Moyamoya Syndrome in a Child with β-Thalassemia Major

2020 ◽  
Vol 18 (04) ◽  
pp. 214-216
Author(s):  
Soumya Roy

AbstractA 9-year-old girl patient presented with left-sided weakness and joint contractures developing over a period of 18 months. She was known to be suffering from β-thalassemia major and was on regular blood transfusions. Eighteen months ago, she had suffered from an episode of ischemic cerebrovascular accident affecting the right side of her brain. Magnetic resonance angiogram revealed vaso-occlusive disease affecting mainly the anterior cerebral circulation, resembling Moyamoya disease. She was advised to carry out regular physiotherapy but her parents discontinued it, which resulted in the gradual development of joint contractures and muscle wasting.

Author(s):  
Gyani J. S. Birua ◽  
Gaurav Tyagi ◽  
Manish Beniwal ◽  
Dwarakanath Srinivas ◽  
Shilpa Rao

AbstractParenchymal perianeurysmal cysts are rare. We report a case of 50-year-old woman who presented with persistent headaches and episodes of vomiting for the last 2 months. Magnetic resonance imaging of the brain showed a well-defined solitary cystic lesion with a mural nodule measuring 5.4 × 5.2 × 4.6 cm in the right basifrontal region. The mural nodule was cortically based. It was hypointense on T2-weighted fluid-attenuated inversion recovery and showed intense contrast enhancement with few nonenhancing areas—no evidence of diffusion restriction. The cyst wall was nonenhancing, and magnetic resonance angiogram was unremarkable. Differential diagnoses included intra-axial gliomas such as ganglioglioma and pleomorphic xanthoastrocytoma. Right pterional craniotomy and a transcortical approach were made. Subtotal excision of cyst and clipping of right middle cerebral artery bifurcation thrombosed aneurysm were done. After 6 months of follow-up, patient is stable without any deficits. A parenchymal perianeurysmal cyst is a rare entity; it is crucial to be considered a differential diagnosis in any cystic lesion with the mural nodule.


2019 ◽  
Vol 12 (12) ◽  
pp. e231320
Author(s):  
Mário José Pereira-Lourenço ◽  
Duarte Vieira-Brito ◽  
João Pedro Peralta ◽  
Noémia Castelo-Branco

This case report describes the case of a 37-year-old man that noticed an intrascrotal right mass with 1 month of evolution. During physical exam presented with a large mass at the inferior portion of the right testicle, clearly separated from the testicle, with a tender consistency and mobile. An ultrasound was performed that showed a solid and subcutaneous nodular lesion, extra testicular, heterogeneous, measuring 7.2 cm. Pelvic magnetic resonance imageMRI showed a lesion compatible with a lipoma. The patient was subjected to surgical excision of the lesion by scrotal access, having histology revealed a lipoblastoma (LB) of the scrotum. Histological diagnosis was obtained by microscopic characteristics (well-circumscribed fatty neoplasm) and immunohistochemistry (stains for CD34, S100 protein and PLAG1 were positive; stains for MDM2 and CDK4 were negative). LB is extremely rare after adolescence in any location, being this first described case of intrascrotal LB described in adulthood.


2020 ◽  
Vol 10 (1) ◽  
pp. 14
Author(s):  
Cezary Grochowski ◽  
Kamil Jonak ◽  
Marcin Maciejewski ◽  
Andrzej Stępniewski ◽  
Mansur Rahnama-Hezavah

Purpose: The aim of this study was to assess the volumetry of the hippocampus in the Leber’s hereditary optic neuropathy (LHON) of blind patients. Methods: A total of 25 patients with LHON were randomly included into the study from the national health database. A total of 15 patients were selected according to the inclusion criteria. The submillimeter segmentation of the hippocampus was based on three-dimensional spoiled gradient recalled acquisition in steady state (3D-SPGR) BRAVO 7T magnetic resonance imaging (MRI) protocol. Results: Statistical analysis revealed that compared to healthy controls (HC), LHON subjects had multiple significant differences only in the right hippocampus, including a significantly higher volume of hippocampal tail (p = 0.009), subiculum body (p = 0.018), CA1 body (p = 0.002), hippocampal fissure (p = 0.046), molecular layer hippocampus (HP) body (p = 0.014), CA3 body (p = 0.006), Granule Cell (GC) and Molecular Layer (ML) of the Dentate Gyrus (DG)–GC ML DG body (p = 0.003), CA4 body (p = 0.001), whole hippocampal body (p = 0.018), and the whole hippocampus volume (p = 0.023). Discussion: The ultra-high-field magnetic resonance imaging allowed hippocampus quality visualization and analysis, serving as a powerful in vivo diagnostic tool in the diagnostic process and LHON disease course assessment. The study confirmed previous reports regarding volumetry of hippocampus in blind individuals.


Diagnostics ◽  
2020 ◽  
Vol 11 (1) ◽  
pp. 1
Author(s):  
Maria Vlachou ◽  
Vasileios Kamperidis ◽  
Efthymia Vlachaki ◽  
Georgios Tziatzios ◽  
Despoina Pantelidou ◽  
...  

Patients with beta-thalassemia major (β-ΤΜ) may develop cardiac arrhythmias through a multifactorial mechanism. The current study evaluated the association of cardiac structure and function on echocardiography with atrial ectopic burden on 24-hour tape recording in β-ΤΜ patients. This prospective study included consecutive β-ΤΜ patients. Demographic, laboratory, echocardiographic, cardiac magnetic resonance (CMR) T2* and 24-hour tape recording data were prospectively collected. The patients were classified according to the median value of premature atrial contractions (PACs) on 24-hour tape. In total, 50 β-TM patients (37.6 ± 9.1 years old, 50% male) were divided in 2 groups; PACs ≤ 24/day and > 24/day. Patients with PACs > 24/day were treated with blood transfusion for a longer period of time (39.0 ± 8.6 vs. 32.0 ± 8.9 years, p < 0.007), compared to their counterparts. Older age (OR: 1.121, 95% CI: 1.032–1.217, p = 0.007), longer duration of blood transfusion (OR:1.101, 95% CI:1.019–1.188, p = 0.014), larger LV end-diastolic diameter (OR: 4.522, 95% CI:1.009–20.280, p = 0.049), higher values of LA peak systolic strain (OR: 0.869, 95% CI: 0.783–0.964, p = 0.008), higher MV E/E′ average (OR: 1.407, 95% CI: 1.028–1.926, p = 0.033) and higher right ventricular systolic pressure (OR: 1.147, 95% CI: 1.039–1.266, p = 0.006) were univariably associated with PACs > 24/day. LA peak systolic strain remained significantly associated with PACs > 24/day after adjusting for the duration of blood transfusions or for CMR T2*. The multivariable model including blood transfusion duration and LA peak systolic strain was the most closely associated with PACs > 24/day. Receiver operating characteristic curve analysis identified a left atrial peak systolic strain of 31.5%, as the best cut-off value (83% sensitivity, 68% specificity) for prediction of PACs > 24/day. In β-TM patients, LA peak systolic strain was associated with the atrial arrhythmia burden independently to the duration of blood transfusions and CMR T2*.


2016 ◽  
Vol 30 (1) ◽  
pp. 88-91 ◽  
Author(s):  
Alfredo Di Gaeta ◽  
Francesco Giurazza ◽  
Eugenio Capobianco ◽  
Alvaro Diano ◽  
Mario Muto

To identify and localize an intraorbital wooden foreign body is often a challenging radiological issue; delayed diagnosis can lead to serious adverse complications. Preliminary radiographic interpretations are often integrated with computed tomography and magnetic resonance, which play a crucial role in reaching the correct definitive diagnosis. We report on a 40 years old male complaining of pain in the right orbit referred to our hospital for evaluation of eyeball pain and double vision with an unclear clinical history. Computed tomography and magnetic resonance scans supposed the presence of an abscess caused by a foreign intraorbital body, confirmed by surgical findings.


1995 ◽  
Vol 18 (2) ◽  
pp. 118-121 ◽  
Author(s):  
Tae Kyoung Kim ◽  
Yeon Hyoen Choe ◽  
Hak Soo Kim ◽  
Jae Kon Ko ◽  
Young Tak Lee ◽  
...  

Metabolites ◽  
2021 ◽  
Vol 11 (3) ◽  
pp. 176
Author(s):  
Justine Deborne ◽  
Noël Pinaud ◽  
Yannick Crémillieux

The aim of this study was to evaluate the potential of a miniaturized implantable nuclear magnetic resonance (NMR) coil to acquire in vivo proton NMR spectra in sub-microliter regions of interest and to obtain metabolic information using magnetic resonance spectroscopy (MRS) in these small volumes. For this purpose, the NMR microcoils were implanted in the right cortex of healthy rats and in C6 glioma-bearing rats. The dimensions of the microcoil were 450 micrometers wide and 3 mm long. The MRS acquisitions were performed at 7 Tesla using volume coil for RF excitation and microcoil for signal reception. The detection volume of the microcoil was measured equal to 450 nL. A gain in sensitivity equal to 76 was found in favor of implanted microcoil as compared to external surface coil. Nine resonances from metabolites were assigned in the spectra acquired in healthy rats (n = 5) and in glioma-bearing rat (n = 1). The differences in relative amplitude of choline, lactate and creatine resonances observed in glioma-bearing animal were in agreement with published findings on this tumor model. In conclusion, the designed implantable microcoil is suitable for in vivo MRS and can be used for probing the metabolism in localized and very small regions of interest in a tumor.


2008 ◽  
Vol 49 (9) ◽  
pp. 1058-1067 ◽  
Author(s):  
L. Han ◽  
X. Zhang ◽  
S. Qiu ◽  
X. Li ◽  
W. Xiong ◽  
...  

Background: Gliosarcomas are rare tumors with mixed glial and mesenchymal components. Many of their radiologic features resemble those of other primary brain malignancies. Purpose: To investigate the magnetic resonance (MR) imaging features of gliosarcomas. Material and Methods: We retrospectively reviewed the MR images, pathology reports, and clinical information of 11 male and four female patients aged 15–71 years to evaluate the location, morphology, enhancement, and other features of their pathologically confirmed gliosarcomas. Results: Apart from one tumor in the right cerebellar hemisphere, all were supratentorial. Two tumors were intraventricular, and four involved the corpus callosum. The tumors were well demarcated, with an inhomogeneous or cystic appearance and moderate-to-extensive surrounding edema. Thick walls with strong rim and ring-like enhancement were observed in 13 (87%). Seven (47%) showed intratumoral paliform enhancement. Conclusion: Gliosarcoma demonstrates certain characteristic MR features, such as supratentorial and peripheral location, well-demarcated, abutting a dural surface, uneven and thick-walled rim-like or ring enhancement, as well as intratumoral strip enhancement. These findings, combined with patient age, can aid the differential diagnosis of gliosarcomas from more common primary brain tumors.


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