Journal of the neurological sciences
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Cerebrovascular lesions in Alzheimer disease (AD) being significantly more frequent than in nondemented elderly subjects suggest overlaps and synergistic effects between both pathologies. Examination of a consecutive series of autopsy-proven AD cases and age-matched controls revealed a higher frequency of vascular lesions and of cerebral amyloid angiopathy (CAA) in AD (57.34% vs. 33.2% and 94.1% vs. 33.3%, respectively). These and previous data on vascular pathology in Parkinson disease emphasize its importance in these disorders. ⋯ The severity of CapCAA not correlating with general CAA showed high correlation with AD pathology, suggesting different pathogenesis of both types of CAA. Its elucidation may have implications for new therapeutic strategies. Considering the variability of vascular pathology in both AD and aged brains, the mechanisms behind their interactions are largely unknown, and further studies are needed to clarify their impact on cognitive impairment.
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Coronary artery bypass grafting (CABG) is the most common major surgical procedure performed worldwide. Neuropsychological deficits are frequent following CABG occurring in up to 80% in the early postoperative period, 20-50% at 6 weeks and 10-30% of patients at 6 months. Transcranial Doppler monitoring is well suited for monitoring the brain during surgery. ⋯ This method can also monitor cerebral hemodynamics during surgery and alert the surgical team when a fall in perfusion pressure occurs. Magnetic resonance imaging (MRI) studies have found evidence which suggests increased water content in the brain following "on-pump" CABG. New postoperative cerebral lesions have also been found in many patients using diffusion-weighted MRI.
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The phenotype and genotype of cerebral autosomal dominant arteriopathy and subcortical infarcts and leukoencephalopathy (CADASIL) in Caucasians have been well characterized, but CADASIL is less recognized in Asian populations. Here we investigated the first known Taiwanese family affected by CADASIL and identified an uncommon NOTCH3 mutation. The family had clinical manifestations in affected members including recurrent strokes, early dementia, and depression, but not migraine. ⋯ Head MRI showed multiple infarcts in bilateral basal ganglia, thalami, periventricular white matter, external capsules, and brainstem, but involvement of the anterior temporal pole was found only in two people with milder symptoms. To our knowledge, the Arg332Cys NOTCH3 mutation at exon 6, which was identified in the studied family, has not been reported in Asian populations. Our findings emphasize the importance of genetic analysis of NOTCH3 for Asians with a phenotype typical of CADASIL.
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Comparative Study
Primary angiitis of the central nervous system: report of five biopsy-confirmed cases from Colombia.
Primary (isolated) angiitis of the central nervous system (PACNS) is a rare cause of cerebrovascular disease (CVD), and few leptomeningeal and brain biopsy (LBB)-confirmed cases have been reported from South America. ⋯ We report five biopsy-proven cases of PACNS from Colombia associated with neurological and neuroimaging abnormalities; these patients presented a mild inflammatory disease that was correlated with few CSF abnormalities and good response to single steroid treatment without relapses. Leptomeningeal and brain biopsy is mandatory for a definitive diagnosis.
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Small fiber neuropathy (SFN) is a neuropathy selectively involving small diameter myelinated and unmyelinated nerve fibers. Interest in this disorder has considerably increased during the past few years. ⋯ Among others, these tests include assessment of epidermal nerve fiber density, temperature sensation tests for sensory fibers and sudomotor and cardiovagal testing (QSART) for autonomic fibers. Unless an underlying disease is identified, treatment is usually symptomatic and directed towards alleviation of neuropathic pain.