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Maternal Autoantibodies in Autism
Braunschweig and Van de WaterArticle develop the position that some cases of autism spectrum disorder may be influenced, or even caused, by fetal-brain reactive maternal antibodies or other in utero immune-related exposures. This is an area of active investigation, and their article reviews the current literature in this area and proposes several directions for future research.
Revised Criteria for Mild Cognitive Impairment May Compromise the Diagnosis of Alzheimer Disease Dementia
MorrisArticle evaluates the potential impact of revised criteria for mild cognitive impairment (MCI), developed by a work group sponsored by the National Institute on Aging and the Alzheimer's Association, on the diagnosis of very mild and mild Alzheimer disease (AD) dementia. His view is that the categorical distinction between MCI and milder stages of AD dementia has been compromised by the revised criteria. The resulting diagnostic overlap supports the premise that “MCI due to AD” represents the earliest symptomatic stage of AD.
Amyloid-β–Associated Clinical Decline Occurs Only in the Presence of Elevated P-tau
Desikan and colleaguesArticle elucidate the relationship between the 2 hallmark proteins of Alzheimer disease (AD), amyloid-β (Aβ) and tau, and clinical decline over time among cognitively normal older individuals. Of considerable note, they find a significant relationship between decreased cerebrospinal fluid (CSF) Aβ1-42 levels and longitudinal change in global Clinical Dementia Rating (CDR), CDR–Sum of Boxes, and Alzheimer Disease Assessment Scale–cognitive subscale in individuals with elevated CSF p-tau181p levels. In the absence of CSF p-tau181p, the effect of CSF Aβ1-42 on longitudinal clinical decline was not significantly different from zero. Editorial perspective is provided by David M. Holtzman, MDArticle.
Evidence of Intrathecal Immunoglobulin Synthesis in Stroke: A Cohort Study
Prüss et alArticle determine the frequency of intrathecal immunoglobulin synthesis in a well-characterized cohort of patients who experienced “noninflammatory” acute stroke. Cerebrospinal fluid–specific immunoglobulin (IgG, IgM, and IgA) synthesis was significantly (P < .001) more frequent after stroke (24.8%) compared with the incidence in age- and sex-matched controls (2.5%). Furthermore, 31.3% of stroke patients demonstrated blood-brain barrier dysfunction and 18.1% displayed pleocytosis.
Novel Infantile-Onset Leukoencephalopathy With High Lactate Level and Slow Improvement
Steenweg and colleaguesArticle describe a novel pattern of magnetic resonance imaging (MRI) abnormalities as well as the associated clinical and laboratory findings. The MRIs of more than 3000 patients with an unclassified leukoencephalopathy were systematically reviewed. Clinical and laboratory data were retrospectively collected. They conclude that these patients represent a single novel leukoencephalopathy, probably caused by a mitochondrial defect.
Restricted Diffusion in Vanishing White Matter
Van der Lei et alArticle investigate the occurrence of restricted diffusion in vanishing white matter, the affected structures, the time of occurrence in the disease course, and the histopathologic correlate. In vanishing white matter, restricted diffusion can be found in relatively spared regions with high cellularity particularly in young patients with short disease duration.
Myopathy Associated With Antibodies to Signal Recognition Particle: Disease Progression and Neurological Outcome
Suzuki and colleaguesArticle characterize the clinical course of myopathy associated with antibodies to signal recognition particle (SRP), or anti-SRP myopathy. They report that a subset of patients with anti-SRP myopathy can show a chronic progressive form associated with severe clinical deficits.
Clinical Prediction of Fall Risk and White Matter Abnormalities: A Diffusion Tensor Imaging Study
Koo et alArticle test the hypothesis that elderly subjects at risk for falling, as determined by the Tinetti scale, have specific patterns of white matter (WM) abnormalities on diffusion tensor imaging. They report that elderly individuals at risk for falls as determined by the Tinetti scale have WM abnormalities in specific locations on diffusion tensor imaging, some of which correlate with cognitive function scores.
Multiple Sclerosis Lesions and Irreversible Brain Tissue Damage: A Comparative Ultrahigh-Field Strength Magnetic Resonance Imaging Study
Sinnecker and colleaguesArticle demonstrate the potential of ultrahigh-field 3-dimensional T1-weighted imaging using magnetization-prepared rapid acquisition and multiple gradient-echoes (MPRAGEs) to detect and characterize white and gray matter pathology in multiple sclerosis (MS). At ultrahigh-field strength, T1-weighted MPRAGE is highly sensitive in detecting MS plaques within the white and the gray brain parenchyma. Their results indicate structural damage beyond demyelination in every lesion depicted, which is in accordance with postmortem histopathological studies. The 7-T MPRAGE clearly delineated every cortical lesion that was visualized by any other magnetic resonance imaging sequence at 1.5 or 7 T.
Mutational Origin in Machado-Joseph Disease in the Australian Aboriginal Communities of Groote Eylandt and Yirrkala
Martins et alArticle determine whether the presence of Machado-Joseph disease (MJD, also spinocerebellar ataxia type 3) among Australian aborigines was caused by a new mutational event or by the introduction of expanded alleles from other populations. They find that a related-extended MJD haplotype shared by Australian aborigines and some Asian families (a Joseph-derived lineage) suggests a common ancestor for all, dating back more than 7000 years.
Anti–Myelin Oligodendrocyte Glycoprotein Antibodies in Pediatric Patients With Optic Neuritis
Rostasy and colleaguesArticle study the humoral immune response directed at myelin oligodendrocyte glycoprotein (MOG) in pediatric patients with isolated and recurrent optic neuritis (ON). They show that high-titer MOG-IgG antibodies are predominantly detected in pediatric patients with recurrent ON, indicating that anti–MOG-specific antibodies may exert a direct role in the pathogenesis of ON in this subgroup.
Myelin oligodendrocyte glycoprotein (MOG)–IgG antibody levels were significantly elevated in children with recurrent episodes of optic neuritis (ON) compared with children with an isolated episode or an ON as part of a clinically isolated syndrome (CIS). The dotted line indicates a titer of 1:160; high titers are considered to be those greater than 1:160. MS indicates multiple sclerosis.
Plasma Signaling Proteins in Persons at Genetic Risk for Alzheimer Disease: Influence of APOE Genotype
Ringman et alArticle study the effect of familial Alzheimer disease (FAD) mutations and APOE genotype on plasma signaling protein levels. They found different patterns of inflammatory markers in young and middle-aged persons among APOE genotype groups. The APOE ϵ4 carriers had the lowest levels of apolipoprotein E. Young ϵ4 carriers have increased inflammatory markers that diminish with age. They demonstrated altered inflammatory responses in young and middle adulthood in ϵ4 carriers that may relate to AD risk later in life.
This Month in Archives of Neurology. Arch Neurol. 2012;69(6):688–689. doi:10.1001/archneurol.2011.1459
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