Clinical Spectrum of Motor Neuron Disease in Hanyang University Hospital ALS Clinic

Abstract

Clinical Spectrum of Motor Neuron Disease in Hanyang University Hospital ALS Clinic

Hyun-dong Park, Hyun-Young Kim, Hee-Jin Kim, Hee-Tae Kim, Juhan Kim, Seung Hyun Kim

Department of Neurology, College of Medicine, Hanyang University

Background & Objectives: Motor neuron disease (MND) are degenerative disorders that involve upper and lower motor neuron in various types. Many factors are associated with MND and understanding of MND variability is important to know prognosis and progression patterns. We performed a study to evaluate the spectrum of motor neuron disease at Hanyang University Hospital ALS (Amyotrophic lateral sclerosis) clinic.

Method: This study was performed at Hanyang University ALS clinic during a 5-year period. Patient and family informations are gathered at the first visit to the clinic. The clinical data on the gender, age, age at onset, initial symptoms, date when respiratory and gastrointestinal support was applied (tracheostomy, noninvasive positive pressure ventilation or gastrostomy), and date of death were colleted. We divided the clinical spectrum of motor neuron disease as follows: ALS, primary lateral sclerosis (PLS), progressive muscular atrophy (PMA), progressive bulbar palsy (PBP), spinobulbar muscular atoprhy (SBMA, Kennedy syndrome), benign focal amyotrophy (BFA), bibrachial amyotrophic diplegia (BAD) and ALS plus syndrome. ALS was classified as sporadic vs. familial, and spinal onset vs. bulbar onset type.

Results: Between March 2003 and August 2008, 241 patients were enrolled in this study. In our study, we diagnosed 218 ALS patients, 6 ALS-Dementia, 12 SBMA, 12 BFA, 6 BAD, 1 PMA and 2 undetermined. Among our patient with ALS, 210 patients were sporadic and 8 patients were familial type. SOD1 mutations were identified in three families with ALS. Male to female ratio was 1.84:1. Mean age at onset of MND was 55.75±12.24 years. Among ALS patients, 25 patients died during follow up period. The most common clinical subtype of ALS was spinal onset type (76.7%) and bulbar onset type was 23.3%. The mean interval from symptom onset to gastrostomy and tracheostomy were 28.7±5.3 months and 31.5±6.5 months.

Conclusion: In our study, gastrostomy interval was shorter and bular onset type ratio was higher than previous studies. As we only studied the epidemiology of MND at our clinic, more studies and multidisciplinary approaches are needed.