Clinical highlights.
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Amyotrophic Lateral Sclerosis↗
Biomedical subjects
Gian Domenico Borasio
Publications and source records attributed to Gian Domenico Borasio.
Carbamazepine-responsive paroxysmal nausea and vomiting in a patient with meningeal carcinomatosis.
In neurology, paroxysmal syndromes are well-known, eg, as manifestations of multiple sclerosis. We report a patient with meningeal carcinomatosis, who presented with therapy-refractory nausea and vomiting. The clinical suspicion of a paroxysmal syndrome prompted a trial of carbamazepine, which resulted in complete cessation of the symptoms. In cancer patients with central nervous system (CNS) involvement and therapy-refractory symptoms with sudden onset, carbamazepine treatment should be considered.
Adult↗
Validation and clinical application of the german version of the palliative care outcome scale.
The Palliative Care Outcome Scale (POS) is a multidimensional instrument covering physical, psychosocial, spiritual, organizational, and practical concerns. This study validated the German version of the POS and used the tool in different palliative care settings in Germany and Austria. Patients and staff were asked to complete the POS three times and evaluate the questionnaire and the translation afterwards. One hundred eighteen patients (44 male, 74 female, mean age 63 years, all suffering from advanced cancer) completed the POS one time, 55 patients two times, and 36 patients three times. Spearman's rho was highly significant for pain, other symptoms, anxiety, and life worthwhile in the first two assessments. The third assessment showed significant correlations for pain, other symptoms, anxiety, and family anxiety. Seventy-seven of 87 patients answered questions regarding the scale and the translation. All questions other than "Over the past 3 days, have you felt good about yourself?" were understandable for patients. Almost half of the staff was undecided whether the tool was reflecting the patients' condition. In contrast, the majority of patients liked it. Thus, the German version of the POS is well accepted by patients and staff and appears to be valid, although there are some areas where the scale would benefit from expansion to more closely capture staff and patient concerns.
Austria↗
Personal values and individual quality of life in palliative care patients.
To evaluate the relationship between personal values and individual quality of life (iQoL) in palliative care patients, 75 patients with advanced cancer or amyotrophic lateral sclerosis (ALS) were asked to complete a self-report questionnaire concerning personal values and a semi-structured interview on their iQoL. Sixty-four patients took part in the study (56% cancer, 44% ALS). The most important personal values were benevolence, self-direction, and universalism, whereas power, achievement, and stimulation were the least important. Self-transcendence values were higher than self-enhancement values in all patients. Compared with healthy adults, palliative care patients scored significantly higher in benevolence and lower in self-enhancement values. Conservation values (security, conformity, tradition) were correlated with higher levels of iQoL (P=0.03). There were no significant differences between ALS and cancer patients. These data suggest that conservation values protect the patients' iQoL in the palliative care situation. The observed shift towards self-transcendence values may be related to coping processes of terminally ill patients. The relationship between self-transcendence values and iQoL should be further investigated.
Adolescent↗
Promoting excellence in end-of-life care in ALS.
The type and quality of end-of-life care varies greatly in ALS; the time to initiate end-of-life care is not defined, and decision making is hampered by logistical and financial barriers. There has been no systematic review of these issues in ALS. The goals of this initiative are to: 1) improve end-of-life care for patients with ALS and families based on what limited evidence is available; 2) increase awareness, interest, and debate on the end-of-life care in ALS; and 3) identify areas needed for new prospective clinical research. The ALS Peer Workgroup reviewed the literature and 1) identified the current state of knowledge, 2) analysed the gaps in care, and 3) provided recommendations for standard of care and future research. It was shown that areas of investigation are needed on the incorporation of an interdisciplinary approach to care in ALS that includes: psychosocial evaluation and spiritual care; the use of validated instruments to assess patient and caregiver quality of life; and the establishment of proactive caregiver programs. Several public policy changes that will improve coverage for medical care, hospice, and caregiver costs are also reviewed. More clinical evidence is needed on how to provide optimal end-of-life care specifically in ALS.
Advance Care Planning↗
Effects of spiritual care training for palliative care professionals.
Little is known about the effects of spiritual care training for professionals in palliative medicine. We therefore investigated prospectively the effects of such training over a six-month period. All 63 participants of the three and a half-day training were asked to fill out three questionnaires: before and after the training, as well as six months later. The questionnaires included demographic data, numeric rating scales about general attitudes towards the work in palliative care, the Self-Transcendence Scale (STS), the spiritual subscale of the Functional Assessment of Chronic Illness Therapy (FACIT-Sp) and the Idler Index of Religiosity (IIR). Forty-eight participants (76%) completed all three questionnaires (91% women, median age 49 years; 51% nurses, 16% hospice volunteers, 14% physicians). Significant and sustained improvements were found in self-perceived compassion for the dying (after the training: P=0.002; 6 months later: P=0.025), compassion for oneself (P <0.001; P =0.013), attitude towards one's family (P =0.001; P =0.031), satisfaction with work (P < 0.001; P = 0.039), reduction in work-related stress (P < 0.001; P = 0.033), and attitude towards colleagues (P = 0.039; P = 0.040), as well as in the FACIT-Sp (P < 0.001; P = 0.040). Our results suggest that the spiritual care training had a positive influence on the spiritual well-being and the attitudes of the participating palliative care professionals which was preserved over a six-month period.
Adult↗
Palliative care in neurology.
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Attitude of Health Personnel↗
Differential expression of mGluR5 in human lumbosacral motoneurons.
Glutamatergic excitotoxicity is one of the main hypotheses to explain motoneuronal degeneration in amyotrophic lateral sclerosis (ALS). Interestingly, autonomic motoneurons remain almost unaffected, even in late stages of the disease. Since glutamate receptors may mediate neurotoxic as well as neuroprotective effects, different expression patterns may contribute to neuronal vulnerability. We and others have previously described a significantly higher expression of group I metabotropic glutamate receptors (mGluRs) in rat autonomic motoneurons compared to somatic motoneurons. Here we show a selective expression of the group I receptor mGluR5 in human parasympathetic Onuf's nucleus. These results are in accordance with previous findings in rat and strengthen the hypothesis that mGluR expression may provide a possible clue to the selective vulnerability in ALS.
Amyotrophic Lateral Sclerosis↗
Sexuality in patients with amyotrophic lateral sclerosis and their partners.
Sexuality in patients with amyotrophic lateral sclerosis (ALS) has received little attention so far. Although sexual function is not directly affected by the disease process, several patients have reported problems within their sexual relationship. We performed a questionnaire survey to ascertain the extent and clinical relevance of sexual problems experienced by patients with ALS and their partners. Of 91 patients and partners asked, 62 agreed to participate in the study. Compared with the time before disease onset, sexual interest had decreased from 72% to 44% for patients and from 78% to 44% for partners. Sexual activity had moderately decreased from 94% to 76% for the patients and from 100% to 79% for the partners. Before the disease, 19% of the patients and 20% of the partners reported sexual problems. This increased to 62% of the patients and 75% of the partners at time of survey. The problems reported were mainly decreased libido, passivity of the partner and own passivity. The most frequent reasons for these problems were the physical weakness and the body image changes due to ALS. The data show that sexuality is an important and problematic issue for a large proportion of ALS patients and their partners. This topic is rarely discussed in the medical setting. Counselling and information should be made available in order to better address this important aspect of quality of life.
Aged↗
Breaking the news in amyotrophic lateral sclerosis.
Diagnosis communication is a mainstay of the patient-physician relationship and in ALS represents the beginning of the treatment. In Europe, the diagnosis is now communicated to most patients, but with some differences from north to south. Communication is the basis of the patient's autonomy. When adequately informed, patients are able to consider their preferences about life-extending interventions. However, this discussion is sometimes entertained too late. Both the style and the context of this discussion have profound impact on patient satisfaction. The content of the communication should include all relevant information about the disease and should be performed honestly while preserving hope. Collusion with relatives can create difficulties in the relationship with a patient and isolate them from their family. Diagnostic communication is an ongoing process and should form part of every follow-up visit. Communication with the dying patient is often reinforced by non-verbal messages, acknowledgements of patients' emotions, and by listening. Written material may increase satisfaction of patients and their relatives, but should never substitute for face-to-face discussion.
Amyotrophic Lateral Sclerosis↗
Individual quality of life is not correlated with health-related quality of life or physical function in patients with amyotrophic lateral sclerosis.
We compared the change over time of individual quality of life (QOL) versus health-related QOL (HRQOL) and functional status in palliative care patients with amyotrophic lateral sclerosis (ALS). Forty-two patients with ALS performed the following assessments: the ALS functional rating scale (ALSFRS), the Sickness Impact Profile (SIP), the Short Form 36 (SF-36), and the Schedule for the Evaluation of Individual QOL-Direct Weighting (SEIQOL-DW). The SF-36 and the SEIQOL-DW were assigned at random. Patients were examined at least three times at 2-month intervals. There was a significant decrease from visit 1 to 3 (4-month period) in the function-based values of the SIP, SF-36, and ALSFRS. Despite this progressive decline of physical function and HRQOL, individual QOL as assessed by the SEIQOL-DW remained stable throughout the observation period. Correspondingly, there was a clear correlation between the ALSFRS, the SIP, and the SF-36, but no correlation between these scales and the SEIQOL-DW. The QOL domains most often named in the SEIQOL-DW were family, friends/social life, health, and profession. Thus, individual QOL appears to be largely independent from physical function in severely ill patients with ALS. Because of the characteristics of the scale, assessment of individual QOL may have an interventional value in palliative care.
Activities of Daily Living↗
Glial proliferation and metabotropic glutamate receptor expression in amyotrophic lateral sclerosis.
Accumulating evidence indicates that alterations in glial activation and disturbances in glial glutamate metabolism may contribute to the pathogenesis of amyotrophic lateral sclerosis (ALS). Metabotropic glutamate receptors (mGluRs) are involved in glutamate homeostasis as well as in glial proliferation. Using in situ hybridization and immunohistochemistry we found a strong upregulation of group I and group II mGluR mRNA and protein in ALS spinal cord as compared to controls (mGluR5 > mGluR1 > mGluR2/3). In vitro, the mGluR group I agonist 3,5-dihydroxyphenylglycine induced proliferation in chick spinal cord astroglial cultures. Moreover, addition of cerebrospinal fluid (CSF) from ALS patients resulted in significantly higher proliferation rates than control CSF. In both cases, the effect could be blocked by addition of the mGluR group I antagonist 1-aminoindan-1,5-dicarboxylic acid. Taken together, our data suggest that stimulation of glial mGluRs through mediators present in the CSF may contribute to glial proliferation and astrogliosis in ALS.
Aged↗
Quality of life and psychosocial issues in ventilated patients with amyotrophic lateral sclerosis and their caregivers.
Non-invasive ventilation (NIV) is an efficient palliative measure for symptoms of chronic hypoventilation in patients with amyotrophic lateral sclerosis (ALS), and can also lengthen survival. A subset of ALS patients undergoes tracheostomy ventilation (TV) for life prolongation. We investigated the quality of life (QOL) and psychosocial situation of 52 home ventilated ALS patients and their caregivers. The battery included sociodemographic, generic, and disease-specific variables, as well as the Profile of Mood States and the Munich Quality of Life Dimensions List. Data were compared between the NIV (n=32) and the TV (n=21) groups. Mean ventilation time was 14 months for NIV and 35 months for TV. Eighty-one percent of TV patients had been tracheotomized without informed consent. The data show a good overall QOL for both NIV and TV patients, but a very high burden of care for TV caregivers, 30% of whom rated their own QOL lower than their patient's QOL. Sexuality was an important issue. Thus, any assessment of QOL in a home palliative care situation should include the primary caregivers.
Aged↗
Riluzole treatment in advanced ALS.
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Amyotrophic Lateral Sclerosis↗
Measures of quality of life: con.
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Amyotrophic Lateral Sclerosis↗