Hepatic glomerulosclerosis, a renal complication of liver cirrhosis, presents challenges in diagnosis and management. This case report discusses the rarity of kidney biopsy findings resembling lecithin-cholesterol acyltransferase (LCAT) deficiency post liver transplantation. We present the case of a patient with end-stage liver disease (ESLD) from alcohol-related cirrhosis, who underwent orthotopic liver transplantation (OLT) with persistent proteinuria after transplantation.
View Article and Find Full Text PDFAdenoviral infections in post-transplant patients have been described in multiple organ systems, most classically the lung, liver, and alimentary tract. In the genitourinary tract, hemorrhagic cystitis is most frequently observed. Clinically apparent renal involvement with adenovirus is rare, and adenovirus-associated interstitial nephritis (AAIN) is an uncommon cause of renal allograft failure.
View Article and Find Full Text PDFGlioneural hamartomas are exceedingly rare lesions. When localized to the internal auditory canal (IAC), they can cause symptoms referrable to seventh and eighth cranial nerve compression. Here, the authors present a rare case of an IAC glioneural hamartoma.
View Article and Find Full Text PDFBackground: Cases of isolated intramedullary spinal neurocysticercosis are extremely rare. Only 25 cases have been reported before 2022. Due to its rarity, the diagnosis of spinal neurocysticercosis may be missed.
View Article and Find Full Text PDFActa Neuropathol Commun
April 2022
Pathologists can label pathologies differently, making it challenging to yield consistent assessments in the absence of one ground truth. To address this problem, we present a deep learning (DL) approach that draws on a cohort of experts, weighs each contribution, and is robust to noisy labels. We collected 100,495 annotations on 20,099 candidate amyloid beta neuropathologies (cerebral amyloid angiopathy (CAA), and cored and diffuse plaques) from three institutions, independently annotated by five experts.
View Article and Find Full Text PDFPurpose Of Review: To discuss the pathophysiology, key clinical features, necessary diagnostic evaluation, and current treatment regimens for granulomatous diseases of the central nervous system.
Recent Findings: The diagnosis and management of granulomatous disease of the central nervous system has been revolutionized by advances in diagnostic imaging. Nevertheless, tissue and/or cerebrospinal fluid (CSF) sampling remains necessary to establish the diagnosis in most cases.
Acta Neuropathol
March 2022
Background: Hemangiopericytomas (HPCs) and solitary fibrous tumors (SFTs) are rare tumors of mesenchymal origin. Here, the authors present a rare case of anaplastic HPC in the jugular foramen (JF). The authors also conduct a systematic review of the literature to examine the current fund of knowledge on JF HPC/SFTs.
View Article and Find Full Text PDFBackground: Hemangioblastomas are benign vascular neoplasms of the central nervous system that may occur sporadically or in association with Von Hippel-Lindau disease. A minority of these lesions can occur in the spine, mostly as intramedullary masses. The authors present a rare case of primary sporadic spinal hemangioblastoma occurring as an intradural extramedullary (IDEM) lesion.
View Article and Find Full Text PDFBackground: Hypertrophic cranial pachymeningitis is a rare inflammatory disorder characterized by thickening of the dura mater and multiple cranial neuropathies. Although an infectious etiology may be present, often no specific cause is discovered.
Observations: The authors described a 71-year-old man with progressive right eye vision loss, ptosis, and complete ophthalmoplegia with imaging findings suggestive of hypertrophic cranial pachymeningitis.
Case reports of acute kidney injury in patients taking the glucagon-like peptide 1 (GLP-1) receptor agonists exenatide and liraglutide have been reported. We report 2 patients with chronic kidney disease due to diabetic kidney disease who experienced rapid worsening of kidney function and increased proteinuria after being prescribed the GLP-1 receptor agonist semaglutide. In 1 patient, kidney biopsy showed advanced diffuse and nodular glomerulosclerosis accompanied by interstitial lymphoplasmacytic and eosinophilic infiltrate and evidence of acute tubular injury.
View Article and Find Full Text PDFFor patients presenting with neurological changes from pituitary tumor apoplexy, urgent surgical intervention is commonly performed for diagnosis, tumor resection, and optic apparatus decompression. Although identification and preservation of the pituitary gland during the time of surgery can be challenging, it may lead to improve endocrine outcomes. A retrospective case series of all patients with macroadenomas presenting with apoplexy at Loyola University Medical Center from 2016 to 2018 was studied.
View Article and Find Full Text PDFMost pituitary adenomas are of soft consistency and can be resected during surgery with routine suction instruments. However, fibrous adenomas may require more aggressive techniques. The ability to predict consistency on magnetic resonance imaging (MRI) would improve preoperative preparation and may have implications on the extent of resection.
View Article and Find Full Text PDFBackground: Malignant transformation of a mature intracranial teratoma into an adenocarcinoma is an extremely rare event and portends a poor prognosis. The clinical progression, radiographic changes, histopathological findings, and immunohistochemistry findings from these unique cases might provide clues regarding this transformation. Caudal homeobox gene-2 (CDX-2) is a specific and robust marker for colonic adenocarcinomas and can also be used to identify differentiation of mature intracranial teratoma into colonic-type adenocarcinoma.
View Article and Find Full Text PDFAssess impact of K -67 labeling index (LI; K -67 LI) on risk of recurrence or progression of WHO grade I meningiomas. Retrospective study of adult patients who underwent resection of cranial base meningioma between 2004 and 2016. 272 patients fulfilled criteria for inclusion in the study.
View Article and Find Full Text PDFBackground And Purpose: The use of ventricular assist devices (VADs) for the treatment of heart failure has become increasingly common. These patients have a considerable risk of cerebral embolism. We describe such a patient and his successful treatment by thrombectomy, compare his attributes with those previously published, and describe the construct of a clinical decision model, whose results bear practical implications for patient management.
View Article and Find Full Text PDFBackground: Pituitary metastasis of cervical adenocarcinoma is an exceedingly rare phenomenon.
Case Description: The authors present a case of a 66-year-old female with cervical adenocarcinoma who was discovered to have a rapidly growing intrasellar mass in the work-up of adrenal insufficiency and hypothyroidism. The patient underwent subsequent endoscopic endonasal subtotal resection of the mass.
Introduction: We sought to establish the relationships between standard postmortem measures of AD neuropathology and antemortem [C]PIB-positron emission tomography ([C]PIB-PET) analyzed with the Centiloid (CL) method, a standardized scale for Aβ-PET quantification.
Methods: Four centers contributed 179 participants encompassing a broad range of clinical diagnoses, PET data, and autopsy findings.
Results: CL values increased with each CERAD neuritic plaque score increment (median -3 CL for no plaques and 92 CL for frequent plaques) and nonlinearly with Thal Aβ phases (increases were detected starting at phase 2) with overlap between scores/phases.