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https://www.readbyqxmd.com/read/28214652/unilateral-oculomotor-palsy-in-charcot-marie-tooth-disease-1a-cmt-1a
#1
A Posa, A Emmer, M E Kornhuber
BACKGROUND: Charcot-Marie-Tooth disease (CMT) type 1A is the most common form of CMT 1 and one of the autosomal dominant demyelinating hereditary motor and sensory neuropathies (HMSN). Cranial nerves may be frequently subclinically affected in CMT disease. However manifest clinical signs of cranial nerve involvement are rare. METHODS: This case comprise neurological, ophthalmological, internal medicine and ear-nose-throat investigation, motor and sensory nerve conduction velocity, auditory evoked potentials and orbicularis-oculi reflex measurements, lumbar puncture and blood examination, inclusive molecular genetic testing, as well as electrocardiogram and cranial imaging such as computer tomography and magnetic resonance imaging RESULTS: The present case shows a Charcot-Marie-Tooth (CMT) 1A patient with complete unilateral oculomotor palsy in combination with predominant ipsilateral subclinical trigeminal demyelination...
February 13, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28213912/diagnostic-red-flags-steroid-treated-malignant-cns-lymphoma-mimicking-autoimmune-inflammatory-demyelination
#2
Alonso Barrantes-Freer, Aylin Sophie Engel, Odir Antonio Rodríguez-Villagra, Anne Winkler, Markus Bergmann, Christian Mawrin, Tania Kuempfel, Hannah Pellkofer, Imke Metz, Annalen Bleckmann, Silvia Hernández-Durán, Sven Schippling, Elisabeth J Rushing, Stephan Frank, Markus Glatzel, Jakob Matschke, Christian Hartmann, Guido Reifenberger, Wolf Müller, Hans-Ulrich Schildhaus, Wolfgang Brück, Christine Stadelmann
The presence of inflammation and demyelination in a central nervous system (CNS) biopsy points towards a limited, yet heterogeneous group of pathologies, of which multiple sclerosis (MS) represents one of the principal considerations. Inflammatory demyelination has also been reported in patients with clinically suspected primary central nervous system lymphoma (PCNSL), especially when steroids had been administered prior to biopsy acquisition. The histopathological changes induced by corticosteroid treatment can range from mild reduction to complete disappearance of lymphoma cells...
February 18, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28212925/reversible-opercular-syndrome-secondary-to-osmotic-demyelination
#3
Shuchit Pandey, Vikram V Holla, Imran Rizvi, Rakesh Shukla
Opercular syndrome (OPS) is characterized by weakness of facial, masticatory, pharyngeal, laryngeal, tongue and brachial muscles on voluntary command with preservation of emotional and reflexive movements. We report a case of 45year old female who developed the features of OPS due to lesions of bilateral frontal opercular region induced by osmotic demyelination secondary to hyperosmolar hyperglycaemia. On follow up at 6 months, she had complete recovery.
February 11, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28212662/marked-central-nervous-system-pathology-in-cd59-knockout-rats-following-passive-transfer-of-neuromyelitis-optica-immunoglobulin-g
#4
Xiaoming Yao, Alan S Verkman
Neuromyelitis optica spectrum disorders (herein called NMO) is an inflammatory demyelinating disease of the central nervous system in which pathogenesis involves complement-dependent cytotoxicity (CDC) produced by immunoglobulin G autoantibodies targeting aquaporin-4 (AQP4-IgG) on astrocytes. We reported evidence previously, using CD59(-/-) mice, that the membrane-associated complement inhibitor CD59 modulates CDC in NMO (Zhang and Verkman, J. Autoimmun. 53:67-77, 2014). Motivated by the observation that rats, unlike mice, have human-like complement activity, here we generated CD59(-/-) rats to investigate the role of CD59 in NMO and to create NMO pathology by passive transfer of AQP4-IgG under conditions in which minimal pathology is produced in normal rats...
February 17, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/28212304/methylglyoxal-derived-advanced-glycation-endproducts-in-multiple-sclerosis
#5
Suzan Wetzels, Kristiaan Wouters, Casper G Schalkwijk, Tim Vanmierlo, Jerome J A Hendriks
Multiple sclerosis (MS) is a demyelinating disease of the central nervous system (CNS). The activation of inflammatory cells is crucial for the development of MS and is shown to induce intracellular glycolytic metabolism in pro-inflammatory microglia and macrophages, as well as CNS-resident astrocytes. Advanced glycation endproducts (AGEs) are stable endproducts formed by a reaction of the dicarbonyl compounds methylglyoxal (MGO) and glyoxal (GO) with amino acids in proteins, during glycolysis. This suggests that, in MS, MGO-derived AGEs are formed in glycolysis-driven cells...
February 15, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28212177/neurocognitive-outcomes-for-acute-global-acquired-brain-injury-in-children
#6
Fenella J Kirkham
PURPOSE OF REVIEW: In children, acute global brain injury from traumatic brain injury (TBI) and nontraumatic coma has a substantial cost to the child, the family, and the society. There have historically been relatively few studies looking at long-term cognition and behavioural outcomes. RECENT FINDINGS: Long-term follow-up studies, population-based studies, and meta-analyses are now available for TBI in children as well as adults and suggest that there is a significant cognitive cost, particularly for processing speed, working memory, and intelligence quotient (IQ) in severe TBI...
February 15, 2017: Current Opinion in Neurology
https://www.readbyqxmd.com/read/28211783/recurrent-tumefactive-demyelinating-lesions-a-pathological-study
#7
Recai Türkoğlu, Hans Lassmann, Fügen Vardar Aker, John Tzartos, Socrates Tzartos, Erdem Tüzün
No abstract text is available yet for this article.
February 17, 2017: Clinical Neuropathology
https://www.readbyqxmd.com/read/28211470/transient-auditory-nerve-demyelination-as-a-new-mechanism-for-hidden-hearing-loss
#8
Guoqiang Wan, Gabriel Corfas
Hidden hearing loss (HHL) is a recently described auditory neuropathy believed to contribute to speech discrimination and intelligibility deficits in people with normal audiological tests. Animals and humans with HHL have normal auditory thresholds but defective cochlear neurotransmission, that is, reduced suprathreshold amplitude of the sound-evoked auditory nerve compound action potential. Currently, the only cellular mechanism known for HHL is loss of inner hair cell synapses (synaptopathy). Here we report that transient loss of cochlear Schwann cells results in permanent auditory deficits characteristic of HHL...
February 17, 2017: Nature Communications
https://www.readbyqxmd.com/read/28211240/phenotypic-spectrum-of-charcot-marie-tooth-disease-due-to-litaf-simple-mutations-a-study-of-18-patients
#9
R Guimarães-Costa, R Iancu Ferfoglia, S Leonard-Louis, F Ziegler, L Magy, E Fournier, O Dubourg, P Bouche, T Maisonobe, A Lacour, A Moerman, P Latour, T Stojkovic
BACKGROUND AND PURPOSE: Charcot-Marie-Tooth (CMT) 1C due to mutations in LITAF/SIMPLE is a rare subtype amongst the autosomal dominant demyelinating forms of CMT. Our objective was to report the clinical and electrophysiological characteristics of 18 CMT1C patients and compare them to 20 patients with PMP22 mutations: 10 CMT1A patients and 10 patients with hereditary neuropathy with liability to pressure palsies (HNPP). METHODS: Charcot-Marie-Tooth 1C patients were followed-up in referral centres for neuromuscular diseases or were identified by familial survey...
March 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28210570/balo-s-concentric-lesions-with-concurrent-features-of-schilder-s-disease-in-relapsing-multiple-sclerosis-neuropathological-findings
#10
Maher Kurdi, David Ramsay
Atypical inflammatory demyelinating syndromes are rare neurological diseases that differ from multiple sclerosis (MS), owing to unusual clinicoradiological and pathological findings, and poor responses to treatment. The distinction between them and the criteria for their diagnoses are poorly defined due to the lack of advanced research studies. Balo's concentric sclerosis (BCS) and Schilder's disease (SD) are two of these syndromes and can present as monophasic or in association with chronic MS. Both variants are difficult to distinguish when they present in acute stages...
October 2016: Autopsy & case reports
https://www.readbyqxmd.com/read/28209583/combining-diffusion-tensor-metrics-and-dsc-perfusion-imaging-can-it-improve-the-diagnostic-accuracy-in-differentiating-tumefactive-demyelination-from-high-grade-glioma
#11
S B Hiremath, A Muraleedharan, S Kumar, C Nagesh, C Kesavadas, M Abraham, T R Kapilamoorthy, B Thomas
BACKGROUND AND PURPOSE: Tumefactive demyelinating lesions with atypical features can mimic high-grade gliomas on conventional imaging sequences. The aim of this study was to assess the role of conventional imaging, DTI metrics (p:q tensor decomposition), and DSC perfusion in differentiating tumefactive demyelinating lesions and high-grade gliomas. MATERIALS AND METHODS: Fourteen patients with tumefactive demyelinating lesions and 21 patients with high-grade gliomas underwent brain MR imaging with conventional, DTI, and DSC perfusion imaging...
February 16, 2017: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/28209311/mri-findings-of-optic-pathway-involvement-in-miller-fisher-syndrome-in-3-pediatric-patients-and-a-review-of-the-literature
#12
Ajay Malhotra, Mia Zhang, Xiao Wu, Shanu Jindal, David Durand, Naila Makhani
BACKGROUND: Miller Fisher syndrome (MFS) is a rare demyelinating condition which may have involvement of cranial nerves. There are a few case reports of optic pathway involvement in children. We describe 3 patients with optic pathway enhancement in pediatric patients with MFS. CASE SERIES: We retrospectively reviewed brain imaging findings in 17 pediatric patients with of Guillain-Barré syndrome (GBS) meeting Brighton criteria who had brain MRIs performed during their acute illness...
February 10, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28203460/the-prevalence-of-anti-aquaporin-4-antibody-in-patients-with-idiopathic-inflammatory-demyelinating-diseases-presented-to-a-tertiary-hospital-in-malaysia-presentation-and-prognosis
#13
S Abdullah, W F Wong, C T Tan
Background. There have been inconsistent reports on the prevalence and pathogenicity of anti-Aquaporin 4 (AQP4) in patients presented with idiopathic inflammatory demyelinating diseases (IIDDs). Objective. To estimate the prevalence of anti-AQP4 antibody in patients with IIDDs presented to University Malaya Medical Centre in terms of patients' clinical and radiological presentations and prognoses. Methods. Retrospective data review of IIDDs patients presented from 2005 to 2015. Patients were classified into classical multiple sclerosis (CMS), opticospinal (OS) presentation, optic neuritis (ON), transverse myelitis (TM), brainstem syndrome (BS), and tumefactive MS...
2017: Multiple Sclerosis International
https://www.readbyqxmd.com/read/28203182/treatable-leukoencephalopathy-in-a-patient-with-hypophosphatemia
#14
Masahiro Okazaki, Makito Hirano, Tomoki Iwatsu, Masaki Yamana, Hidekazu Suzuki, Takao Satou, Susumu Kusunoki
We report the first patient with pathologically proven leukoencephalopathy associated with hypophosphatemia. A 61-year-old woman had repetitive episodes of decreased consciousness with pontine and pallidal lesions and extensive leukoencephalopathy on MRI, later found to be associated with hypophosphatemia. Although hypophosphatemia has been linked to central pontine and extrapontine myelinolysis (osmotic myelinolysis), lesions in the deep white matter have not been reported. Brain biopsy performed during the first diagnosis process revealed nonspecific demyelination with gliosis, a finding similar to that of chronic osmotic myelinolysis...
September 2016: Case Reports in Neurology
https://www.readbyqxmd.com/read/28202068/the-dna-damage-response-promotes-polyomavirus-jc-infection-by-nucleus-to-cytoplasm-nf-kappab-activation
#15
Martyn K White, Anna Bellizzi, Gabriele Ibba, Valeria Pietropaolo, Anna T Palamara, Hassen S Wollebo
BACKGROUND: Infection of glial cells by human neurotropic polyomavirus JC (JCV), the causative agent of the CNS demyelinating disease progressive multifocal leukoencephalopathy (PML), rapidly inflicts damage to cellular DNA. This activates DNA damage response (DDR) signaling including induction of expression of DNA repair factor Rad51. We previously reported that Rad51 co-operates with the transcription factor NF-κB p65 to activate JCV early transcription. Thus Rad51 induction by JCV infection may provide positive feedback for viral activation early in JCV infection...
February 15, 2017: Virology Journal
https://www.readbyqxmd.com/read/28198408/therapeutic-effects-of-human-adipose-tissue-derived-stem-cell-hadsc-transplantation-on-experimental-autoimmune-encephalomyelitis-eae-mice
#16
Jia Li, Ying Chen, Zhibo Chen, Yuanyuan Huang, Dehao Yang, Zhongqian Su, Yiyun Weng, Xiang Li, Xu Zhang
This study is to investigate the therapeutic effects of human adipose tissue-derived stem cell (hADSC) transplantation on experimental autoimmune encephalomyelitis (EAE) in mice. EAE mouse model was established by MOG35-55 immunization. Body weight and neurological function were assessed. H&E and LFB staining was performed to evaluate histopathological changes. Flow cytometry was used to detect Th17 and Treg cells. ELISA and real-time PCR were performed to determine transcription factor and pro-inflammatory cytokine levels...
February 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28197184/the-role-of-interleukin-6-in-central-nervous-system-demyelination
#17
Filip Petković, Bernardo Castellano
No abstract text is available yet for this article.
December 2016: Neural Regeneration Research
https://www.readbyqxmd.com/read/28197176/multiple-sclerosis-integration-of-modeling-with-biology-clinical-and-imaging-measures-to-provide-better-monitoring-of-disease-progression-and-prediction-of-outcome
#18
REVIEW
Shikha Jain Goodwin
Multiple Sclerosis (MS) is a major cause of neurological disability in adults and has an annual cost of approximately $28 billion in the United States. MS is a very complex disorder as demyelination can happen in a variety of locations throughout the brain; therefore, this disease is never the same in two patients making it very hard to predict disease progression. A modeling approach which combines clinical, biological and imaging measures to help treat and fight this disorder is needed. In this paper, I will outline MS as a very heterogeneous disorder, review some potential solutions from the literature, demonstrate the need for a biomarker and will discuss how computational modeling combined with biological, clinical and imaging data can help link disparate observations and decipher complex mechanisms whose solutions are not amenable to simple reductionism...
December 2016: Neural Regeneration Research
https://www.readbyqxmd.com/read/28197174/extremely-low-frequency-electromagnetic-fields-stimulation-modulates-autoimmunity-and-immune-responses-a-possible-immuno-modulatory-therapeutic-effect-in-neurodegenerative-diseases
#19
REVIEW
Fabio Guerriero, Giovanni Ricevuti
Increasing evidence shows that extremely low frequency electromagnetic fields (ELF-EMFs) stimulation is able to exert a certain action on autoimmunity and immune cells. In the past, the efficacy of pulsed ELF-EMFs in alleviating the symptoms and the progression of multiple sclerosis has been supported through their action on neurotransmission and on the autoimmune mechanisms responsible for demyelination. Regarding the immune system, ELF-EMF exposure contributes to a general activation of macrophages, resulting in changes of autoimmunity and several immunological reactions, such as increased reactive oxygen species-formation, enhanced phagocytic activity and increased production of chemokines...
December 2016: Neural Regeneration Research
https://www.readbyqxmd.com/read/28196893/phosphorylation-of-%C3%AE-b-crystallin-supports-reactive-astrogliosis-in-demyelination
#20
Hedwich F Kuipers, Jane Yoon, Jack van Horssen, May H Han, Paul L Bollyky, Theo D Palmer, Lawrence Steinman
The small heat shock protein αB-crystallin (CRYAB) has been implicated in multiple sclerosis (MS) pathogenesis. Earlier studies have indicated that CRYAB inhibits inflammation and attenuates clinical disease when administered in the experimental autoimmune encephalomyelitis model of MS. In this study, we evaluated the role of CRYAB in primary demyelinating events. Using the cuprizone model of demyelination, a noninflammatory model that allows the analysis of glial responses in MS, we show that endogenous CRYAB expression is associated with increased severity of demyelination...
February 14, 2017: Proceedings of the National Academy of Sciences of the United States of America
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