Sorenson, Eric J.

AbstractIntroductionOur research aim was to develop a novel clinimetric scale sensitive enough to detect disease progression in primary lateral sclerosis (PLS).MethodsA prototype of the PLS Functional Rating Scale (PLSFRS) was generated. Seventy‐seven participants with PLS were enrolled and evaluated at 21 sites that comprised the PLSFRS study group. Participants were assessed using the PLSFRS, Neuro‐Quality of Life (QoL), Schwab‐England Activities of Daily Living (ADL), and the Clinical Global Impression of Change scales. Participants completed telephone assessments at 12, 24, and 48 weeks after enrollment.ResultsThe PLSFRS demonstrated internal consistency as well as intrarater, interrater, telephone test‐retest reliability, and construct validity. Significant changes in disease progression were detected at 6 and 12 months; changes measured by the PLSFRS vs the ALSFRS‐R were significantly higher.DiscussionThe PLSFRS is a valid tool to assess the natural history of PLS in a shorter study period.

Purpose: We present a unique technique applying F-wave latencies to assist in the diagnosis of peripheral neuropathy within the pediatric age group. Methods: We calculated an F-wave estimated deviance adjusted for limb length measurements, distal motor conduction velocity, and distal motor latencies. We compared the F-wave estimated deviance from the normal subjects with those with peripheral neuropathy (both axonal and demyelinating) to establish diagnostic accuracy in pediatric patients. Results: The normal range for the F-wave estimated deviance in the upper limb was −1 to +6 ms and in the lower limb was −8 to +5 ms. When compared with 82 subjects with peripheral neuropathy, there was a significant difference between the normal subjects and those with neuropathy (P values between 0.002 and 0.0005 for each of the individual nerves tested). Conclusions: The F-wave estimated deviance is independent of age and can be accurately applied to the electrodiagnostic testing of pediatric patients with suspected neuropathy.

ABSTRACT Introduction Existing normal value references for pediatric nerve conduction studies (NCS) are based on limited sample sizes with uncertain reliability, suggesting a need for better normative data. Methods Electronic medical records were reviewed for pediatric patients (0 to <18 years) with normal findings on electromyography and NCS during the period from January 1, 1997 through September 20, 2017. Electrodiagnostic and demographic data were collected. Gaussian and descriptive statistics were used to establish normal values by age group. Results In this study we analyzed 1,918 normal NCS on 1,849 unique pediatric patients. Patients were stratified by age: 0 to <1 month; 1 to <6 months; 6 to <12 months; 12 to <24 months; 2 to <3 years; 3 to <4 years; 4 to <5 years; 5 to <10 years; 10 to <15 years; and 15 to <18 years. Normal reference ranges for amplitude, conduction velocity, and distal latency were established for each age group for 4 motor and 4 sensory nerves. Discussion The large sample size of this study provides reliable reference values for interpreting pediatric NCS. Muscle Nerve 60 : 155–160, 2019

Objective. To evaluate longitudinal cognitive/behavioral change over 12 months in participants enrolled in the ALS Multicenter Cohort Study of Oxidative Stress (ALS COSMOS). Methods. We analyzed data from 294 ALS participants, 134 of whom were studied serially. Change over time was evaluated controlling for age, sex, symptom duration, education, race, and ethnicity. Using multiple regression, we evaluated associations among decline in ALS Functional Rating Scale-Revised (ALSFRS-R) scores, forced vital capacity (FVC), and cognitive/behavioral changes. Change in cognitive/behavioral subgroups was assessed using one-way analyses of covariance. Results. Participants with follow-up data had fewer baseline behavior problems compared to patients without follow-up data. We found significant worsening of behavior (ALS Cognitive Behavioral Screen (ALS CBS) behavioral scale, p<0.001; Frontal Behavioral Inventory-ALS (FBI-ALS) disinhibition subscale, p=0.044). Item analysis suggested change in frustration tolerance, insight, mental rigidity, and interests (p<0.05). Changes in ALSFRS-R correlated with the ALS CBS. Worsening disinhibition (FBI-ALS) did not correlate with ALSFRS-R, FVC, or disease duration. Conclusion. We did not detect cognitive change. Behavioral change was detected, and increased disinhibition was found among patients with abnormal baseline behavioral scores. Disinhibition changes did not correlate with disease duration or progression. Baseline behavioral problems were associated with advanced, rapidly progressive disease and study attrition.

ABSTRACTIntroduction: Isolated musculocutaneous neuropathy is uncommon. In this study we aimed to determine its causes and clinical presentation and interpret the electrodiagnostic findings associated with this condition. Methods: Our investigation was a retrospective review of patients diagnosed with musculocutaneous neuropathy at the Mayo Clinic (Rochester, Minnesota) electromyography (EMG) laboratory between 1997 and 2015. Results: Thirty‐two patients with musculocutaneous neuropathy and 5 patients with lateral antebrachial cutaneous neuropathy were identified. The most common cause was acute trauma or surgery (65%). Fourteen percent of the cases were idiopathic and 14% were inflammatory. Pain and sensory disturbance were more common presentations than weakness. Weakness from nerve injury was not noted in 2 patients, suggesting that other muscles may provide adequate elbow flexion/supination. The bilateral absence of lateral antebrachial cutaneous nerve sensory responses suggests an inflammatory cause. Discussion: Musculocutaneous neuropathy usually results from trauma or iatrogenic injury. Nerve conduction studies alone are insufficient to confirm neuropathy, and needle EMG examination should be a routine part of the diagnostic evaluation. Muscle Nerve 58: 726–729, 2018