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Withdrawal and withholding of treatment - at the end of life
Abstracts / Journal of the Neurological Sciences 357 (2015) e457–e512
e499
(POTS), occasionally reflect a peripheral autonomic neuropathy or ganglionopathy.
pre-implantation genetic diagnosis and the prospects for gene therapy.
doi:10.1016/j.jns.2015.09.295
doi:10.1016/j.jns.2015.09.297
1722 WFN15-1842 Autonomic Nervous System T 1.1 Assessment and treatment of male and female sexual dysfunction
1724 WFN15-1779 Ethics T 10.1 Withdrawal and withholding of treatment - at the end of life
M. Hilz. Department of Neurology, University of Erlangen-Nuremberg, Erlangen, Germany
D. Oliver. Tizard Centre, University of Kent, Canterbury, United Kingdom
In the USA, 43% of women and 31% of men report sexual dysfunction. Assessment of sexual dysfunction should be patientspecific, including a detailed history, physical, genital and neurologic examination, neurophysiologic testing, such as genital quantitative sensory testing, endocrine and psychological work-ups. Treatment of female hypoactive sexual desire disorder is mainly non-pharmacologic. For sexual arousal disorder, various treatment options have been tested, for example transdermal testosterone substitution which is not approved in the USA. Treatment of orgasmic disorders may require a combination of therapies. Patients with sexual pain require specific treatment depending on the particular cause. Male erectile dysfunction (ED) probably affects 20 to 30 million men in the USA. Diagnosis may include nocturnal tumescence testing or Doppler sonographic measurements of penile perfusion. PDE5 inhibitors are one of the pharmacologic treatment options in ED patients. However, in patients with reduced nitric oxide production, such as diabetics, PDE5 inhibitors may fail. Patients with high cardiovascular risk should defer sexual activity. PDE5 inhibitors are contraindicated in these patients and in patients on nitrates. Premature ejaculation (PE) is the most common male sexual dysfunction affecting up to 21% of men between 18 and 59 years of age in the USA. Patients with PE may benefit from behavioral, cognitive therapy, “squeeze techniques”, serotonin reuptake inhibitors (SSRIs), and topical anesthetics. In several countries, the shortacting Dapoxetine is approved while most drugs are not officially approved for PE therapy. SSRI treatment should be used with caution due to the risk of a life-threatening serotonin syndrome. doi:10.1016/j.jns.2015.09.296
1723 WFN15-1932 Ethics T 10.1 Huntington's disease and ALS - the ethical challenges for families and professionals facing genetic disease C. Shaw. Department of Basic and Clinical Neuroscience, Institute of Psychiatry Psychology and Neuroscience King's College London and King's Health Partners, London, United Kingdom Huntington’s disease (HD) and amyotrophic lateral sclerosis (ALS) are both relentlessly progressive neurodegenerative disorders for which diagnostic and predictive gene testing have been available for ~20 years. HD is a single gene autosomal dominant disorder whereas ALS is highly heterogeneous and complex. ALS is familial in ~ 10% of cases and currently 13 + genes account for 60% of familial cases but the same genes are also present in 10% of sporadic cases due in large part to incomplete penetrance. Significant differences in the rate and effects of symptomatic progression have influenced the uptake of predictive gene testing. This talk will discuss the major issues that face affected individuals and families with HD and ALS and our current practice for diagnostic and predictive gene testing,
As a patient with a progressive neurological disease deteriorates there may be an increasing need to consider new options for the management of symptoms, including the consideration of withdrawing existing treatment options or withholding treatments. Ethically the withdrawal or withholding of treatment are similar – as the goals of any medical treatment should be to benefit the patient by restoring or maintaining health, maximising benefit and minimising harm. If a treatment is felt not to provide net benefit to the patient it may be, legally and ethically, withheld or withdrawn, with the goals of care shifting to the palliation of symptoms. These issues can be seen when there is consideration of the withdrawal of non-invasive ventilation (NIV) in a patient with amyotrophic lateral sclerosis. There may be consideration of a tracheostomy, to maintain ventilation, although this may often seem to be too burdensome. However as the disease progresses and the patient continues to deteriorate they may ask for the NIV to be withdrawn. This is ethically acceptable, at the clear request of the patient or in the presence of a clear advance directive. There is a need to ensure that symptoms are adequately treated and distress avoided and there may be complex discussion within the team with the patient and family. The complex discussion and involvement at these times is a crucial part of the palliative care of a person with a progressive neurological disease, ensuring that all aspects of care – physical, psychological, social and spiritual – are considered and supported. doi:10.1016/j.jns.2015.09.298
1725 WFN15-1868 Neuro-Otology T 12.1 Diagnosis of acute vertigo A. Bronstein. Clinical Neuro-otology, Imperial College London/ Charing Cross Hospital, London, United Kingdom Acute vertigo and dizziness is common in emergency departments. It creates confusion amongst emergency doctors as a very similar presentation can be due to a very benign self-limiting condition (e.g. vestibular neuritits, BPPV, migraine) or to a life threating disease (e.g. posterior fossa stroke). The majority of cases are due to benign peripheral vestibular disorders or migraine that can be easily treated so in this lecture we will emphasise the ‘red flags’ that, in the presence of acute vertigo, suggest structural, central disease and warrant acute MRI. These red flags can be summarised as follows (Seemungal and Bronstein, in Practical Neurology): 1) acute unilateral deafness (may be Meniere’s disease but suspect AICA stroke), 2) acute occipital headache (may be migraine but suspect posterior fossa stroke), 3) any central neurological symptoms or signs (ask for them, look for them) and 4) a negative (i.e. normal) head-impulse test. doi: 10.1016/j.jns.2015.09.299
e499
(POTS), occasionally reflect a peripheral autonomic neuropathy or ganglionopathy.
pre-implantation genetic diagnosis and the prospects for gene therapy.
doi:10.1016/j.jns.2015.09.295
doi:10.1016/j.jns.2015.09.297
1722 WFN15-1842 Autonomic Nervous System T 1.1 Assessment and treatment of male and female sexual dysfunction
1724 WFN15-1779 Ethics T 10.1 Withdrawal and withholding of treatment - at the end of life
M. Hilz. Department of Neurology, University of Erlangen-Nuremberg, Erlangen, Germany
D. Oliver. Tizard Centre, University of Kent, Canterbury, United Kingdom
In the USA, 43% of women and 31% of men report sexual dysfunction. Assessment of sexual dysfunction should be patientspecific, including a detailed history, physical, genital and neurologic examination, neurophysiologic testing, such as genital quantitative sensory testing, endocrine and psychological work-ups. Treatment of female hypoactive sexual desire disorder is mainly non-pharmacologic. For sexual arousal disorder, various treatment options have been tested, for example transdermal testosterone substitution which is not approved in the USA. Treatment of orgasmic disorders may require a combination of therapies. Patients with sexual pain require specific treatment depending on the particular cause. Male erectile dysfunction (ED) probably affects 20 to 30 million men in the USA. Diagnosis may include nocturnal tumescence testing or Doppler sonographic measurements of penile perfusion. PDE5 inhibitors are one of the pharmacologic treatment options in ED patients. However, in patients with reduced nitric oxide production, such as diabetics, PDE5 inhibitors may fail. Patients with high cardiovascular risk should defer sexual activity. PDE5 inhibitors are contraindicated in these patients and in patients on nitrates. Premature ejaculation (PE) is the most common male sexual dysfunction affecting up to 21% of men between 18 and 59 years of age in the USA. Patients with PE may benefit from behavioral, cognitive therapy, “squeeze techniques”, serotonin reuptake inhibitors (SSRIs), and topical anesthetics. In several countries, the shortacting Dapoxetine is approved while most drugs are not officially approved for PE therapy. SSRI treatment should be used with caution due to the risk of a life-threatening serotonin syndrome. doi:10.1016/j.jns.2015.09.296
1723 WFN15-1932 Ethics T 10.1 Huntington's disease and ALS - the ethical challenges for families and professionals facing genetic disease C. Shaw. Department of Basic and Clinical Neuroscience, Institute of Psychiatry Psychology and Neuroscience King's College London and King's Health Partners, London, United Kingdom Huntington’s disease (HD) and amyotrophic lateral sclerosis (ALS) are both relentlessly progressive neurodegenerative disorders for which diagnostic and predictive gene testing have been available for ~20 years. HD is a single gene autosomal dominant disorder whereas ALS is highly heterogeneous and complex. ALS is familial in ~ 10% of cases and currently 13 + genes account for 60% of familial cases but the same genes are also present in 10% of sporadic cases due in large part to incomplete penetrance. Significant differences in the rate and effects of symptomatic progression have influenced the uptake of predictive gene testing. This talk will discuss the major issues that face affected individuals and families with HD and ALS and our current practice for diagnostic and predictive gene testing,
As a patient with a progressive neurological disease deteriorates there may be an increasing need to consider new options for the management of symptoms, including the consideration of withdrawing existing treatment options or withholding treatments. Ethically the withdrawal or withholding of treatment are similar – as the goals of any medical treatment should be to benefit the patient by restoring or maintaining health, maximising benefit and minimising harm. If a treatment is felt not to provide net benefit to the patient it may be, legally and ethically, withheld or withdrawn, with the goals of care shifting to the palliation of symptoms. These issues can be seen when there is consideration of the withdrawal of non-invasive ventilation (NIV) in a patient with amyotrophic lateral sclerosis. There may be consideration of a tracheostomy, to maintain ventilation, although this may often seem to be too burdensome. However as the disease progresses and the patient continues to deteriorate they may ask for the NIV to be withdrawn. This is ethically acceptable, at the clear request of the patient or in the presence of a clear advance directive. There is a need to ensure that symptoms are adequately treated and distress avoided and there may be complex discussion within the team with the patient and family. The complex discussion and involvement at these times is a crucial part of the palliative care of a person with a progressive neurological disease, ensuring that all aspects of care – physical, psychological, social and spiritual – are considered and supported. doi:10.1016/j.jns.2015.09.298
1725 WFN15-1868 Neuro-Otology T 12.1 Diagnosis of acute vertigo A. Bronstein. Clinical Neuro-otology, Imperial College London/ Charing Cross Hospital, London, United Kingdom Acute vertigo and dizziness is common in emergency departments. It creates confusion amongst emergency doctors as a very similar presentation can be due to a very benign self-limiting condition (e.g. vestibular neuritits, BPPV, migraine) or to a life threating disease (e.g. posterior fossa stroke). The majority of cases are due to benign peripheral vestibular disorders or migraine that can be easily treated so in this lecture we will emphasise the ‘red flags’ that, in the presence of acute vertigo, suggest structural, central disease and warrant acute MRI. These red flags can be summarised as follows (Seemungal and Bronstein, in Practical Neurology): 1) acute unilateral deafness (may be Meniere’s disease but suspect AICA stroke), 2) acute occipital headache (may be migraine but suspect posterior fossa stroke), 3) any central neurological symptoms or signs (ask for them, look for them) and 4) a negative (i.e. normal) head-impulse test. doi: 10.1016/j.jns.2015.09.299