Primary lateral sclerosis: clinical, neurophysiological, and magnetic resonance findings.
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.
Kuipers-Upmeijer
J
J
Department of Neurology, University Hospital Groningen, PO Box 30.001, 9700 RB Groningen, The Netherlands.
de Jager
A E
AE
Hew
J M
JM
Snoek
J W
JW
van Weerden
T W
TW
D000328:Adult; D001284:Atrophy / Q000473:pathology; D001921:Brain / Q000473:pathology* / Q000503:physiopathology*; D003402:Creatine Kinase / Q000097:blood; D004185:Disability Evaluation; D004576:Electromyography; D019054:Evoked Potentials, Motor / Q000502:physiology; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000175:diagnosis* / Q000503:physiopathology*; D009046:Motor Neurons / Q000502:physiology; D009431:Neural Conduction / Q000502:physiology; D009778:Occipital Lobe / Q000473:pathology; D010296:Parietal Lobe / Q000473:pathology; D012720:Severity of Illness Index; D013997:Time Factors
D003402:Creatine Kinase