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Annals of Neurology

Daniel L Kenney-Jung, Annamaria Vezzani, Robert J Kahoud, Reghann G LaFrance-Corey, Mai-Lan Ho, Theresa Wampler Muskardin, Elaine C Wirrell, Charles L Howe, Eric T Payne
Febrile infection-related epilepsy syndrome (FIRES) is a devastating epileptic encephalopathy with limited treatment options and an unclear etiology. Anakinra is a recombinant version of the human interleukin-1 receptor antagonist used to treat autoinflammatory disorders. This is the first report of anakinra for treatment of a child with super-refractory status epilepticus secondary to FIRES. Anakinra was well-tolerated and effective. Cerebral spinal fluid analysis revealed elevated levels of proinflammatory cytokines before treatment that normalized on anakinra, suggesting a potential pathogenic role for neuroinflammation in FIRES...
October 22, 2016: Annals of Neurology
Joan Yw Liu, Cheryl Reeves, Beate Diehl, Antonietta Coppola, Aliya Al-Hajri, Chandrashekar Hoskote, Salim Al Mughairy, Mohamed Tachrount, Michael Groves, Zuzanna Michalak, Kevin Mills, Andrew W McEvoy, Anna Miserocchi, Sanjay M Sisodiya, Maria Thom
OBJECTIVE: This study reports on a novel brain pathology in young patients with Frontal Lobe Epilepsy that is distinct from Focal Cortical Dysplasia. METHODS: Surgical specimens from twenty young adults with frontal lobe epilepsy (mean age, 30 years) were investigated with histological/immunohistochemical markers for cortical laminar architecture, mammalian target of rapamycin pathway activation and inhibition, cellular autophagy, and synaptic vesicle-mediated trafficking as well as proteomics analysis...
October 20, 2016: Annals of Neurology
Linda Chang, Gro C Løhaugen, Tamara Andres, Caroline S Jiang, Vanessa Douet, Naomi Tanizaki, Christina Walker, Deborrah Castillo, Ahnate Lim, Jon Skranes, Chad Otoshi, Eric N Miller, Thomas M Ernst
OBJECTIVES: We aimed to evaluate the effectiveness of an adaptive-working memory training (WMT) program, the corresponding neural correlates, and LMX1A-rs4657412 polymorphism on the adaptive-WMT, in HIV-participants compared to seronegative controls (SN). METHODS: 201/206 qualified participants completed baseline assessments before randomization to 25-sessions of adaptive-WMT or non-adaptive-WMT. 74/76 (34HIV, 42SN) completed adaptive-WMT and all 40 completed non-adaptive-WMT (20HIV, 20SN) and were assessed after 1-month, and 55 adaptive-WMT-participants were also assessed after 6-months...
October 19, 2016: Annals of Neurology
Rivka Inzelberg, Shira Flash, Eitan Friedman, Esther Azizi
The mechanisms underlying the high prevalence of cutaneous malignant melanoma (CMM) in Parkinson's disease (PD) are unclear, but plausibly involve common pathways. 129Ser-phosphorylated-α-synuclein, a pathological PD hallmark, is abundantly expressed in CMM, but not in normal skin. In inherited PD, PARK genes harbor germline mutations; the same genes are somatically mutated in CMM, or their encoded proteins involved in melanomagenesis. Conversely, genes associated with CMM, affect PD risk. PD/CMM targeted cells share the neural crest origin and melanogenesis capability...
October 19, 2016: Annals of Neurology
Martin R Turner, Raph Goldacre, Kevin Talbot, Michael J Goldacre
It is recognized that neuropsychiatric conditions are over-represented in ALS patient kindreds and psychiatric symptoms may precede the onset of motor symptoms. Using a hospital record linkage database, hospitalization with a diagnosis of schizophrenia, bipolar disorder, depression or anxiety was significantly associated with a first diagnosis of ALS within the following year. This is likely to specifically reflect the clinicopathological overlap of ALS with frontotemporal dementia. A diagnosis of depression was significantly associated with a first record of ALS five or more years later, in keeping with growing evidence for major depressive disorder as an early marker of cerebral neurodegeneration...
October 19, 2016: Annals of Neurology
Amy Krans, Michael G Kearse, Peter K Todd
OBJECTIVE: Repeat associated non-AUG (RAN) translation drives production of toxic proteins from pathogenic repeat sequences in multiple untreatable neurodegenerative disorders. Fragile X-associated tremor/ataxia syndrome (FXTAS) is one such condition, resulting from a CGG trinucleotide repeat expansion in the 5' leader sequence of the FMR1 gene. RAN proteins from the CGG repeat accumulate in ubiquitinated inclusions in FXTAS patient brains and elicit toxicity. In addition to the CGG repeat, an antisense mRNA containing a CCG repeat is also transcribed from the FMR1 locus...
October 19, 2016: Annals of Neurology
Guy Bar-Klein, Rebecca Klee, Claudia Brandt, Marion Bankstahl, Pablo Bascuñana, Kathrin Töllner, Hotjensa Dalipaj, Jens P Bankstahl, Alon Friedman, Wolfgang Löscher
OBJECTIVE: Acquired epilepsy is a devastating long-term risk of various brain insults, including trauma, stroke, infections, and status epilepticus (SE). There is no preventive treatment for patients at risk. Due to the complex alterations involved in epileptogenesis, it is likely that multi-targeted approaches are required for epilepsy prevention. We report novel preclinical findings with isoflurane, which exerts various non-anesthetic effects that may be relevant for anti-epileptogenesis...
October 19, 2016: Annals of Neurology
Christof Dame
No abstract text is available yet for this article.
October 19, 2016: Annals of Neurology
Juan Song, Xiaoyang Wang, Changlian Zhu
No abstract text is available yet for this article.
October 19, 2016: Annals of Neurology
Orrin Devinsky, Martin A Samuels
No abstract text is available yet for this article.
October 17, 2016: Annals of Neurology
Daniel Garbin Di Luca, David J Adams
No abstract text is available yet for this article.
October 17, 2016: Annals of Neurology
Oscar H Del Brutto
No abstract text is available yet for this article.
October 12, 2016: Annals of Neurology
Arturo Carpio, Agnes Fleury, Matthew Romo, Josemir Sander
No abstract text is available yet for this article.
October 12, 2016: Annals of Neurology
Alefiya Dhilla Albers, Josephine Asafu-Adjei, Mary K Delaney, Kathleen E Kelly, Teresa Gomez-Isla, Deborah Blacker, Keith A Johnson, Reisa A Sperling, Bradley T Hyman, Rebecca A Betensky, Lloyd Hastings, Mark W Albers
OBJECTIVE: To relate a novel test of identifying and recalling odor percepts to biomarkers of Alzheimer's Disease (AD) in well-characterized elderly individuals, ranging from cognitively normal to demented. METHODS: 183 participants (cognitively normal: n=70, subjective cognitive concerns: n=74, Mild Cognitive Impairment (MCI): n=29, AD dementia: n=10) were administered novel olfactory tests: the Odor Percept IDentification (OPID) and the Percepts of Odor Episodic Memory (POEM) tests...
October 1, 2016: Annals of Neurology
Keith A Vossel, Kamalini G Ranasinghe, Alexander J Beagle, Danielle Mizuiri, Susanne M Honma, Anne F Dowling, Sonja M Darwish, Victoria Van Berlo, Deborah E Barnes, Mary Mantle, Anna M Karydas, Giovanni Coppola, Erik D Roberson, Bruce L Miller, Paul A Garcia, Heidi E Kirsch, Lennart Mucke, Srikantan S Nagarajan
OBJECTIVE Seizures are more frequent in patients with Alzheimer's disease (AD) and can hasten cognitive decline. However, the incidence of subclinical epileptiform activity in AD and its consequences are unknown. Motivated by results from animal studies, we hypothesized higher than expected rates of subclinical epileptiform activity in AD with deleterious effects on cognition. METHODS We prospectively enrolled 33 patients (mean age 62 years) who met criteria for AD, but had no history of seizures, and 19 age-matched, cognitively normal controls...
October 1, 2016: Annals of Neurology
Elena Herranz, Costanza Giannì, Céline Louapre, Constantina A Treaba, Sindhuja T Govindarajan, Russell Ouellette, Marco L Loggia, Jacob A Sloane, Nancy Madigan, David Izquierdo-Garcia, Noreen Ward, Gabriel Mangeat, Tobias Granberg, Eric C Klawiter, Ciprian Catana, Jacob M Hooker, Norman Taylor, Carolina Ionete, Revere P Kinkel, Caterina Mainero
Objective In multiple sclerosis (MS), using simultaneous magnetic resonance-positron emission tomography (MR-PET) imaging with (11) C-PBR28, we quantified expression of the 18kDa translocator protein (TSPO), a marker of activated microglia/macrophages, in cortex, cortical lesions, deep gray matter (GM), white matter (WM) lesions and normal-appearing WM (NAWM) to investigate the in vivo pathological and clinical relevance of neuroinflammation. Methods Fifteen secondary-progressive MS (SPMS) and 12 relapsing-remitting MS (RRMS) cases, and 14 matched healthy controls underwent (11) C-PBR28 MR-PET...
September 30, 2016: Annals of Neurology
Megan S Keiser, Alejandro Mas Monteys, Romuald Corbau, Pedro Gonzalez-Alegre, Beverly L Davidson
OBJECTIVE: Spinocerebellar ataxia type 1 is an autosomal dominant fatal neurodegenerative disease caused by a polyglutamine expansion in the coding region of ATXN1. We showed previously that partial suppression of mutant ataxin-1 (ATXN1) expression, using virally-expressed RNAi triggers, could prevent disease symptoms in a transgenic mouse model and a knock in mouse model of the disease, using a single dose of virus. Here, we set out to test if RNAi triggers targeting ATXN1 could not only prevent, but also reverse disease readouts when delivered after symptom onset...
September 30, 2016: Annals of Neurology
Kristien Peeters, Paulius Palaima, Ana L Pelayo-Negro, Antonio García, Elena Gallardo, Rosario García-Barredo, Ligia Mateiu, Jonathan Baets, Björn Menten, Els De Vriendt, Peter De Jonghe, Vincent Timmerman, Jon Infante, José Berciano, Albena Jordanova
OBJECTIVE: To identify the unknown genetic cause in a large pedigree previously classified with a distinct form of axonal Charcot-Marie-Tooth disease type 2G (CMT2G) and to explore its transcriptional consequences. METHODS: Clinical reevaluation of the pedigree was performed, followed by linkage analysis with the redefined disease statuses, and whole genome and exome sequencing. The impact of the mutation was investigated by immunoblotting and transcriptome sequencing...
September 30, 2016: Annals of Neurology
Clifford B Saper
No abstract text is available yet for this article.
September 27, 2016: Annals of Neurology
Wendy K Roberts, Nathalie E Blachère, Mayu O Frank, Athanasios Dousmanis, Richard M Ransohoff, Robert B Darnell
No abstract text is available yet for this article.
September 26, 2016: Annals of Neurology
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