Adult presentation of subacute necrotizing encephalomyelopathy in two siblings

Subacute necrotizing encephalomyelopathy (SNE) or Leigh syndrome is a rare progressive neurodegenerative mitochondrial disorder typically manifesting in the pediatric age group with variable clinical presentation and genetic heterogeneity. Late-onset varieties are extremely rare and only few cases have been reported globally. Neuroimaging however shows characteristic symmetrical necrotic lesions in the basal ganglia and/or brainstem. We report cases of two siblings who had history of seizures, but presented to us in adulthood. They had similar clinical presentation and radiological features on magnetic resonance imaging (MRI) and were subsequently diagnosed with SNE primarily based on imaging

Lumbar artery pseudoaneurysm following renal biopsy.

Lumbar artery pseudoaneurysms have previously been described as rare iatrogenic complications following percutaneous interventional procedures involving the flanks. We describe a case of a 71-year-old man who became unstable and dropped 3 grams of hemoglobin within 24 hours following renal biopsy. A post-biopsy hemorrhage was suspected, and a pseudoaneurysm of his second right lumbar (L2) artery was found on computed tomography angiogram (CTA). Successful coil embolization was performed in the right L2 artery. This case discusses the diagnostic and therapeutic challenges of this unusual complication as well as the anatomical and technical factors involved in the embolization of the lumbar arteries

MELAS: a complex and challenging diagnosis

Mitochondrial encephalopathy, lactic acidosis and stroke-like episodes (MELAS) is a rare multisystem disorder and is the most common maternally inherited mitochondrial disease. This condition has a special predilection for the nervous system and muscles. Typical findings on brain imaging include stroke-like areas, calcification of basal ganglia and brain atrophy. This accounts for the disease being, both clinically and radiologically, mistaken for ischemic stroke. The differentiation features from stroke include comparatively young age of the patients, site of the lesions, and relative overlap between the cerebral vasculature territories. In this case report, we discuss a 16-year male with clinical and radiological features highly suggestive of MELAS syndrome. Since this disease is rare and its clinical presentation is complex, it is among the most challenging to diagnose. It is particularly difficult to differentiate between ischemic stroke and MELAS. Magnetic resonance imaging (MRI) with diffusion weighted imaging (DWI), susceptibility weighted imaging (SWI) sequences and MR spectroscopy may aid in establishing the diagnosis. A particularly characteristic feature of MELAS syndrome is that recurrence may occur in locations different than previously noted, which was also seen in our patien

Imaging patterns of encephalopathy in patients with COVID-19

The aim of this retrospective observational study was to describe the neuroimaging manifestations of patients with COVID-19. This study was conducted at Aga Khan University Hospital, Karachi, Pakistan from March to July 2020. COVID-19 patients with neurological symptoms and positive neuroimaging were included after confirmation of COVID-19 by polymerase chain reaction test (PCR). In the 12 included patients, seizures and altered mentation were predominant neurological manifestations. Three cases had acute watershed infarcts (25%), two cases had posterior cerebral artery territorial infarcts (16.7%), two cases had periventricular corona radiata infarcts (16.7%), three cases had hypoxic ischemic encephalopathy (25%), two cases had posterior reversible encephalopathy syndrome (16.7%), and there was one case each of cerebral venous sinus thrombosis, pontine infarct, and bithalamic lesions (8.3%). This study highlights the diagnostic approaches in COVID-19-associated encephalopathy and the variable imaging features that clinicians and neuroradiologists should be aware of, as the pandemic progresses. Key Words: COVID-19, Neuroimaging, Encephalopathy, Magnetic resonance imaging, Coronavirus

Utility and accuracy of primary and secondary ultrasonographic signs for diagnosing acute appendicitis in pediatric patients

Introduction Making an accurate diagnosis of acute appendicitis (AA) is vital to prevent the morbid complications associated with untreated AA. This is challenging in up to 30% of pediatric patients which is a significantly high number. Ultrasound (US) has been generally used as the initial mode of imaging in pediatric patients due to the lack of ionizing radiation. Given its variable accuracy, adjuvants such as secondary signs can be used to aid the radiologist in making an accurate diagnosis. Materials and methods Patients between the ages of two and sixteen years with acute abdominal pain suspicious for AA, who underwent right lower quadrant US between 2003 and 2016, were retrospectively identified. Corresponding computed tomography (CT) and histopathology findings were noted. Based on the presence of primary and secondary signs, results were classified into three groups to determine accuracy. Group 1 included all patients with a normal appendix or if the appendix was not visualized, no secondary signs were present. Group 2 patients were those in which the appendix was not clearly seen and they had one or more secondary signs of AA. Group 3 included all patients with primary signs of AA. The number of secondary signs and cases with perforated appendices were also correlated with sonographic accuracy. Results One thousand one hundred and fifteen patients met the inclusion criteria of which 29% had confirmatory AA. The positive appendectomy rate was 89% (337/380). Using a 3-category classification of US results, the sensitivity was 79%, specificity 97%, positive predictive value was 93%, negative predictive value was 91% and the overall accuracy was 91%. The presence of two or more secondary signs had a high likelihood of appendicitis. The perforation rate was 10% with the highest percentage seen in Group 2 patients. Conclusion Despite inescapable limiting factors, US should be used as first-line imaging for suspected appendicitis in pediatric patients especially since its accuracy rivals CT when the appendix is visualized. The use of secondary sonographic signs has solid potential to aid the radiologist in making an accurate diagnosis with our study demonstrating a proportional relationship between the number of secondary signs and the likelihood of true appendicitis. However, further investigation is needed to determine the individual accuracy of secondary signs and whether a certain combination of secondary signs has a higher association with appendicitis

Metachronous renal ewing sarcoma/primitive neuroectodermal tumour in a survivor of burkitt lymphoma

We present a case of a 14-year-old girl who was diagnosed with Burkitt lymphoma in 2014. She was managed with chemotherapy and remained in remission for 3 years. On her surveillance imaging in 2017, a left-sided renal neoplastic mass was incidentally discovered. She underwent nephrectomy and pathology of the resected specimen revealed small cell tumour of the kidney with features favouring renal Ewing sarcoma/primitive neuroectodermal tumour. Molecular genetic analysis by fluorescence in situ hybridisation was performed which showed translocation of 22q12, thereby confirming the diagnosis. This is a rare secondary malignancy and an unusual association. This case highlights the importance and diagnostic dilemmas of rare secondary tumours in patients with such haematological malignancies and discusses its possible pathogenetic aspects

Non-surgical retrieval of dislodged or misplaced intravascular/ ndoluminal foreign objects.

Abstract Interventional procedures involving the use of intravascular or endoluminal objects have rapidly increased over the years with advancements in minimally invasive techniques. These foreign objects such as endovascular coils, guidewires, and endoluminal catheters, if lost or malpositioned, are a potential threat, which can result in complications such as embolization, perforation, infections, and arrhythmias. Therefore, timely removal of these foreign bodies is essential. In this technical report, we have described our experience with different scenarios in which percutaneous interventional techniques for retrieval of such foreign bodies were performed at our institute

A Curious Case of Intermittent Left Bundle Branch Block Associated with Cough

A handful of cases of voluntary control of left bundle branch block (LBBB) have been described in the literature. We report the case of a middle-aged man who was found to have LBBB on baseline electrocardiogram (ECG) which disappeared on coughing and then reappeared with the same maneuver. Subsequent myocardial perfusion scan showed reduced count in the anteroseptal region likely attributed to LBBB. It is possible that the intermittent conduction changes may be due to the alteration in the vagal tone associated with cough as reflected in the change in the PR interval on the ECG

Cesarean scar pregnancy: an experience of three cases with review of literature

Cesarean scar pregnancy (CSP), often considered the rarest form of ectopic pregnancy, is a result of implantation of the gestational sac into the fibrous tissue scar of a previous cesarean section. With an increase in the rate of cesarean sections, along with better awareness and improvement in sonographic diagnosis, the number and detection of scar pregnancies are on the rise. Because of its early invasion of the myometrium, usually in the first trimester, CSP is considered to be potentially lethal, leading to high risks of uterine rupture. We report a series of three cases of scar pregnancy that presented at different gestational ages and were managed by different methods. The aim of this case series is to share our experience with CSP, review previous literature, and emphasize on the radiological criteria to making a confident diagnosis. Diagnosis and management of CSP needs considerable expertise and a multidisciplinary approach to prevent complications. KEYWORDS

Polypoid adenomyoma of the uterus.

Polypoid adenomyoma is a rare uterine endometrial polypoid tumor of mixed epithelial and mesenchymal origin. Although the clinical and pathologic features of polypoid adenomyomas have been described extensively, imaging findings for these tumors have not been frequently reported in the literature. On imaging, their features may be confused with prolapsed leiomyomas or malignancy. Hemorrhagic cystic spaces in a prolapsed uterine tumor within the vagina should raise consideration of a diagnosis of polypoid adenomyoma. Such blood-containing cystic spaces would be unusual findings in leiomyomas and malignancy. Diagnosing polypoid adenomyoma is vital because it can potentially be managed by hysteroscopic resection, unlike an ordinary form of adenomyosi