Extranodal Rosai-Dorfman Disease of Breast Mimicker of Breast Malignancy.

Rosai-Dorfman-Destombes (RDD) disease is also known as sinus histiocytosis with massive lymphadenopathy. It is an uncommon heterogeneous disease of children and young adults. Most of the patients with RDD generally present with painless lymphadenopathy, while extranodal and multisystem manifestation of the disease is unusual. The diagnosis is based on the imaging with clinicopathological correlation. Flourine-18 fluorodeoxyglucose positron emission tomography/computed tomography is useful for the initial staging of the RDD lesions, which have similar appearance and avidity like intermediate and high-grade lymphomas. Here, we present the case of a 55-year-old female presented with left breast mass that turned out to be the extranodal Rosai-Dorfman disease.

Role of Clinical and Multimodality Neuroimaging in the Evaluation of Brain Death/Death by Neurologic Criteria and Recent Highlights from 2023 Updated Guidelines.

Purpose of Review: This review aims to provide a comprehensive overview of the diagnosis of brain death/death by neurologic criteria (BD/DNC) by emphasizing the clinical criteria established by the American Academy of Neurology (AAN) in light of their updated guidelines released in 2023. In this review, we will focus on the current implementation of ancillary tests including the catheter cerebral angiogram, nuclear scintigraphy, and transcranial Doppler, which provide support in diagnoses when clinical examination and apnea tests are inconclusive. Finally, we will also provide examples to discuss the implementation of certain imaging studies in the context of diagnosing BD/DNC. Recent Findings: Recent developments in the field of neurology have emphasized the importance of clinical criteria for diagnosing BD/DNC, with the AAN providing clear updated guidelines that include coma, apnea, and the absence of brainstem reflexes. Current ancillary tests, including the catheter cerebral angiogram, nuclear scintigraphy, and transcranial Doppler play a crucial role in confirming BD/DNC when the clinical assessment is limited. The role of commonly used imaging studies including computed tomography and magnetic resonance angiographies of the brain as well as CT/MR perfusion studies will also be discussed in the context of these new guidelines. Summary: BD/DNC represents the permanent cessation of brain functions, including the brainstem. This review article provides the historical context, clinical criteria, and pathophysiology that goes into making this diagnosis. Additionally, it explores the various ancillary tests and selected imaging studies that are currently used to diagnose BD/DNC under the newly updated AAN guidelines. Understanding the evolution of how to effectively use these diagnostic tools is crucial for healthcare professionals who encounter these BD/DNC cases in their practice.

Case 325: Persistent Craniopharyngeal Canal.

HISTORY: A 76-year-old female patient with a history of rheumatoid arthritis, Sjögren syndrome, and hypertension presented with headache, fever, and dysphagia. The patient was taking prednisone and leflunomide to treat rheumatoid arthritis. The headache was primarily left sided and occasionally radiated to the occipital region. The patient had a high-grade fever spike, with a temperature of 104 °F (40 °C). Results of a prior lumbar puncture and temporal artery biopsy from an outside hospital were negative. Findings of a neurologic examination were unremarkable. Oropharyngeal examination for dysphagia revealed minimal pharyngeal motility, with pooling of food in the pharynx, which was thought to be due to cranial nerve dysfunction. Laboratory analysis revealed that, except for anemia, complete blood count parameters were within normal limits. C-reactive protein level (49.7 mg/L; reference range, 0.0-8.0 mg/L), erythrocyte sedimentation rate (>140 mm/h with Westergren method; reference range, 0-27 mm/h), and brain-type natriuretic peptide level (145 pg/mL; reference range, 0-100 pg/mL) were elevated. Blood and urine cultures were negative. A lumbar puncture was performed, which revealed cloudy cerebrospinal fluid (CSF), with an elevated CSF protein level (78 mg/dL; reference range, 7.0-35.0 mg/dL) and a low CSF glucose level (37 mg/dL [2.05 mmol/L]; reference range, 45-70 mg/dL [2.50-3.89 mmol/L]); otherwise, CSF encephalopathy, an autoimmune panel, and cultures were negative. CT and MRI of the brain with paranasal sinus were performed. Nasal endoscopy-guided drainage of the preclival fluid collection was performed, and biopsy of nasopharyngeal tissue was performed. Fluid culture revealed the growth of Candida albicans, as well as Pseudomonas and Enterobacter aerogenes. The patient received fluconazole, ceftriaxone, vancomycin, and metronidazole. Follow-up MRI was performed after 2 weeks.

Revisiting the "Puffed Cheek" Technique: Advantages, Fallacies, and Potential Solutions.

The "puffed cheek" technique is routinely performed during CT neck studies in patients with suspected oral cavity cancers. The insufflation of air within the oral vestibule helps in the detection of small buccal mucosal lesions, with better delineation of lesion origin, depth, and extent of spread. The pitfalls associated with this technique are often underrecognized and poorly understood. They can mimic actual lesions, forfeiting the technique's primary purpose. This review provides an overview of the puffed cheek technique and its associated pitfalls. These pitfalls include pneumoparotid, soft palate elevation that resembles a nasopharyngeal mass, various tongue displacements or distortions that obscure tongue lesions or mimic them, sublingual gland herniation, an apparent exacerbation of the airway edema, vocal cord adduction that hinders glottic evaluation, and false indications of osteochondronecrosis in laryngeal cartilage. Most stem from a common underlying mechanism of unintentional Valsalva maneuver engaged in by the patient while trying to perform a puffed cheek, creating a closed air column under positive pressure with resultant surrounding soft-tissue displacement. These pitfalls can thus be avoided by instructing the patient to maintain continuous nasal breathing while puffing out their cheek during image acquisition, preventing the formation of the closed air column. Keywords: CT, Head/Neck © RSNA, 2024.

Potential Role of Bone Scintigraphy in the Diagnosis of Calciphylaxis.

Nonosseous abnormalities are often seen on bone scans and can be related to a wide variety of pathology ranging across vascular, infection, and inflammatory etiology. Diffuse soft tissue radiotracer uptake on bone scans is typically attributed to renal or metabolic derangements. Calciphylaxis is the deposition of calcium in small blood vessels, skin, and other organs leading to vascular obstruction and skin necrosis. It is a rare disorder with unknown pathophysiology. Diagnosis of calciphylaxis is challenging and requires an interdisciplinary approach including clinical findings, laboratory results, medical imaging, and skin biopsy. An early diagnosis is important as the disease is associated with high morbidity and mortality. The purpose of this review article is to highlight the role of bone scintigraphy in the evaluation of calciphylaxis and to correlate the findings with other imaging modalities and histopathology.

Case 324: CHANTER Syndrome.

HISTORY: A 69-year-old right-handed man with a history of substance use disorder, hypertension, and diabetes presented to the emergency department in an unresponsive state. Upon examination, apart from tachycardia (heart rate, 108 beats per minute), vital signs were within normal ranges (blood pressure, 134/102 mm Hg; temperature, 97.9 ºF (36.6 ºC); respiratory rate, 16 per minute; oxygen saturation, 96%). The patient had a Glasgow coma scale score of 8. Otherwise, the physical examination revealed no abnormalities. Prior psychiatric and surgical histories were unremarkable. There was no history of recent travel, camping, hiking, or vaccination. No family history could be obtained. Laboratory work-up revealed an elevated creatine kinase level (49 006 U/L [818.4 µkat/L]; normal reference range, 10-205 U/L [0.17-3.42 µkat/L]). An electrocardiogram showed sinus tachycardia without evidence of cardiac ischemia. An echocardiogram was unremarkable. Alanine aminotransferase (126 U/L [2.10 µkat/L]; normal reference range, 0-40 U/L [0-0.67 µkat/L]) and aspartate aminotransferase (488 U/L [8.15 µkat/L]; normal reference range, 3-44 U/L [0.05-0.74 µkat/L]) levels were elevated. Polymerase chain reaction results were negative for HIV-1, HIV-2, syphilis treponemal, and COVID-19 antibodies. The remaining routine laboratory work-up findings were within normal limits. Urine drug screening was positive for cocaine, marijuana, fentanyl, and benzodiazepines. Naloxone was administered, but the patient remained unresponsive. Intubation was performed for airway protection. Noncontrast and contrast-enhanced CT of the head and CT angiography were performed in the emergency department to rule out an acute intracranial abnormality. Multisequence MRI of the brain with administration of intravenous contrast material was ordered for further assessment. CT of the abdomen and pelvis was unremarkable (images not shown).

Imaging review of the atypical spinal epidural space pathologies.

Pathologies affecting the spinal epidural space (SES) comprise various abnormalities. However, they all have the potential to cause thecal sac narrowing or spinal cord compression. In this review, we group these pathologies into degenerative, infective, neoplastic, vascular, traumatic, and others, focusing on their imaging features. Degenerative pathologies of the SES range from disc to facet disease, with a particular emphasis on the less common degenerative pathologies in this review. Infective pathologies affecting the epidural space include spondylodiscitis and associated epidural phlegmon and abscess. Neoplasms arising from typical SES components include neurofibroma, hemangioma, and liposarcoma. MRI is the best modality to assess the anatomy and abnormalities of the epidural space. MRI, combined with computed tomography, or a radiograph, is useful for the evaluation of bones or radiopaque foreign bodies.

Case 325.

HISTORY: A 76-year-old woman with a history of rheumatoid arthritis, Sjögren syndrome, and hypertension presented with a headache, fever, and dysphagia. The patient was taking prednisone and leflunomide to treat rheumatoid arthritis. Her headache was primarily left sided and occasionally radiated to the occipital region. The patient had a high-grade fever spike, with a temperature of 104°F (40°C). Results of a prior lumbar puncture and temporal artery biopsy from an outside hospital were negative. Findings of a neurologic examination were unremarkable. Oropharyngeal examination for dysphagia revealed minimal pharyngeal motility, with pooling of food in the pharynx, which was thought to be due to cranial nerve dysfunction. Laboratory analysis revealed that, except for anemia, complete blood count parameters were within normal limits. C-reactive protein level (49.7 mg/L; reference range, 0.0-8.0 mg/L), erythrocyte sedimentation rate (>140 mm/h with Westergren method; reference range, 0-27 mm/h), and brain-type natriuretic peptide level (145 pg/mL; reference range, 0-100 pg/mL) were elevated. Blood and urine cultures were negative. A lumbar puncture was performed, which revealed cloudy cerebrospinal fluid (CSF), with an elevated CSF protein level (78 mg/dL; reference range, 7.0-35.0 mg/dL) and a low CSF glucose level (37 mg/dL [2.05 mmol/L]; reference range, 45-70 mg/dL [2.50-3.89 mmol/L]); otherwise, CSF encephalopathy, an autoimmune panel, and cultures were negative. MRI and CT scans of the brain with paranasal sinus were performed (Figs 1-3). Nasal endoscopy-guided drainage of the preclival fluid collection was performed, and biopsy of nasopharyngeal tissue was performed. Fluid culture revealed the growth of Candida albicans, as well as Pseudomonas and Enterobacter aerogenes. The patient received fluconazole, ceftriaxone, vancomycin, and metronidazole. Follow-up MRI was performed after 2 weeks (Fig 4).

Case 324.

HISTORY: A 69-year-old right-handed man with a history of substance use disorder, hypertension, and diabetes presented to the emergency department in an unresponsive state. Upon examination, apart from tachycardia (heart rate, 108 beats per minute), vital signs were within normal ranges (blood pressure, 134/102 mm Hg; temperature, 97.9°F [36.6°C]; respiratory rate, 16 breaths per minute; oxygen saturation, 96%). He had a Glasgow coma scale score of 8. Otherwise, the physical examination revealed no abnormalities. His prior psychiatric and surgical histories were unremarkable. There was no history of recent travel, camping, hiking, or vaccination. No family history could be obtained. Laboratory work-up revealed an elevated creatine kinase level (49 006 U/L [818.4 µkat/L]; normal reference range, 10-205 U/L [0.17-3.42 µkat/L]). An electrocardiogram showed sinus tachycardia without evidence of cardiac ischemia. An echocardiogram was unremarkable. Alanine aminotransferase (126 U/L [2.10 µkat/L]; normal reference range, 0-40 U/L [0-0.67 µkat/L]) and aspartate aminotransferase (488 U/L [8.15 µkat/L]; normal reference range, 3-44 U/L [0.05-0.74 µkat/L]) levels were elevated. Polymerase chain reaction results were negative for HIV-1, HIV-2, syphilis treponemal, and COVID-19 antibodies. The rest of the routine laboratory work-up findings were within normal limits. Urine drug screening was positive for cocaine, marijuana, fentanyl, and benzodiazepines. Naloxone was administered, but the patient remained unresponsive. Intubation was performed for airway protection. Noncontrast and contrast-enhanced CT of the head (Fig 1) and CT angiography were performed in the emergency department to rule out an acute intracranial abnormality. Multisequence MRI of the brain with administration of intravenous contrast material was ordered for further assessment (Figs 2-4). CT of the abdomen and pelvis was unremarkable (images not shown).

Case 317: Adult-Onset Leukoencephalopathy with Axonal Spheroids and Pigmented Glia.

HISTORY: A 44-year-old previously healthy man with a 9-month history of progressive cognitive decline, depression, urinary incontinence, and inability to perform tasks of daily living presented to the emergency department with worsening cognitive and neuropsychiatric symptoms. He had become more distressed, and his family noticed him departing the house without closing doors, leaving water faucets running, and sending his children to school on Sundays. History taken from the patient's wife revealed that his brother had passed away in his late 30s after a slowly progressing functional and cognitive decline over the course of 5 years. No further detailed family history could be obtained. The review of systems was negative; he had no prior medical, psychiatric, or surgical history; and he denied any history of recent travel, camping, hiking, or vaccination. The patient was not taking any dietary supplements, nor was he taking any over-the-counter or prescription medication. Examination revealed vital signs were within normal limits. Neurocognitive assessment revealed a conscious, coherent, and alert patient with impaired memory and concentration. He showed poor attention, depressed mood, and restricted affect. He was unable to spell the word world forward, nor was he able to understand a request to spell it backward. The rest of the physical and neurologic examination revealed no abnormalities. Extensive laboratory work-up was conducted and included the following: toxicology screening; screening for HIV-1, HIV-2, and syphilis treponemal antibodies; COVID-19 polymerase chain reaction; and measurement of B1 and B12 levels. The results of screening were negative. Cerebrospinal fluid (CSF) assays, including CSF oligoclonal bands and CSF flow cytometry, revealed values within normal limits. CT of the brain without intravenous contrast material was performed in the emergency department to rule out acute intracranial abnormality. Multiplanar multisequence MRI of the brain without and with intravenous contrast material was ordered for further assessment. CT images of chest, abdomen, and pelvis were unremarkable (images not shown).

Case 313: Cerebral Venous Infarct Due to Internal Cerebral Vein Thrombosis in the Setting of COVID-19 Infection.

HISTORY: A 45-year-old woman presented to the emergency department of an outside hospital with shortness of breath and cough. Five days after initial presentation, the patient presented again to the same emergency department with worsening headache and progressive left arm and left leg weakness. She was transferred to the neuroscience intensive care unit of our hospital with concern for an intracranial abnormality based on her work-up at the outside hospital. Her past medical history was notable only for a large uterine fibroid. In our hospital, CT of the brain and chest and MRI of the brain, including perfusion studies, were performed. Additionally, CT venography of the brain was performed.

Case 313.

HISTORY: A 45-year-old woman presented to the emergency department of an outside hospital with shortness of breath and cough. Five days after initial presentation, the patient presented again to the same emergency department with worsening headache and progressive left arm and left leg weakness. She was transferred to the neuroscience intensive care unit of our hospital with concern for an intracranial abnormality based on her work-up at the outside hospital. Her past medical history was notable only for a large uterine fibroid. In our hospital, CT of the brain and chest (Figs 1, 2) and MRI of the brain, including perfusion studies (Figs 3, 4), were performed. Additionally, CT venography of the brain was performed (Fig 5).

Iatrogenic watering-can perineum and osteomyelitis of pubic ramus as a complication of post-urethral calculus removal UTI.

We present a case of a 55-year-old male with a history of urethroscopic calculus removal who later developed urinary tract infection (UTI), complicated by periurethral abscess formation with osteomyelitis of the inferior pubic ramus and a urethrocutaneous fistula after surgical drainage of the abscess. UTI with periurethral abscess and urethrocutaneous fistula (watering-can perineum) is a rare complication of UTI. A periurethral abscess with pubic osteomyelitis has not been previously reported.

Incidental diagnosis of liver tuberculosis in a patient with jaundice.

A 30-year-old afebrile woman presented with anorexia, yellowish discolouration of the sclera and bilateral pedal oedema. Blood investigations revealed severe anaemia, raised erythrocyte sedimentation rate and thrombocytopaenia. Liver function tests were abnormal with raised bilirubin, alanine transaminase and prothrombin time. Chest roentgenogram was negative for tuberculosis. Abdominal ultrasonography (USG) revealed coarsened echotexture of the liver with surface nodularity. Contrast-enhanced CT scan revealed heterogeneity with surface and parenchymal nodularity scattered throughout the liver parenchyma. USG-guided liver biopsy was performed, which showed changes in granulomatous hepatitis with positive Ziehl-Neelsen staining for acid-fast bacilli. The patient was started on antituberculous drugs. After completion of the antituberculous regimen, the patient made an uneventful recovery with normal range of aspartate aminotransferase, serum albumin and prothrombin time. In summary, isolated liver tuberculosis is rare and a high index of suspicion is required in a patient from an area where tuberculosis is endemic, after excluding other common diffuse liver pathologies.