Effect of Liver Transplant on Neurologic Manifestations and Brain Magnetic Resonance Imaging Findings in Wilson Disease.

Objectives: Wilson disease is an autosomal recessive disorder of copper metabolism. It leads to copper accumulation in various organs (liver, eye, brain) and deteriorates their functions. Symptoms usually appear in the second and third decades of life.

Reliability of Real-Time Sonoelastography in the Diagnosis of Supraspinatus Tendinopathy.

The practicality of real-time sonoelastography in the diagnosis of tendinopathy is being discussed since the beginning of its use in musculoskeletal system. The aims of this study were to compare the elasticity of pathologic supraspinatus tendon with the uninvolved side by sonoelastography and to determine the relationship between the sonoelastographic findings and magnetic resonance imaging (MRI) grade of the tendinosis. Eighty-2 patients (50 males, 32 females, mean ± SD age = 53.

Assessment of Subclinical Atherosclerosis in Vitamin D Deficiency.

Purpose: Patients with vitamin D deficiency (VDD) have potential to have increased cardiovascular morbidity and mortality. In this study, we aimed to discover the findings of early atherosclerosis in patients with VDD by measuring carotid intima-media thickness (CIMT) and epicardial fat thickness (EFT).

Materials And Methods: The study population includes 52 patients with VDD (n = 30 [57% female], mean ± SD age 54.

The prevalence of spondyloarthropathy in fibromyalgia patients.

Objective: To document the prevalence of Spondyloarthropathy (SpA) with an evaluation of patients previously diagnosed with fibromyalgia syndrome (FMS).

Methods: The patients diagnosed with FMS before telephoned and asked three questions to determine for inflammatory back pain. American College of Rheumatology (ACR) Fibromyalgia criteria 1990 and ACR 2010 and for diagnosing patients with SpA; criteria from the European Spondyloarthropathy Study Group (ESSG), and Amor were applied.

MRI Findings in Childhood PRES: What is Different than the Adults?

Purpose: Posterior reversible encephalopathy syndrome (PRES) is a clinical scenario with convulsion, vision abnormalities, altered mental status, and headaches in the presence of an underlying etiology, and the diagnosis can be made by support of radiological studies. In this study, we evaluated the magnetic resonance imaging (MRI) findings of PRES in children and compared our findings with that of the known features in adults, and reviewed the possible pathophysiological reasons that may cause the difference.

Materials And Methods: A total of 29 children (13 male, 16 female, aged 1-17 years, mean age: 10 years) diagnosed as having PRES were retrospectively reviewed.

Intraosseous angiolipoma of the frontal bone with a unique location: a clinical and pathological case illustration and review of the literature.

Here, we report a case of a 16-year-old female patient was referred with scalp swelling and headache. Her neurological examination was normal and imaging of the skull revealed a well-defined lytic lesion measuring 15 mm × 6 mm to the right of the frontal bone. She was operated on with a prediagnosis of Langerhans cell histiocytosis.

A collodion baby with facial dysmorphism, limb anomalies, pachygyria and genital hypoplasia: a mild form of Neu-laxova syndrome or a new entity?

Neu-Laxova syndrome is a rare, lethal, autosomal recessive disorder characterized by intrauterine growth retardation, central nervous system anomalies, skin findings, such as ichthyosis, edema, collodion baby and harlequin fetus, facial dysmorphic features, limb anomalies and genital hypoplasia. Although it is generally a lethal condition, cases of such patients who lived beyond 6 months and 10 months of age have been reported. Here, we describe an 8-year-old boy who was born with collodion membrane, facial dysmorphic features, limb anomalies, genital hypoplasia and pachygyria.

Diagnosis of early sacroiliitis in seronegative spondyloarthropathies by DWI and correlation of clinical and laboratory findings with ADC values.

Purpose: Sacroiliitis is one of the diagnostic criteria of seronegative SpA. The purpose of our study is to show the signal characteristics of the sacral and iliac surfaces by DWI which may contribute in early diagnosis of sacroiliitis and investigate the correlation between ADC values and clinical and laboratory parameters.

Materials And Methods: 62 patients with inflammatory low back pain, with a history or suspect of seronegative SpA are enrolled into the study.

Embolic infarct presented with bilateral thalamic lesions possibly initiated by an emboli to the artery of Percheron.

Bilateral thalamic infarcts are rare and present with varying symptoms. Cardioembolism and small artery disease are the most common etiologic factors. The occlusion of a rare arterial variant called the artery of Percheron results in bilateral thalamic infarcts.

Dual anca positivity in a child with moyamoya-like cerebral vascular changes: an unusual presentation with sudden homonymous hemianopsia.

A 12-year-old girl presented with a sudden decrease in her right visual acuity and homonymous hemianopsia. An angiography of the retinal arteries demonstrated recanalized occlusion of the right retinal artery. Cerebral angiography showed bilateral internal carotid artery stenosis associated with the development of collateral circulation.

Tissue Doppler study of the right ventricle with a multisegmental approach: comparison with cardiac magnetic resonance imaging.

Objective: We investigated whether systolic right ventricular (RV) function and myocardial performance index (MPI) studied with a multisegmental approach improve the accuracy of RV function estimation based on tricuspid lateral annulus.

Methods: Systolic and isovolumic contraction velocities, isovolumic acceleration, regional MPI from the tricuspid lateral and septal annulus, and basal and apical RV free wall and blood pool MPI were obtained in 69 patients and compared with RV ejection fraction (EF) by cardiac magnetic resonance.

Results: Average systolic velocity from 2 annular sites had the highest correlation to the RVEF (r = 0.

The effects of interferon beta-1a on proton MR spectroscopic imaging in patients with multiple sclerosis, a controlled study, preliminary results.

To evaluate the effects of interferon beta-1a(INFbeta-1a) on brain metabolites in patients with multiple sclerosis (MS), we performed Magnetic Resonance Spectroscopy Imaging (MRSI) on five patients treated with INFbeta-1a (Rebif 44 microg), and on five untreated patients. Six healthy volunteers were used as controls. Patients were evaluated at the beginning, in the first, third, sixth, and twelfth month.

[Hemicentral retinal artery occlusion associated with moyamoya syndrome].

Moyamoya syndrome is a rare cerebrovascular disorder characterized by bilateral progressive supraclinoidal stenosis or occlusion of the internal carotid artery and development of collateral vessels in the lenticulostriate region. The syndrome manifests itself in association with another disease or other clinical symptoms. We present the case of a 12-year-old girl with hemicentral retinal artery occlusion due to moyamoya syndrome associated with clinical and laboratory findings of presumed systemic lupus erythematosus.

Brachydactyly short-stature hypertension syndrome: a case with associated vascular malformations.

Brachydactyly short-stature hypertension syndrome, also known as hypertension-with-brachydactyly (HTNB) syndrome, is a rare autosomal dominant disorder that was first described by Bilginturan and colleagues in 1973. Many familial cases of HTNB have been reported, but the first sporadic case of this condition was published only recently. This article describes a case of HTNB syndrome in a 16-year-old boy.

Value of whole-body turbo short tau inversion recovery magnetic resonance imaging with panoramic table for detecting bone metastases: comparison with 99MTc-methylene diphosphonate scintigraphy.

Objective: The objectives of this study were to assess the efficacy and reliability of whole-body turbo short tau inversion recovery (STIR) magnetic resonance imaging (MRI) for detecting skeletal metastasis and to compare the results with those of bone scintigraphy.

Methods: Twenty-six patients with primary cancer (mean age=56 years, age range: 34-75 years) were assessed for bone metastasis with whole-body MRI and bone scintigraphy. Eight bone regions in each patient were assessed (total of 208 sites) with each of these 2 techniques.

Two cases of glycine encephalopathy accompanied by pes equinovarus.

Glycine encephalopathy is a rare autosomal recessive metabolic disease characterized by glycine accumulation in body fluids owing to a defect in the glycine cleavage system. There are several forms of glycine encephalopathy. In the classic or neonatal form, symptoms usually develop as neurologic symptoms in the first few days of life.

Familial arachnoid cysts in association with autosomal dominant polycystic kidney disease.

Autosomal dominant polycystic kidney disease (ADPKD) is a systemic disorder well-known for its association with intracranial aneurysms. Recently, intracranial arachnoid cysts have also been reported to be associated with ADPKD. We describe a father and daughter who each has a posterior fossa arachnoid cyst and asymptomatic ADPKD.

Lumbar myxopapillary ependymoma mimicking neurofibroma.

Objective: To report a case of lumbar myxopapillary ependymoma in whom neuroradiological and surgical findings strongly suggested neurofibroma.

Clinical Presentation: The patient presented with a 2 year history of progressive monoparesthesia and monoparesia of his right leg. He reported having minimal fecal and urinary incontinence.

A case of incidentally detected Castleman's disease with retroperitoneal paravertebral localization.

Castleman's disease, or angiofollicular lymph node hyperplasia, is a fairly rare benign tumor of lymphoid origin. The expected localization is mediastinum and rarely retroperitoneum. An asymptomatic case is reported with multimodality imaging and postoperative findings.

Giant posterior fossa teratoma.

In this study we report a rare case of a giant midline posterior fossa teratoma; its clinical presentation, radiological appearance, treatment and outcome, with an extensive review of the literature.