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Ladogana et al. analyzed the available data on 4,441 cases of CJD provided from an international study. The overall annual mortality rate for sporadic CJD was 1.39 per million, with similar figures in all participating countries. Mortality rates differed for genetic, iatrogenic, and variant CJD.
see page 1586
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PRNP analysis is crucial for genetic transmissible spongiform encephalopathies diagnosis
Ladogana et al. sequenced the PRNP gene in 643 cases with suspected human transmissible spongiform encephalopathies (TSE) and found that 130 cases carried pathogenic mutations. About 60% of them had no relatives with TSE and had clinical and laboratory findings comparable to sporadic CreutzfeldtJakob disease. Genetic TSE patients would have been misclassified without PRNP gene analysis.
see page 1592
Cholesterol and dementia
Dufouil et al. evaluated the association of cholesterol levels, lipid-lowering agent (LLA) intake, and APOE genotype with dementia prevalence in a population-based study of 9,294 subjects. LLA intake was associated with decreased risk of dementia whereas hyperlipidemia was associated with increased odds for non-AD type dementia.
see page 1531
Aß immunization in patients with Alzheimer disease
Gilman et al. studied Aß immunization in 372 patients with AD. Although the trial was interrupted because of meningoencephalitis in 6% of immunized patients, cognitive performance was better and CSF tau levels were lower in antibody responders than placebo cases, suggesting a downstream neuropathologic benefit of targeting Aß.
see page 1553
The accompanying article by Fox et al. reports on cerebral volume changes in antibody responders from the same study. Responders had greater brain volume loss but better cognitive performance compared to placebo cases. The volume loss in responders may be due in part to amyloid removal.
see page 1563
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Migraine with aura and ischemic stroke
Stang et al. studied the association of migraine with ischemic stroke in 12,750 middle-aged adults. Those with a history of migraine accompanied by aura had a higher occurrence of ischemic stroke. In contrast, there was little association between migraine without aura and ischemic stroke.
see page 1573
The editorial by Diener and Kurth concludes that there is good evidence that migraine with aura is associated with an increased risk of ischemic stroke, which appears to be stronger among the young but may persist in the elderly. Prospective data do not support an association between migraine without aura and ischemic stroke. Strict definitions of migraine and stroke as well as a prospective assessment of the migrainestroke association are essential.
see page 1496
The ability of persons with AD to make a decision about using treatment for AD
Karlawish et al. studied the ability of persons with mild to moderate stage Alzheimer disease (AD) to understand and appreciate the risks and benefits involved in the decision to take an AD-slowing medication. They found that impairments in these abilities as well as in the process of reasoning that flows from them was significant among persons with moderate stage dementia and those who lacked awareness of their diagnosis, symptoms, or prognosis. Such persons were more likely to be judged not competent.
see page 1514
Who can say yes (or no) to a physician and how does the physician know they can?
The accompanying editorial by McQuillen and Tariot notes that an essential message from this study is one of sensitivity, respect, and humility: sensitivity to the dimensions of a multifaceted illness such as dementia; respect for persons, even when demented; and humility in recognizing the degree to which a bond of trust influences decision-making. The authors show us that the mere presence of a dementia diagnosis does not preclude the patient from participating in decisions about his or her own care.
see page 1494
Use of tacrolimus in myasthenia gravis
Ponseti et al. present the long-term results of tacrolimus in 79 cyclosporine- and prednisone-dependent patients with myasthenia gravis. Ninety-two percent of patients had received tacrolimus for more than 3 years. Tacrolimus was well-tolerated and did not have major adverse effects related to long-term treatment. All but two patients were withdrawn from prednisone.
see page 1641
Ventriculoperitoneal shunt for leptomeningeal metastasis
Omuro et al. reviewed patients with leptomeningeal metastasis and found they frequently developed intracranial hypertension and often had hydrocephalus. Shunting rapidly relieved symptoms of intracranial hypertension; median survival was only 2 months after shunt but some of the patients survived for years.
see page 1625
Orthotic device effective in primary writing tremor
A writing device significantly benefited drawing and writing in patients with primary writing tremor. Espay et al. suggest applying this simple, noninvasive intervention before pharmacotherapy or functional surgery in task-specific tremor.
see page 1648
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