J Clin Neurol.  2012 Dec;8(4):293-300. 10.3988/jcn.2012.8.4.293.

Current Status of the Diagnosis and Management of Amyotrophic Lateral Sclerosis in Korea: A Multi-Center Cross-Sectional Study

• Bae JS
• Hong YH
• Baek W
• Sohn EH
• Cho JY
• Kim BJ
• Kim SH
• The Korean ALS/MND Research Group

• KMID: 2287581
• DOI: http://doi.org/10.3988/jcn.2012.8.4.293

Abstract

BACKGROUND AND PURPOSE
Recently published, evidence-based guidelines should alter the management of amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND). However, the newest recommendations for ALS/MND therapy are not reflected in actual clinical practice. We sought to evaluate the current status of the diagnosis and management of ALS in Korea.
METHODS
The Korean ALS/MND research group was organized in 2010, involving more than 50 neurologists from neuromuscular centers in Korea. Participating centers collected data from April to September 2010 on the diagnosis and management of patients with ALS. Data forms from the ALS patient care database, which is a component of the ALS clinical assessment, research, and education program (http://www.outcomes-umassmed.org/ALS/), were modified and used for data collection.
RESULTS
In total, 373 sporadic ALS cases from 35 centers were enrolled. The demographic features and clinical findings were similar to those in previous reports from other countries. The mean age at onset was 50-60 years, and a slight male predominance was observed. The enrolled patients predominantly showed focal onset of cervical or lumbosacral symptoms. Only about one-half of the indicated patients (31.4%) received a physician's recommendation for a parenteral gastrostomy, and 18.1% underwent the procedure. Noninvasive ventilation was recommended in 23% of patients, but applied in only 9.5% of them. Tracheostomy was performed in 12.7% of patients.
CONCLUSIONS
The demographic and clinical features of the diagnosis and management of ALS in Korea are similar to those reported in other countries; however, supportive management, as recommended in evidence-based guidelines, are not yet widely recommended or performed for patients with ALS in Korea.

Keyword

amyotrophic lateral sclerosis; diagnosis; Korea; palliative care; treatment

MeSH Terms

Amyotrophic Lateral Sclerosis
Cross-Sectional Studies
Gastrostomy
Humans
Korea
Male
Neurons
Noninvasive Ventilation
Palliative Care
Patient Care
Tracheostomy

Figure

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  Fig. 1 Patient distribution. Data were collected from 35 institutions by either a cross-sectional survey or retrospective review of medical records. Data were sorted according to the total number of patients (white bars) in descending order. Gray bars represent the cross-sectional survey.

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  Fig. 2 Clinical features of 373 patients with sporadic amyotrophic lateral sclerosis (ALS). Age and sex distributions (A), first symptoms at onset (B), level of diagnostic certainty according to the revised (and modified) El Escorial Criteria (C), distribution of clinical evidence for upper and lower motor neuron dysfunction, and distribution of electromyographic evidence for lower motor neuron loss across four regions (D).

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  Fig. 3 Rates of percutaneous endoscopic gastrostomy (PEG), noninvasive intermittent positive-pressure ventilation (NIPPV), and tracheostomy. Seven institutions that contributed the greatest number of patients were chosen and are represented by different colors. Numbers within parentheses indicate the number of patients whose data were collected in the corresponding institution. The rates of supportive intervention varied between the centers, including of PEG and NIPPV (p=0.028 and 0.016, respectively).

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