Choline chloride in the treatment of ataxia.
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Cerebellar Ataxia↗
Biomedical subjects
F L Mastaglia
Publications and source records attributed to F L Mastaglia.
Drug therapy: Disorders of neuromuscular transmission caused by drugs.
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Aged↗
Drug-induced peripheral neuropathies.
Review of the various drugs in current clinical use showed that over 50 of them may cause a purely sensory or mixed sensorimotor neuropathy. These include antimicrobials, such as isoniazid, ethambutol, ethionamide, nitrofurantoin, and metronidazole; antineoplastic agents, particularly vinca alkaloids; cardiovascular drugs, such as perhexiline and hydrallazine; hypnotics and psychotropics, notable methaqualone; antirheumatics, such as gold, indomethacin, and chloroquine; anticonvulsants, particularly phenytoin; and other drugs, including disulfiram, calcium carbimide, and dapsone. Patients receiving drug treatment who complain of paraesthesie, pain, muscle cramps, or other abnormal sensations and those without symptoms who are receiving drugs that are known or suspected to be neurotoxic should undergo neurological examination and studies of motor and sensory nerve conduction. This will allow the incidence of drug-induced peripheral neuropathy to be determined more precisely.
Anti-Infective Agents↗
'Locked-in coma' in postinfective polyneuropathy.
A patient with postinfective cranial and peripheral polyneuropathy exhibited the electroencephalographic and behavioral features of "alpha coma". The relation of this form of extensive peripheral disconnection to those cases with central disconnection due to pontomesencephalic lesions is discussed. We conclude that in both situations further evaluation of brain stem and cortical function is necessary to determine whether or not consciousness is preserved, rather than relying solely on the presence of ocular movements and reactivity of the electroencephalogram.
Coma↗
Alpha and beta coma in drug intoxication uncomplicated by cerebral hypoxia.
Four patients who were rendered comatose or stuporous by drug intoxication, but who were not hypoxic, are described. Three patients received high doses of chlormethiazole for alcohol withdrawal symptoms, and one took a suicidal overdose of nitrazepam. The patient with nitrazepam overdose and two of those with chlormethiazole intoxication conformed to the criteria of 'alpha coma', showing non-reactive generalized or frontally predominant alpha activity in the EEG. The fourth patient who was unconscious after chlormethiazole administration exhibite generalized non-reactive activity in the slow beta range. All four recovered completely without neurological sequelae following the withdrawal of the offending agents. The similarities between the effects of structural lesions and pharmacological depression of the brain stem reticular formation are discussed. It is suggested that in both situations disturbed reticulo-thalamic interactions are important in the pathogenesis of alpha coma. It is concluded that when this electroencephalographic and behavioural picture is seen in drug intoxication, in the absence of significant hypoxaemia, a favourable outcome may be anticipated.
Adult↗
Leber's optic neuropathy: a clinical and visual evoked potential study of affected and asymptomatic members of a six generation family.
Fourteen clinically affected and 40 asymptomatic members of a six generation family with Leber's optic neuropathy have been studied clinically and by recording pattern-reversal visual evoked potentials. While 12 of the affected members had suffered the typical sequential bilateral failure of vision, in 2 the condition was still monocular after periods of 12 and 14 years. Reduced vascularity of the optic nerve head was found in 11 of these cases, all of whom had some degree of optic atrophy, and showed a significant correlation with the visual acuity. Excessive tortuosity of peripheral retinal vessels was noted in 6 cases and was a prominent feature in the unaffected eye of one of the subjects with monocular visual impairment. In cases with advanced visual impairment the VEP was absent bilaterally, while in those with less severe involvement responses which were delayed, desynchronized and much small than normal could still be recorded. Two subjects with early bilateral clinical involvement had normal or minimally abnormal responses. Repeat studies in 6 subjects after intervals of up to fifteen months showed no change in 4 and a deterioration in 2. It is concluded that the VEP findings in clinically affected subjects are in keeping with a severe demyelinating lesion of the optic nerve with associated nerve fibre loss. Mild impairment of colour vision, pallor or reduced vascularity of the optic nerve head, excessive tortuosity of retinal vessels, a small central scotoma, and/or mild abnormalities or atypical features of the VEP were found in 16 of the 40 asymptomatic family members studied. Such abnormalities were present in 50 per cent of descendants from the female lineage who were at risk of developing the disease, and also in 30 per cent of descendants from male lineages who were not at risk. These findings suggest that there is a stage prior to the onset of visual impairment during which subtle abnormalities may be detected in individuals at risk of developing or transmitting the disease. The finding of asymptomatic abnormalities in descendants from male lineages could be accounted for by transmission of a partial form of the disease by affected or unaffected males, which would be in accord with a cytoplasmic mechanism of transmission for the disease.
Adolescent↗
Quantitative studies of saccadic and pursuit eye movements in multiple sclerosis.
Ocular movements were studied in 108 patients with established or suspected multiple sclerosis using an on-line computer-based electro-oculographic technique. In one group of patients peak eye movement velocities alone were measured during horizontal refixation saccades. In a second group saccade reaction times and accuracies were measured in addition to velocities, while in a subgroup a quantitative analysis of horizontal pursuit eye movements was also carried out. With the saccade velocity test abnormalities were present in 44 per cent of cases studied and were subclinical in 18 per cent. Abnormalities were found in 57 per cent of cases in whom the detailed saccade analysis was performed, including 48 per cent of patients with clinically normal eye movements. Saccade reaction time and accuracy were more sensitive parameters than saccade velocity, and the highest yield of abnormalities was obtained when all three were taken into consideration. Abnormalities of pursuit movements were found in 71 per cent of cases studied and were frequently subclinical. Abnormalities of saccadic and pursuit movements were not always present together in the same patient, and the overall yield of abnormalities was higher when the results of both types of study were taken into account. The yield of abnormalities with the eye movement studies was somewhat lower than with the pattern-reversal VEP in the clinically definite multiple sclerosis group, but was higher in patients in the other categories. Subclinical abnormalities of eye movement were found in a significant number of patients with normal VEPs. The finding of such an abnormality in patients with spinal cord syndromes allowed reclassification of 14 patients to a category with a higher degree of diagnostic certainty. It is concluded that quantitative electro-oculography is a valuable adjunct to the clinical evaluation of eye movements and has an important role in the investigation of patients suspected of multiple sclerosis.
Adult↗
Epsilon aminocaproic acid (EACA) myopathy.
Two female patients developed a severe, painful proximal myopathy after taking 18--30 g of epsilon-aminocaproic acid daily for 5 weeks. Marked elevations of serum aminotransferases, creatine kinase and aldolase levels were found and the first patient had electromyographic and muscle biopsy changes of an acute monophasic, necrotising myopathy at the height of the illness. Resolution occurred in both cases on stopping the drug and the second patient had no electromyographic or muscle biopsy abnormalities 3 weeks later. Only 2 recognized cases of the condition have been reported previously but a review of the literature revealed several other possible examples.
Aged↗
Optic neuropathy and ophthalmoplegia in herpes zoster oticus.
A 55-year-old man with herpes zoster oticus and minimal cutaneous involvement developed reversible optic neuropathy, and ocular motor and cerebellar abnormalities. Serologic changes confirmed infection with herpes zoster. A demyelinating process seems likely to have been responsible for these lesions. It is suggested that herpes zoster antibody titers should be measured whenever the syndrome of polyneuritis cranialis of acute onset is being investigated.
Antibodies, Viral↗
Drug-induced myopathies in man.
Drug-induced diseases constitute up to 5% of hospital admissions,a figure which almost certainly understates the total morbidity due to drugs1. Sever drug-induced myopathies are uncommon, but milder forms may be more prevalent than is generally appreciated, since skeletal muscle constitutes some 45% of total body-weight and has a major metabolic role in addition to its mechanical function2. Knowledge of possible effects of drugs on the neuromuscular system is of increasing importance both because the range of therapeutic agents continues to expand and because the resulting syndromes, through usually reversible at the outset, may progress and lead to grave consequences if the drug responsible is not stopped. Drug-induced neuropathies3 will not be considered here, but it will be appreciated that muscle weakness may also be feature of such disorders and that some drugs may cause both a neuropathy and a myopathy. The features of the main drug-induced syndromes are summarised in the table. To these one could justifiably add the unwanted effects of srugs given for the treatment of central-nervous-system or neuromuscular disorders per se-e.g., the cholinergic block which may be produced by anticholinesterases alone or with corticosteroids in the myasthenic,4 and the profound weakness which may supervene after relief of spasticity with dantrolene sodium5.
Acute Disease↗
Slowing of conduction in visual pathway in hypothyroidism.
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Adult↗
On-line electro-oculography.
The design and use of an on-line PDP 11/40-based system for quantitative study of ocular kinetics are described. The system can be used in neurophysiological or ophthalmological applications. Two different techniques are presented. In one, horizontal, vertical or oblique eye motion can be studied, in the other horizontal eye motion only. Several parameters of eye motion can be measured including saccadic velocity, eye movement latency and accuracy of refixation. For ophthalmological EOG applications the system allows measurement of the absolute voltage excursion corresponding to a horizontal eye movement of a specific amplitude. The system consists of five software programs and supporting signal processing equipment. The software package runs under the RSX11M executive.
Computers↗
Computerized tomography of brain and optic nerve in multiple sclerosis. Observations in 100 patients, including serial studies in 16.
Computerized tomography (CT) of the brain was carried out in 100 patients with established or suspected multiple sclerosis (MS). The optic nerves were also examined in 53 of these patients. Areas compatible with demyelinating lesions were found in the cerebral hemisphere white matter and less frequently in the brain stem in 47% of cases. The hemisphere lesions were commonly multiple, typically situated in the deep white matter and periventricular regions, and were often asymptomatic. Small areas with unduly low attenuation coefficients were found in one or both optic nerves in 52% of patients in whom the optic nerves were examined. While these areas may represent demyelinating lesions their significance remains uncertain in view of poor correlation with clinical and electrophysiological parameters of optic nerve damage. Cerebral cortical atrophy and/or ventricular dilatation was found in 44% of cases, the frequency and severity of atrophy increasing with age and duration of disease. Serial studies after intervals of up to 21 months were performed in 16 patients, providing the opportunity to study the natural history of the cerebral lesions. While in some cases no significant change occurred, in others white matter lesions underwent an increase or a reduction in size, and in some cases new lesions appeared. In some patients minor degrees of atrophy became apparent over the period of the study. The value of CT in the investigation of patients with suspected MS and as a means of studying the natural history of the disease is discussed.
Adolescent↗
Pattern-reversal visual evoked potential. Method of analysis and results in multiple sclerosis.
A detailed method of analysis of the pattern-reversal visual evoked potential is presented. This method takes into account a number of parameters in addition to the latency of the major surface-positive component (P2) and has been tested in a group of 50 normal subjects and in 98 patients with established or suspected multiple sclerosis (MS). It was found that this more detailed form of analysis improved the detection rate of abnormal responses in the MS subjects particularly in those classified in the suspected category. The potential value of this form of analysis, particularly in clinical neurophysiology laboratories where the recording of visual evoked potentials is the only technique employed in the investigation of patients with suspected MS, is discussed.
Adolescent↗
Brain atrophy and intellectual impairment in heavy drinkers--a clinical, psychometric and computerized tomography study.
The frequency of cerebral and cerebellar atrophy was assessed by computerized tomography (CT) in 26 heavy drinkers. Findings were correlated with clinical deficits, with the results of psychometric testing using the Wechsler Adult Intelligence Scale (WAIS), and with the alcoholic history and nutritional status. Cerebral atrophy was present in 19 cases (73%), 16 of whom also had cerebellar atrophy. There was a good correlation between the degree of cerebral hemisphere atrophy and age and length of drinking history, but a poor correlation between neurological deficits and atrophy. Impairment of visuo-spatial and visuo-motor functions with sparing of other non-dominant hemisphere functions and of verbal skills was the characteristic pattern found with the WAIS in 18 subjects; four showed a more global depression of intellectual function. The degree of cerebral hemisphere atrophy correlated significantly with the impairment of non-dominant hemisphere functions but not with total IQ.
Adult↗
The contribution of evoked potentials in the functional assessment of the somatosensory pathway.
The value of evoked potentials in studying conduction in the somatosensory pathway was assessed in patients with various neurological disorders. In patients with multiple sclerosis (MS) abnormalities of the cervical response (N14) were found particularly in longstanding cases but also in the early stages of the disease, even in patients without sensory symptoms or signs, and were reversible in some patients. The cortical response was also abnormal in some cases but the two were not always affected together. In Friedreich's ataxia both the cervical and cortical responses were usually abnormal. Subclinical abnormalities of the cervical responses were found in some patients with hereditary spastic paraparesis or mixed forms of spinocerebellar ataxia. The cervical responses were also abnormal in patients with peripheral neuropathy and cervical radiculopathy, and in some patients with brain-stem or thalamic lesions. Cervical and cortical responses were normal in the lateral medullary syndrome, whereas the cortical response was markedly abnormal in patients with high brain-stem or cerebral hemisphere vascular lesions. Cortical and subcortical responses were abnormal in some patients with stereotactic thalamic lesions. Enhanced cortical responses were found in patients with lesions at different levels in the CNS. The most marked enhancement was observed in patients with familial myoclonic epilepsy. Lesser degrees were found in some patients with MS, progressive supranuclear palsy, thalamic lesions, brain-stem encephalitis and syringomyelia. Enhanced responses were usually found in patients with minimal or no clinical sensory involvement. It is postulated that this type of abnormality results from an interference to the inhibitory mechanisms which normally operate at various levels in the somatosensory pathway. It is concluded that evoked potential studies are a valuable adjunct to the clinical evaluation of sensation, and that they may provide useful information on the pathophysiology of conduction in the somatosensory pathway.
Brain Diseases↗
Vertical gaze palsy in barbiturate intoxication.
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Adult↗
Vacuolar myopathy associated with chloroquine, lupus erythematosus and thymoma. Report of a case with unusual mitochondrial changes and lipid accumulation in muscle.
Pathological and biochemical observations are presented in a 55-year-old woman with lupus erythematosus and thymoma who developed a vacuolar myopathy while being treated with chloroquine. Electromyography showed prominent spontaneous muscle activity including myotonic discharges. Vacuoles were present in all fibre types but, in contrast to previous cases of chloroquine myopathy, were most prominent in intermediate fibres. Electron microscopy showed cytoplasmic sequestration by membranes in proximity to the t-system, many autophagic vacuoles, tubular networks, and a variety of membranous bodies, some identical to those found in certain forms of cerebral lipidosis. Other features not previously described in chloroquine myopathy included prominent mitochondrial vacuolation and sequestration of glycogen within mitochondria. Thin-layer chromatography of muscle homogenates showed an increase in all major neutral and phospholipid fractions.
Animals↗