BACKGROUND: Motor neuron diseases can affect the upper motor neuron and/or the lower motor neuron. Both amyotrophic lateral sclerosis (ALS) and primary lateral sclerosis (PLS) are motor neuron diseases, and there is much debate as to whether these are 2 separate disorders or simply 2 points on a continuum. OBJECTIVE: To determine which clinical features at onset and during follow-up could help differentiate between PLS and ALS. DESIGN: Retrospective study comparing patients with a diagnosis of PLS or ALS for differences in symptoms or signs at disease onset and during follow-up. SETTING: Tertiary referral center. Patients Six hundred sixty-one patients with ALS and 43 patients with PLS were included in the study. RESULTS: At presentation, stiffness was the only symptom that was significantly different between patients with PLS and patients with ALS (observed in 47% and 4% of patients, respectively; P<.001). During follow-up, limb wasting was rare in patients with PLS (2%, compared with 100% in patients with ALS; P<.001). Disease duration was significantly longer in patients with PLS compared with patients with ALS (mean +/- SD, 11.2 +/- 6.1 vs 3.8 +/- 4.2 years, respectively; P<.001). During the 16 years of follow-up, the mortality rate was significantly lower in patients with PLS compared with patients with ALS (only 33% vs 89%, respectively; P<.001). CONCLUSION: Our findings suggest that a patient presenting with spasticity who does not develop wasting within 3 years most likely has PLS.
BACKGROUND: Motor neuron diseases can affect the upper motor neuron and/or the lower motor neuron. Both amyotrophic lateral sclerosis (ALS) and primary lateral sclerosis (PLS) are motor neuron diseases, and there is much debate as to whether these are 2 separate disorders or simply 2 points on a continuum. OBJECTIVE: To determine which clinical features at onset and during follow-up could help differentiate between PLS and ALS. DESIGN: Retrospective study comparing patients with a diagnosis of PLS or ALS for differences in symptoms or signs at disease onset and during follow-up. SETTING: Tertiary referral center. Patients Six hundred sixty-one patients with ALS and 43 patients with PLS were included in the study. RESULTS: At presentation, stiffness was the only symptom that was significantly different between patients with PLS and patients with ALS (observed in 47% and 4% of patients, respectively; P<.001). During follow-up, limb wasting was rare in patients with PLS (2%, compared with 100% in patients with ALS; P<.001). Disease duration was significantly longer in patients with PLS compared with patients with ALS (mean +/- SD, 11.2 +/- 6.1 vs 3.8 +/- 4.2 years, respectively; P<.001). During the 16 years of follow-up, the mortality rate was significantly lower in patients with PLS compared with patients with ALS (only 33% vs 89%, respectively; P<.001). CONCLUSION: Our findings suggest that a patient presenting with spasticity who does not develop wasting within 3 years most likely has PLS.
Authors: Andrew D Schweitzer; Tian Liu; Ajay Gupta; Karen Zheng; Stephen Seedial; Alexander Shtilbans; Mona Shahbazi; Dale Lange; Yi Wang; A John Tsiouris Journal: AJR Am J Roentgenol Date: 2015-05 Impact factor: 3.959
Authors: F Geser; D Prvulovic; L O'Dwyer; O Hardiman; P Bede; A L W Bokde; J Q Trojanowski; H Hampel Journal: Prog Neurobiol Date: 2011-09-03 Impact factor: 11.685
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