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Journal of Neuropathology & Experimental Neurology:
doi: 10.1097/NEN.0000000000000053
In This Issue

In This Issue

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Immune To Memory - Or Not?

In this review the author advances the interesting hypothesis that an impaired blood-brain barrier in Alzheimer disease allows the immune system to attack neurons involved in memory. He further theorizes that these neurons are targeted because they synthesize proteins via epigenetic modifications that are unique to memory neurons.

see page 192

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Inflammatory Pathogenesis Implicated in Focal Cortical Dysplasia (FCD)

Upregulation of the inflammatory cytokine IL-2 and its receptor was identified in the cortex of patients with intractable seizures and was closely correlated with abnormal neurons in FCD type II. Activation of downstream factors further supports a role for this signaling pathway in the pathogenesis of FCD type II.

see page 206

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It All Begins with the Cytoskeleton

The INF2 gene, which encodes a protein regulating actin polymerization, is mutated in patients with renal dysfunction and a dominant intermediate form of Charcot-Marie-Tooth disease (CMTDIE). Mathis et al report that CMTDIE nerve biopsies show both axon loss and Schwann cell pathology characterized by atypical whorl-like proliferations and β-actin accumulation. These findings suggest that INF2 mutation induces a neuropathy by disrupting the Schwann cell actin cytoskeleton, which in turn alters Schwann cell polarization and myelin gene expression.

see page 223

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Treating Niemann-Pick Disease Type C

Loss of cholinergic neurons is common in neurodegenerative processes Niemann-Pick disease type C (NPC). Accordingly, acetylcholinesterase inhibitors (ACEI) hold the potential for therapy. Using a NPC mouse model, Seo et al find efficacy with one ACEI, donepezil.

see page 234

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Silent Neurodegenerative Pathology in the Elderly is More Common than Expected

In a community-based autopsy series of more than 100 aged individuals who were not affected neurologically in their last 5 years, a clinicopathological diagnosis was made in 25% of cases, while 75% of the remaining cases which were not neurologically affected at the time of death displayed variable neurodegenerative pathology, notably severe Alzheimer changes, incidental α-synucleinopathy, and progressive supranuclear palsy.

see page 244

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Arx Marks the Spot

Sunnen et al investigated the effects of loss of the aristaless-related homeobox (ARX) gene, which in humans results in a spectrum of structural and functional nervous system disorders including lissencephaly, movement disorders, intellectual disabilities, and epilepsy as well as hypothalamic dysfunction. Mice null for Arx showed abnormal formation and loss of tyrosine hydroxylase, a dopaminergic marker, of thalamic reticular nucleus (TRN) and zona incerta (ZI), while retaining expression in other thalamic nuclei and in the hypothalamus. The findings suggest that ARX deficiency may contribute to the hypothalamic dysfunction observed in some patients.

see page 253

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Sharpening CLIPPERS

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) was first described in 2010. It has since been the subject of a surprisingly large number of clinical and neuroimaging reports and a few neuropathological studies. Kleinschmidt-DeMasters and West report the pathology of a recent case of a biopsy of the frontal lobe and review the current literature. Their report further documents extrapontine involvement and emphasizes the finding of multifocal small vessel hyalinization as a chronic feature in this disorder.

see page 262

Copyright © 2014 by the American Association of Neuropathologists, Inc.

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