Annals of Neurology

The global SARS-CoV-2 pandemic has contributed to more than 163 million confirmed infections and 3.3 million deaths worldwide. The severity of the pandemic has led to an unprecedented effort to develop multiple effective vaccines. Due to excellent safety and efficacy data from clinical trials, several vaccines were approved. We report a case series of postvaccinal encephalitis in temporal correlation to vaccination with ChAdOx1 nCov-19. The diagnostic criteria for possible autoimmune encephalitis were fulfilled...

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No abstract text is available yet for this article.

OBJECTIVE: To examine the Alzheimer's Disease metabolite signature through magnetic resonance spectroscopy in adults with Down syndrome and its relation with Alzheimer's Disease biomarkers and cortical thickness. METHODS: We included 118 adults with Down syndrome from the Down Alzheimer Barcelona Imaging Initiative and 71 euploid healthy controls from the Sant Pau Initiative on Neurodegeneration cohort. We measured the levels of myo-inositol (a marker of neuroinflammation) and N-acetyl-aspartate (a marker of neuronal integrity) in the precuneus using magnetic resonance spectroscopy...

No abstract text is available yet for this article.

No abstract text is available yet for this article.

OBJECTIVE: We utilized human midbrain-like organoids (hMLOs) generated from human pluripotent stem cells (hPSCs) carrying glucocerebrosidase gene (GBA1) and α-synuclein (SNCA) perturbations to investigate genotype-to-phenotype relationships in Parkinson's disease, with the particular aim of recapitulating α-syn and Lewy body-related pathologies and the process of neurodegeneration in the hMLO model. METHOD: We generated and characterized hMLOs from GBA1-/- and SNCA O/E isogenic embryonic stem cells (ESCs) and also generated Lewy body-like inclusions (LBLIs) in GBA1/SNCA dual perturbation hMLOs and conduritol-b-epoxide-treated SNCA triplication hMLOs...

OBJECTIVE: Psychoses affecting people with epilepsy increase disease burden and diminish quality of life. We characterised post-ictal psychosis, which comprises about one-quarter of epilepsy-related psychoses, and has unknown causation. METHODS: We conducted a case-control cohort study including patients diagnosed with post-ictal psychosis, confirmed by psychiatric assessment, with available data regarding epilepsy, treatment, psychiatric history, psychosis profile and outcomes...

OBJECTIVE: Unidentified mechanisms largely restrict the viability of effective therapies in pharmacoresistant epilepsy. Our previous study revealed that hyperactivity of the subiculum is crucial for the genesis of pharmacoresistance in temporal lobe epilepsy (TLE), but the underlying molecular mechanism is not clear. METHODS: Here, we examined the role of subicular caspase-1, a key neural pro-inflammatory enzyme, in pharmacoresistant TLE. RESULTS: We found that the expression of activated caspase-1 in the subiculum, but not the CA1, was upregulated in pharmacoresistant amygdaloid-kindled rats...

OBJECTIVE: We aimed to estimate the incidence of cerebral sinus and venous thrombosis (CVT) within one month from first-dose administration and the frequency of vaccine-induced immune thrombotic thrombocytopenia (VITT) as the underlying mechanism after vaccination with BNT162b2, ChAdOx1, and mRNA-1273, in Germany. METHODS: A web-based questionnaire was e-mailed to all Departments of Neurology. We asked to report cases of CVT within one month of a COVID-19 vaccination...

OBJECTIVE: To identify disease relevant genes and explore underlying immunological mechanisms that contribute to early and late onset forms of myasthenia gravis. METHODS: We used a novel genomic methodology to integrate genome wide association study (GWAS) findings in myasthenia gravis with cell-type specific information such as gene expression patterns and promotor-enhancer interactions, in order to identify disease relevant genes. Subsequently, we conducted additional genomic investigations including an expression quantitative analysis of 313 healthy people to provide mechanistic insights...

OBJECTIVE: The hippocampus is connected to two distinct cortical brain networks, the posterior-medial and the anterior-temporal networks, involving different medial temporal lobe (MTL) subregions. The aim of this study was to assess the functional alterations of these two networks, their changes over time and links to cognition in Alzheimer's disease. METHODS: We assessed MTL connectivity in 53 amyloid-β positive patients with mild cognitive impairment and Alzheimer dementia and 68 healthy elderly controls, using resting-state functional magnetic resonance imaging, cross-sectionally and longitudinally...

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In Parkinson's disease, both motor and neuropsychiatric complications unfold as consequence of both incremental striatal dopaminergic denervation and intensifying long-term dopaminergic treatment. Together, this leads to 'dopaminergic sensitization' steadily increasing motor and behavioral responses to dopaminergic medication that result in the detrimental sequalae of long-term dopaminergic treatment. We review the clinical presentations of 'dopaminergic sensitization', including rebound off and dyskinesia in the motor domain, and neuropsychiatric fluctuations and behavioral addictions with impulse control disorders and dopamine dysregulation syndrome in the neuropsychiatric domain...

OBJECTIVE: Histology reveals that early active multiple sclerosis lesions can be classified into three main inter-individually heterogeneous but intra-individually stable immunopathological patterns of active demyelination (patterns I-III). In pattern I and II, a T-cell- and macrophage-associated demyelination is suggested, with pattern II only showing signs of a humoral immune response. Pattern III is characterized by inflammatory lesions with an oligodendrocyte degeneration. Patterns suggest pathogenic heterogeneity, and we postulated that they have distinct MRI correlates that may serve as biomarkers...

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No abstract text is available yet for this article.