Teaching NeuroImages: Myeloperoxidase-anti-neutrophil cytoplasmic antibody-positive hypertrophic pachymeningitis
A 49-year-old woman with chronic epistaxis presented with painless left monocular vision loss. Notable findings on examination of the left eye included visual acuity of 20/200 and relative afferent pupillary defect. MRI of the brain revealed enhancement of the left optic nerve sheath and diffuse dural thickening (figure). Laboratory workup yielded only lymphocytic pleocytosis (12 white blood cells) and positive serologies for perinuclear anti-neutrophil cytoplasmic antibodies (ANCA) and anti-myeloperoxidase (MPO) antibodies. Dural biopsy showed multifocal dense lymphoplasmacytic infiltration with vasculitis and reactive fibroplasia. A diagnosis of MPO-ANCA-positive hypertrophic pachymeningitis was made. This phenotypic variant of granulomatosis with polyangiitis is typically restricted to the CNS and upper airway; treatment involves immunosuppression.1 The patient improved clinically and radiographically with prednisone and rituximab.
Authors: Martin-Marcuartu JJ, Lojo-Ramirez JA, Pachón-Garrudo VM, Jimenez-Hoyuela JM PMID: 29776655 [PubMed - as supplied by publisher]
Authors: Protopopov M, Poddubnyy D Abstract INTRODUCTION: Non-radiographic axial spondyloarthritis (nr-axSpA) represents a subtype of axial spondyloarthritis (axSpA) with no significant structural damage in sacroiliac joints and spine. Despite that, patients with nr-axSpA demonstrate a substantial burden of illness, and a considerable share of them might progress to radiographic axSpA (r-axSpA) over time. The amount and quality of published data allows crude estimation of progression rate and factors related to a higher risk of progression. Areas covered: This review discusses the available data reporting the rates...
CONCLUSIONS: Sufficient attention is required for middle-aged males before and during exposure to long-term ADV therapy, regardless of nationality. The clinical picture, laboratory and radiograph alterations are important clues for those patients and are usually characterized by polyarthralgia, renal tubular dysfunction and mineralization defects. Implementation of an early renal tubular injury index is recommended for patients with higher risk, which would prevent further renal injury. PMID: 29769119 [PubMed - in process]
We present a girl with severe multisystem LCH who responded only to vemurafenib. After 8 months of treatment, vemurafenib was tapered and replaced by prednisone and vinblastine, a strategy which has not been described to date. Despite chemotherapy, early relapse occurred, but remission was achieved by re-institution of vemurafenib. Further investigation needs to address the optimal duration of vemurafenib therapy in LCH and whether and which chemotherapeutic regimen may prevent disease relapse after cessation of vemurafenib. PMID: 29774135 [PubMed]
Conclusions: When AA was administered without goserilin, only 78% achieved castration levels. AA combined with SRT and goserilin did not increase pelvic toxicity, but lead to an unsuspected high frequency of grade 3 liver toxicity. The phase II recommended dose of AA combined to goserelin and SRT is 750 mg. PMID: 29774129 [PubMed]
Authors: Bhatti M, Ippolito T, Mavis C, Gu J, Cairo MS, Lim MS, Hernandez-Ilizaliturri F, Barth MJ Abstract Though outcomes for pediatric Burkitt lymphoma (BL) have improved significantly in recent decades with intensive multi-agent chemotherapy and the addition of rituximab, chemotherapy resistance remains a significant impediment to cure following relapse. Activation of the PI3K/AKT pathway has been implicated in Burkitt lymphomagenesis and increased PI3K/AKT activation has been associated with worse outcomes in adults with aggressive B-cell non-Hodgkin lymphoma (B-NHL). Inhibitors of the PI3K/AKT pathway have be...
We describe the case of a 35-year-old woman with solitary osseous anaplastic plasmacytoma that presented initially with a pathological fracture following minor trauma. The patient was immunocompetent and had no predisposing conditions for a plasma cell tumor. Left lower extremity radiographs revealed an oblique fracture of the distal femur, and CT imaging indicated a primary osseous lesion at the fracture site. MRI confirmed the diagnosis of pathological fracture. Initial surgical pathology of the lesion was concerning because it could have been an osteosarcoma. Further immunostaining demonstrated CD138 positivity and kapp...
AbstractMulticentric carpotarsal osteolysis (MCTO) is a rare skeletal disorder characterized by progressive carpal and tarsal destruction. The upper and lower limbs may be involved, leading to deformities and joint limitation. These anatomic features may be associated with progressive renal failure. The radiographs obtained during childhood showed a carpal and tarsal osteolysis and an asymmetrical involvement. Here, we report on the long-term clinical and radiological findings of three patients with skeletal manifestations of MCTO.
This report provides support in differential diagnosis to help discriminate potentially self-limiting conditions from other diseases that may require invasive diagnosis and/or therapy.
ConclusionMRI of the left hand is a feasible alternative to hand radiographs for skeletal age estimation in adolescents using the GP criteria with 2 SD. Using 1 SD, the age of healthy volunteers tended to be estimated as higher than the chronologic age. Future studies should evaluate the results in a larger number of participants.