Lancet neurology
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Multicenter Study
Natural history of multiple system atrophy in the USA: a prospective cohort study.
Multiple system atrophy is a rare, fatal neurodegenerative disorder with symptoms of autonomic failure plus parkinsonism, cerebellar ataxia, or both. We report results of the first prospective natural history study of multiple system atrophy in the USA, and the effects of phenotype and autonomic failure on prognosis. ⋯ US National Institutes of Health, Mayo Clinic, and Kathy Shih Memorial Foundation.
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High-frequency deep brain stimulation (DBS) with a single electrical source is effective for motor symptom relief in patients with Parkinson's disease. We postulated that a multiple-source, constant-current device that permits well defined distribution of current would lead to motor improvement in patients with Parkinson's disease. ⋯ Boston Scientific.
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Sleep-wake disturbances are extremely common after a traumatic brain injury (TBI). The most common disturbances are insomnia (difficulties falling or staying asleep), increased sleep need, and excessive daytime sleepiness that can be due to the TBI or other sleep disorders associated with TBI, such as sleep-related breathing disorder or post-traumatic hypersomnia. ⋯ Sleep-wake disturbances associated with TBI warrant treatment. Although evidence specific to patients with TBI is still scarce, cognitive-behavioural therapy and medication could prove helpful to alleviate sleep-wake disturbances in patients with a TBI.
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Tuberous sclerosis (also known as tuberous sclerosis complex [TSC]) is a multisystem genetic disorder that affects almost every organ in the body. Mutations in the TSC1 or TSC2 genes lead to disruption of the TSC1-TSC2 intracellular protein complex, causing overactivation of the mammalian target of rapamycin (mTOR) protein complex. The surveillance and management guidelines and clinical criteria for tuberous sclerosis were revised in 2012, and mTOR inhibitors are now recommended as treatment options for subependymal giant cell astrocytomas and renal angiomyolipomas-two common features of the disease. ⋯ Clinical trials of mTOR inhibitors to treat seizures and TAND are underway. Management of the neurological and neuropsychiatric manifestations of the disorder should be coordinated with treatment of other organ systems. In view of the age-related expression of manifestations from infancy to adulthood, continuity of clinical care and ongoing monitoring is paramount, and particular attention is needed to plan transition of patient care from childhood to adult services.
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For several years, the only therapy with proven efficacy for acute ischaemic stroke was alteplase, which is approved for use within 4·5 h after stroke onset in many countries, but only within 3 h in the USA. However, the recanalisation rate with alteplase is modest. Several trials have shown substantial clinical benefit of neurothrombectomy within 6 h of ischaemic stroke onset, which has initiated a new era of acute stroke therapy. ⋯ Continuing clinical trials with several types of advanced MRI and CT imaging to enhance patient selection are investigating alteplase, other thrombolytic drugs, and novel endovascular devices, for use in later time periods from stroke onset. Consequently, the organisation and implementation of future clinical trials will need to adapt to what has been learned from the present generation of trials. The delivery of care to patients with acute stroke will also need to incorporate newly proven therapies, and much additional work is needed to maximise outcomes in as many patients as possible.