Hybrid peripheral nerve sheath tumors (HPNST) were first included in the 4th edition of the World Health Organization (WHO) Classification of Central Nervous System in 2016. These tumors are a combination of schwannoma and neurofibroma, and are typically found in the limbs, trunk, and soft tissues. In this case report, we present a rare intracranial localization of neurofibroma/schwannoma with histological examination. Our patient was a 36-year-old man without any tumor genetic syndromes. The tumor measured 1.6 × 1.1 cm and was completely excised. The pathology report showed no significant nuclear atypia, mitotic activity, or necrosis. At the time of writing, the patient is alive and well with no recurrence. It is important to note that HPNSTs are usually benign tumors, but can occur in various locations, including the intracranial region. Further studies with a larger number of cases are needed to determine the exact pathogenetic basis of these tumors.
Neurotrauma, especially repetitive neurotrauma, is associated with the development of progressive neurodegeneration leading to chronic traumatic encephalopathy (CTE). Exposure to neurotrauma regularly occurs during sports and military service, often not requiring medical care. However, exposure to severe and/or repeated sub-clinical neurotrauma has been shown cause physical and psychological disability, leading to reduce life expectancy. Misfolding of proteins, or proteinopathy, is a pathological hallmark of CTE, in which chronic injury leads to local and diffuse protein aggregates. These aggregates are an overlapping feature of many neurodegenerative diseases such as CTE, Alzheimer's Disease, Parkinsons disease. Neurotrauma is also a significant risk factor for the development of these diseases, however the mechanism's underlying this association are not well understood. While phosphorylated tau aggregates are the primary feature of CTE, amyloid-beta, Transactive response DNA-binding protein 43 (TDP-43), and alpha-synuclein (αSyn) are also well documented. Aberrant misfolding of these proteins has been shown to disrupt brain homeostasis leading to neurodegeneration in a disease dependent manor. In CTE, the interaction between proteinopathies and their associated neurodegeneration is a current area of study. Here we provide an update on current literature surrounding the prevalence, characteristics, and pathogenesis of proteinopathies in CTE.
PURPOSES: Cine PC-MRI has shown that CSF oscillations increase with age, while the ratio between the CSF Oscillation in the aqueduct and the spinal canal is constant. Our aim was to test whether the CSF hydrodynamic can bring complementary information to study pediatric population with an increase of the CSF volume. Material and Method: Forty three patients, newborns and children (mean age: 31 ± 32 months; 5 days - 111 months) with an intracranial CSF volume increase (ventricular or/and subarachnoid spaces) underwent a morphological MRI along with cine PC-MRI to quantify CSF oscillations. We defined a ratio of the ventricular area to that of the intracranial subarachnoid spaces (CSFratio). We also determined an index called CSFdynamic, which equals the CSF aqueduct stroke volume (SVAq) divided by the cervical stroke volume (SVC2C3) at the level C2C3 in the spine. RESULTS: Twenty-three patients presented only ventricular dilatation: CSFdynamic = 20 ± 25; CSFratio: 120 ± 151; with no significant correlation, (rs = 0.152, p = 0.48). Sixteen patients presented both ventricular and subarachnoid space dilations: CSFdynamic = 18 ± 17; CSFratio = 1.67 ± 0.81; with significant positive correlation (rs = 0.5911 p = 0.016). CONCLUSIONS: In pediatric population, the absence of correlation between the dynamic of the CSF and its volume shows that the CSF oscillation does not result only of the size of the ventricles and/or the subarachnoid spaces. The CSF oscillations bring complementary information concerning the active aspect of the CSF.
Nuclear factor-kappa B (NF-κB) is a key modulator of inflammation and secondary injury responses in neurodegenerative disease, including spinal cord injury (SCI). Inhibition of astroglial NF-κB reduces inflammation, enhances oligodendrogenesis and improves functional recovery after SCI, however the contribution of neuronal NF-κB to secondary inflammatory responses following SCI has yet to be investigated. We demonstrate that conditional ablation of IKK2 in Synapsin 1-expressing neurons in mice (Syn1creIKK2fl/fl) reduces activation of the classical NF-κB signaling pathway, resulting in impaired motor function and altered memory retention under naïve conditions. Following induction of a moderate SCI phosphorylated NF-κB levels decreased in the spinal cord of Syn1creIKK2fl/fl mice compared to controls, resulting in improvement in functional recovery. Histologically, Syn1creIKK2fl/fl mice exhibited reduced lesion volume but comparable microglial/leukocyte responses after SCI. In parallel, interleukin (IL)-1β expression was significantly decreased within the lesioned spinal cord, whereas IL-5, IL-6, IL-10, tumor necrosis factor (TNF) and chemokine (C-X-C motif) ligand 1 were unchanged compared to control mice. We conclude that conditional ablation of IKK2 in neurons, resulting in reduced neuronal NF-B signaling, and lead to protective effects after SCI and propose the neuronal classical NF-κB pathway as a potential target for the development of new therapeutic, neuroprotective strategies for SCI.
