Handbook of clinical neurology
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Anorexia nervosa is a chronic and debilitating psychiatric disorder associated with one of the highest mortality rates of any psychiatric condition. Despite advances in neuroimaging, genetics, pharmacology, and psychosocial interventions in the last half-century, little progress has been made in altering the natural history of the condition or its outcomes. ⋯ Abnormal reward processing, compulsive hyperactivity, chronic anxiety, and depression, all suggest that anorexia nervosa shares much in common with other conditions, such as major depression and obsessive-compulsive disorder, for which surgical therapy with deep brain stimulation (DBS) has been tried, with promising results. As a result, the use of DBS in treatment-resistant anorexia nervosa should be evaluated in carefully designed, early-phase feasibility trials.
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Tourette syndrome is a neuropsychiatric disorder characterized by motor and vocal tics, often associated with behavioral disorders, with typical onset in early childhood. In most patients, the symptoms decrease spontaneously when adulthood is reached, or can be treated with behavioral therapy or medication. Only a small proportion of patients are candidates for surgical treatment. ⋯ Current knowledge of cortical-basal ganglia-thalamocortical circuits provides explanations for the beneficial effects of DBS on tics. Inclusion and exclusion criteria have been formulated to identify good candidates for DBS. Because of the small number of patients, there is a strong need for multicenter double-blind trials with standard protocols.
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Lyme neuroborreliosis (LNB) designates the nervous system disorders caused by the tick-borne spirochete Borrelia burgdorferi (Bb). The clinical syndromes are usually distinct and are classified as early and the rare late or chronic LNB. Early LNB occurs 3-6 weeks after infection most frequently as a lymphocytic meningoradiculoneuritis (LMR). ⋯ In the rare chronic or late LNB the pathology and thus the clinical presentation is primarily due to chronic meningitis and meningovascular CNS involvement, whereas the peripheral nervous system is not primarily affected. In early and late LNB the diagnosis is based on a characteristic clinical appearance and CSF inflammation with Bb-specific intrathecal antibody production. Both conditions, but not the ACA-associated neuropathy, respond to antibiotic therapy.
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Multiple system atrophy (MSA) is a sporadic and fatal α-synuclein-linked oligodendrogliopathy manifesting with progressive autonomic failure, poorly levodopa-responsive parkinsonism, and cerebellar ataxia, in any combination. Here we review key aspects of MSA integrating important insights from rapidly emerging fields such as genetics, diagnostic work-up including imaging, and translational therapies aimed at disease modification.
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Several countries have adopted laws that regulate physician assistance in dying. Such assistance may consist of providing a patient with a prescription of lethal medication that is self-administered by the patient, which is usually referred to as (physician) assistance in suicide, or of administering lethal medication to a patient, which is referred to as euthanasia. The main aim of regulating physician assistance in dying is to bring these practices into the open and to provide physicians with legal certainty. ⋯ Arguments against the legal regulation of physician assistance in dying include principled arguments, such as the wrongness of hastening death, and arguments that emphasize the negative consequences of allowing physician assistance in dying, such as a devaluation of the lives of older people, or people with chronic disease or disabilities. Opinion polls show that some form of accepting and regulating euthanasia and physician assistance in suicide is increasingly supported by the general population in most western countries. Studies in countries where physician assistance in dying is regulated suggest that practices have remained rather stable in most jurisdictions and that physicians adhere to the legal criteria in the vast majority of cases.