OBJECTIVE: To determine how clinical features at the first evaluation and in follow-up can be used to suggest a diagnostic outcome for patients with only upper motor neuron (UMN) signs at disease onset. METHODS: We reviewed the records of 34 patients (9 primary lateral sclerosis [PLS], 15 UMN-dominant amyotrophic lateral sclerosis [ALS], and 10 randomly selected control patients with ALS) seen in 1984-2007. Analysis of variance F tests for continuous variables and chi2 tests for categorical variables analyzed differences in baseline data among the diagnostic categories. Linear and generalized mixed effects models assessed the relation between examination data and diagnostic group over time. RESULTS: At first examination, the lowest score of the weakest muscle (p < 0.001), the site of onset (p = 0.041), and time to evaluation (p = 0.05) discriminated between eventual diagnostic group; patients with PLS were stronger, slower in progressing, and more likely to have limb onset than the other groups. Strength < or = 4 on any muscle was associated with the diagnosis of ALS (p = 0.0001), but not PLS. Across all visits, muscle strength (p = 0.003), ALS Functional Rating Scale score (p = 0.009), and vital capacity (p = 0.026) predicted group assignment. UMN-dominant and ALS groups had more weight loss (p = 0.004), even when controlled for dysphagia (p = 0.021) and muscle atrophy (p = 0.009), and patients with ALS were more likely to have hyporeflexia (p = 0.001). CONCLUSIONS: Features at baseline most suggestive of eventual lower motor neuron signs were focal muscle weakness or bulbar onset. Later, weight loss, reduced forced vital capacity, and limb weakness predicted lower motor neuron dysfunction. We suggest that patients with only upper motor neuron signs have periodic evaluations of strength, weight, forced vital capacity, Amyotrophic Lateral Sclerosis Functional Rating Scale score, and EMG, because a change in any can signal the imminent development of lower motor neuron signs.
OBJECTIVE: To determine how clinical features at the first evaluation and in follow-up can be used to suggest a diagnostic outcome for patients with only upper motor neuron (UMN) signs at disease onset. METHODS: We reviewed the records of 34 patients (9 primary lateral sclerosis [PLS], 15 UMN-dominant amyotrophic lateral sclerosis [ALS], and 10 randomly selected control patients with ALS) seen in 1984-2007. Analysis of variance F tests for continuous variables and chi2 tests for categorical variables analyzed differences in baseline data among the diagnostic categories. Linear and generalized mixed effects models assessed the relation between examination data and diagnostic group over time. RESULTS: At first examination, the lowest score of the weakest muscle (p < 0.001), the site of onset (p = 0.041), and time to evaluation (p = 0.05) discriminated between eventual diagnostic group; patients with PLS were stronger, slower in progressing, and more likely to have limb onset than the other groups. Strength < or = 4 on any muscle was associated with the diagnosis of ALS (p = 0.0001), but not PLS. Across all visits, muscle strength (p = 0.003), ALS Functional Rating Scale score (p = 0.009), and vital capacity (p = 0.026) predicted group assignment. UMN-dominant and ALS groups had more weight loss (p = 0.004), even when controlled for dysphagia (p = 0.021) and muscle atrophy (p = 0.009), and patients with ALS were more likely to have hyporeflexia (p = 0.001). CONCLUSIONS: Features at baseline most suggestive of eventual lower motor neuron signs were focal muscle weakness or bulbar onset. Later, weight loss, reduced forced vital capacity, and limb weakness predicted lower motor neuron dysfunction. We suggest that patients with only upper motor neuron signs have periodic evaluations of strength, weight, forced vital capacity, Amyotrophic Lateral Sclerosis Functional Rating Scale score, and EMG, because a change in any can signal the imminent development of lower motor neuron signs.
Authors: Jeffrey M Statland; Richard J Barohn; April L McVey; Jonathan S Katz; Mazen M Dimachkie Journal: Neurol Clin Date: 2015-09-08 Impact factor: 3.806
Authors: Christina Schuster; Elisabeth Kasper; Judith Machts; Daniel Bittner; Jörn Kaufmann; Reiner Benecke; Stefan Teipel; Stefan Vielhaber; Johannes Prudlo Journal: J Neurol Date: 2013-08-31 Impact factor: 4.849
Authors: Christina Schuster; Elisabeth Kasper; Judith Machts; Daniel Bittner; Jörn Kaufmann; Reiner Benecke; Stefan Teipel; Stefan Vielhaber; Johannes Prudlo Journal: J Neurol Date: 2014-07-15 Impact factor: 4.849
Authors: Jeffrey M Statland; Richard J Barohn; Mazen M Dimachkie; Mary Kay Floeter; Hiroshi Mitsumoto Journal: Neurol Clin Date: 2015-09-08 Impact factor: 3.806
Authors: John Ravits; Stanley Appel; Robert H Baloh; Richard Barohn; Benjamin Rix Brooks; Lauren Elman; Mary Kay Floeter; Christopher Henderson; Catherine Lomen-Hoerth; Jeffrey D Macklis; Leo McCluskey; Hiroshi Mitsumoto; Serge Przedborski; Jeffrey Rothstein; John Q Trojanowski; Leonard H van den Berg; Steven Ringel Journal: Amyotroph Lateral Scler Frontotemporal Degener Date: 2013-05 Impact factor: 4.092