Syncope in a patient with relapsing polychondritis.
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Adult↗
Biomedical subjects
L H Goldstein
Publications and source records attributed to L H Goldstein.
The management of motor neurone disease.
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Gastrostomy↗
Correlates of Quality of Life in people with motor neuron disease (MND).
OBJECTIVES: Previous work has not found correlations between standardized questionnaire measures of quality of life (QoL) and physical strength/functional ability in people with motor neuron disease (MND). Little is known about the relationship between ratings on an abbreviated self-generated measure of QoL, the Schedule for Evaluation of Individual Quality of Life-Direct Weighting (SEIQoL-DW), and measures of functional status such as the Sickness Impact Profile (SIP), although the former has been rated by people with MND as providing a more valid measure of their own QoL than the latter. The aim of this study was to examine whether self-generated ratings of QoL correlated with measures of physical impairment and self-reported functional status, psychological wellbeing and self-reported cognitive functioning, and with factors such as social support, which elsewhere has been reported to be a determinant of QoL in MND. DESIGN: The present cross-sectional study investigated between SEIQoL-DW ratings and SIP and the relationship ALS Severity Scale (ALSSS) scores, as well as with self-reported anxiety, depression, social support and everyday cognitive functioning in 31 people with MND. RESULTS: Overall QoL ratings on the SEIQoL-DW failed to correlate with any of the ALSSS or SIP subscale scores. This was despite the fact that health was nominated as an important QoL-related category by 64.5% of the sample. QoL scores were, however, found to correlate positively with the existence of confiding and emotional support; they also correlated negatively with the presence of self-rated everyday cognitive difficulties but not with affective state. CONCLUSIONS: Current findings support recent observations that individuals' ratings of their QoL cannot simply be equated with their physical impairment and functional limitations, and that support systems may be important. Cognitive functioning, known to be impaired in some people with MND, should also be considered when evaluating QoL.
Cognition Disorders↗
High and low perceived self-control of epileptic seizures.
PURPOSE: To define behaviours and to identify psychological, demographic, and epilepsy-related variables associated with high as opposed to low perceived self-control of seizures. METHOD: In a semistructured interview, 100 adults with intractable seizures were asked about their seizure precipitants and attempts at self-control of seizures. They also completed four psychological questionnaires. Latent Class Analysis was used to analyse the interview data to create two groups, High Controllers and Low Controllers, who were then compared on demographic, epilepsy, and psychological characteristics. RESULTS: Being able to identify and seeking out low-risk-for-seizure situations, avoiding high-risk-for-seizure situations, and making attempts at seizure inhibition were seizure behaviours that discriminated High from Low Controllers. The general probability of being a High Controller was greater than that of being a Low Controller. Perceived high self-control of seizures was associated with low chance-health locus of control. For Low Controllers, current age, age at onset of seizures, and duration of epilepsy history were related to psychological variables. A significantly higher proportion of the Low Controllers than High Controllers were women. CONCLUSIONS: Many people with intractable seizures do not accept their epilepsy as a condition over which they have no control. Perceived self-control of seizures, however, involves a complex interaction between epilepsy and psychological factors, with health locus of control an apparently important discriminator between High and Low Controllers.
Adult↗
The effect of non-invasive positive pressure ventilation (NIPPV) on cognitive function in amyotrophic lateral sclerosis (ALS): a prospective study.
OBJECTIVES: Neuropsychological investigations have shown a degree of cognitive dysfunction in a proportion of non-demented patients with ALS. Respiratory muscle weakness in ALS can lead to nocturnal hypoventilation, resulting in sleep disturbance and daytime somnolence. Sleep deprivation of this type may cause impairments in cognitive function, but this has not been formally evaluated in ALS. METHODS: Cognitive functioning was evaluated in nine patients with ALS with sleep disturbance caused by nocturnal hypoventilation (NIPPV group), and in a comparison group of 10 similar patients without ventilation problems (control group). The NIPPV group then started non-invasive positive pressure ventilation (NIPPV) at night. After about 6 weeks, change in cognitive function was evaluated. RESULTS: Statistically significant improvement in scores on two of the seven cognitive tests was demonstrated in the NIPPV group postventilation, and a trend towards significant improvement was found for two further tests. Scores in the control group did not improve significantly for these four tests, although an improvement was found on one other test. CONCLUSIONS: Nocturnal hypoventilation and sleep disturbance may cause cognitive dysfunction in ALS. These deficits may be partially improved by NIPPV over a 6 week period. This has important implications for investigations of both cognitive dysfunction in non-demented patients with ALS, and the effect of ventilation on quality of life.
Aged↗
Attributions, strain and depression in carers of partners with MND: a preliminary investigation.
Further examination of the data of Goldstein et al. (J Neurol Sci 160 (1998) S114) on the impact of MND in their spouses/partners on 19 caregivers investigated whether carers' depression and strain scores could be related to their attributional style. Contrary to expectations, carers' scores on a measure of depressed mood were not correlated with measures of internal/external, stable or global attributions. However, perceived strain was greater in carers who viewed their partners' illness as having a more global impact on their lives, and was related to self-perceived control over their thoughts about their partner. Female carers anticipated experiencing less strain and distress in the following year than male carers, even though they did not differ in current strain levels. Male and female carers received similar numbers of formal services. The availability of informal support (family and friends) was associated with reduced anticipation of future strain and distress and with reduced perception of the pervasive impact of their partners' MND on their lives.
Adaptation, Psychological↗
Verbal fluency and executive dysfunction in amyotrophic lateral sclerosis (ALS).
Neuropsychological investigations of amyotrophic lateral sclerosis (ALS) patients have revealed variable results on specific tests, despite a similar overall cognitive profile of predominantly executive dysfunction with some evidence of memory impairment. The most striking and consistent deficit is found using tests of verbal fluency. The current investigation explored why verbal fluency is particularly sensitive to the impairment in ALS, by investigating some of the underlying cognitive processes: (i) intrinsic response generation; (ii) phonological loop functions; and (iii) simple word retrieval. Twenty-two ALS patients and 25 healthy controls were investigated. The battery included: (i) written and spoken letter-based fluency, category fluency, design fluency; (ii) the Phonological Similarities effect and Word Length Effect; and (iii) computerised sentence completion and confrontational naming. The tests were designed to control for motor speed and to accommodate for the range of disabilities that are present in ALS patients. Significant impairments were found on some tests of intrinsic response generation, namely the Written Verbal Fluency Test, Category Fluency Test (generation of animal names) and Design Fluency Test. Phonological loop functions appeared to be intact with evidence of both the Phonological Similarities and Word Length Effects, but the ALS patients displayed significantly reduced working memory capacity. No deficits were found on tests of simple word retrieval. The findings indicate that verbal fluency impairments in ALS patients result from a higher order dysfunction, implicating deficits in the supervisory attentional system or central executive component of working memory, and are not caused or exaggerated by an impairment in phonological loop functions or in primary linguistic abilities. The study also demonstrates the importance of controlling for differences in motor speed, which may have served to exaggerate the presence of cognitive deficits in ALS patients reported by some other studies.
Amyotrophic Lateral Sclerosis↗
Seizure precipitants and perceived self-control of seizures in adults with poorly-controlled epilepsy.
This study was set up in order to investigate the prevalence and nature of seizure precipitants and self-control behaviours in adults with intractable seizures. A semi-structured interview was conducted with 100 patients attending neurology or neuropsychiatry epilepsy out-patient clinics. The interview included six questions regarding seizure precipitants, the extent to which the patients seek and avoid precipitants, and their ability to induce or abort seizures. The study revealed that over 90% of the participants could identify at least one seizure precipitant. Stress, depression, tiredness and the menstrual cycle were the most common precipitants reported. In addition, 65% of the participants could identify at least one 'low-risk' situation in which seizures were unlikely to occur. Fifteen percent reported they could induce a seizure, 52% said that they consciously try to avoid seizure precipitants and 47% said they could sometimes stop their seizures from happening. These results indicate that the majority of the sample could identify factors which trigger their seizures, and that some of the participants engage in attempts to reduce their seizure frequency by avoiding these factors and by controlling the onset of their seizures. The possible mechanisms involved in the relationship between precipitants and seizure genesis are discussed.
Adult↗
Dissociation, hypnotizability, coping styles and health locus of control: characteristics of pseudoseizure patients.
Although literature in this area is relatively sparse, the occurrence of psychogenic non-epileptic seizures (pseudoseizures) has been linked to stress, anxiety and possible dissociative tendencies. An association between dissociation and hypnotic susceptibility has also been proposed and dissociative tendencies have themselves been found to relate to the use of emotion-focused coping strategies. In order to investigate the hypothesis that pseudoseizure patients may exhibit higher levels of dissociation, a more emotion-focused coping style, and greater hypnotic susceptibility than the general population, the questionnaire responses of 20 patients with pseudoseizures were compared with those obtained from a non-clinical control group. As predicted, pseudoseizure patients demonstrated some evidence of higher levels of dissociation and escape-avoidance coping strategies. They also expressed a greater belief in external control over health and higher depression scores, compared to the control group, but the previously reported elevation in hypnotizability scores in the pseudoseizure patients was not found. Possible explanations for this pattern of results are discussed.
Adaptation, Psychological↗
The emotional lability questionnaire: a new measure of emotional lability in amyotrophic lateral sclerosis.
In an ALS Clinic, use of the Pathological Laughter and Crying Scale of Robinson et al. [Robinson RG, Parikh RM, Lipsey JR, Starkstein SE, Price TR. Pathological laughter and crying following stroke: validation of a measurement scale and double-blind treatment study. American Journal of Psychiatry 1993;150(2):286-293] revealed several problems: reliance on self-rating, insufficient period of assessment, inadequate exploration of 'appropriateness of emotion', lack of an item for abnormal smiling, amusement rather than happiness being the cause of laughter in ALS, and a frequency-based rating system. The necessary modifications produced a new Emotional Lability Questionnaire (ELQ) that was tested in 43 ALS patients and 43 healthy controls. The self-rated version of the ELQ was administered as a structured interview to each participant, and the independent-relationship between subscales of the ELQ for both versions, thus confirming its internal validity. Greater emotional lability appeared associated with pseudobulbar symptoms. The question why 14 patients rated themselves as severely labile in the crying domain alone and four in the laughter domain alone, required further study.
Adult↗
Reduction in seizure frequency following a short-term group intervention for adults with epilepsy.
A preliminary investigation of the efficacy of a group intervention combining a range of psychological approaches and techniques for seizure management in adults with poorly controlled epilepsy. An uncontrolled AB group design was employed. Seven adults with intractable seizures took part in 8, weekly group sessions which included providing information, employed cognitive-behavioural techniques and addressed emotional difficulties. Weekly seizure logs were kept by participants during the intervention and the following 3 months. Five questionnaires were administered before and after the intervention and at 2-months follow-up to provide an indication of psychosocial well-being. Seizure frequency and scores on the questionnaires were used as outcome measures. There was a significant reduction in seizure frequency in the group, which persisted at follow-up. There were no significant changes on any of the questionnaires. The results suggest that a group-based intervention incorporating a range of psychological techniques may be effective for improving seizure control. The link between seizure reduction and psychological and psychosocial well-being needs further investigation.
Adaptation, Psychological↗
Social dominance and cardiovascular reactivity in preschoolers. Associations with SES and health.
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Blood Pressure↗
The psychological impact of MND on patients and carers.
Nineteen patients with Motor Neurone Disease (MND) who had been living with their partners for at least two years prior to the onset of their illness, together with their partners, completed self-report questionnaires to investigate the impact of MND on both patients and carers. Physical disability and impact of the illness on aspects of everyday functioning were related to levels of anxiety and depression in the patients; psychological coping strategies adopted depended to some extent on symptom duration. Carers also demonstrated signs of anxiety and depression, with the latter correlating with aspects of the patients' functional impairment. Perceived strain in carers over caring for the patient correlated with a loss in intimacy in their relationship, which in turn was predicted by patients' cognitive/behavioural and communication changes. Changes in patients' social performance also correlated with the extent to which carers felt that the illness was affecting other areas of their life, the extent to which their partner dominated their thoughts and the extent to which they could control their reactions when thinking about the patient. Satisfaction with formal services and the number of social groups to which carers belonged correlated with carers' self-predicted future ability to cope.
Caregivers↗
Exploring the temporal nature of hemodynamic responses of cortical motor areas using functional MRI.
OBJECTIVE: To use functional MRI (fMRI) to study grouped patterns of cerebral activation and the course of hemodynamic responses during performance of two activation tasks (paradigms) using a hand-held joystick to perform movements in a repetitively fixed direction and movements in freely selected random directions. BACKGROUND: Evidence from lesion, electrophysiologic, and functional imaging studies implicates prefrontal and mesial frontal cortex in motor preparation and primary motor cortex in motor execution. fMRI can be used to study cerebral activation and has practical advantages over other methods of functional neuroimaging. METHODS: We acquired 100 multislice T2*-weighted data sets from five healthy volunteers during performance of each paradigm using conventional fMRI. For each paradigm, rest and movement epochs were alternated every 30 seconds. After coregistration and spatial normalization, we combined the data for group studies. We used statistical parametric mapping to compare the early (first 15 seconds) components of the movement epochs with rest as well as the late (last 15 seconds) components of the movement epochs with rest. RESULTS: During the early phase of both paradigms, significant activation was present in rostral and caudal mesial premotor cortex. Right prefrontal cortex was significantly activated during the early component of freely selected joystick movements. Activation of rostral supplementary motor area was maintained during the late component of freely selected movements but decreased during repetitively fixed movements. In contrast, significant activation in contralateral sensorimotor cortex was maintained during both early and late components of both paradigms. CONCLUSIONS: fMRI can detect cortical activation. The temporal resolution of fMRI also allows adaptation of blood oxygenation level-dependent (BOLD) contrast signal to be detected in association cortex. However, the level of BOLD contrast signal in primary motor cortex remained significantly elevated throughout task performance.
Adult↗
Fear of seizures: an investigation and treatment.
Despite the wealth of epidemiological evidence showing the comorbidity of epilepsy with various forms of psychopathology, there has been little systematic investigation of its relationship with affective disorders. This study aims to explore the phenomenon of seizure fear. Survey data have suggested that patients commonly fear death and/or brain damage as a result of their seizures, but documented cases of seizure phobia are rare and infrequently address issues of treatment. The study describes the exploration and successful cognitive behavioural treatment of a case of seizure phobia in a 26 year old woman with a 9 year history of epilepsy following a subarachnoid haemorrhage. The findings represent an important development in the much-neglected study of seizure fear. The case highlights some of the factors which may contribute to the genesis and maintenance of epilepsy-related psychopathologies and consequently has important implications for theoretical modelling in this area. Furthermore, it provides encouraging evidence to support the use of cognitive-behavioural techniques in treatment of these disorders.
Adaptation, Psychological↗
Are what people know about their epilepsy and what they want from an epilepsy service related?
This study set out to investigate the level of knowledge about epilepsy in general and in relation to the patients' own condition, in patients attending a tertiary referral epilepsy outpatient clinic. It also sought to investigate patient satisfaction with the service and whether knowledge acquired about epilepsy related to that satisfaction. Seventy out of 94 patients responded to the Epilepsy Knowledge Profile Questionnaires (general and personal knowledge of epilepsy) and a questionnaire assessing service satisfaction. Patients were found to know more about epilepsy in general than about their own condition. In particular some patients were unable to give accurate indications of their drug regimes. Over 91% were satisfied with the serviced they received but this bore no relation to information they had acquired or wanted about epilepsy. Multidisciplinary services were requested by a sizeable percentage of patients but especially access to a specialist nurse in epilepsy. The study highlights the need for clinicians to check patients' knowledge about their condition and for further work to clarify the source of patients' satisfaction with service delivery.
Adolescent↗
Patients' and referring doctors' perceptions of treatment on an in-patient neuropsychiatry/epilepsy unit: a study of three cohorts.
Three cohorts of patients, discharged within three, 12-month periods from a tertiary referral Neuropsychiatry/Epilepsy Unit, were asked whether they felt their condition had improved as a result of their admission, how they valued the admission and how easy they had found the admission to manage. They were also asked about their psychological and psychosocial state. For the first cohort a longer admission was more greatly valued and was associated with a better self-perceived outcome, and for the second cohort with the manageability of the admission. Replies to questionnaires sent to the referring doctors of two of the three cohorts of patients indicated that, in addition to their being satisfied with the service, there was a positive correlation between their ratings of patients' improvement and those ratings offered by the patients themselves. Issues relating to length and purpose of admissions, finances and the distribution of assessment and treatment of patients across different services are discussed in the light of the findings.
Adaptation, Psychological↗
Relation between cognitive dysfunction and pseudobulbar palsy in amyotrophic lateral sclerosis.
OBJECTIVES: To examine the relation between cognitive dysfunction and pseudobulbar features in patients with amyotrophic lateral sclerosis (ALS). METHODS: The performance of two patient groups, ALS with pseudobulbar palsy (n = 24) and ALS without pseudobulbar palsy (n = 28), was compared with 28 healthy age matched controls on an extensive neuropsychological battery. Tests used were the national adult reading test, short form of the WAIS-R, recognition memory test, Kendrick object learning test, paired associate learning, Wisconsin card sorting test, verbal fluency, Stroop and negative priming tests, a random movement joystick test, and a computerised Tower of Hanoi test. RESULTS: Tests of executive function showed a pronounced deficit on written verbal fluency in both ALS groups in comparison to controls, which tended to be more prominent in patients with ALS with pseudobulbar palsy. The random movement joystick test (a non-verbal test of intrinsic movement generation) showed an impairment in the generation of random sequences in patients with pseudobulbar palsy only. The computerised Tower of Hanoi showed a subtle planning impairment (shorter planning times) in all the patients with ALS compared with controls on trials requiring more complex solutions. In addition the pseudobulbar patients displayed shorter planning times on complex trials, and tended to solve these trials less accurately. There was also evidence of a deficit for all patients with ALS in comparison with controls on total errors and number of categories achieved on the Wisconsin card sorting test and a strong tendency towards an impairment on a task of selective attention and cognitive inhibition (negative priming). A word recognition memory deficit was showed across both ALS groups. CONCLUSIONS: This study elicited cognitive deficits (involving predominantly executive processes, with some evidence of memory impairment) in patients with ALS and further strengthened the link between ALS and frontal lobe dysfunction, this being more prominent in patients with pseudobulbar palsy. However, cognitive impairments suggestive of extramotor cortical involvement were not exclusive to this subgroup.
Adult↗