Showing papers in "Journal of Neurology, Neurosurgery, and Psychiatry in 1988"
TL;DR: Associated pathological findings suggest that cases of incidental Lewy body disease are presymptomatic cases of Parkinson's disease, and confirm the importance of age (time) in the evolution of the disease.
Abstract: The Lewy body is a distinctive neuronal inclusion that is always found in the substantia nigra and other specific brain regions in Parkinson's disease. It is mainly composed of structurally altered neurofilament, and occurs wherever there is excessive loss of neurons. It occurs in some elderly individuals and rarely in other degenerative diseases of the central nervous system. In 273 brains of patients dying from disorders other than Parkinson's disease, the age-specific prevalence of Lewy bodies increased from 3.8% to 12.8% between the sixth and ninth decades. Associated pathological findings suggest that these cases of incidental Lewy body disease are presymptomatic cases of Parkinson's disease, and confirm the importance of age (time) in the evolution of the disease. In view of the common and widespread occurrence of this disorder we propose that endogenous mechanisms operating in early life may be more important than environmental agents in the pathogenesis of Lewy bodies and Parkinson's disease.
3,223Â citations
TL;DR: The committee took into account an analysis of data from the international cooperative aneurysm study which contained 3521 patients from 68 countries showed that the two most important prognostic factors were the level ofconsciousness and the presence or absence of hemiparesis and/or aphasia.
Abstract: Sir: At its meeting in Barcelona in September 1987, the Executive Committee of World Federation of Neurosurgical Societies received and unanimously approved a report from a committee that had been working for six years to devise a simple, reliable, clinically valid scale for grading patients with a subarachnoid haemorrhage. The committee's view was that the scale was needed for describing changes in an individual patient at different times, for estimating prognosis and for standardising assessment of management in different groups of patients; a requirement was that the scale would meld with currently used scaling systems. The committee took into account an analysis of data from the international cooperative aneurysm study which contained 3521 patients from 68 countries;' this showed that the two most important prognostic factors were the level ofconsciousness (important for the prediction of both death and disability) and the presence or absence of hemiparesis and/or aphasia (important only for disability in survivors). The analysis had shown that ifconsciousness was normal, headache and/or a stiff neck did not significantly affect outcome. The committee resolved that five grades only should be used for patients with subarachnoid haemorrhage; patients with an unruptured aneurysm should be identified separately, or classified as 'zero'. It believed that the Glasgow Coma Scale2 should be used to assess the level of consciousness, because of its world wide acceptance in assessment of coma from head injury. The only additional factor should be the presence or absence of major focal deficit to differentiate between grades two and three. The committee also resolved that in assessing outcome from subarachnoid haemorrhage, categories of the Glasgow Outcome Scale should be used3: Dead Vegetative Survival, Severely disabled; Moderately disabled; good recovery.
631Â citations
TL;DR: The study protocol combined rapid clinical assessment of patients with accurate diagnosis of the pathological type of stroke by CT or necropsy, whether or not they were admitted to hospital, to ensure complete case ascertainment.
Abstract: A prospective study of acute cerebrovascular disease in a community of about 105,000 people is reported. The study protocol combined rapid clinical assessment of patients with accurate diagnosis of the pathological type of stroke by CT or necropsy, whether or not they were admitted to hospital. The study population was defined as those people who were registered with one of 50 collaborating general practitioners (GPs). Referrals to the study were primarily from the GPs though, to ensure complete case ascertainment, hospital casualty and admission registers, death certificates and special data from the Oxford Record Linkage Study were also scrutinized. Six hundred and seventy five cases of clinically definite first-ever in a lifetime stroke were registered in four years yielding a crude annual incidence of 1.60/1,000 or 2.00/1,000 when adjusted to the 1981 population of England and Wales. The age and sex specific incidence rates for first stroke showed a steep rise with age for both sexes. The odds of a male sustaining a first stroke were 26% greater than those of a female. Ninety one per cent of patients were examined in a median time of four days after the event by a study neurologist and 88% had cerebral CT or necropsy.
618Â citations
TL;DR: Comparisons of DFT and Alzheimer patients revealed qualitative differences in clinical presentation, neurological signs, profile of psychological disability, electroencephalography, single photon emission tomography and demography, which suggests DFT may be more common than is often recognised.
Abstract: A significant proportion of patients with presenile dementia due to primary cerebral atrophy do not have Alzheimer's disease. One form of non-Alzheimer dementia may be designated as dementia of frontal lobe type (DFT), on the basis of a characteristic neuropsychological picture suggestive of frontal lobe disorder, confirmed by findings on single photon emission tomography. The case histories of seven patients exemplify the disorder: a presentation of social misconduct and personality change, unconcern and disinhibition, in the presence of physical well-being and few neurological signs. Assessment revealed economic and concrete speech with verbal stereotypes, variable memory impairment, and marked abnormalities on tasks sensitive to frontal lobe function. Visuo-spatial disorder was invariably absent. Comparisons of DFT and Alzheimer patients revealed qualitative differences in clinical presentation, neurological signs, profile of psychological disability, electroencephalography, single photon emission tomography and demography. DFT, which may represent forms of Pick's disease, may be more common than is often recognised.
489Â citations
TL;DR: Baseline measurement of the ability to execute a given plan of action, to generate low level strategies required for efficient searching, and spatial working memory capacity, established that the Parkinson's disease group was unimpaired on any of these measures.
Abstract: The higher level cognitive function of planning was studied in a group of medicated Parkinson's disease patients and a group of matched control subjects, using a computerised version of Shallice's Tower of London task. Baseline measurement of the ability to execute a given plan of action, to generate low level strategies required for efficient searching, and spatial working memory capacity, all of which contribute to performance on the planning task, established that the Parkinson's disease group was unimpaired on any of these measures. On the Tower of London task, the Parkinson's disease group was also unimpaired in terms of the average number of moves required to solve a problem. However, a specific planning deficit was evident when "thinking" times were analysed, and this was after the confounding influence of motor initiation and execution times had been carefully extracted from total performance times. This finding is discussed in relation to putative functions of the frontal lobes and basal ganglia, and an attention-switching hypothesis is developed to account for it.
380Â citations
TL;DR: A prospective study in South-East England during 15 months in 1983-1984 recruited 100 patients with acute idiopathic neuropathy and found that after 12 months 67% had recovered completely, 20% were still significantly disabled and 13% had died.
Abstract: A prospective study in South-East England during 15 months in 1983-1984 recruited 100 patients with acute idiopathic neuropathy. After 12 months 67% had recovered completely, 20% were still significantly disabled and 13% had died. Ten of the 13 deaths were attributable to the neuropathy. The major features in the initial assessment which were associated with persistent disability were the time taken to become bedbound, requirement for ventilation, age greater than 40 years, and small or absent compound abductor pollicis brevis muscle action potentials elicited by stimulation of the median nerve at the wrist. These four variables have been combined in a statistical model to predict outcome for individual patients with acute idiopathic neuropathy.
364Â citations
TL;DR: The selective affection of this putative cholinergic nucleus in PSP and Parkinson's disease appears to be related to motor dysfunctions in these disorders.
Abstract: Significant loss of neurons in the pedunculopontine nucleus pars compacta (PPNc), a putative cholinergic nucleus involved in modulating somatic motor activities, has been demonstrated in progressive supranuclear palsy (PSP) and Parkinson's disease but not in Alzheimer's disease. A morphometric study of this nucleus was performed in two cases of PSP and in a cohort of cases of Parkinson's disease, Alzheimer's disease, senile dementia of Alzheimer type (SDAT), and age-matched controls. In PSP a significant 60% neuronal loss in PPNc was associated with neurofibrillary tangles in 40 to 64% of the remaining neurons. In Parkinson's disease there was a significant decrease in cell numbers and density by 53 and 51%, respectively, with Lewy bodies involving 6 to 39% of all neurons. In Alzheimer's disease and SDAT, large neurons were reduced by 29 and 33.8%, respectively, with tangles in 9 to 38% of the remaining cells. The selective affection of this putative cholinergic nucleus in PSP and Parkinson's disease appears to be related to motor dysfunctions in these disorders.
357Â citations
TL;DR: The natural history and response to different treatments have been evaluated, and a family history of blepharospasm or dystonia elsewhere was found in 9.5% of cases, which suggests a genetic predisposition.
Abstract: The natural history and response to different treatments have been evaluated in 264 patients with blepharospasm. The mean age of onset was 55.8 years and there was a female preponderance of 1.8 to 1. Dystonia elsewhere was found in 78% of patients, usually in the cranial-cervical region, and appeared to follow a somatotopic progression. A family history of blepharospasm or dystonia elsewhere was found in 9.5% of cases, which suggests a genetic predisposition. Ocular lesions preceded the onset of blepharospasm in 12.1% of cases. The response to drugs was inconsistent, although initial improvement was experienced by one fifth of patients treated with anticholinergics. Twenty-nine bilateral facial nerve avulsion operations were performed with benefit in 27 cases; but recurrences appeared in 22, on average one year after surgery. Botulinum toxin injections were performed in 151 patients. Significant improvement was achieved in 118 cases. Mean duration of benefit was 9.2 weeks. Transient ptosis and diplopia were the commonest side effects.
307Â citations
TL;DR: Possible explanations for the association of these agents with acute idiopathic neuropathy include possession of antigens shared with myelin and inhibition of suppressor mechanisms.
Abstract: The incidence of antecedent events and serological evidence of preceding infection were studied in 100 patients with acute idiopathic neuropathy and age and sex matched control subjects in South-East England. Symptoms of respiratory infections occurred within one month before onset of neuropathic symptoms in 38% of patients and 12% of controls (p less than 0.001) and symptoms of gastrointestinal infections in 17% of patients and 3% of controls (p less than 0.005). Immunisations, insect bites and animal contact were equally common in the patient and control subjects. Eight per cent of patients had undergone an operation within the preceding 3 months. Six per cent of patients had co-existing "autoimmune" diseases. Serological evidence of recent infection was identified in 31% of patients. Campylobacter jejuni (14%) and cytomegalovirus (11%) were both significantly more frequently demonstrated in patients than controls. Serological evidence of recent infection with mycoplasma (1%), Epstein Barr virus (1-2%) and parvovirus B19 (4%) was also identified in the patients but not more frequently than in the controls. Possible explanations for the association of these agents with acute idiopathic neuropathy include possession of antigens shared with myelin and inhibition of suppressor mechanisms.
301Â citations
TL;DR: In 15 patients with dystonia or tremor in whom the onset of abnormal movements was related, in time and in distribution, to injury of a body part, possible predisposing factors may have contributed to the pathogenesis of the trauma induced abnormal involuntary movements.
Abstract: Movement disorders are usually of central origin, but sometimes involuntary movements occur after peripheral trauma. Twenty eight patients, 13 women and 15 men, mean age 37 years (range 15-78), were studied with dystonia or tremor in whom the onset of abnormal movements was related, in time and in distribution, to injury of a body part. Among 23 patients with latency of less than one year after injury, focal dystonia of the involved body part was found in 18, nine of whom had associated reflex sympathetic dystrophy (RSD). One of five patients with peripherally induced tremor had RSD. Abnormal electromyography or nerve conduction velocities were found in the affected limb in four patients, but other electrophysiologic techniques provided evidence for disturbed central function. In 15 patients (65%) possible predisposing factors may have contributed to the pathogenesis of the trauma induced abnormal involuntary movements.
257Â citations
TL;DR: A patient with a 5 year progressive defect of naming and auditory verbal comprehension, the pathological nature of which was presumably degenerative, showed loss of the verbal knowledge of the physical attributes of the concepts corresponding to the words he was unable to understand.
Abstract: A patient is described with a 5 year progressive defect of naming and auditory verbal comprehension, the pathological nature of which was presumably degenerative. The auditory comprehension defect unevenly affected different semantic categories, and was particularly severe for the names of animals, fruits and vegetables. The patients showed loss of the verbal knowledge of the physical attributes of the concepts corresponding to the words he was unable to understand, and sparing of the verbal knowledge of the functional attributes. His performance was defective also on the colour-figure and sound-picture matching test.
TL;DR: It appears that stimulus content and spatial array affect neglect behaviour in patients with right-sided lesions and that a lack of systematic visual exploration within the extrapersonal space is one factor that contributes to visual hemispatial inattention.
Abstract: Patients with unilateral hemispheric lesions were given visual target cancellation tasks. As expected, marked contralateral and less severe ipsilateral visual inattention were observed in patients with right-sided cerebral lesions whereas those with left-sided lesions showed only mild contralateral neglect. Stimulus material (shapes vs letters) and array (random vs structured) interacted in a complex manner to influence target detection only in patients with right-sided lesions. Furthermore, the search strategy of these patients tended to be erratic, particularly when the stimuli were in an unstructured array. A structured array prompted a more systematic and efficient search. It appears, therefore, that stimulus content and spatial array affect neglect behaviour in patients with right-sided lesions and that a lack of systematic visual exploration within the extrapersonal space is one factor that contributes to visual hemispatial inattention.
TL;DR: The nature and organisation of anticipatory postural adjustments (APA) associated with the early phase of a voluntary upper limb movement were studied and appear to counteract the disturbing effects of the forthcoming voluntary movement.
Abstract: The nature and organisation of anticipatory postural adjustments (APA) associated with the early phase of a voluntary upper limb movement were studied. Upper limb elevations, performed at maximal velocity, were studied according to three conditions: bilateral flexions (BF) and unilateral flexions without and with an additional inertia (respectively OUF and IUF). Activities of the anterior part of the deltoid (DA) and of main muscles of the lower limbs, pelvis, trunk and scapular girdle were recorded by surface electromyography. Miniature-accelerometers enabled the recording of the tangential acceleration of the arm at wrist level (Aw) and the antero-posterior accelerations of various body links. Systematic investigations allow a precise description of the segmental phenomena which precede the onset of the voluntary movement. Before the activation of the anterior deltoid, a sequence of EMG modifications occurred in muscles of lower limbs, pelvis and trunk. The onset of Aw was preceded by anticipatory local accelerations of all the body links. Anticipatory EMG activities and local accelerations were organised according to patterns which were specific to the forthcoming voluntary movement. By comparing anticipatory EMG activities with anticipatory local accelerations, the nature of anticipatory postural movements can be determined. They appear to counteract the disturbing effects of the forthcoming voluntary movement. Because of their reproducibility and specificity, the anticipatory postural movements can be considered as preprogrammed. Postural adjustments and voluntary movement appear to be parts of the same motor program. Anticipatory postural movements should result from muscular functional synergies selected from a pre-evaluation of the perturbative aspects of the forthcoming movement.
TL;DR: Of the 27 patients with clinical diagnosis of vascular dementia, 23 showed multiple cerebral infarcts but senile plaques and neurofibrillary tangles were absent or in insignificant numbers, giving an accuracy of 85%, a figure higher than previously documented.
Abstract: Brains from a prospective study of demented patients were investigated post mortem. Of the 27 patients with clinical diagnosis of vascular dementia, 23 showed multiple cerebral infarcts but senile plaques and neurofibrillary tangles were absent or in insignificant numbers. This gives an accuracy of 85%, a figure higher than previously documented.
TL;DR: The muscular activity acting about different joints in the different movements was found to correlate with the predictions of activity needed to compensate for net joint reaction moments arising from the focal movement.
Abstract: Normal subjects performed bilaterally symmetric rapid elbow flexions or extensions ("focal movements") while standing. Specific patterns of electromyographic activity in leg and trunk muscles ("associated postural adjustments") were seen for each type of movement. The biomechanical significance of these postural adjustments was analysed by means of the ground reaction forces and motion of the various body segments. Experimental data were compared with that from a theoretical model of the body consisting of a six segment kinetic chain with rigid links. Distinct patterns of the ground reaction forces with elbow flexion were opposite in direction to those seen with elbow extension. Movements of the various body segments were small and specific for a certain focal movement. Dynamic perturbations arising from the arm movement in an anteroposterior direction were found to be compensated by postural adjustments, whereas vertical perturbations were not compensated. The muscular activity acting about different joints in the different movements was found to correlate with the predictions of activity needed to compensate for net joint reaction moments arising from the focal movement. Motion of the various body segments could be understood as resulting from the interplay of the net reaction moments and the net muscular moments at the different joints. Dynamic postural requirements are accomplished by a precise active compensation initiated before the focal movement.
TL;DR: The results suggest that the pathologic process responsible for CT changes in schizophrenia is static and is not affected by 8 years of neuroleptic medication and institutionalisation.
Abstract: Earlier cross-sectional studies have suggested that CT findings of ventricular enlargement and increased cortical markings in schizophrenic patients are not progressive, but individual patients have rarely been followed prospectively. Fifteen patients with chronic schizophrenia were rescanned on the same model machine after 7 to 9 years of continuous illness and, in seven cases, of continuous hospitalisation. It was not possible to demonstrate significant changes in either ventricular-brain ratio or frontal atrophy scores. These results suggest that the pathologic process responsible for CT changes in schizophrenia is static and is not affected by 8 years of neuroleptic medication and institutionalisation.
TL;DR: The unilateral facial flushing and sweating induced by heat in all five patients was thus a normal or excessive response by an intact sympathetic pathway, the other side failing to respond because of a sympathetic deficit.
Abstract: Facial flushing and sweating were investigated in five patients who complained of the sudden onset of unilateral facial flushing in hot weather or when exercising vigorously. One patient probably suffered a brainstem infarct at the time that the unilateral flush was first noticed, and was left with a subtle Horner's syndrome on the side opposite to the flush. The other four had no other neurological symptoms and no ocular signs of Horner's syndrome. Thermal and emotional flushing and sweating were found to be impaired on the non-flushing side of the forehead in all five patients whereas gustatory sweating and flushing were increased on that side in four of the five patients, a combination of signs indicating a deficit of the second sympathetic neuron at the level of the third thoracic segment. CT and MRI of this area failed to disclose a structural lesion but latency from stimulation of the motor cortex and thoracic spinal cord to the third intercostal muscle was delayed on the non-flushing side in one patient. The complaint of unilateral flushing and sweating was abolished in one patient by ipsilateral stellate ganglionectomy. The unilateral facial flushing and sweating induced by heat in all five patients was thus a normal or excessive response by an intact sympathetic pathway, the other side failing to respond because of a sympathetic deficit. The onset in the four cases of peripheral origin followed strenuous exertion, which suggested that an anterior radicular artery may have become occluded at the third thoracic segment during torsion of the thoracic spine.
TL;DR: The stereotyped anatomical and neuropsychological changes lead to the belief that the virus may recognise, within the limbic system, particular cellular "strains" on the basis of their molecular specificity.
Abstract: The clinical, neuropsychological and neuroradiological features of two patients affected by herpes simplex virus type 1 (HSV-1) encephalitis are described. An experimental study for the assessment of naming, recognition and description displayed in one patient a persistent significant impairment in naming living things. The other patient showed a failing "semantic memory" for the same categories, although a significant impairment emerged only for plants. In both patients, the late neuroradiological sequelae were localised mainly in the inferior and middle gyri of the left temporal lobe and in the left-side insula. In one patient, the right-side insula was also involved. The selective cerebral damage induced by HSV-1 is stressed and a correlation between the neuroradiological and neuropsychological findings is attempted. The stereotyped anatomical and neuropsychological changes lead to the belief that the virus may recognise, within the limbic system, particular cellular "strains" on the basis of their molecular specificity.
TL;DR: The cerebral vein wall in multiple sclerosis is subject to chronic inflammatory damage, which promotes haemorrhage and increased permeability, and constitutes a form of vasculitis.
Abstract: Evidence of damage to cerebral vein walls was sought in 70 cases of multiple sclerosis. Seventy control cases were also examined. The multiple sclerosis cases showed venous intramural fibrinoid deposition (7%), recent haemorrhages (17%), old haemorrhages revealed by haemosiderin deposition (30%), thrombosis (6%) and thickened veins (19%). In all, 41% of all multiple sclerosis cases showed some evidence of vein damage. Occasional control cases showed haemosiderin deposition in the brain but, unlike the multiple sclerosis cases, these were diffuse and almost entirely related to coexistent cardiovascular or cerebrovascular disease. Haemosiderin deposition was common in the substantia nigra and other pigmented nuclei in all cases. It is concluded that the cerebral vein wall in multiple sclerosis is subject to chronic inflammatory damage, which promotes haemorrhage and increased permeability, and constitutes a form of vasculitis.
TL;DR: The corticospinal direct response to stimulation of the motor cortex exposed for intracranial surgery was recorded from wire electrodes inserted into the spinal epidural space and was resistant to anaesthesia and unaffected by muscle relaxants.
Abstract: The corticospinal direct (D) response to stimulation of the motor cortex exposed for intracranial surgery was recorded in 20 cases from wire electrodes inserted into the spinal epidural space. The D response was obtained from stimulation of restricted areas of the cerebral cortex, that is, the hand, trunk and thigh areas of the motor cortex. The D response was resistant to anaesthesia and unaffected by muscle relaxants. Thus, recordings of the D response are useful for identifying the location of the motor cortex during intracranial surgery under general anaesthesia.
TL;DR: Results from an item analysis suggested that depression in Parkinson's disease patients is not a somatic artifact and indicated that Parkinsonian symptoms of depression can be measured separately from symptoms of the disease.
Abstract: Although the Beck Depression Inventory (BDI) is one of the most frequently employed measures of depression in Parkinson's disease, the somatic items included in the scale raise questions about its ability to differentiate depression from manifestations of the disease. The internal consistency and validity of the BDI as a measure of depression in a sample of 119 Parkinson's disease patients and 76 controls were studied. Results from an item analysis suggested that depression in Parkinson's disease patients is not a somatic artifact. Internal consistency reliability within the Parkinson's disease group was high. The factorial validity of the BDI was confirmed in both Parkinson's disease and control subjects. The results also indicated that Parkinsonian symptoms of depression can be measured separately from symptoms of the disease. These data suggest that the BDI including the somatic items is a reliable and valid measure of depression in Parkinson's disease and control subjects.
TL;DR: A relationship between severity of aphasia and degree of cortical hypoperfusion was found, in both the acute and the follow up assessments, in the aphasic subgroup of patients with unilateral subcortical vascular lesions.
Abstract: Cortical regional cerebral perfusion was assessed by N, N, N1-trimethyl-N1-(2)-hydroxy-3-methyl-5-(I-123) iodobenzyl-1, 3-propanediamine 2 HCl I-123 (HIPDM) and single photon emission computerised tomography (SPECT) in six aphasic and two neglect patients with unilateral subcortical vascular lesions. Assessments were carried out both in the acute phase and after a period ranging from 1 to 6 months after stroke onset. In all patients an almost complete spontaneous recovery occurred and was associated with a significant improvement of cortical perfusion. A relationship between severity of aphasia and degree of cortical hypoperfusion was found, in both the acute and the follow up assessments, in the aphasic subgroup.
TL;DR: CO2 and argon lasers were used as thermal stimulators, and the intra-individual variations obtained in sensory and pain thresholds were very low, suggesting that the method can be applied for clinical purposes.
Abstract: The clinical applications of thermal sensory and pain thresholds have been very limited due to large intra-individual variations. In the present paper CO2 and argon lasers were used as thermal stimulators, and the different factors (stimulus parameters and skin conditions) affecting the thresholds are described. The intra-individual variations obtained in sensory (9.3%) and pain (4.3%) thresholds were very low, which suggests that the method can be applied for clinical purposes.
TL;DR: Magnetic stimulation of the brain and spinal column was used to assess conduction in the descending central motor pathways controlling arm and leg muscles of patients with multiple sclerosis and normal subjects and provides clinically relevant information in the diagnosis and monitoring of multiple sclerosis patients.
Abstract: Magnetic stimulation of the brain and spinal column was used to assess conduction in the descending central motor pathways controlling arm and leg muscles of 20 patients with multiple sclerosis, and 10 normal subjects. The multiple sclerosis patients had relapsing and remitting disease but all were ambulant and in stable clinical remission. Increased central motor conduction times (CMCTs), up to three times normal, were frequently encountered in multiple sclerosis patients and in leg muscles these correlated closely with clinical signs of upper motor neuron disturbance; in the upper limb muscles a higher proportion of subclinical lesions was present. Weak muscles were almost invariably associated with abnormal central conduction but increased CMCTs were also found for 52 of the 104 muscles with normal strength. CMCTs for lower limb muscles were directly related (p less than 0.005) to functional motor disability (Kurtzke and Ambulatory Index Scales). No patient developed clinical evidence of relapse during follow-up of at least 8 months. Magnetic brain stimulation is easy to perform, painless, and safe, and provides clinically relevant information in the diagnosis and monitoring of multiple sclerosis patients.
TL;DR: Although there was no confirmed instance of familial Creutzfeldt-Jakob disease in the case-control study, dementia in close relatives was significantly more common than in controls and the age-specific mortality rate reached a peak in the seventh decade.
Abstract: An attempt was made to ascertain all cases of Creutzfeldt-Jakob disease occurring in England and Wales during the 5 year period 1980-1984. The mean annual mortality rate was 0.49/million; women were more frequently affected than men. The age-specific mortality rate reached a peak in the seventh decade. A case-control study involving 92 of the 122 definite and probable cases ascertained failed to confirm the reality of previously suspected aetiological agents in the environment. Although there was no confirmed instance of familial Creutzfeldt-Jakob disease in the case-control study, dementia in close relatives was significantly more common than in controls.
TL;DR: Seizures were evenly distributed among all pathological stroke sub-types but were restricted to lesions in the carotid artery territory, indicating a poorer prognosis over the first 2 days.
Abstract: In a series of 230 patients admitted to hospital with a stroke, the frequency and significance of epileptic seizures at onset was assessed. Thirteen (5.7%) suffered single or multiple witnessed seizures at the onset of their stroke. Seizures were evenly distributed among all pathological stroke sub-types but were restricted to lesions in the carotid artery territory. They indicated a poorer prognosis over the first 2 days. Six of the 13 presenting with stroke and seizures had prior seizures and, if they survived, continued to have fits. The five patients surviving with stroke and a first seizure were all fit-free after 30 months follow-up.
TL;DR: The dysfunctional pattern of muscle recruitment resembled that characteristic of dystonia elsewhere in the body and was indistinguishable in patients with idiopathic anismus and those with extrapyramidal motor disturbance due to Parkinson's disease, suggesting that anismu may be a focal dystonic phenomenon.
Abstract: Anismus, or constipation due to functional obstruction at the pelvic outlet by paradoxical contraction of the striated sphincter muscles during defaecation straining, is described in ten constipated patients and four patients with Parkinson's disease and constipation. The dysfunctional pattern of muscle recruitment resembled that characteristic of dystonia elsewhere in the body and was indistinguishable in patients with idiopathic anismus and those with extrapyramidal motor disturbance due to Parkinson's disease. These findings suggest that anismus may be a focal dystonic phenomenon.
TL;DR: The P300 latency was significantly prolonged in 12 patients with dementia compared with age-matched controls and showed a significant negative correlation with the score of Wechsler Adult Intelligence Scale (WAIS), especially with that of Digit Span subtest.
Abstract: An auditory discrimination paradigm was employed to elicit event-related brain potentials in 13 patients with Alzheimer's disease and 14 patients with multi-infarct dementia. The P300 latency was significantly prolonged in 12 patients with dementia compared with age-matched controls and showed a significant negative correlation with the score of Wechsler Adult Intelligence Scale (WAIS), especially with that of Digit Span subtest. There was no disease specificity. After physostigmine treatment, P300 latency decreased and WAIS score increased in 6 among 10 cases.
TL;DR: Findings indicate that the different clinical syndromes caused by cortical auditory lesions form a spectrum of related auditory processing disorders.
Abstract: The symptoms of two patients with bilateral cortical auditory lesions evolved from cortical deafness to other auditory syndromes: generalised auditory agnosia, amusia and/or pure word deafness, and a residual impairment of temporal sequencing. On investigation, both had dysacusis, absent middle latency evoked responses, acoustic errors in sound recognition and matching, inconsistent auditory behaviours, and similarly disturbed psychoacoustic discrimination tasks. These findings indicate that the different clinical syndromes caused by cortical auditory lesions form a spectrum of related auditory processing disorders. Differences between syndromes may depend on the degree of involvement of a primary cortical processing system, the more diffuse accessory system, and possibly the efferent auditory system.
TL;DR: This behavioural syndrome was produced by disconnecting the dorsomedian nucleus from the frontal lobe and limbic system and resolution of conflicting tasks was severely impaired, suggesting frontal lobe dysfunction.
Abstract: A disinhibition syndrome affecting speech (with logorrhoea, delirium, jokes, laughs, inappropriate comments, extraordinary confabulations), was the main manifestation of a right-sided thalamic infarct involving the dorsomedian nucleus, intralaminar nuclei and medial part of the ventral lateral nucleus. Resolution of conflicting tasks was severely impaired, suggesting frontal lobe dysfunction. These abnormalities correlated with the finding on SPECT of a marked hypoperfusion in the overlying hemisphere predominating in the frontal region. We suggest that this behavioural syndrome was produced by disconnecting the dorsomedian nucleus from the frontal lobe and limbic system.