Abstract Details

Title Distribution of Serum Creatine Kinase Levels in Amyotrophic Lateral Sclerosis

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) P5 - Poster Session 5 (8:00 AM-9:00 AM)

Poster/Presentation
Number 1-010

Objective

We determined the range of creatinine kinase (CK) levels in patients with amyotrophic lateral sclerosis (ALS) using the Pooled Resource Open-Access ALS Clinical Trials (PRO-ACT) database.

Background We recently saw a 58 year-old man with progressive fatigue, weight loss, and weakness. Exam revealed intrinsic hand and right thigh atrophy and weakness. Electrodiagnostic studies demonstrated active and chronic neuropathic changes in paraspinal musculature and the weak muscles of the upper and lower extremities. CK was 1,905 units/liter (Ref 0-200 units/liter). Due to high CK, the patient underwent a muscle biopsy to exclude myopathy. Pathology showed denervation patterns without concomitant myopathy. He later developed pathologically brisk reflexes, meeting El Escorial criteria for probable ALS. Recognizing our error in misattributing the high CK to another process, we investigated the distribution of CK in ALS.

Design/Methods We downloaded patient data from the publicly available PRO-ACT database containing over 8 million data points for 8,600 de-identified ALS patients previously enrolled in clinical trials, therefore meeting strict ALS diagnostic criteria. We used Statistical Package for the Social Sciences (SPSS) v25 for analyses.

Results 4,452 ALS patients had 39,249 CK tests. Values were logarithmically distributed. 51.4% of patients had normal CK, 3% had CK over 1,000 units/liter, and 0.3% (n=13) had CK over 2,000 units/liter. CK was not correlated with age, weight, riluzole use, or sex (p>0.20).

Conclusions While most ALS patients have normal or mildly elevated CK, some individuals have elevations over 2,000 units/liter. A literature review reveals another author who confused early lower motor neuron predominant ALS with myositis due to high CK. Undoubtedly other unpublished cases exist. Clinicians should be aware of the wide range of CK in ALS, and consider muscle biopsies only when clinical or electrodiagnostic evidence suggests myopathy.

Authors/Disclosures
Devin E. Prior, MD (Providence Regional Medical Center) PRESENTER	No disclosure on file
Elijah Stommel, MD, F�鶹��ýӳ�� (Dartmouth-Hitchock Med Ctr/Dept of Neuro)	Dr. Stommel has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for Suslick Attorney. The institution of Dr. Stommel has received research support from CDC.
Victoria Lawson, MD, F�鶹��ýӳ�� (Department of Neurology, Dartmouth-Hitchcock Medical Center)	Dr. Lawson has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sanofi.
	No disclosure on file
Nathaniel M. Robbins, MD (MGB)	Dr. Robbins has received personal compensation in the range of $0-$499 for serving as a Consultant for Red Nucleus. Dr. Robbins has received personal compensation in the range of $0-$499 for serving as a Consultant for TDG Health. Dr. Robbins has received personal compensation in the range of $0-$499 for serving as a Consultant for Jupiter Life Science Consulting. Dr. Robbins has received personal compensation in the range of $50,000-$99,999 for serving as an Expert Witness for Vaccine Injury Compensation Program. The institution of Dr. Robbins has received research support from Diamond Endowment Fund. The institution of Dr. Robbins has received research support from Reeves Endowment Fund. The institution of Dr. Robbins has received research support from Institute for Ethnomedicine. The institution of Dr. Robbins has received research support from Theravance. The institution of Dr. Robbins has received research support from Dysautonomia International. The institution of Dr. Robbins has received research support from National Institute of Health. The institution of Dr. Robbins has received research support from Vertex pharmaceutical. Dr. Robbins has received personal compensation in the range of $50,000-$99,999 for serving as a Locums neurohospitalist with Hayes Locums. Dr. Robbins has received personal compensation in the range of $500-$4,999 for serving as a Speaker with The Dysautonomia Project.

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