Absence of cognitive, behavioral, or emotional dysfunction in progressive muscular atrophy.
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Adult↗
Biomedical subjects
L H Goldstein
Publications and source records attributed to L H Goldstein.
Longitudinal predictors of psychological distress and self-esteem in people with ALS.
OBJECTIVE: To identify predictors of psychological distress (measured by anxiety and depression) and low self-esteem and to determine whether these change over time in people with ALS. METHOD: We interviewed 50 patients with ALS living with a spouse/partner; further interviews were held at median intervals of 6 and then 5 months. Although carers were interviewed, we report the patients' data. Patients completed measures about their social support and marital relationship; the functional impact of ALS; everyday cognitive, emotional, and behavioral changes; symptoms of anxiety and depression; and self-esteem. The ALS Severity Scale was also completed. RESULTS: From the initial sample of 50, 26 patients were interviewed on all three occasions. At the first interview, negative social support and bulbar impairment were most predictive of psychological distress; pre-illness marital intimacy was the best predictor of patients' self-esteem. Over time, negative social support and pre-illness marital intimacy retained an ability to predict patients' affective state and self-esteem. CONCLUSIONS: Social factors are important in determining longer-term psychological well-being in people with ALS who are in the relatively early stages of the disease.
Adult↗
The effect of noninvasive ventilation on ALS patients and their caregivers.
BACKGROUND: Noninvasive ventilation (NIV) reduces mortality and improves some aspects of quality of life (QoL) in ALS. However, concerns remain that progressive disability may negate these benefits and unnecessarily burden caregivers. METHODS: Thirty-nine patients requiring NIV were offered treatment. Twenty-six were established on NIV, but 13 declined or could not tolerate NIV. Fifteen patients without respiratory muscle weakness (RMW) but with similar ALS severity and age were studied in parallel. Caregivers of 21 NIV, 7 untreated, and 10 patients without RMW participated. Patients and caregivers had detailed QoL measurements for 12 months. NIV patients underwent cognitive testing before and after treatment. RESULTS: RMW correlated with lower QoL. The median survival of untreated patients (18 days; 95% CI 11 to 25 days) was shorter than for NIV patients (298 days; 95% CI 192 to 404 days) and non-RMW patients (370 days; 95% CI 278 to 462 days; log rank test [2 df] = 81, p = 0.00001). A wide range of QoL measures improved within 1 month of starting NIV, and improvements were maintained for 12 months. QoL of non-RMW patients declined as RMW progressed. Caregivers of NIV and non-RMW patients showed similar increases in burden, but NIV patient caregivers developed a deterioration in the Short Form-36 Vitality score. No improvements were found on measures of learning and recall in the NIV patients. CONCLUSIONS: Respiratory muscle weakness has a greater impact on quality of life (QoL) than overall ALS severity. Noninvasive ventilation (NIV) improves QoL despite ALS progression. NIV has no impact on most aspects of caregiver QoL and does not significantly increase caregiver burden or stress.
Adult↗
Predictors of psychological distress in carers of people with amyotrophic lateral sclerosis: a longitudinal study.
BACKGROUND: The majority of people providing informal care for people with amyotrophic lateral sclerosis (ALS) are spouses. This prospective study set out to examine changes in and predictors of psychological distress in spouse carers of people with ALS. METHOD: Fifty spouse carers of people with ALS underwent an initial interview and at least 21 underwent two further interviews, at median intervals of approximately 5-6 months. They rated the functional impact of their partner's ALS on everyday activities and everyday cognitive, emotional and behavioural changes that might have occurred in the person with ALS. They also rated their own social support and marital relationship, and completed measures of mood, burden and strain. The ALS Severity Scale was also completed for their partner with ALS. RESULTS: Over time, carers' psychological distress (a global measure combining mood, burden and strain) increased significantly. Initially carers' psychological distress was best predicted by the psychosocial impact of their spouse/partners' ALS, the extent to which their partner demonstrated emotional lability and how many other people were considered as dependents of the carer. Subsequently, carer distress was best predicted by an initial measure of negative social support and by their initial satisfaction with their social relationships. CONCLUSIONS: Despite the significant physical impairment associated with ALS, psychosocial factors appear important in determining short- and longer-term psychological well-being in carers of people with ALS and may help clinicians to predict which carers are likely to experience psychological difficulties as part of their caring role.
Aged↗
Ictal symptoms of anxiety, avoidance behaviour, and dissociation in patients with dissociative seizures.
OBJECTIVE: To examine anxiety related seizure symptoms and avoidance behaviour in adults with dissociative (psychogenic non-epileptic) seizures (DS) in comparison with a group suffering from partial epilepsy. METHODS: 25 DS and 19 epilepsy patients completed an attack symptom measure, the hospital anxiety and depression scale, the dissociative experiences scale, and the fear questionnaire. RESULTS: DS patients reported the presence of significantly greater numbers of somatic symptoms of anxiety during their attacks than the epilepsy group, despite not reporting subjectively higher levels of anxiety. The DS patients also reported higher levels of agoraphobic-type avoidance behaviour than the epilepsy group. Measures of dissociation were higher in the DS group, who also reported greater symptoms of depression. CONCLUSIONS: The findings support a model whereby DS occur as a paroxysmal, dissociative response to heightened arousal in the absence of raised general anxiety levels. The model has practical implications for clinical assessment and treatment: in clinical practice, inquiry about these symptoms may help in the diagnosis of DS; with respect to treatment, the anxiety related symptoms and avoidance behaviour prevalent in DS are a potential focus for a cognitive behavioural approach analogous to that used in the treatment of other anxiety disorders.
Adult↗
[The risks of combining medicine and herbal remedies].
The risks of using herbal remedies, considered 'natural', should not be disregarded, as some have serious side effects and some interact with and influence conventional medical therapeutics. The effect may be pharmacokinetic by altering absorption or metabolism, and may be pharmacodynamic, by changing the final effect of the drug. St. John's wort, for example, an antidepressant herbal remedy, may pharmacodynamically interact with specific serotonin reuptake inhibitors (SSRI's), causing a serotonin syndrome. St. Johns wort also causes serious pharmacokinetic interactions by activating the cytochrome CYP3A4, dangerously decreasing blood levels of cyclosporin, warfarin, and theophylline, and reducing the efficacy of contraceptive pills and AIDS therapy. The article presents a review of a number of herbal remedies, commonly used in Israel, that have documented drug interactions, providing details of common indications, adverse reactions and drug interactions of each herbal remedy. Physicians should recognize the fact that patients use herbal remedies, purchased directly at pharmacies or health stores, and be aware of the potential interactions of these remedies with conventional drugs.
Antidepressive Agents↗
Psychological treatments for epilepsy.
BACKGROUND: Psychological interventions such as relaxation therapy, cognitive behaviour therapy, bio-feedback and educational interventions have been used alone or in combination in the treatment of epilepsy, to reduce the seizure frequency and improve the quality of life. OBJECTIVES: To assess whether the treatment of epilepsy with psychological methods is effective in reducing seizure frequency and/or leads to a better quality of life. SEARCH STRATEGY: We searched the Cochrane Epilepsy Group's Specialized Register (July 2005), the Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library Issue 2, 2005), and MEDLINE (1966 to March 2005). No language restrictions were imposed. We checked the reference lists of retrieved studies for additional reports of relevant studies. SELECTION CRITERIA: Randomized or quasi-randomized studies assessing one or more types of psychological or behaviour modification techniques for people with epilepsy. DATA COLLECTION AND ANALYSIS: Two review authors independently assessed the trials for inclusion and extracted data. Primary analyses were by intention to treat. Outcomes included reduction in seizure frequency and quality of life. MAIN RESULTS: We found three small trials (50 participants) of relaxation therapy. They were of poor methodological quality and a meta-analysis was therefore not undertaken. No study found a significant effect of relaxation therapy on seizure frequency. One trial found cognitive behavioural therapy to be effective in reducing depression, among people with epilepsy with a depressed affect, whilst another did not. One trial of group cognitive therapy found no significant effect on seizure frequency. Two trials of combined relaxation and behaviour therapy and one of EEG bio-feedback and four of educational interventions did not provide sufficient information to assess their effect on seizure frequency. One small study of galvanic skin response biofeedback reported significant reduction in seizure frequency. Combined use of relaxation and behaviour modification was found beneficial for anxiety and adjustment in one study. In one study EEG bio-feedback was found to improve the cognitive and motor functions in individuals with greatest seizure reduction. Educational interventions were found to be beneficial in improving the knowledge and understanding of epilepsy, coping with epilepsy, compliance to medication and social competencies. AUTHORS' CONCLUSIONS: In view of methodological deficiencies and limited number of individuals studied, we have found no reliable evidence to support the use of these treatments and further trials are needed.
Biofeedback, Psychology↗
Cognitive change in ALS: a prospective study.
OBJECTIVES: To investigate longitudinally the profile of cognitive impairment in nondemented patients with ALS. METHODS: Twenty nondemented patients with ALS and 18 controls were interviewed at two time points separated by a 6-month interval. The extensive battery was designed to accommodate the range of physical disability present in ALS, and included measures of executive, memory, language, and visuospatial functions, everyday behavior, and emotion. RESULTS: On a test of simple word retrieval (Computerised Sentence Completion Test) patients with ALS became slower over time, while controls became faster at completing sentences with appropriate words. This effect remained when the analysis accommodated for progressive speech disability. Patients with ALS also displayed an impairment in both written and spoken verbal fluency indices (time to think of each word) at both time points, but there was no evidence of deterioration over time. On the Short Inventory of Minor Lapses, carers of patients with ALS displayed increased awareness of cognitive dysfunction in everyday behavior while controls' ratings of their partners decreased. In addition, patients displayed more depressive symptomology (although well below clinical levels) on the second interview vs controls. Patients with ALS also displayed emotional lability at both time points, although this did not increase over time. CONCLUSIONS: Cognitive deterioration in nondemented patients with ALS is a relatively slow process. Selective cognitive impairment in the form of verbal fluency deficits, most likely indicating executive dysfunction, appears relatively early on in the course of the disease, although language functions may become vulnerable as the disease progresses.
Adult↗
Changes in memory for emotional material in amyotrophic lateral sclerosis (ALS).
The current study sought to examine the performance of non-demented ALS patients on neuropsychological tests involving emotional perception and memory. Nineteen non-demented patients with ALS were compared with 20 healthy controls on assessments of facial expression recognition, social judgement ratings of faces and recognition memory for emotional words. Patients and controls were well matched to exclude a range of potentially confounding variables. The patients and controls demonstrated significant differences on only one test of cognitive functioning. The ALS group demonstrated a failure to show the normative pattern of enhanced recognition memory for emotional words compared to neutral words and produced higher scores than controls on recognition memory for neutral words. These findings suggest that patients with ALS show a different pattern of cognitive performance with respect to memory for emotional material when compared to healthy adults.
Adult↗
Cytomegalovirus encephalitis in an immunocompetent pregnant woman.
Presented here is the case of an immunocompetent pregnant woman with probable cytomegalovirus encephalitis. The clinical picture was characterized by diffuse headache, drowsiness and the development of an acute confused state. Diagnosis was based on the documentation of recent cytomegalovirus seroconversion with low avidity for the immunoglobulin G class antibodies. Initially, the diagnosis of encephalitis was challenging due to the subtle findings in cerebrospinal fluid and the normal electroencephalograph results. However, repeated tests revealed findings compatible with the diagnosis of encephalitis. Due to the suspicion of herpes simplex encephalitis the patient was treated with acyclovir. Within a few days rapid resolution of the fever and complete recovery were observed. Cytomegalovirus encephalitis should be considered early in the evaluation of pregnant women if appropriate clinical symptoms are present.
Acyclovir↗
Word retrieval in amyotrophic lateral sclerosis: a functional magnetic resonance imaging study.
The cognitive impairment revealed in some non- demented amyotrophic lateral sclerosis (ALS) patients is characterized by executive dysfunction with widely repeated deficits on tests of verbal (letter) fluency. However, conflicting evidence exists of an impairment on other word retrieval tasks, such as confrontation naming, which do not place heavy demands on executive processes. Previous research has demonstrated intact confrontation naming in the presence of verbal fluency deficits, although naming deficits have been described in other studies. In this investigation, functional MRI (fMRI) techniques were employed to explore whether word retrieval deficits and underlying cerebral abnormalities were specific to letter fluency, which are more likely to indicate executive dysfunction, or were also present in confrontation naming, indicating language dysfunction. Twenty-eight non-demented ALS patients were compared with 18 healthy controls. The two groups were matched for age, intelligence quotient, years of education, and anxiety and depression scores. Two compressed-sequence overt fMRI activation paradigms were employed, letter fluency and confrontation naming, which were developed for use with an older and potentially impaired population. In ALS patients relative to controls, the letter fluency fMRI task revealed significantly impaired activation in the middle and inferior frontal gyri and anterior cingulate gyrus, in addition to regions of the parietal and temporal lobes. The confrontation naming fMRI task also revealed impaired activation in less extensive prefrontal regions, including the inferior frontal gyrus and regions of the temporal, parietal and occipital lobes. These changes were present despite matched performance between patients and controls during each activation paradigm. The pattern of dysfunction corresponded to the presence of cognitive deficits on both letter fluency and confrontation naming in the ALS group. This study provides evidence of cerebral abnormalities in ALS in the network of regions involved in language and executive functions. Moreover, the findings further illustrate the heterogeneity of cognitive and cerebral change in ALS.
Aged↗
Pneumococcal sepsis due to functional hyposplenism in a bone marrow transplant patient.
Encapsulated bacteria can cause severe infections following bone marrow transplantation, usually in patients with chronic graft-versus-host disease (GVHD). Presented here is the case of an allogenic bone marrow transplantation recipient with chronic GVHD who developed overwhelming pneumococcal sepsis 3 years following transplantation. One year earlier the male patient had developed non-meningococcal, non-gonococcal neisseria infection. The infection recurred repeatedly despite monthly replacement immunoglobulin prophylaxis. These infections were attributed to functional hyposplenism after a prominent number of Howell-Jolly bodies was noticed in a peripheral blood smear during the patient's most recent admission. The case report is followed by a discussion of the policy of administering antibiotic prophylaxis to patients with chronic GVHD.
Adult↗
Toxic myopathy induced by the ingestion of loquat leaf extract.
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Adult↗
Assessment of patients with psychogenic non-epileptic seizures.
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Humans↗
Fructose-1,6-diphosphatase deficiency: a rare cause of prolonged prothrombin time.
A 20-year-old woman presented with severe life-threatening metabolic acidosis and hypoglycemia. In addition, her blood tests revealed elevated hepatic enzymes and a prolonged prothrombin time, with a reduction in factor VII activity. After treatment with a glucose and bicarbonate-containing intravenous infusion, there was a dramatic clinical improvement and normalization of the prothrombin time within 2 days. The patient was found to have fructose-1,6-diphosphatase deficiency, a rare metabolic disorder which has not been described previously as causing coagulation defects.
Acidosis↗
Subacute necrotizing fasciitis caused by gas-producing Staphylococcus aureus.
Presented here is a case of necrotizing fasciitis that developed bilaterally on the thighs of a 54-year-old diabetic woman following subcutaneous insulin injection. Severe localized pain was the presenting symptom; later, soft-tissue gas appeared. Incisional biopsy, performed on day 10 following admission, confirmed the diagnosis. Staphylococcus aureus was the only pathogen isolated. The disease had a slowly progressive course despite appropriate medical treatment, and recovery of the patient was achieved only after fasciotomy, drainage, and debridement of necrotic tissue was undertaken 4 weeks following admission. Staphylococcus aureus may cause subacute necrotizing fasciitis, and infection with this organism should be considered in cases of soft-tissue infection with gas formation in diabetics. The development of soft-tissue infection at the site of insulin injection should alert physicians to the possibility of infection with Staphylococcus aureus.
Anti-Bacterial Agents↗
Cognitive behaviour therapy with adults with intractable epilepsy and psychiatric co-morbidity: preliminary observations on changes in psychological state and seizure frequency.
Cognitive behaviour therapy (CBT) was undertaken with six adults with chronic, poorly controlled seizures and co-existing psychiatric and/or psychosocial difficulties. During 12 sessions of CBT from an experienced CBT Nurse Specialist, treatment focused concurrently on epilepsy-related problems, associated psychopathology and on the development of psychological strategies to reduce seizure occurrence. At the end of treatment participants rated their initial epilepsy-related problem as having less impact on their daily lives and at one-month follow-up reported less deleterious impact on everyday life in terms of their psychological difficulties. In addition participants demonstrated significant improvements in terms of their self-rated work and social adjustment, and in their decreased use of escape-avoidance coping strategies. These positive findings occurred despite the absence of a significant decrease in seizure frequency. Issues raised by the complexity and severity, both of these patients' psychological/psychosocial difficulties and their epilepsy, are discussed in relation to the optimal length of treatment that may be required when adopting a CBT model in this patient group.
Adaptation, Psychological↗
Health-related locus of control: does it change in motor neurone disease (MND)?
OBJECTIVES: Previous studies have attempted to describe locus of control beliefs in people with MND. This exploratory, longitudinal study set out to examine some of the possible correlations of health-related locus of control beliefs and the stability of these beliefs. METHOD: 32 people with Motor Neurone Disease completed the Multi-dimensional Health Locus of Control (MHLC) scale, initially on average 10.3 months after diagnosis, and again on average 16.4 months after diagnosis. Physical symptoms were assessed at both times. RESULTS: Initially there were no correlations between MHLC beliefs or disease duration and physical symptomatology, although longer disease duration was associated with greater beliefs in the role of powerful others in health control. At the second assessment, belief in the role of powerful others controlling health had increased, with this increase relating significantly to a worsening in physical symptoms. At this second assessment, neither duration of symptoms nor time since diagnosis correlated with MHLC beliefs. CONCLUSIONS: Whilst health locus of control beliefs do appear to change in MND, current findings suggest that this does not occur simply as a function of the passage of time. How symptoms change seems to be of particular importance when considering health locus of control beliefs in people with MND. Suggestions are made concerning other factors that might usefully be examined in future studies of this type.
Aged↗