Accurate ALSFRS-R scores can be generated from retrospective review of clinic notes

Lechtzin, Noah; Maragakis, Nicholas J.; Kimball, R. F.; Busse, Anne M.; Hoffman, Virginia; Clawson, Lora

doi:10.1080/17482960802502993

Cited by 5 publications

(3 citation statements)

References 12 publications

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“…The ALSFRS-R is among the most frequently used scales in the neurological and functional assessment of patients with ALS, and survival time is reportedly shorter in ALS patients with lower ALSFRS-R scores (28). The range of ALSFRS-R scores was 20-42 with a mean 33.04±6.89 in the present study, and therefore patients with an ALSFRS-R score <33 points were separated from those with a score ≥33 points for analysis, as in previous studies (28,29). .…”

Section: Methodssupporting

confidence: 51%

Serum ferritin is a candidate biomarker of disease aggravation in amyotrophic lateral sclerosis

Wang

et al. 2018

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Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease. The mechanism that defines the loss of neurons in ALS is still not clearly understood, and there is no effective therapy to block its progression. Previous studies indicate that a disorder of iron homeostasis exists in ALS and based on this, the change of serum iron and ferritin and the association between iron metabolism and clinical features in Chinese Han patients with ALS was further investigated in the present study, in order to define its pathogenesis. Two cohorts were established: An ALS group consisting of 24 patients and a control group consisting of 38 healthy volunteers. Venous blood samples were collected for serum iron and ferritin analysis. The results indicated that the levels of serum iron were significantly decreased in patients with ALS (P<0.05), while there was no significant difference in serum ferritin between the ALS and control groups. However, the levels of serum ferritin were increased significantly in ALS patients with bulbar-onset (vs. limb-onset in females), dysphagia (vs. without dysphagia), longer disease duration (>12 months vs. ≤12 months in males) and lower ALS Functional Rating Scale-Revised score (<33 vs. ≥33; P<0.05). These results suggested that there was dysregulation of iron metabolism in Chinese Han patients with ALS and that serum ferritin may be a candidate biomarker of aggravation in these patients.

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Section: Methodssupporting

confidence: 51%

Serum ferritin is a candidate biomarker of disease aggravation in amyotrophic lateral sclerosis

Wang

et al. 2018

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“…It is not clear if MUNE techniques are equivalent or even better markers for disease progression than established outcome measures like the forced vital capacity (FVC) or ALSFRS‐R 5, 8. The ALSFRS‐R has been shown to have high reproducibility and linearity and can even be assessed by phone 1, 17. However, in addition to a functional measure a marker of motor neuron loss that tracks the underlying pathology seems prudent.…”

Section: Discussionmentioning

confidence: 99%

Motor unit number index (MUNIX): A novel neurophysiological technique to follow disease progression in amyotrophic lateral sclerosis

et al. 2010

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Motor unit number estimation techniques in amyotrophic lateral sclerosis (ALS) patients are technically challenging and time-consuming. The Motor Unit Number Index (MUNIX) is a novel technique based on surface-EMG recordings and requires only 3-5 minutes per muscle. The objective was to explore the feasibility of longitudinal MUNIX measurements in ALS patients. In seven patients enrolled in a clinical trial, eight muscles were studied every 2 months for up to 15 months in addition to the revised ALS-functional rating scale, slow vital capacity, and compound muscle action potentials. The method was well tolerated and easy to perform. Initial MUNIX measures were significantly reduced compared to controls (487 +/- 194 vs. 1459 +/- 113; P < 0.001). Relative drop from baseline paralleled the clinical course and was greater than the drop of other markers of disease progression. MUNIX measurements in multiple muscles are suitable for serial neurophysiologic investigations in ALS. Further longitudinal data are needed for reliability validation.

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“…Clinically, amyotrophic lateral sclerosis (ALS) can be monitored with different scoring methods, such as the ALS Functional Rating Scale (ALSFRS-R), which also includes respiratory status. [1][2][3] The clinical assessment provides the functional output as a combination of central factors, motor neuron degeneration, and compensatory sprouting. A progressive loss of motor neurons may be clinically masked by the reinnervation process.…”

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confidence: 99%

Motor unit number index (MUNIX): principle, method, and findings in healthy subjects and in patients with motor neuron disease

Nandedkar¹,

2010

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The motor unit number index (MUNIX) is a method for assessment of number and size (MUSIX) of motor units (MUs) using the compound muscle action potential (CMAP) and surface electromyographic interference pattern (SIP). This method was used to study the hypothenar muscle in 34 healthy subjects to define normal range, and to study reproducibility. Four healthy subjects and 13 patients with amyotrophic lateral sclerosis (ALS) were studied serially over a 1-year period. In healthy subjects, MUNIX showed good reproducibility. In serial studies, healthy subjects showed no change in the CMAP amplitude and MUNIX. ALS patients with minimal change in CMAP amplitude had a significant drop in MUNIX and increase in MUSIX, indicating MU loss compensated by reinnervation. When the CMAP changed significantly (>30%) in 1 year, the CMAP and MUNIX decreased in parallel. MUNIX would be useful to study MU loss in degenerative diseases of motor neurons. Muscle Nerve 42: 798-807, 2010.

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Accurate ALSFRS-R scores can be generated from retrospective review of clinic notes

Cited by 5 publications

References 12 publications

Serum ferritin is a candidate biomarker of disease aggravation in amyotrophic lateral sclerosis

Serum ferritin is a candidate biomarker of disease aggravation in amyotrophic lateral sclerosis

Motor unit number index (MUNIX): A novel neurophysiological technique to follow disease progression in amyotrophic lateral sclerosis

Motor unit number index (MUNIX): principle, method, and findings in healthy subjects and in patients with motor neuron disease

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