OBJECTIVE: To describe the clinical, neurophysiological, and MRI findings in 10 patients with primary lateral sclerosis (PLS). RESULTS: The course of the disease was very slowly progressive. Spasticity due to upper motor neuron dysfunction was the most prominent sign, but EMG showed slight lower motor neuron signs, such as a mixed pattern on maximal voluntary contraction and enlarged motor unit potentials. One patient had clinically mild lower motor neuron involvement. Central motor conduction times (CMCT) were more prolonged in PLS than is the case in ALS. Minor sensory signs were found on neurophysiological examination, comparable with those in ALS. In four patients serum creatine kinase activity was raised. On MRI cortical atrophy was seen, most pronounced in the precentral gyrus and expanding into the parietal-occipital region. CONCLUSIONS: PLS is a distinct clinical syndrome, part of the range of motor neuron diseases. Besides pronounced upper motor neuron symptoms, mild lower motor neuron symptoms can also be found, as well as (subclinical) sensory symptoms. PLS can be distinguished from ALS by its slow clinical course, a severely prolonged MEP, and a more extensive focal cortical atrophy.
OBJECTIVE: To describe the clinical, neurophysiological, and MRI findings in 10 patients with primary lateral sclerosis (PLS). RESULTS: The course of the disease was very slowly progressive. Spasticity due to upper motor neuron dysfunction was the most prominent sign, but EMG showed slight lower motor neuron signs, such as a mixed pattern on maximal voluntary contraction and enlarged motor unit potentials. One patient had clinically mild lower motor neuron involvement. Central motor conduction times (CMCT) were more prolonged in PLS than is the case in ALS. Minor sensory signs were found on neurophysiological examination, comparable with those in ALS. In four patients serum creatine kinase activity was raised. On MRI cortical atrophy was seen, most pronounced in the precentral gyrus and expanding into the parietal-occipital region. CONCLUSIONS:PLS is a distinct clinical syndrome, part of the range of motor neuron diseases. Besides pronounced upper motor neuron symptoms, mild lower motor neuron symptoms can also be found, as well as (subclinical) sensory symptoms. PLS can be distinguished from ALS by its slow clinical course, a severely prolonged MEP, and a more extensive focal cortical atrophy.
Authors: N Le Forestier; T Maisonobe; L Spelle; A Lesort; F Salachas; L Lacomblez; Y Samson; P Bouche; V Meininger Journal: J Neurol Sci Date: 2001-04-01 Impact factor: 3.181
Authors: M D Caramia; P Cicinelli; C Paradiso; R Mariorenzi; F Zarola; G Bernardi; P M Rossini Journal: Electroencephalogr Clin Neurophysiol Date: 1991-08
Authors: D S Younger; L P Rowland; N Latov; W Sherman; M Pesce; D J Lange; W Trojaborg; J R Miller; R E Lovelace; A P Hays Journal: Neurology Date: 1990-04 Impact factor: 9.910
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