Abstract Details

Title Characteristics of respiratory dysfunction in amyotrophic lateral sclerosis: a retrospective cohort study in 236 cases

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) P1 - Poster Session 1 (5:30 PM-6:30 PM)

Poster/Presentation
Number 12-023

Objective To access the characteristics of respiratory deterioration in Amyotrophic lateral sclerosis (ALS) patients, including the effect of the type of disease onset, as well as the progression rates.

Background Respiratory deterioration is the main cause of ALS. Currently, it is still controversial of the characteristics of respiratory deterioration in ALS patients.

Design/Methods We performed a retrospective cohort study including 236 ALS patients at PUMCH. Pulmonary function tests (PFT) values (forced vital capacity (FVC%), forced expired volume in one second (FEV1%) and peak expiratory flow (PEF%)) records as well as other clinical features were collected. Patents were subdivided into bulbar-onset and limb-onset as well as bulbar-involved and non-bulbar involved group. ΔPFT was calculated as follows: value of PFT at the first examination - value of PEF at the last examination/time gap between the examinations (months).

Results Bulbar-onset of symptoms was seen in 19.9% patients, while 48.9% cases showed bulbar involvement during the courses. Mean FVC%,FEV1% and PEF% value was 88.9±21.5%,87.7±19.8% and 84.7±22.5%,respectively. Non-bulbar involved group showed higher PFT value (FVC%, FEV1% and PEF%) than the bulbar-involved group (p=0.004,0.002 and <0.001). But no significant difference was observed between the bulbar-onset and limb-onset group. After full multivariable adjustment, ALS patients without bulbar involvement were 6.38% (-0.12,12.87), 6.32% (0.41,12.22) and 7.49% (0.59,14.40) higher than those with bulbar involved (p=0.056, p=0.038 and p=0.035). Other risk factors correlated with poorer PEF values were male, age over 60, long disease duration and poor ALSFRS-R score. ΔFVC%?ΔFEV1% and ΔPEF% of patients in bulbar-involved group were significantly higher than those in limb-involved group with no difference found between bulbar and limb onset groups.

Conclusions Bulbar involvement predicted poorer respiratory function in ALS patients compared with limb-only involvement, no matter whether the involvement was the onset symptom. The progression rate was higher in the bulbar involved patients than those with limb-only involvement.

Authors/Disclosures
PRESENTER	No disclosure on file
Dongchao Shen (Peking Union Medical College Hospital)	No disclosure on file
Kang Zhang	No disclosure on file
	No disclosure on file
	No disclosure on file
Liying Cui, PhD (Peking Union Medical College Hospital)	No disclosure on file

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