Protean Neurologic Manifestations of Two Rare Dermatologic Disorders: Sweet Disease and Localized Craniofacial Scleroderma

AbstractPurpose of ReviewTo describe diverse neurologic and neuroradiologic presentations of two rare, immunologically mediated skin conditions: Sweet disease and localized scleroderma (morphea).Recent FindingsCore syndromes of neuro-Sweet disease (NSD) are steroid responsiveness, recurrent meningitis, and encephalitis. Focal neurologic, neuro-vascular, and neuro-ophthalmologic syndromes have been reported recently in NSD. A variety of steroid-sparing treatments and biologics have been used for relapsing NSD. Localized craniofacial scleroderma is associated with seizures, headaches, and, less commonly, focal deficits and cognitive decline. Immunosuppressive therapy may be required in patients with disease progression; some refractory cases have responded to IL-6 inhibition.SummaryOur review provides an up-to-date reference for neurologists faced with a patient with a history or skin findings consistent with Sweet disease or localized scleroderma. We hope that it will stimulate collaborative studies aimed at unraveling the pathogenesis of these disorders, better characterization of their neurologic manifestations, and discovery of optimal therapeutic solutions.
Source: Current Neurology and Neuroscience Reports - Category: Neuroscience Source Type: research

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In conclusion, our data show how oncogenic and tumor-suppressive drivers of cellular senescence act to regulate surveillance processes that can be circumvented to enable SnCs to elude immune recognition but can be reversed by cell surface-targeted interventions to purge the SnCs that persist in vitro and in patients. Since eliminating SnCs can prevent tumor progression, delay the onset of degenerative diseases, and restore fitness; since NKG2D-Ls are not widely expressed in healthy human tissues and NKG2D-L shedding is an evasion mechanism also employed by tumor cells; and since increasing numbers of B cells express NKG2D ...
Source: Fight Aging! - Category: Research Authors: Tags: Newsletters Source Type: blogs
Publication date: Available online 16 August 2019Source: The LancetAuthor(s): Michel D Ferrari, Hans Christoph Diener, Xiaoping Ning, Maja Galic, Joshua M Cohen, Ronghua Yang, Matthias Mueller, Andrew H Ahn, Yael Carmeli Schwartz, Melissa Grozinski-Wolff, Lindsay Janka, Messoud AshinaSummaryBackgroundAntibodies targeting calcitonin gene-related peptide (CGRP) or its receptor have shown efficacy in the prevention of migraine attacks. We investigated the efficacy and tolerability of fremanezumab, a fully humanised CGRP antibody, in patients with migraine who had previously not responded to two to four classes of migraine pre...
Source: The Lancet - Category: General Medicine Source Type: research
Publication date: Available online 16 August 2019Source: The LancetAuthor(s): Alessandro Tessitore, Antonio Russo
Source: The Lancet - Category: General Medicine Source Type: research
Publication date: Available online 16 August 2019Source: Journal of Clinical NeuroscienceAuthor(s): A. Skalnaya, V. Fominykh, R. Ivashchenko, D. Averchenkov, L. Grazhdantseva, N. Frigo, E. Negasheva, O. Dolya, L. Brylev, A. GuekhtAbstractThe term of neurosyphilis (NS) refers to infection of central nervous system by Treponema pallidum. Classically, it has been divided into early (meningitis, meningovascular) and late forms (general paresis and tabes dorsalis). The availability of penicillin and high sensitivity of Treponema pallidum to this antibiotic has led to a widely held perception about rarity of syphilitic forms wit...
Source: Journal of Clinical Neuroscience - Category: Neuroscience Source Type: research
In this report, considering also the literature information, a case of G.sanguinis which is thought to cause meningitis was presented. Our case is a 39-year-old female patient with a lumboperitoneal shunt. The patient was admitted to the neurosurgery clinic with a headache and vision loss and was hospitalized in the service with a pre-diagnosis of pseudotumor cerebri. Neurological examination revealed no pathological findings. Eye examination revealed mild papillary edema, local retinal hemorrhage, and bilateral expansion in retinal vascularization. There was no pathologic findings in the brain magnetic resonance imaging. ...
Source: Mikrobiyoloji Bulteni - Category: Microbiology Tags: Mikrobiyol Bul Source Type: research
ConclusionsUtilities estimated from the general population sample may be used to represent route of administration and AEs in cost-utility models. Results from the patient sample indicate that these treatment characteristics have an impact on patient preference.
Source: Quality of Life Research - Category: Health Management Source Type: research
We report a case of K. variicola meningitis after neurosurgery. The bacterium was isolated from blood and cerebrospinal fluid, and bacterial species identification was carried out by using both matrix-assisted laser-desorption/ionization time-of-flight mass spectroscopy (MALDI-TOF MS) and whole genome sequencing. Initially, the organism was misidentified as K. pneumoniae by VITEK®2; automated system in the clinical laboratory examination. The patient recovered with the combination of surgical drainage and antimicrobial treatment. To our knowledge, this is the first case report of post-surgical meningitis caused by K. v...
Source: IDCases - Category: Infectious Diseases Source Type: research
Background: Few studies report hearing preservation following middle cranial fossa (MCF) floor defect repair. Objective: To investigate audiological outcomes following MCF floor defect repair using a modified MCF suprapetrous approach. Study Design: Retrospective cohort. Setting: Tertiary referral center. Patients: Eleven patients, with MCF floor defects. Interventions: MCF floor defect surgical repairs with either fascia or fascia and bone graft. Main Outcome Measured: Hearing outcomes. Results: Eleven patients (two men) aged 34 to 82 years (median, 62 yrs) were identified. All patients were operate...
Source: Otology and Neurotology - Category: ENT & OMF Tags: MIDDLE EAR AND MASTOID DISEASE Source Type: research
AbstractThe role of otolaryngologist in the management of CSF rhinorrhea is expanding. It is prudent to evaluate all cases of CSF leak, understand the etiological causes and formalize a working approach to management of such cases to yield better outcome. A retrospective descriptive study of 39 cases of proven CSF rhinorrhea treated at a tertiary care center between January 2014 to August 2019. The data retrieved were analyzed for age, gender, weight, etiology, recurrence of the disease, history of meningitis, nasal or neurosurgical intervention in past, imaging inform of CT and MR cisternography as and when required. This...
Source: Indian Journal of Otolaryngology and Head and Neck Surgery - Category: ENT & OMF Source Type: research
Our technical consulting group at Nelson Laboratories acts as a bridge between medical device companies and regulatory bodies, putting us in a good position to understand both sides and what they need from each other. This also creates an opportunity for us to recognize the typical deficiencies, mishaps, or errors that are identified by regulatory agencies during the submission process and to provide our expertise to medical device manufacturers about how to structure and document their submission paperwork to avoid some of these common mistakes. Oftentimes we receive feedback from FDA or a notified body that a submission ...
Source: MDDI - Category: Medical Devices Authors: Tags: Regulatory and Compliance Source Type: news
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