SAPHO Syndrome Presenting With Atlo-Epistrophic Synovitis and Left Vocal Cord Paresis: A Challenging Diagnosis.

SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) is a rare syndrome mainly characterized by cutaneous and osteoarticular manifestations. The most typical osteoarticular manifestations are localized to the anterior chest wall and include a usually noninfectious osteitis, hyperostosis, and synovitis of the sternoclavicular joints. However, clinical presentation of SAPHO syndrome can be quite heterogeneous.

Cerebral Microstructure Analysis by Diffusion-Based MRI in Systemic Lupus Erythematosus: Lessons Learned and Research Directions.

Diffusion-based magnetic resonance imaging (MRI) studies, namely diffusion-weighted imaging (DWI) and diffusion-tensor imaging (DTI), have been performed in the context of systemic lupus erythematosus (SLE), either with or without neuropsychiatric (NP) involvement, to deepen cerebral microstructure alterations. These techniques permit the measurement of the variations in random movement of water molecules in tissues, enabling their microarchitecture analysis. While DWI is recommended as part of the initial MRI assessment of SLE patients suspected for NP involvement, DTI is not routinely part of the instrumental evaluation for clinical purposes, and it has been mainly used for research.

Longitudinal changes in cerebral white matter microstructure in newly diagnosed systemic lupus erythematosus patients.

Objectives: To evaluate longitudinal variations in diffusion tensor imaging (DTI) metrics of different white matter (WM) tracts of newly diagnosed SLE patients, and to assess whether DTI changes relate to changes in clinical characteristics over time.

Methods: A total of 17 newly diagnosed SLE patients (19-55 years) were assessed within 24 months from diagnosis with brain MRI (1.5 T Philips Achieva) at baseline, and after at least 12 months.

Bilateral strio-pallido-dentate calcinosis (Fahr's disease): report of seven cases and revision of literature.

Background: Fahr's disease is rare a neurodegenerative idiopathic condition characterized by symmetric and bilateral calcifications of basal ganglia, usually associated with progressive neuropsychiatric dysfunctions and movement disorders. The term "Fahr's syndrome" is used in presence of calcifications secondary to a specific cause, but the variability of etiology, pathogenesis, and clinical picture underlying this condition have raised the question of the real existence of a syndrome. Several classifications based on the etiology, the location of brain calcifications and the clinical presentation have been proposed.

Cerebral Blood Volume ASPECTS Is the Best Predictor of Clinical Outcome in Acute Ischemic Stroke: A Retrospective, Combined Semi-Quantitative and Quantitative Assessment.

Introduction: The capability of CT perfusion (CTP) Alberta Stroke Program Early CT Score (ASPECTS) to predict outcome and identify ischemia severity in acute ischemic stroke (AIS) patients is still questioned.

Methods: 62 patients with AIS were imaged within 8 hours of symptom onset by non-contrast CT, CT angiography and CTP scans at admission and 24 hours. CTP ASPECTS was calculated on the affected hemisphere using cerebral blood flow (CBF), cerebral blood volume (CBV) and mean transit time (MTT) maps by subtracting 1 point for any abnormalities visually detected or measured within multiple cortical circular regions of interest according to previously established thresholds.

Fluctuation relations for driven coupled classical two-level systems with incomplete measurements.

We theoretically investigate fluctuation relations in a classical incomplete measurement process where only partial information is available. The scenario we consider consists of two coupled single-electron boxes where one or both devices can undergo a nonequilibrium transformation according to a chosen protocol. The entropy production of only one of the two boxes is recorded and fluctuation relations for this quantity are put to a test, showing strong modifications whose nature depends upon the specific case study.

Temporal changes in perihematomal apparent diffusion coefficient values during the transition from acute to subacute phases in patients with spontaneous intracerebral hemorrhage.

Introduction: Diffusion-weighted imaging (DWI) studies focusing on apparent diffusion coefficient (ADC) abnormalities have provided conflicting results about the nature and fate of perihematomal edema.

Methods: We investigated 35 patients with supratentorial spontaneous intracerebral hemorrhage (SICH) by using DWI scanning obtained at 48 h and 7 days after symptom onset. Regional ADC (rADC) values were measured in three manually outlined regions of interest: (1) the perihematomal hyperintense area, (2) 1 cm of normal appearing brain tissue surrounding the perilesional hyperintense rim, and (3) a mirror area, including the clot and the perihematomal region, located in the contralateral hemisphere.

Central nervous system involvement in Sjögren's syndrome: unusual, but not unremarkable--clinical, serological characteristics and outcomes in a large cohort of Italian patients.

Objectives: To perform an observational retrospective cross-sectional case-control study to evaluate prevalence, clinical patterns and outcomes of CNS involvement in a large cohort of primary SS (pSS) patients.

Methods: A total of 424 pSS patients, diagnosed according to the 2002 criteria proposed by the American-European Consensus Group, were checked for CNS involvement after exclusion of secondary causes. Demographic, clinical, seroimmunological data were compared between patients with and without CNS involvement.

Potential role of CT perfusion parameters in the identification of solitary intra-axial brain tumor grading.

In this study, neoplastic perfusion abnormalities were investigated by computed tomography perfusion (CTP) scanning in 38 patients with solitary intra-axial brain tumors (19 with high grade gliomas, 7 with low grade gliomas and 12 with brain metastasis). Regional cerebral blood flow (rCBF), cerebral blood volume (rCBV), mean transit time (rMTT) and permeability surface flow (rPSF) levels were measured in two different regions of interest: (1) enhancing or non-enhancing tumor tissue and (2) a mirror area of apparently normal brain tissue located in the contralateral hemisphere. rCBF mean levels were greater in tumoral tissue than in the contralateral area for high-grade gliomas (p < 0.

Percutaneous vertebroplasty and spinal cord compression: a case report.

This report describes a 60-year-old woman with intensive back pain due to metastatic vertebral body collapse, who underwent percutaneous vertebroplasty. Subsequently, the patient developed metastatic lesion extrusion into the spinal canal because of pressure of the cement, with compression of the left anterolateral spinal cord. During percutaneous vertebroplasty procedure in patient with malignant tumors, the complication rate increases owing to the risk of leakage of cement resulting from the vertebral body destruction, but as also seen in our case, for the extrusion of the neoplastic tissue and increase of the pressure in the vertebral body due to the introduction of the cement.

Thrombosis of the cerebral veins and sinuses in acute promyelocytic leukemia after all-trans retinoic acid treatment: a case report.

Thrombosis of the cerebral veins or sinuses is a rare cerebrovascular disorder, which seldom represents a complication of acute promyelocytic leukemia. To the best of our knowledge, it never occurred during treatment with all-trans retinoic acid. We report a case of a 35-year-old woman affected by acute promyelocytic leukemia, who developed massive thrombosis of the cerebral sinuses and veins when she was in complete morphological and molecular remission after all-trans retinoic acid and idarubicin treatment.

CT perfusion mapping of hemodynamic disturbances associated to acute spontaneous intracerebral hemorrhage.

Introduction: We sought to quantify perfusion changes associated to acute spontaneous intracerebral hemorrhage (SICH) by means of computed tomography perfusion (CTP) imaging.

Materials And Methods: We studied 89 patients with supratentorial SICH at admission CT by using CTP scanning obtained within 24 h after symptom onset. Regional cerebral blood flow (rCBF), cerebral blood volume (rCBV) and mean transit time (rMTT) levels were measured in four different regions of interest manually outlined on CT scan: (1) hemorrhagic core; (2) perihematomal low-density area; (3) 1 cm rim of normal-appearing brain tissue surrounding the perilesional area; and (4) a mirrored area, including the clot and the perihematomal region, located in the non-lesioned contralateral hemisphere.

Merkel cell tumour of the external ear. Report of a case.

Merkel cells carcinoma (MCC) is an uncommon skin lesion, considered a malignancy of the neuroendocrine system, which is found mainly in elderly people. Its incidence is highly correlated with sun exposure or immunodeficiency syndromes. MCC is often an aggressive tumour with high tendency for local recurrence, lymph node involvement and distant metastasis.

Neurological involvement in North Italian patients with Behçet disease.

The aim of this study was to evaluate neurological involvement in a series of 110 North Italian patients with Behçet disease (BD), a multisystemic vasculitis of unknown origin, followed up for a period of 5 years. During this time, 27 (24.5%) patients with neuro-BD were identified.