A comprehensive analysis of the epidemiology and clinical characteristics of anti-LRP4 in myasthenia gravis

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Bmp7 Regulates the Survival, Proliferation, and Neurogenic Properties of Neural Progenitor Cells during Corticogenesis in the Mouse

Bone morphogenetic proteins (BMPs) are considered important regulators of neural development. However, results mainly from a wide set of in vitro gain-of-function experiments are conflicting since these show that BMPs can act either as inhibitors or promoters of neurogenesis. Here, we report a specific and non-redundant role for BMP7 in cortical neurogenesis in vivo using knockout mice. Bmp7 is produced in regions adjacent to the developing cortex; the hem, meninges, and choroid plexus, and can be detected in the cerebrospinal fluid. Bmp7 deletion results in reduced cortical thickening, impaired neurogenesis, and loss of radial glia attachment to the meninges. Subsequent in vitro analyses of E14.5 cortical cells revealed that lack of Bmp7 affects neural progenitor cells, evidenced by their reduced proliferation, survival and self-renewal capacity. Addition of BMP7 was able to rescue these proliferation and survival defects. In addition, at the developmental stage E14.5 Bmp7 was also required to maintain Ngn2 expression in the subventricular zone. These data demonstrate a novel role for Bmp7 in the embryonic mouse cortex: Bmp7 nurtures radial glia cells and regulates fundamental properties of neural progenitor cells that subsequently affect Ngn2-dependent neurogenesis

Anti-myelin-associated glycoprotein polyneuropathy coexistent with CREST syndrome

Clinical involvement of the peripheral nervous system in the calcinosis cutis, raynaud′s phenomenon, esophageal dismotility, sclerodactyly and telangiectasia (CREST) variant of systemic sclerosis occurs infrequently and is characterized by axonal degeneration due to necrotizing vasculitis. We report a female patient with a known history of CREST syndrome, which developed a slowly progressive, distal symmetric demyelinating sensorimotor polyneuropathy (PN), with tremor and ataxia as prominent features, compatible with anti-myelin associated glycoprotein (MAG) PN. The diagnosis of PN was established by the presence of monoclonal immunoglobulin M anti-MAG antibodies (Thin-Layer Chromatography, Western Blot and enzyme-linked immunoabsorbent assay). Given the evidence that in CREST activation of T-helper cells is observed and that anti-MAG antibodies, despite the fact that they are T-cell-independent, may be influenced by an increase in T-helper function, the coexistence of these two rare autoimmune disorders in the same patient may not be incidental but related to the underlying immunological mechanisms involved

AchR-positive myasthenia gravis with MRI evidence of early muscle atrophy

Muscle atrophy, when it occurs in myasthenia gravis (MG), is usually associated with long-standing disease or with chronic corticosteroid treatment. Early muscle atrophy in a steroid-naive patient has been reported previously only in muscle-specific tyrosine kinase (MuSK)-MG. We report a 63-year-old male patient with acetylcholine receptor (AchR)-positive MG with a short duration of disease, no steroid treatment and MRI evidence of muscle atrophy. © 2012 Elsevier Ltd. All rights reserved

Patients with ocular symptoms referred for electrodiagnosis: how many of them suffer from myasthenia gravis?

The aim of this study was the diagnosis of patients with isolated ocular manifestations (ptosis and/or diplopia) referred for electrophysiological evaluation to the electrodiagnostic laboratory of a University Neurological Department. Examination was performed either in inpatient status or in outpatient basis. We analyzed the clinical, electrophysiological and other laboratory data in 79 subjects. Myasthenia gravis (MG) was diagnosed in 38 %, 45.6 % in other diseases (Graves disease, blepharospasm, IIId cranial verve palsy, multiple sclerosis, stroke, etc.), while in 16.5 %, the cause remained unidentified. Symptoms fluctuation was significantly more frequent in the myasthenic patients, compared to patients with other diseases. The presence of both diplopia and ptosis are more likely due to MG rather than other pathology. © 2015, Belgian Neurological Society