Epidemiology Of Amyotrophic Lateral Sclerosis In Texas Print E-mail

The AANEM 59th annual meeting, October 3-6, 2012; Orlando Florida

Introduction: Amyotrophic Lateral Sclerosis (ALS) is a progressive disease causing degeneration of upper and lower motor neurons.

By: A. M. Paker, K. Sheikh*, A. Shaibani* (Galveston, TX; Houston, TX)

Objective: Aim of our study was to investigate the epidemiological features of the ALS patients in Texas. Methods: The authors identified 64 patients diagnosed with ALS seen in a tertiary neuromuscular center, from January 2007 till December 2011. Other potentially treatable or definable diseases were ruled out. Results: All 64 patients were diagnosed with definite or probable ALS as per revised El Escorial criteria. Eight (12.5%) patients were diagnosed with progressive bulbar palsy (PBP). Men to woman ratio were 1.4:1 (p=0.211), but 63% of patients with PBP were women. Median age of onset was 63 years. Patients with PBP were older than spinal onset ALS 66.7 ± 9.8 years vs. 61.1 ± 12.2 years. There was no difference in survival between these two groups (p=0.395). Mean interval from onset to diagnosis was 12.5±11.9 months. 28 patients (43.75%) died at mean age of 67.7 ±10 years after mean 23.8 ± 18 months of the onset of symptoms. Survival ranged from 5 months to 11.3 year. The median survival time was 38 months. The 3 year survival rate from onset of symptoms was 51.7% (95% CI 37%–64.4&). Increasing age at onset of symptoms was significantly associated with shorter disease duration (p<0.0001). 16 (26%) patients reported psychiatric problems including emotional lability and depression.

Conclusion: Epidemiology of ALS in Texas is very similar to the national trends, except gender ratio which is approaching equality.

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