Showing papers in "Journal of Neurology, Neurosurgery, and Psychiatry in 1984"

Late outcome of very severe blunt head trauma: a 10-15 year second follow-up.

Abstract: Forty patients with very severe blunt head trauma (post-traumatic amnesia greater than or equal to 1 month) were initially examined at an average of 4.5 months after the injury. The patients were visited in their homes 2.5 years and 10-15 years after the accident and questionnaires were presented to patients, relatives and/or staff. Though physical impairment, dysarthria and defects of memory remained severe in many cases, the psychosocial sequelae presented the most serious problems. Permanent changes in personality and emotion were reported in two thirds and were especially frequent among the youngest patients. The worse overall outcome was seen in cases with severe brainstem involvement or anterior lesions or both. In spite of the great frequency of deficits long-term improvement of functional state was common and several regained at least some work capacity.

Sympathetic skin response--a method of assessing unmyelinated axon dysfunction in peripheral neuropathies.

Abstract: The sympathetic skin response (SSR) was measured in 33 patients with peripheral neuropathies and in 30 normal control subjects. Abnormalities of the response were correlated with clinical, pathologic, and EMG observations. The response was usually absent in axonal neuropathies, but present in demyelinating disorders. Abnormalities of the sympathetic skin response did not correlate well with clinical evidence of dysautonomia, but were a reliable indicator of disorders affecting unmyelinated axons.

Polyneuropathy in critically ill patients

Abstract: Five patients developed a severe motor and sensory polyneuropathy at the peak of critical illness (sepsis and multiorgan dysfunction complicating a variety of primary illnesses). Difficulties in weaning from the ventilator as the critical illness subsided and the development of flaccid and areflexic limbs were early clinical signs. However, electrophysiological studies, especially needle electrode examination of skeletal muscle, provided the definite evidence of polyneuropathy. The cause is uncertain, but the electrophysiological and morphological features indicate a primary axonal polyneuropathy with sparing of the central nervous system. Nutritional factors may have played a role, since the polyneuropathy improved in all five patients after total parenteral nutrition had been started, including the three patients who later died of unrelated causes. The features allow diagnosis during life, and encourage continued intensive management since recovery from the polyneuropathy may occur.

Mini-mental state examination in neurological patients.

Abstract: The Mini-Mental State examination has been found to be a quick and valuable test for simple bedside screening, and for serial assessment of cognitive function in a population of 126 neurological patients. Amongst those with cognitive impairment, there was a close relation between the Mini-Mental State examination and the conventional Weschler Adult Intelligence Scale (WAIS). However, the Mini-Mental test was not a sensitive indicator of focal versus diffuse hemisphere disease. Further refinement in the areas of language and visuo-spatial function may improve its value.

The effects of movement velocity, mass displaced, and task certainty on associated postural adjustments made by normal and hemiplegic individuals.

Abstract: The activation times for trunk and leg muscles were examined in normal and left hemiplegic subjects who raised their right arms at different velocities in self-paced or reaction time conditions Activity in these postural muscles preceded arm displacement, and they were activated in a similar sequence during all types of rapid movements The presence and sequencing of associated postural adjustments were more variable during slow movements

Cognitive and motor shifting aptitude disorder in Parkinson's disease.

Abstract: Eighteen patients suffering from Parkinson's disease and nineteen control subjects, who were matched for age and intelligence, were compared in tests measuring "shifting aptitude" at cognitive and motor levels (word production, sorting blocks or animals, and finger pushing sequences). It was found that Parkinson patients produced fewer different names of animals and professions in one minute than control subjects, needed more trials for detecting a shift in a sorting criterion, and produced fewer finger responses in a change of pushing sequence than control subjects. These results are interpreted as reflecting a central programming deficit that manifests itself in verbal, figural and motor modalities, that is, a diminished "shifting aptitude" characteristic of patients with dysfunctioning basal ganglia. The results are discussed in relation to changes of behaviour organisations in animals with dysfunctioning basal ganglia.

Neuropsychological rehabilitation after closed head injury in young adults.

Abstract: Cognitive and personality disturbances following severe closed head injury in young adults are associated with poor rehabilitation outcome. Yet systematic programmes for dealing with these disturbances have generally not appeared. The present report briefly describes the Neuropsychological Rehabilitation Program (NRP) at Presbyterian Hospital in Oklahoma City and the initial outcome data on eighteen closed head injury patients and seventeen untreated controls. Greater improvement in neuropsychological functioning occurred in the NRP patient group on selected variables, but generally the effects were modest. Emotional distress, however, substantially decreased in treated patients. Fifty percent of the NRP patients maintained productivity 75% of the time or more following rehabilitation, compared to 36% of the controls. Treatment successes showed less personality disturbances than treatment failures and better learning and memory scores post-treatment.

The brief scale for anxiety: a subdivision of the comprehensive psychopathological rating scale.

Abstract: A rating scale suitable for recording anxious symptoms is described. It is a subdivision of the Comprehensive Psychopathological Rating Scale and comprises 10 items, all of which are rated on a 7 point scale. It is suitable for the rating of pathological anxiety alone or for anxiety occurring in the setting of other psychological or medical disorder.

The olfactory bulbs in Alzheimer's disease.

Abstract: The olfactory bulbs have been examined in patients with Alzheimer's disease and compared with those in elderly undemented and younger undemented control patients. In Alzheimer's disease neurofibrillary tangles were found in the anterior olfactory nucleus but not elsewhere in the olfactory bulb. Cell loss in the anterior olfactory nucleus was also found in Alzheimer's disease. It is clear that the olfactory sensory pathway is pathologically affected in Alzheimer's disease and would merit further study.

Increased rate of suicide among patients with Huntington's disease.

Abstract: The proportion of deaths attributed to suicide was examined among 506 deceased individuals with diagnosed or suspected Huntington's Disease from New England USA. Comparison of this proportion with that of the general population indicated that the odds of a death being due to suicide in the Huntington's disease group is 8.2 times that of the Massachusetts population for persons aged 50 to 69 yr, but no difference appears in the 10 to 49 yr age group. Among the 157 Huntington's disease patients for whom cause of death was known, the corresponding odds estimates are 23.0 for the 50 to 69 yr age group and 2.7 for the 10 to 49 yr age group. More than half of the suicides occurred in individuals who showed early signs of the illness but who had not been diagnosed, suggesting that suicide may occur more frequently in the early stages of the illness.

Postural adjustments associated with rapid voluntary arm movements 1. Electromyographic data.

Abstract: Normal subjects made bilaterally symmetric rapid elbow flexions or extensions ("focal movement") while free standing or when supported by being strapped to a firm wall behind them (different "postural set"). In some trials a load opposed the movement two thirds of the way into its course. Electromyographic activity in leg and trunk muscles ("associated postural adjustments") demonstrated specific patterns for each type of movement. Activity in these muscles began prior to activity in the arm muscles and demonstrated a distal-to-proximal order of activation. The EMG patterns were characterised by alternating activity in the antagonist pairs similar to the triphasic pattern seen in the arm muscles. When the movement type was changed change of the pattern of the postural muscles occurred over several trials. It is concluded that the associated postural adjustments are pre-programmed motor activity linked to the focal movement, specific for the focal movement including anticipated events and the postural set.

Adverse effects of the management of malignant spinal cord compression.

Abstract: As a prelude to further work which attempts to improve the management of metastatic spinal cord compression the efficacy and adverse effects of existing therapy has been assessed. All papers dealing with the management of malignant spinal cord compression since 1960 have been reviewed. Data from this review is presented in a novel manner in order to identify not only the degree of successful return to ambulation achieved but more importantly the extent of the adverse effects which occur during existing management. It is seen that, while in general some 35% of patients treated in any manner retain or return to the ability to walk, some 20% to 25% sustain major neurological deterioration. In addition, those patients treated by laminectomy who do deteriorate may be subject to a significant rate of perioperative mortality and major structural complications related to the surgical wounds. In the light of the adverse factors described, the role of laminectomy as first-line management of malignant cord compression is questioned. Alternative modes of treatment are discussed and a tentative scheme of management described which it is hoped will lead to a better quality of survival of the group as a whole in addition to maintaining, or perhaps, improving the rate of successful return to ambulation.

Essential tremor in Rochester, Minnesota: a 45-year study.

Abstract: A 45-year (1935-79) retrospective study of essential tremor based on original medical records on residents of Rochester, Minnesota, is presented. The age and sex adjusted incidence for the most recent 15 year period was 23.7 per 100 000 for US white population. The prevalence rate, age and sex adjusted to 1970 US white population on January 1, 1979 was estimated at 305.6 per 100 000. Survival after diagnosis of essential tremor is comparable to age and sex matched population of West North Central United States. Mean age at diagnosis was 58 (range 2-96) years. Age adjusted annual incidence rate was not different in males (18.3/100 000) and females (17.1/100 000). Functional handicap was reported by four (1.5%) of the 266 incidence cases in school, 13 (5%) cases at work and five cases (2%) retired prematurely. Excessive use of alcohol was noted in 16% and 6% were diagnosed as alcoholic. Torticollis was diagnosed in 3% cases and an additional diagnosis of Parkinson's disease after the index date was made in 2% of incidence cases. Subsequent emergence of Parkinsonism was regarded as incidental. Diagnosis of hypertension was made at some time in 30% of incidence cases during the period (mean 37 years) for which the medical records were available. Risk of hypertension after onset of essential tremor in the cases was not different from that in a control group.

Predicting the outcome of acute stroke: a prognostic score.

Abstract: A prognostic score was derived from a prospective study of 148 consecutively admitted patients, aged less than 76 years, who survived the first 24 hours after an acute stroke. Multivariate analysis was used to compare the presenting clinical features of 137 (93%) of these patients with their outcome after two months. Little change in the level of residual disability was detected in 128 of these patients after a further four months. The features which were found to predict functional dependence or death included older age, complete limb paralysis, depression of conscious level and the combination of hemiplegia and hemianopia with higher cerebral dysfunction. Hemiparesis uncomplicated by hemianopia or higher cerebral dysfunction predicted a return to functional independence. A discriminant function derived from this analysis can be used to calculate the likelihood of recovery to independent function for an individual patient following an acute stroke.

A retrospective study of Creutzfeldt-Jakob disease in England and Wales 1970-79. I: Clinical features.

Abstract: A comprehensive search yielded 121 confirmed cases of Creutzfeldt-Jakob disease who died in England and Wales in the decade 1970-79, and 31 probable cases. The clinical features and the results of investigation are described. Three subgroups of patients were distinguished by clinical characteristics and contrasting course.

A double-blind trial of chronic cerebellar stimulation in twelve patients with severe epilepsy.

Abstract: Twelve patients with severe intractable epilepsy were treated by chronic cerebellar stimulation under double-blind conditions for six months. No reduction in seizure frequency occurred that could be attributed to stimulation, though eleven of the patients considered that the trial had helped them. One patient experienced fewer episodes of incontinence during stimulation. Cerebellar stimulation in its present form cannot be recommended for the treatment of severe intractable epilepsy.

Why do bridging veins rupture into the virtual subdural space

Abstract: Electron microscopic data on human bridging veins show thin walls of variable thickness, circumferential arrangement of collagen fibres and a lack of outer reinforcement by arachnoid trabecules, all contributory to the subdural portion of the vein being more fragile than its subarachnoid portion. These features explain the laceration of veins and the subdural location of resultant haematomas.

Presenile dementia with motor neuron disease in Japan: clinico-pathological review of 26 cases.

Abstract: The clinico-pathological findings of 26 cases of presenile dementia with motor neuron disease in Japan are reviewed. The characteristic features include: (1) Progressive dementia with slowly progressive onset in the presenile period. (2) Neurogenic muscular wasting during the course of illness. (3) A duration of illness to death of from one to three years. (4) Absence of extrapyramidal symptoms and definite sensory deficits. (5) No characteristic abnormalities in the CSF or EEG. (6) No known parental consanguinity of familial occurrence. (7) Non-specific mild degenerative changes throughout the CNS without evidence of cerebrovascular disease or primary degenerative dementia, but with the presence of pathological findings of motor neuron disease. The possibility that this is a new disease entity is suggested.

Pure psychic akinesia with bilateral lesions of basal ganglia.

Abstract: Three patients showed dramatic psychic akinesia after recovery from toxic encephalopathy. They had no or only mild motor disorders. The spontaneous psychic akinesia was reversible when the patient was stimulated, as if there was a loss of self psychic activation. Intellectual capacities were normal. Two patients had stereotyped behaviours resembling compulsions. In all patients CT cans showed bilateral lesions in the basal ganglia, mainly within the globus pallidus.

Deficient learning and memory in early and middle phases of multiple sclerosis.

Abstract: Forty-three patients with multiple sclerosis showed disturbances in short-term memory, learning, and delayed recall which were associated with years of active disease (average was 4.5 years), age, presence of flareup, but not steroid/ACTH treatment. Unrecognised memory loss might be prevalent early in the natural history of multiple sclerosis and deserves neuropsychological assessment.

Damage to the innervation of the voluntary anal and periurethral sphincter musculature in incontinence: an electrophysiological study

Abstract: In 40 women with idiopathic (neurogenic) faecal incontinence, 20 of whom also had stress urinary incontinence, single fibre EMG studies showed an increased fibre density in the external anal sphincter muscle. All these patients showed excessive descent of the pelvic floor on straining. The mean terminal motor latencies in the pudendal and perineal nerves, measured by a digitally-directed intrarectal stimulating technique, were increased when compared with 20 control subjects (p less than 0.01). The perineal nerve terminal motor latency was more markedly increased in the 20 patients with double incontinence than in those with faecal incontinence alone (p less than 0.01). These results provide direct electrophysiological evidence of damage to the innervation of the pelvic floor musculature in idiopathic faecal and double incontinence, and imply that idiopathic stress urinary incontinence may have a similar cause.

Normal proximal and delayed distal conduction in the pudendal nerves of patients with idiopathic (neurogenic) faecal incontinence.

Abstract: The latency of the response in the external anal sphincter muscle following transcutaneous stimulation of the cauda equina at the L1 vertebral level was measured in nine women with neurogenic faecal incontinence (mean 7.3 SD 0.7 ms) and 11 normal subjects (mean 5.6 SD 0.6 ms) (p = 0.01). There was no difference in conduction velocity between the L1 and L4 vertebral levels thus supporting the suggestion that conduction delay in faecal incontinence occurs distally.

A clinical study of Gilles de la Tourette syndrome in the United Kingdom.

Abstract: The clinical features of 53 British-born patients with Gilles de la Tourette syndrome are described. The mean age at onset of body tics was seven years and for vocalisations 11 years. Coprolalia was present in 39%, copropraxia in 21%, echolalia in 46% and echopraxia in 21%. Complicated antics and mannerisms were also common, often involving the compulsive touching of objects or self-injurious behaviour. Forty-six per cent of cases had a family history of tics in a single close relative and in two individuals a further member of the family had Gilles de la Tourette syndrome. Focal dystonia was present in four patients who had never received neuroleptics drugs and chorea was seen in two other untreated patients. In three patients acoustic startle consistently induced brief eye blink followed by a whole body jerk or jump. Rapid repetitive movements of the hands increased the frequency and severity of tics in 13 patients, but the performance of mental arithmetic under time pressure had a much more unpredictable effect. Electroencephalographic abnormalities occurred in eight (13%) but no definite CT brain scan abnormalities were detected. The incidence of left handedness did not differ from that in the general population and no evidence to suggest organic impairment was found on neuropsychological testing. This study provides no support for the notion that Gilles de la Tourette syndrome is a degenerative disorder of the central nervous system but provides some evidence for heterogeneity.

Measurement of reaction time following minor head injury.

Abstract: A four-choice reaction time test was carried out on 45 minor head injury cases, 24 hours after the injury and 6 weeks later. Twenty-eight subjects were re-tested after a six month interval. Reaction time measures were also obtained in a matched, general practice control group. The concussion cases displayed significantly poorer performances than the matched controls in four measures, at day 0 and at 6 weeks. The patients also showed serial improvement in these measures up to six months after the injury, when their scores excelled those of the matched controls.

Alterations in cognitive performance and affect-arousal state during fluctuations in motor function in Parkinson's disease.

Abstract: Sixteen patients with idiopathic Parkinson's disease were selected who were all showing severe fluctuations in motor function ("on-off" phenomenon). Measures of cognitive function and of subjective affect/arousal state were taken on two occasions, once when "on" and once when "off". Twenty-five matched normal controls were also assessed on the same measures. Results revealed, on the average, a drop in cognitive function plus an adverse swing in affect/arousal state, in the patient group in the "off" condition, compared to the levels when "on". Analysis of the data suggested that the main factor associated with cognitive function when "off" was not the severity of disability but the level of affect/arousal. The fluctuations in cognitive function found tended to be mild relative to the severe changes in motor ability, and were present in only a proportion of patients.

Tension development and muscle activation in the leg during gait in spastic hemiparesis: independence of muscle hypertonia and exaggerated stretch reflexes.

Abstract: In 15 patients with spastic hemiparesis the development of tension of calf muscles in relation to their electrical activation and their stretching period was studied on both sides during locomotion. Only in the spastic leg did isolated small biphasic potentials appear in the gastrocnemius E.M.G. with monosynaptic latency at the beginning of the stance phase, while the remaining gastrocnemius activation was reduced compared to the unaffected side. Perturbations of gait were followed in the spastic leg by a large monosynaptic response, while the polysynaptic reflex response was reduced. In the unaffected leg only a strong polysynaptic response appeared, which suggests a reciprocal modulation of monosynaptic and polysynaptic reflex responses. Tension development paralleled the gastrocnemius E.M.G. in the unaffected leg, while in the spastic leg tension was more closely correlated to muscle stretch. It is concluded that in spasticity the exaggerated monosynaptic reflexes represent only a small part of leg extensor activation during gait and that the tension development does not depend on these reflexes.

Studies on regional cerebral oxygen utilisation and cognitive function in multiple sclerosis.

Abstract: Regional cerebral oxygen utilisation (rCMRO2), oxygen extraction (rOER), blood flow (rCBF), and blood volume (rCBV) have been determined for fifteen patients with multiple sclerosis in remission using positron emission tomography (PET). Cerebral oxygen utilisation and blood flow were significantly reduced in both white matter and peripheral cortical grey matter in the multiple sclerosis patients compared to a group of normal controls. No evidence of regional cerebral ischaemia in the multiple sclerosis group was found. Lowest levels of cerebral oxygen utilisation were found in patients with cerebral atrophy, and in patients in whom a significant fall in present full-scale IQ from estimated pre-morbid levels had occurred. No correlation was found between rCMRO2 values and severity of locomotor dysfunction or clinical disease duration.

Levodopa-induced dyskinesia and thalamotomy

Abstract: Levodopa-induced dyskinesia of the limbs in thirteen cases of Parkinsonism, which was choreic, ballistic or dystonic in type, was alleviated almost completely by stereotaxic surgery using a microelectrode technique for the ventralis oralis anterior and posterior nuclei of the thalamus, but much less by the ventralis intermedius nucleus. Control of levodopa-induced dyskinesias by thalamic lesions in the course of routine treatment of Parkinsonism is discussed.

Chronic polyneuropathy of undetermined cause.

Abstract: The case histories of 519 patients with peripheral neuropathy on whom sural nerve biopsy had been performed were reviewed. In 67 patients (50 males, 17 females) (13%) who had symptoms of a symmetrical polyneuropathy for more than one year, the cause remained undiagnosed in spite of intensive investigation. Patients with inflammatory neuropathy were not included, but represented 17% of the whole series. The mean age of onset of symptoms was 50.6 years, and the median time from onset of symptoms to initial investigation was 2 years. Males were affected more commonly than females in a ratio of 3:1. The clinical features in 43 patients were those of a mixed motor and sensory neuropathy, in 17 patients a predominantly sensory neuropathy and in 7 patients a predominantly motor neuropathy. The mean CSF protein was 0.73 g/l and in only six patients was it greater than 1 g/l. Nerve conduction studies most commonly demonstrated mild slowing of motor conduction and impairment of sensory conduction. The usual pathological changes on sural nerve biopsy were those of chronic axonal degeneration. Forty seven patients (70%) were re-examined at intervals of time which ranged from 4 months to 12 years after their initial presentation and nerve biopsy (median, 3 years). As a group, they were only mildly disabled, the condition had a very slowly progressive course and there had been little change in their disability. A possible aetiological factor was found in 17 of the 47 patients (36%) and included malignancy, alcoholism, and benign paraproteinaemia. It is concluded that with intensive investigation the cause of chronic polyneuropathy of duration greater than one year remains undetermined in only about 13% of patients and that continued follow-up is worthwhile since a diagnosis may be established on re-examination.

The treatment of severe dystonia in children and adults.

Abstract: Twenty-three children (aged less than 18 years) and 17 adults with severe widespread dystonia were treated with high doses of benzhexol (up to 130 mg daily introduced slowly over many weeks). Children tolerated higher doses (median 30 mg/day) than adults (median 20 mg/day). 52% of the children gained useful benefit, many (43%) without unwanted side effects. Such an approach was less successful in adults; 41% gained benefit, but only 35% had no side effects. Twelve adults with severe axial dystonia, and two children with life-threatening generalised dystonia were treated with a combination of a low constant dose of tetrabenazine to which were added pimozide and benzhexol as necessary. The dose of tetrabenazine was aimed at 75 mg daily; pimozide was increased (6 to 25 mg/day) until the dystonia was relieved or Parkinsonism and other side-effects prevented further increments; if necessary benzhexol (6 to 30 mg/day) then was added to control side-effects and to provide additional benefit. 75% of the adults with severe axial dystonia, and one of the two children with life threatening generalised dystonia gained useful benefit from this regime. It is concluded that high dose benzhexol is the present first treatment of choice for children with severe dystonia, and is worth a try in adults but with less expectation of success. When benzhexol treatment alone fails in adults with severe disabling axial dystonia, or in children with life-threatening generalised dystonia, combined therapy with tetrabenazine, pimozide and benzhexol may give valuable symptomatic relief.