Clinical and imaging features of primary lateral sclerosis
- VernacularTitle:原发性侧索硬化的临床和影像学特征
- Author:
Qi SUN
;
Wen LIU
;
Li WANG
- Publication Type:Journal Article
- Keywords:
primary lateral sclerosis;
motor neuron disease;
MRI
- From:
Journal of Clinical Neurology
2001;0(05):-
- CountryChina
- Language:Chinese
-
Abstract:
Objective To explore the clinical and imaging features of primary lateral sclerosis(PLS).Methods The clinical and imaging data of 3 patients with PLS were analysed.Results 3 patients included 2 females and 1 male.The onset of the disease was insidious.The initial symptoms included rigidity and weakness of right upper limb(case 1),rigidity and weakness of legs(case 2),and pseudobulbar palsy(case 3). As the disease progressed slowly,the patients presented with spastic paralysis of four extremities,reduced muscle strength(3 to 4 degree),clasp-knife like of muscular tension,hyperactivity of tendon reflexes,ankle clonus and presence of Babinski sign.In T2-weighted images of the brain MRI,the abnormal high signals were symmetrically situated in the motor cortex,the posterior limbs of internal capsules,cerebral peduncles and basilar part of pons.1H magnetic resonance spectroscopy(1HMRS) showed decreases of N-acetylasartate(NAA) and the ratio of N-acetylaspartate/Creatine(NAA/Cr) in the focal regions.Conclusion PLS presents with characteristic features of clinical symptoms,signs and abnormal changes of MRI.In addition,1HMRS may be helpful in diagnosis of this disease.