neurol, 57, pp.109~113 (2000)
- Yamada M, "Amyotrophic lateral sclerosis: frequent complications by cervical spondylosis", J Orthop Sci, 8, pp.878~881 (2003)
- Brooks BR, "Defining optimal management in ALS: from first symptoms to announcement", Neurology, 53(Suppl 5), pp.S1~S3 (1999)
- Traynor BJ, "Clinical features of amyotrophic lateral sclerosis according to the El Escorial and Airlie House diagnostic criteria: a population-based study", Arch Neurol, 57, pp.1171~1176 (2000)
- Brooks BR, "El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. Subcommittee on Motor Neuron Diseases", Amyotrophic Lateral Sclerosis of the World Federation of Neurology Research Group on Neuromuscular Diseases and the El Escorial "Clinical limits of amyotrophic lateral sclerosis" workshop contributors, 1994, pp.124, 96~107
- Srinivasan J, "Inappropriate surgeries resulting from misdiagnosis of early amyotrophic lateral sclerosis", Muscle Nerve, 34, pp.359~360 (2006)
- Yoshor D, "Incidence and characteristics of spinal decompression surgery after the onset of symptoms of amyotrophic lateral sclerosis", Neurosurgery, 57, pp.984~989 (2005)
- Meininger V, "Getting the diagnosis right: beyond El Escorial", J Neurol, 246(Suppl 3), pp.10~12 (1999)
- Traynor BJ, "Clinical features of amyotrophic lateral sclerosis according to the El Escorial and Airlie House diagnostic criteria: a population-based study", Arch Neurol, 57, pp.1171~1176 (2000)