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Journal of Neuropathology & Experimental Neurology:
doi: 10.1097/NEN.0000000000000088
In This Issue

In This Issue

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Primary CNS Lymphoma Revisited

In this review the authors focus on what constitutes a primary CNS lymphoma (large B-cell type) while they address the subtypes. They also describe the clinical and microscopic features of these lymphomas and provide a practical approach to diagnosis.

see page 478

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Developing New Blood Vessels in the Developing Brain

Hypoxic-ischemic injury (HII) remains as the most important cause of neurocognitive morbidity and mortality in the premature infant. While significant work has focused on the neuron during HII in the newborn, new work now begins to focus on the response of blood vessels in the developing brain.

see page 495

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Eyes before EAE

Sühs et al demonstrate the therapeutic benefits of memantine, a non-competitive inhibitor of NMDA receptors, in ameliorating loss of retinal ganglion cells during the pre-clinical stages of experimental autoimmune encephalomyelitis (EAE) in rats. Their results suggest that NMDA receptor blockade protected retinal ganglion cells from excitotoxicity directly and that the protection was independent of effects on oligodendrocytes or inflammatory cells.

see page 507

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Saying NO to Synapses

In spinal muscular atrophy (SMA), α-motoneuron death occurs late in the course of the disease, suggesting that other as yet unknown changes precede neuron death and are responsible for the initial clinical findings in SMA patients. Using a mouse model, Tarabal et al found that α-motoneuron death is preceded by a loss of excitatory glutamatergic synapses acting on these cells. Their findings implicate NO signaling and the RhoA/ROCK signaling in this synaptic pathology, suggesting this signaling cascade may be a useful therapeutic target in SMA.

see page 519

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Oligodendrocyte-Neuron Interactions in Fucosidosis: Black, White and Gray Areas

Investigating the role of oligodendrocytes in the neurodegenerative lysosomal storage disease fucosidosis, Fletcher et al make some unexpected observations. Although oligodendrocytes were maximally involved with vacuoles by 16 weeks they do not continue to undergo apoptosis beyond that period. Thus, this is an unlikely mechanism to explain hypomyelination. Early oligodendrocyte loss does, however, appear to contribute to Purkinje cell loss. These results and gene array data are described in this unique canine model of fucosidosis.

see page 536

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Blowing Bubbles Improves Stroke

Wang et al show that delivering microbubble encapsulated vascular endothelial growth factor (VEGF) plasmid via ultrasound-targeted microbubble destruction reduced infarct size while increasing angiogenesis and neurological recovery. This approach could lead to more efficacious methods for gene therapy in a variety of neurological disorders.

see page 548

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Spinal Muscular Atrophy: Not Just A Neuronal Disease?

Spinal muscular atrophy (SMA) is characterized by loss of spinal motor neurons with corresponding muscle denervation and profound weakness. Martínez-Hernández et al examined several muscle cytoskeletal components (slow, fast and developmental myosin, desmin and vimentin) and found evidence supporting the hypothesis that a delay in muscle maturation is one of the primary pathological components of SMA.

see page 559

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Chronic Proximal Axonopathy May Not Be Only Proximal

Soler-Martín et al report loss of neurofilaments, reduced content in synaptic vesicles, and accumulation of residual bodies in terminal axons of neuromuscular junctions in rodents treated with 3,3’-iminodipropionitrile. Results indicate abnormal nerve terminal structure, and suggest concomitant impaired neuromuscular synaptic function in this model of chronic proximal axonopathy.

see page 568

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Rosette-Forming Glioneuronal Tumors (RGNTs) Struggle to Find Their True “Molecular Identity”

RGNTs are rare low-grade gliomas. Gessi et al found FGFR-1 hotspot mutations in 2 of 8 RGNTs of the 4th ventricle. The tumor recurred in both cases and one patient also had a noncontiguous pilocytic astrocytoma with pilomyxoid features. The findings suggest that FGFR-1 mutations may contribute to MAPK pathway activation in a subset of RGNTs, which may share this molecular feature with pilocytic astrocytomas.

see page 580

© 2014 by American Association of Neuropathologists, Inc.