Scholars@Duke
Karra Anne Jones

Karra Anne Jones

Associate Professor of Pathology
Pathology
karra.jones@duke.edu
Duke Box 3712, Durham, NC 27710
Rm 3093, Yellow Zone, 40 Duke Medicine Circle, Durham, NC 27710

Selected Publications

Clinical Reasoning: A 60-Year-Old Woman With Rapidly Progressive Muscle Weakness and Ophthalmoparesis.

Journal Article Neurology · August 13, 2024 The clinical assessment of patients with proximal muscle weakness represents a frequent yet intricate challenge. We present the case of a 60-year-old woman who experienced a progressive, symmetrical weakness in proximal limbs and bulbar muscles over 6 mont ... Full text Link to item Cite

Iron-based biomarkers for personalizing pharmacological ascorbate therapy in glioblastoma: insights from a phase 2 clinical trial.

Journal Article J Neurooncol · February 2024 BACKGROUND: Pharmacological ascorbate (intravenous delivery reaching plasma concentrations ≈ 20 mM; P-AscH-) has emerged as a promising therapeutic strategy for glioblastoma. Recently, a single-arm phase 2 clinical trial demonstrated a significant increase ... Full text Link to item Cite

Coexisting Biopsy-Diagnosed Dementia and Glioblastoma.

Journal Article Brain Sci · January 30, 2024 Both glioblastoma (GBM) and dementia are devastating diseases with limited treatments that are usually not curative. Having clinically diagnosed dementia with an associated biopsy-proven etiology and a coexisting GBM diagnosis is a rare occurrence. The rel ... Full text Link to item Cite

Magnetic Resonance Imaging of Iron Metabolism with T2* Mapping Predicts an Enhanced Clinical Response to Pharmacologic Ascorbate in Patients with GBM.

Journal Article Clin Cancer Res · January 17, 2024 PURPOSE: Pharmacologic ascorbate (P-AscH-) is hypothesized to be an iron (Fe)-dependent tumor-specific adjuvant to chemoradiation in treating glioblastoma (GBM). This study determined the efficacy of combining P-AscH- with radiation and temozolomide in a p ... Full text Link to item Cite

Effects of gene replacement therapy with resamirigene bilparvovec (AT132) on skeletal muscle pathology in X-linked myotubular myopathy: results from a substudy of the ASPIRO open-label clinical trial.

Journal Article EBioMedicine · January 2024 BACKGROUND: X-linked myotubular myopathy (XLMTM) is a rare, life-threatening congenital muscle disease caused by mutations in the MTM1 gene that result in profound muscle weakness, significant respiratory insufficiency, and high infant mortality. There is ... Full text Link to item Cite

Ventriculus terminalis cyst in an infant: a case report.

Journal Article J Med Case Rep · January 23, 2023 BACKGROUND: Filar cysts are frequently found on neonatal ultrasound and are physiologically involuting structures with natural resolution. Hence, there has been no previous histologic correlation. Ventriculus terminalis is a focal central canal dilation in ... Full text Link to item Cite

Challenging Neuromuscular Disease Cases.

Journal Article Semin Neurol · December 2022 The diagnosis of neuromuscular disorders requires a thorough history including family history and examination, with the next steps broadened now beyond electromyography and neuropathology to include genetic testing. The challenge in diagnosis can often be ... Full text Link to item Cite

The inflammatory pathology of dysferlinopathy is distinct from calpainopathy, Becker muscular dystrophy, and inflammatory myopathies.

Journal Article Acta Neuropathol Commun · February 8, 2022 The descriptions of muscle pathology in dysferlinopathy patients have classically included an inflammatory infiltrate that can mimic inflammatory myopathies. Based on over 20 years of institutional experience in evaluating dystrophic and inflammatory myopa ... Full text Link to item Cite

Catalase Overexpression Drives an Aggressive Phenotype in Glioblastoma.

Journal Article Antioxidants (Basel) · December 14, 2021 Glioblastoma remains the deadliest form of brain cancer, largely because these tumors become resistant to standard of care treatment with radiation and chemotherapy. Intracellular production of reactive oxygen species (ROS) is necessary for chemo- and radi ... Full text Link to item Cite

Vascular Involvement in Neurosarcoidosis: Early Experiences From Intracranial Vessel Wall Imaging.

Journal Article Neurol Neuroimmunol Neuroinflamm · November 2021 BACKGROUND AND OBJECTIVES: Cerebrovascular manifestations in neurosarcoidosis (NS) were previously considered rare but are being increasingly recognized. We report our preliminary experience in patients with NS who underwent high-resolution vessel wall ima ... Full text Link to item Cite

A Review of Clinical and Imaging Findings in Tumefactive Demyelination.

Journal Article AJR Am J Roentgenol · July 2021 OBJECTIVE. Tumefactive demyelination mimics primary brain neoplasms on imaging, often necessitating brain biopsy. This article reviews the literature for the clinical and radiologic findings of tumefactive demyelination in various disease processes to faci ... Full text Link to item Cite

Machine Learning-Based Multiparametric Magnetic Resonance Imaging Radiomics for Prediction of H3K27M Mutation in Midline Gliomas.

Journal Article World Neurosurg · July 2021 OBJECTIVE: H3K27M mutation in gliomas has prognostic implications. Previous magnetic resonance imaging (MRI) studies have reported variable rates of tumoral enhancement, necrotic changes, and peritumoral edema in H3K27M-mutant gliomas, with no distinguishi ... Full text Link to item Cite

Teaching Pathology in an Integrated Preclinical Medical School Curriculum and Adaptations to COVID-19 Restrictions.

Journal Article Acad Pathol · 2021 The coronavirus disease 2019 (COVID-19) pandemic has had a major impact on education at all age levels, including professional schools and health professions programs. We describe the experience of adapting preclinical medical school courses within an inte ... Full text Link to item Cite

Deep Neural Network Analysis of Pathology Images With Integrated Molecular Data for Enhanced Glioma Classification and Grading.

Journal Article Front Oncol · 2021 Gliomas are primary brain tumors that originate from glial cells. Classification and grading of these tumors is critical to prognosis and treatment planning. The current criteria for glioma classification in central nervous system (CNS) was introduced by W ... Full text Link to item Cite

Presurgical detection of brain invasion status in meningiomas based on first-order histogram based texture analysis of contrast enhanced imaging.

Journal Article Clin Neurol Neurosurg · November 2020 OBJECTIVE: Invasion of brain parenchyma by meningioma can be a critical factor in surgical planning. The aim of this study was to determine the diagnostic utility of first-order texture parameters derived from both whole tumor and single largest slice of T ... Full text Link to item Cite

Interpretable multimodal deep learning for real-time pan-tissue pan-disease pathology search on social media.

Journal Article Mod Pathol · November 2020 Pathologists are responsible for rapidly providing a diagnosis on critical health issues. Challenging cases benefit from additional opinions of pathologist colleagues. In addition to on-site colleagues, there is an active worldwide community of pathologist ... Full text Link to item Cite

LIMB GIRDLE MUSCULAR DYSTROPHIES

Conference Neuromuscular Disorders · October 2020 Full text Cite

NEW GENES AND DISEASES / NGS & RELATED TECHNIQUES

Conference Neuromuscular Disorders · October 2020 Full text Cite

Liver metastases from pituitary carcinomas mimicking visceral well-differentiated neuroendocrine tumors: a series of four cases.

Journal Article Diagn Pathol · July 4, 2020 BACKGROUND: Pathologists frequently encounter neuroendocrine tumors (NETs) presenting as multiple liver masses in routine practice. Most often, these are well-differentiated tumors with characteristic histologic features. In contrast, pituitary carcinoma i ... Full text Link to item Cite

Survival after reoperation for recurrent glioblastoma.

Journal Article J Clin Neurosci · March 2020 Determining which patients will benefit from reoperation for recurrent glioblastoma remains difficult and the impact of the volume of FLAIR signal hyperintensity is not well known. The primary purpose of this study is to analyze the impact of preoperative ... Full text Link to item Cite

Adult Medulloblastoma Presenting with Extensive Supratentorial Metastasis with Radiographically Occult Infratentorial Disease: Case Report and Review of the Literature

Journal Article American Journal of Surgical Case Reports · January 30, 2020 This case represents the unique occurrence of supratentorial dissemination of medulloblastoma in the absence of overt cerebellar disease. The authors present the case of a 64-year-old man who presented with headaches and intractable nausea with ima ... Full text Cite

Dysferlinopathy muscle pathology does not mimic myositis

Conference JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY · 2020 Cite

First-in-Human Phase I Clinical Trial of Pharmacologic Ascorbate Combined with Radiation and Temozolomide for Newly Diagnosed Glioblastoma.

Journal Article Clin Cancer Res · November 15, 2019 PURPOSE: Standard treatment for glioblastoma (GBM) includes surgery, radiation therapy (RT), and temozolomide (TMZ), yielding a median overall survival (OS) of approximately 14 months. Preclinical models suggest that pharmacologic ascorbate (P-AscH-) enhan ... Full text Link to item Cite

Clinical, genetic, and pathologic characterization of FKRP Mexican founder mutation c.1387A>G.

Journal Article Neurol Genet · April 2019 OBJECTIVE: To characterize the clinical phenotype, genetic origin, and muscle pathology of patients with the FKRP c.1387A>G mutation. METHODS: Standardized clinical data were collected for all patients known to the authors with c.1387A>G mutations in FKRP. ... Full text Link to item Cite

Glioma grading using structural magnetic resonance imaging and molecular data.

Journal Article J Med Imaging (Bellingham) · April 2019 A glioma grading method using conventional structural magnetic resonance image (MRI) and molecular data from patients is proposed. The noninvasive grading of glioma tumors is obtained using multiple radiomic texture features including dynamic texture analy ... Full text Link to item Cite

BCR-NTRK2 fusion in a low-grade glioma with distinctive morphology and unexpected aggressive behavior.

Journal Article Cold Spring Harb Mol Case Stud · April 2019 A 52-yr-old man was found to have a 6.6-cm left frontotemporal mass. Biopsy revealed a low-grade astrocytic neoplasm with significant infiltration and an unusual morphologic appearance. Only rare mitotic figures were seen and the Ki-67 proliferative index ... Full text Link to item Cite

Novel phenotype in GFPT1 congenital myasthenic syndrome

Conference JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY · 2019 Cite

Adding Pompe disease to the list of autophagic vacuolar myopathies with sarcolemmal features

Conference JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY · 2019 Cite

Granulomatous fasciitis followed by morphea profunda: Is granulomatous fasciitis part of a spectrum of deep morphea? A case report and review of the literature.

Journal Article Clin Case Rep · August 2018 Although eosinophilic fasciitis is known to be part of the deep morphea spectrum, this first report of the coexistence of granulomatous fasciitis and morphea profunda suggests that granulomatous fasciitis may also be a part of the spectrum of deep morphea. ... Full text Link to item Cite

Postnatal hypoxia evokes persistent changes within the male rat's dopaminergic system.

Journal Article Sleep Breath · May 2018 PURPOSE: Hypoxic insults occurring during the perinatal period remain the leading cause of permanent brain impairment. Severe cognitive and motor dysfunction, as seen in cerebral palsy, will occur in 4-10% of post-hypoxic newborns. Subtle cognitive impairm ... Full text Link to item Cite

Serous Carcinoma Mimicking Primary Urothelial Carcinoma on Clinical Evaluation and Pathology: A Potential Diagnostic Pitfall.

Journal Article Arch Pathol Lab Med · February 2018 CONTEXT: - Serous carcinoma of the gynecologic tract often involves the external bladder wall and can occasionally mimic primary urothelial carcinoma of the bladder. OBJECTIVE: - To define the spectrum of morphologic and immunohistochemical features that c ... Full text Link to item Cite

A Fatal Case of Herpes Simplex Encephalitis with Two False-Negative Polymerase Chain Reactions.

Journal Article Case Rep Neurol · 2018 An 88-year-old man presented with a 1-month history of altered mental status and seizures. His electrographic and imaging findings were suggestive of herpes simplex encephalitis (HSE), for which he was empirically treated with acyclovir. He underwent two l ... Full text Link to item Cite

Erratum to: Imaging correlates for the 2016 update on WHO classification of grade II/III gliomas: implications for IDH, 1p/19q and ATRX status.

Journal Article J Neurooncol · December 2017 In the initial online publication, the values in the last two rows in Table 1 were in the wrong rows. The original article has been corrected. ... Full text Link to item Cite

Imaging correlates for the 2016 update on WHO classification of grade II/III gliomas: implications for IDH, 1p/19q and ATRX status.

Journal Article J Neurooncol · December 2017 The 2016 World Health Organization Classification of Tumors of the Central Nervous System incorporates the use of molecular information into the classification of brain tumors, including grade II and III gliomas, providing new prognostic information that c ... Full text Link to item Cite

CLINICAL RELEVANCE OF MOLECULAR MARKERS IN GLIOMAS

Journal Article Revista Medica Clinica Las Condes · May 1, 2017 Molecular markers are increasingly being utilized in diagnosing, prognosticating and predicting response to therapy of gliomas. The 4th edition of the World Health Organization (WHO) Classification of Tumours of the Central Nervous System (CNS) was updated ... Full text Cite

Non-Midline H3 K27M-Mutant Glioma

Conference JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY · 2017 Cite

Muscle Pathology in Patients with the FKRP Mexican Founder Mutation

Conference JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY · 2017 Cite

Anti-tubulin drugs conjugated to anti-ErbB antibodies selectively radiosensitize.

Journal Article Nat Commun · October 4, 2016 Tumour resistance to radiotherapy remains a barrier to improving cancer patient outcomes. To overcome radioresistance, certain drugs have been found to sensitize cells to ionizing radiation (IR). In theory, more potent radiosensitizing drugs should increas ... Full text Link to item Cite

Selective coexpression of VEGF receptor 2 in EGFRvIII-positive glioblastoma cells prevents cellular senescence and contributes to their aggressive nature.

Journal Article Neuro Oncol · May 2016 BACKGROUND: In glioblastoma (GBM), the gene for epidermal growth factor receptor (EGFR) is frequently amplified. EGFR mutations also are common, including a truncation mutation that yields a constitutively active variant called EGFR variant (v)III. EGFRvII ... Full text Link to item Cite

Intracranial Rhabdomyoma: Case Report and Review of the Literature.

Journal Article Cureus · April 29, 2016 A 24-year-old male presented with eight months of increasingly severe frontal headaches, decreased right facial sensation, and periodic vertigo. Magnetic resonance imaging demonstrated a heterogeneously contrast-enhancing mass involving and expanding the r ... Full text Link to item Cite

Maternal dietary supplementation with omega-3 polyunsaturated fatty acids confers neuroprotection to the newborn against hypoxia-induced dopamine dysfunction.

Journal Article Sleep Sci · 2016 INTRODUCTION: Up to 84% of prematurely born infants suffer hypoxic, anoxic, and ischemic insults. Those infants with subsequent behavioral, motor or cognitive dysfunction represent 8-11% of all live births. Yet, no interventions employed during pregnancy a ... Full text Link to item Cite

Breaking Bad: Chromothripsis and Malignancy in CNS tumors

Conference JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY · 2016 Cite

Molecularly-Defined Secondary and "Stealth" Glioblastomas: An Institutional Experience

Conference JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY · 2016 Cite

p16INK4A expression is frequently increased in periorbital and ocular squamous lesions.

Journal Article Diagn Pathol · September 24, 2015 BACKGROUND: p16 expression is a well established biomarker of cervical dysplasia and carcinoma arising from high risk human papilloma virus infection. Increased p16 expression is also seen in squamous neoplasms arising at other sites, including head, neck, ... Full text Link to item Cite

Neutralizing the EGF receptor in glioblastoma cells stimulates cell migration by activating uPAR-initiated cell signaling.

Journal Article Oncogene · July 30, 2015 In glioblastoma (GBM), the EGF receptor (EGFR) and Src family kinases (SFKs) contribute to an aggressive phenotype. EGFR may be targeted therapeutically; however, resistance to EGFR-targeting drugs such as Erlotinib and Gefitinib develops quickly. In many ... Full text Link to item Cite

Soluble Urokinase Receptor Is Released Selectively by Glioblastoma Cells That Express Epidermal Growth Factor Receptor Variant III and Promotes Tumor Cell Migration and Invasion.

Journal Article J Biol Chem · June 12, 2015 Genomic heterogeneity is characteristic of glioblastoma (GBM). In many GBMs, the EGF receptor gene (EGFR) is amplified and may be truncated to generate a constitutively active form of the receptor called EGFRvIII. EGFR gene amplification and EGFRvIII are a ... Full text Link to item Cite

Tumor radiosensitization by monomethyl auristatin E: mechanism of action and targeted delivery.

Journal Article Cancer Res · April 1, 2015 Intrinsic tumor resistance to radiotherapy limits the efficacy of ionizing radiation (IR). Sensitizing cancer cells specifically to IR would improve tumor control and decrease normal tissue toxicity. The development of tumor-targeting technologies allows f ... Full text Link to item Cite

Arteriovenous malformation within an isocitrate dehydrogenase 1 mutated anaplastic oligodendroglioma.

Journal Article Surg Neurol Int · 2015 BACKGROUND: The co-occurrence of intracranial arteriovenous malformations (AVMs) and cerebral neoplasms is exceedingly rare but may harbor implications pertaining to the molecular medicine of brain cancer pathogenesis. CASE DESCRIPTION: Here, we present a ... Full text Link to item Cite

Anaplastic Pleomorphic Xanthoastrocytoma Versus Glioblastoma: A Histopathologic and Molecular Case Study

Conference JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY · 2015 Cite

Combining diffusion and perfusion differentiates tumor from bevacizumab-related imaging abnormality (bria).

Journal Article J Neurooncol · December 2014 A subset of patients with high-grade glioma and brain metastases who are treated with bevacizumab develop regions of marked and persistent restricted diffusion that do not reflect recurrent tumor. Here, we quantify the degree of restricted diffusion and th ... Full text Link to item Cite

Endometriosis of the conus medullaris causing cyclic radiculopathy.

Journal Article J Neurosurg Spine · November 2014 Intramedullary spinal cord hematomas are a rare neurosurgical pathological entity typically arising from vascular and neoplastic lesions. Endometriosis is an extremely rare cause of intramedullary spinal cord hematoma, with only 5 previously reported cases ... Full text Link to item Cite

Orbital granulomatosis with polyangiitis (Wegener granulomatosis): clinical and pathologic findings.

Journal Article Arch Pathol Lab Med · August 2014 The pathology of granulomatosis with polyangiitis (GPA), formerly Wegener granulomatosis, typically features a granulomatous and sometimes necrotizing vasculitis targeting the respiratory tract and kidneys. However, orbital involvement occurs in up to 60% ... Full text Link to item Cite

The Cancer Genome Atlas expression profiles of low-grade gliomas.

Journal Article Neurosurg Focus · April 2014 Differentiating between low-grade gliomas (LGGs) of astrocytic and oligodendroglial origin remains a major challenge in neurooncology. Here the authors analyzed The Cancer Genome Atlas (TCGA) profiles of LGGs with the goal of identifying distinct molecular ... Full text Link to item Cite

Bevacizumab: radiation combination produces restricted diffusion on brain MRI.

Journal Article CNS Oncol · 2014 AIMS: The purpose of this paper is to investigate the effect of bevacizumab (BEV) on the diffusion properties of irradiated brain gliomas. MATERIALS & METHODS: Neuroimaging studies and medical records of 44 patients undergoing treatment for cerebral glioma ... Full text Link to item Cite

Restriction-Spectrum Imaging of Bevacizumab-Related Necrosis in a Patient with GBM.

Journal Article Front Oncol · 2013 IMPORTANCE: With the increasing use of antiangiogenic agents in the treatment of high-grade gliomas, we are becoming increasingly aware of distinctive imaging findings seen in a subset of patients treated with these agents. Of particular interest is the de ... Full text Link to item Cite

Trigeminal Nerve Neuromuscular Choristoma Versus Rhabdomyoma

Conference JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY · 2013 Cite

Pre- and Post-Bevacizumab Glioblastoma Histopathology, Biomarkers and Imaging: An Autopsy Study

Conference JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY · 2013 Cite

Abnormal muscle spindle innervation and large-fiber neuropathy in diabetic mice.

Journal Article Diabetes · June 2008 OBJECTIVE: Large-fiber diabetic polyneuropathy (DPN) leads to balance and gait abnormalities, placing patients at risk for falls. Large sensory axons innervating muscle spindles provide feedback for balance and gait and, when damaged, can cause altered sen ... Full text Link to item Cite

Differential effects of modulation of docosahexaenoic acid content during development in specific regions of rat brain.

Journal Article Lipids · May 2006 Variation in brain FA composition, particularly decreased DHA (22:6n-3), affects neurodevelopment, altering visual, attentional, and cognitive functions, and is implicated in several neuropsychiatric disorders. To further understand how specific brain proc ... Full text Link to item Cite

Reduced extracellular dopamine and increased responsiveness to novelty: neurochemical and behavioral sequelae of intermittent hypoxia.

Journal Article Sleep · February 2005 STUDY OBJECTIVES: Vesicular monoamine transporter and dopamine D1-receptor protein expression are upregulated within the striatum of adults rats exposed to intermittent hypoxic insults as neonates. These observations prompted us to test the hypothesis that ... Full text Link to item Cite