Learn More
In amyotrophic lateral sclerosis the striated pelvic floor sphincter muscles are functionally uninvolved, and pathological studies have confirmed the relative resistance of the Onuf nucleus motor neurons. We have evaluated the external anal sphincter (EAS) muscle in 16 patients with ALS using single fiber EMG, and compared the results with the findings in(More)
SFEMG recordings were carried out in patients with amyotrophic lateral sclerosis, progressive muscular atrophy, familial spinal muscular atrophy and syringomyelia. The fibre density was increased in all conditions, especially in progressive muscular atrophy indicating marked collateral sprouting. The duration of the action potential was increased indicating(More)
The development of cochlear implantation has allowed the majority of patients deafened after the development of language to regain significant auditory benefit. In a subset of patients, however, loss of hearing results from destruction of the cochlear nerves, rendering cochlear implantation ineffective. The most common cause of bilateral destruction of the(More)
Motor units in patients with motor neurone disease and polyneuropathy were studied with a new multielectrode technique allowing fibre mapping. In these disorders which are characterised by reinnervation the motor unit fibre distribution is changed from a normal scattered pattern to grouping often in clinically uninvolved muscles without definite EMG(More)
The motor unit in the normal EDC and biceps brachii muscle was studied with a multielectrode technique permitting recording from 44 sites 300 mum apart. It was found that the muscle fibres in man are scattered with no evidence of grouping, similar to the findings in glycogen depletion experiments in animals.
Computed transverse axial tomography (CT) was employed to examine brain morphometrics in 30 healthy men, aged 21 to 81 years. Seven consecutive CT slices 30 to 80 mm above the inferior orbitomeatal line were analyzed. CT numbers in gray and white matter regions were not correlated significantly with age (p greater than 0.05), nor were right/left ratios for(More)
BACKGROUND Developing methods for protecting organisms in metal-polluted environments is contingent upon our understanding of cellular detoxification mechanisms. In this regard, half-molecule ATP-binding cassette (ABC) transporters of the HMT-1 subfamily are required for cadmium (Cd) detoxification. HMTs have conserved structural architecture that(More)
Regional cerebral blood flow (rCBF) was measured in 23 patients with organic dementia (17 patients with Alzheimer's disease and six with low-pressure hydrocephalus [LPH]). The flow of gray matter was significantly reduced, especially in occipito-parieto-temporal regions, as well as frontally in some patients. During activation with psychologic tests,(More)
We assessed the hypothesis that psychopathology is overrepresented in patients with temporal lobe epilepsy (TLE) who experience ictal fear. Eleven such patients were matched with two control groups: patients who had TLE without ictal fear and patients who had generalized epilepsy. On several MMPI scales, the TLE-ictal fear group scored significantly higher(More)
A girl with rapid-onset, bulbospinal muscular atrophy and deafness is described. The patient's mother and brother showed EMG features consistent with subclinical involvement. T is bulbospinal form of spinal muscular atrophy associated with deafness described by Vialetto and van Laere closely resembles the Madras type of motor neuron disease, also associated(More)