Current neurology and neuroscience reports
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Curr Neurol Neurosci Rep · Aug 2013
ReviewParoxysmal sympathetic hyperactivity after acute brain injury.
Paroxysmal sympathetic hyperactivity is a syndrome associated with brain trauma, stroke, encephalitis, and other forms of brain injury. It is characterized by uncontrolled episodes of unbalanced sympathetic surges causing hyperthermia, diaphoresis, tachycardia, hypertension, tachypnea, and dystonic posturing. Patients who develop paroxysmal sympathetic hyperactivity have worse neurologic outcomes, longer hospital stays, and more complications. ⋯ Treatments should focus on decreasing the frequency and intensity of episodes with regularly scheduled doses of medications, such as long-acting benzodiazepines, nonselective β-blockers, α2-agonists, morphine, baclofen, and gabapentin, usually in combination. Treatment of acute breakthrough episodes should focus on doses of as-needed morphine and short-acting benzodiazepines. A balance between control of symptoms without oversedation is the goal.
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Curr Neurol Neurosci Rep · Aug 2013
ReviewThe association between ß-glucocerebrosidase mutations and parkinsonism.
Mutations in the ß-glucocerebrosidase gene (GBA), which encodes the lysosomal enzyme ß-glucocerebrosidase, have traditionally been implicated in Gaucher disease, an autosomal recessive lysosomal storage disorder. Yet the past two decades have yielded an explosion of epidemiological and basic-science evidence linking mutations in GBA with the development of Parkinson disease (PD) as well. ⋯ Furthermore, multiple lines of evidence suggest that although GBA-associated PD closely mimics idiopathic PD (IPD), it may present at a younger age, and is more frequently complicated by cognitive dysfunction. Understanding the clinical association between GBA and PD, and the relationship between ß-glucocerebrosidase and α-synuclein, may enhance understanding of the pathogenesis of IPD, improve prognostication and treatment of GBA carriers with parkinsonism, and furthermore inform therapies for IPD not due to GBA mutations.
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Palliative care for Parkinson disease (PD) is a new concept in neurodegenerative care. Abundant evidence exists supporting PD as increasing risk of death, most commonly from aspiration pneumonia despite improvements in motor and non-motor symptom management. ⋯ Evaluation using the Edmonton Symptom Assessment Scale-PD can help practitioners identify symptoms requiring intervention and track their response to interventions. Adopting palliative care principles will allow neurologists to fulfill the needs of PD patients in advanced stages to the end of life.