Extraocular muscle enlargement in retinoencephalofacial angiomatosis.

A 22-year-old female presented for evaluation of five years of progressive left exophthalmos and intermittent blurred vision. She had previously received laser treatment for peripheral retinal neovascularization and had undergone lip reconstruction for a left-sided congenital vascular facial malformation. Magnetic resonance imaging demonstrated diffuse enlargement of the left extraocular and temporalis muscles, with prominent vessels in the temporalis muscle and intraconal fat. Left fundoscopic examination revealed grossly enlarged, tortuous retinal vessels extending from the optic disc to the peripheral retina and an abnormal network of capillaries. On the basis of these findings, a diagnosis of retinoencephalofacial angiomatosis was established. Retinoencephalofacial angiomatosis is a rare, non-hereditary disorder associated with ipsilateral retinal, brain, and facial arteriovenous malformations. This is the first report, to the authors' knowledge, of retinoencephalofacial angiomatosis presenting with exophthalmos secondary to extraocular muscle enlargement.

Traumatic Brain Injury and Vision.

Traumatic brain injury disrupts the complex anatomy of the afferent and efferent visual pathways. Injury to the afferent pathway can result in vision loss, visual field deficits, and photophobia. Injury to the efferent pathway primarily causes eye movement abnormalities resulting in ocular misalignment and double vision. Injury to both the afferent and efferent systems can result in significant visual disability.

Clinical and neuroradiologic characteristics in varicella zoster virus reactivation with central nervous system involvement.

OBJECTIVE: To investigate the clinical and magnetic resonance imaging (MRI) characteristics of patients with varicella zoster virus (VZV) reactivation involving the cranial nerves and central nervous system (CNS). METHODS: This is a retrospective, multi-center case-series of 37 patients with VZV infection affecting the cranial nerves and CNS. RESULTS: The median age was 71 years [IQR 51.5-76]; 21 (57%) were men. Cerebrospinal fluid (CSF) was available in 24/37 (65%); median CSF white blood cell count was 11 [IQR 2-23] cells/µL and protein was 45.5 [IQR 34.5-75.5] mg/dL. VZV polymerase chain reaction (PCR) assays were positive in 6/21 (29%) CSF and 8/9 (89%) ocular samples. Clinical involvement included the optic nerve in 12 (32%), other cranial nerves in 20 (54%), brain parenchyma in 12 (32%) and spinal cord or nerve roots in 4 (11%). Twenty-seven/28 immunocompetent patients' MRIs were available for review (96%). Of the 27, 18 had T1 postcontrast fat saturated sequences without motion artifact to evaluate for cranial nerve enhancement and optic perineuritis (OPN). Eight/18 (44%) demonstrated OPN. All 8 experienced vision loss: 3 optic neuritis, 1 acute retinal necrosis, and 3 CNS vasculitis with 1 central and 1 branch retinal artery occlusion and 1 uveitis. Diplopic patients had cranial nerve and cavernous sinus enhancement. All immunosuppressed patients were imaged. Seven/9 (88%) had extensive neuraxis involvement, including encephalitis, vasculitis and transverse myelitis; one case had OPN. CONCLUSION: OPN is a frequent manifestation in VZV-associated vision loss among immunocompetent patients. Immunosuppressed patients had greater neuraxis involvement. Optimizing MRI protocols may improve early diagnosis in VZV reactivation.

Nodular Pachymeningitis Associated With Relapsing Polychondritis and Crohn Disease Responsive to Adalimumab and Prednisone.

OBJECTIVES: To review the previous literature on the associations of pachymeningitis with Crohn disease (CD) and relapsing polychondritis (RP) and to describe a new case occurring in association with both in addition to highlighting its positive response to steroid and adalimumab treatment. METHODS: We review the patient's clinical presentation, diagnostic workup (serum and CSF testing), and MRI findings in detail and chronicle the response of the pachymeningitis to intensive immunotherapy. We contrast this case against previous reports of pachymeningitis occurring in association with RP and inflammatory bowel disease that were found on PubMed. RESULTS: Only 2 cases of ulcerative colitis and 5 cases of RP were found in association with pachymeningitis; there were no cases in association with CD. Our patient presented with symptoms isolated to a steroid-responsive headache in the setting of normal neurologic and rheumatologic examinations. Her preceding history was notable for long-standing CD and increasingly active symptoms referable to RP. Focal nodular pachymeningitis was seen overlying the left hemisphere on brain MRI. An extensive serum and CSF workup and body fluorodeoxyglucose-PET scan failed to identify an alternative etiology beyond her underlying autoimmune inflammatory disorders. After adding prednisone and adalimumab to her preexisting treatment of methotrexate, she responded dramatically both clinically and radiographically. CONCLUSIONS: Although exceptionally rare, pachymeningitis may occur as a neuroinflammatory complication of CD and RP.

Susceptibility-weighted imaging reveals cerebral microvascular injury in severe COVID-19.

We evaluated the incidence, distribution, and histopathologic correlates of microvascular brain lesions in patients with severe COVID-19. Sixteen consecutive patients admitted to the intensive care unit with severe COVID-19 undergoing brain MRI for evaluation of coma or neurologic deficits were retrospectively identified. Eleven patients had punctate susceptibility-weighted imaging (SWI) lesions in the subcortical and deep white matter, eight patients had >10 SWI lesions, and four patients had lesions involving the corpus callosum. The distribution of SWI lesions was similar to that seen in patients with hypoxic respiratory failure, sepsis, and disseminated intravascular coagulation. Brain autopsy in one patient revealed that SWI lesions corresponded to widespread microvascular injury, characterized by perivascular and parenchymal petechial hemorrhages and microscopic ischemic lesions. Collectively, these radiologic and histopathologic findings add to growing evidence that patients with severe COVID-19 are at risk for multifocal microvascular hemorrhagic and ischemic lesions in the subcortical and deep white matter.

Evaluating Factors and Resources Affecting Ranking of Diagnostic Radiology Residency Programs by Medical Students in 2016-2017.

RATIONALE AND OBJECTIVES: Recent changes in radiology curriculum and access to residency program information, including the introduction of various online resources and the Interventional Radiology integrated pathway, may influence the rank list order of medical student applicants. The purpose of this study is to assess factors that affect the rank lists of medical students applying to our radiology residency program in the 2016-2017 academic year. MATERIALS AND METHODS: After IRB approval, an anonymous online 19 question survey was emailed to 622 applicants to our diagnostic radiology and/or interventional radiology integrated pathway. Applicants ranked 35 unique factors that may influence their residency rank list order from 1 (not important at all) to 5 (very important), listed their top five 'very important' factors, and ranked various sources of information used to learn about residency programs. General applicant demographic questions were also included. RESULTS: Response rate was 18.8% (117/622). The 5 most important factors affecting applicant ranking of programs are perceived happiness of the residents and faculty (4.69), fellowship and job placement of recent graduates (4.34), interactions with programs' current residents (4.33), stability of the department and program (4.29), and geographic location of the program (4.27). The top 5 resources for learning about residency programs were interactions with current residents at the program (4.47), program director (3.87), and interviewing faculty (3.87). Individual program websites were ranked more highly than internet message boards and forums as an information source. CONCLUSION: Medical students consider a large number of factors and resources in determining their rank lists, with factors encountered during the interview day playing a significant role in shaping the applicants' view of a residency program.