J Neuropathol Exp Neurol Vol. 77, No. 4, 2018, p. 267 doi: 10.1093/jnen/nly016 was HIV-positive. They note that meningovascular and cortical Primary CNS Lymphomas (PCNSL) and forms of the disease may occur concurrently. Peripheral Diffuse Large B-Cell Lymphomas see page 296 (DLBCL): Differences in Drug Targets but Common Pathways in Pathogenesis Adult Brainstem Gliomas: The Enigma of H3K27M Marosvari et al report on the differences in the molecular biology of the mTOR pathway between DLBCL and PCNSL H3K27M mutations initially ascribed only to pediatric mid- with implications for targeted drug therapies. The authors line gliomas have since been identified in adults as well. In a suggest that alterations in PAS domain-containing serine/ relatively large series of 25 adult brainstem gliomas, the threonine-protein kinase (PASK) may be involved in the investigators correlated clinical, radiological and pathological pathogenesis of both DLBCL and PCNSL. data with mutation status, with interesting results. see page 268 see page 302 The Source of Neuropathic Pain in Targeting Immune Checkpoints for CNS Charcot-Marie-Tooth Disease Germinomas? Some patients with Charcot-Marie-Tooth disease type 1A Wildeman et al demonstrate that the majority of 21 CNS ger- (CMT1A) experience neuropathic pain. This led Duchesne et minomas harbor germ cell components and/or lymphocytes al to ask whether unmyelinated fibers are lost in skin biopsies that immunostain positively for the programmed death recep- from CMT1A patients. They report that mean intraepidermal tor PD-L1; PD-1 expression was largely confined to lympho- nerve fiber densities and the density of epidermal Langerhans cytes. These observations suggest the possibility that immune cells is decreased in CMT1A, raising the question of whether checkpoint inhibitors of PD-1/PD-L1 interactions could ther- factors other than PMP22 gene dosage contribute to small fi- apeutically overcome tolerance of tumor-infiltrating lympho- ber pathology in this disease. cytes to CNS germinomas. see page 274 see page 312 Broadening the Possibilities for Gene Spinal MotorNeuronLossIsthe Basisof Therapy in Myotubular Myopathy Neck Extensor Weakness in Multiple System X-linked myotubular myopathy is usually a lethal congeni- Atrophy (MSA) tal muscular dystrophy caused by mutations in the myotu- Saito et al identified 3 subjects (out of a total population of 75 bularin gene (MTM1). Some success has been achieved with pathologically confirmed MSA) who had dropped head treating an animal model using retroviral mediated gene (DH) during life. They determined the numbers of ventral therapy to replace MTM1.Danie`le et al show that the re- horn neurons in the C4 level of the spinal cord in these sub- lated gene, MTMR2, which shares considerable homology jects and found a greater decrease in neuron numbers com- with MTM1, can partially rescue Mtm1 mutant mice, indi- pared to 3 subjects each of MSA and Parkinson disease cating that MTM1 and MTMR2 have some redundant without DH, and neurologically normal controls. They con- functions. clude that upper cervical motor neuron loss contributes to see page 282 weakness of neck extensor muscles in MSA. see page 317 Clinicopathologic Correlations of Neurosyphilis Neurosyphilis is still encountered in both HIV-positive as well Repairing Roots as HIV-negative patients. Experience with the latter was re- Ruven et al investigated strategies to protect motor neurons cently detailed in a large Chinese cohort of 834 HIV-negative and muscle after avulsion injury to spinal roots, a common patients from Shanghai with primary, secondary, or latent syph- problem in traumatic injuries, including to the brachial plexus. ilis (J Eur Acad Dermatol Venereol 2016; 30:659-66) where Whereas treatment with either glial cell-derived neurotrophic neurosyphilis was found to be more likely in male than female factor or fetal lumbar cell transplantation alone did not result patients (1.52-fold), patients aged >60 years of age, and those in good functional recovery, their use in combination did, with a higher serum RPR titers. Mao et al draw on their experi- offering promise for effective clinical therapies. ence with neurosyphilis, this time from Beijing, to detail histo- see page 325 logical features of 3 neurosyphilis cases, in which only 1 of 3 V C 2018 American Association of Neuropathologists, Inc. All rights reserved. 267 Downloaded from https://academic.oup.com/jnen/article-abstract/77/4/267/4938880 by Ed 'DeepDyve' Gillespie user on 16 March 2018
Journal of Neuropathology & Experimental Neurology – Oxford University Press
Published: Apr 1, 2018
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