BackgroundAtrial myxomas are generally considered benign neoplasms. The majority of tumors are sporadic and less than 10% are associated with an autosomal dominant condition known as the Carney complex, which is most often caused by germline mutation in the gene PRKAR1A. Whether this gene plays a role in the development of sporadic myxomas has been an area of debate, although recent studies have suggested that some fraction of sporadic tumors also carry mutations in PRKARIA. Extra-cardiac complications of atrial myxoma include dissemination of tumor to the brain; however, the dissemination of viable invasive tumor cells is exceedingly rare.Case presentationWe present here a 48-year-old white woman who developed multiple intracranial hemorrhagic lesions secondary to tumor embolism that progressed to ‘false’ aneurysm formation and invasion through the vascular wall into brain parenchyma 7 months after resection of an atrial myxoma. Whole exome sequencing of her tumor revealed multiple mutations in PRKAR1A not found in her germline deoxyribonucleic acid (DNA), suggesting that the myxoma in this patient was sporadic.ConclusionsOur patient illustrates that mutations in PRKAR1A may be found in sporadic lesions. Whether the presence of this mutation affects the clinical behavior of sporadic tumors and increases risk for metastasis is not clear. Regardless, the protein kinase A pathway which is regulated by PRKAR1A represents a possible target for treatment in patients with metastatic cardiac myxomas harboring mutations in the PRKARIA gene.
Neurologists regularly confront complex clinical scenarios that require the application of ethical principles to achieve a respectful and fair resolution. In this article, we describe the types of ethically precarious scenarios neurologists encounter, the current status of standards for ethics and communication training for neurology residents, and the present practice of ethics training in neurology residency programs. We make recommendations for optimizing bioethics training for neurology residents and suggest methods for assessing the efficacy of these training initiatives. We emphasize the current need for strengthening the practical bioethics skills of neurologists.
Powassan virus (POWV), a rare flavivirus that may be transmitted by a tick bite, causes rare but severe cases of encephalitis, meningitis, and meningoencephalitis in humans. We present the case of a 62-year-old man with prior Lyme disease and reactive arthritis who presented to the hospital with symptoms of fever, headache, and fatigue. The patient developed rapid deterioration of mental status including profound expressive aphasia and required intubation and high-dose steroids. Cerebrospinal fluid (CSF) serologies were found to be positive for the POWV.
A 57-year-old man presented with sudden neck pain radiating down his arms. This pain progressed to bilateral upper and subsequently lower extremity weakness and numbness. His vitals were notable for systolic blood pressures lower than his baseline (down to 90 mm Hg). The patient’s neurological examination as well as magnetic resonance imaging of the cervical and thoracic spine localized to a lesion in the anterior spinal cord. The differential diagnosis for such an acute presentation included stroke, demyelination, intramedullary neoplasm, infection, metabolic myelopathy, and a dural arteriovenous fistula. Further imaging with angiography demonstrated that our patient lacked an anterior spinal artery. In its place, collateral flow from cervical artery branches provided sustenance to the anterior spinal cord. In the setting of hemodynamic instability, this variant anatomy likely predisposed the patient to ischemia, leading to the classic presentation of anterior cord syndrome.
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