Amyotrophic Lateral Sclerosis at Geisinger Medical Center: Patient Demographics and Clinical Characteristics
Minali Tare1, Anvi Kumar1, J. David Avila2
1Geisinger Commonwealth School of Medicine, 2Geisinger Medical Center
Objective:
This study investigated the clinical features and demographics of Amyotrophic Lateral Sclerosis (ALS) patients at our ALS Multidisciplinary Clinic, aiming to compare these findings with broader population trends.
Background:
ALS is a progressive neurodegenerative disease characterized by motor neuron destruction, resulting in muscle weakness and inability to control movements typically starting between ages 51-66. The ALS Functional Rating Scale (FRS) assesses disease severity based on ease of completing activities of daily living. The median duration of disease is approximately 3.5 years after symptom onset.
Design/Methods:
This retrospective study at our ALS Multidisciplinary Clinic reviewed 44 patient charts using EPIC electronic medical record system. Data collected included demographics, disease onset, progression, diagnostic delays, and genetic testing outcomes.
Results:
There was a predominance of limb-onset ALS (79.5%) compared to bulbar-onset ALS, consistent with national averages. Age at diagnosis and diagnostic delay were also comparable to national standards, with medians at 63 years and 11.5 months, respectively. Disease progression as measured by ALS-FRS scores mirrored the general population at a median rate of decline of 0.92 points per month. Genetic causes were identified in 14% of patients, whereas familial ALS is typically seen in less than 10% of cases. Our population exhibited a higher proportion of female ALS patients compared to the male predominance seen in the general population. Disease duration varied significantly, with a maximum of 132 months post-symptom onset.
Conclusions:
Overall, the study concluded that the patients seen at our ALS clinic had similar clinical traits to the broader population of ALS patients. Further analyses of the data can determine if the diagnostic delay in our patient population correlates with their rate of disease progression. Additionally, we can investigate if variables such as genetic testing results and initiation of therapies impact the overall duration of disease in these patients.
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