ABSTRACT
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.
Subject(s)
Brain/pathology , Brain/physiopathology , Magnetic Resonance Imaging , Motor Neuron Disease/diagnosis , Motor Neuron Disease/physiopathology , Adult , Atrophy/pathology , Creatine Kinase/blood , Disability Evaluation , Electromyography , Evoked Potentials, Motor/physiology , Female , Humans , Male , Middle Aged , Motor Neurons/physiology , Neural Conduction/physiology , Occipital Lobe/pathology , Parietal Lobe/pathology , Severity of Illness Index , Time FactorsABSTRACT
OBJECTIVE: Quantification and evaluation of five neurological balance tests as part of the routine neurological examination in order to obtain objective test results for healthy individuals and several neurological patient groups. SETTING: Department of neurology, University Hospital Groningen, the Netherlands. DESIGN: Prospective, controlled study. METHODS: 220 subjects (113 healthy individuals and 107 neurological patients) participated in a balance test investigation comprising gang board, Romberg's test, tandem gait, one-leg-stance, hopping. Tests were performed on a hopscotch (3 meters long, divided in 6 rectangles of 20 by 50 cm) and using a stopwatch to register times and faults. RESULTS: All five quantified tests were feasible for healthy individuals. Up to the age of 70 results gradually declined, above 70 a strong decline was observed. Patients with gait and balance disorders performed less well in all tests except the Romberg's test. CONCLUSIONS: Taking into account a strict standardisation of these five existing tests standard values were obtained for a fast and simple quantification of balance in the routine neurological examination, which can also be used to monitor progress. Especially the results obtained in tandem gait, one-leg-stance and hopping differ significantly between healthy individuals and patients. The gang board appears to be useful for quantifying serious balance disorders. Romberg's test is a specific test for the perceptual sensibility and does not belong in the routine neurological examination of balance.
Subject(s)
Nervous System Diseases/physiopathology , Neurologic Examination/methods , Postural Balance , Adult , Aged , Aged, 80 and over , Humans , Middle Aged , Nervous System Diseases/diagnosis , Neurologic Examination/standards , Prospective Studies , Reference ValuesABSTRACT
OBJECTIVE: Quantification of the stepping test in routine neurological examination. SETTING: University Hospital Groningen, the Netherlands. DESIGN: Prospective, controlled study. METHODS: The stepping test was assessed in 41 healthy subjects, in 7 subjects with artificially induced vertigo and in 83 patients with disturbances of gait or equilibrium resulting from different affections of the central nervous system. Displacement and rotation to the right or the left side were measured. The different groups were then compared with each other. RESULTS: We found a great inter- and intra-individual variation in direction and width of rotation and in displacement. The results of the test are not influenced by right(+)-or left(-)-handedness or by dominance of the right or the left leg. CONCLUSION: The current test does not appear to be useful for detection of abnormalities in the vestibular system or for distinguishing normal individuals from patients.
