Ectopic cervical thymus: Recognising the characteristic 'embroidery yarn' appearance on ultrasound.

Introduction: The thymus normally forms in the neck from the third pharyngeal pouch and descends to its normal position in the mediastinum. Arrest of descent or sequestration of thymic tissue can occur at any point along its path leading to an ectopic thymus which can present as a neck mass, usually in the paediatric age group. Purpose and Case Report: Ultrasound is generally performed in the presence of a neck mass in children. Although a characteristic 'starry sky' appearance of the thymus has been described on ultrasound, it is not considered sufficiently specific and cross-sectional imaging with magnetic resonance imaging is usually performed. On magnetic resonance imaging, the ectopic thymus appears as a homogeneous T1 isointense and T2 hyperintense mass and may actually appear ominous due to the tendency of ectopic thymus to sometimes show diffusion restriction unlike the normal thymus. Subsequent invasive biopsy or surgical removal is usually necessary to rule out a neoplastic lesion. In our observation, the ultrasound appearance of thymus is sufficiently distinctive to be confidently diagnosed as ectopic thymic tissue. This appearance is similar to the high-resolution appearance of an embroidery yarn. The reason most radiologists are not aware of the same is because the normal mediastinal thymus is not usually imaged by ultrasound. Conclusion: An accurate diagnosis on ultrasound would mean avoidance of expensive cross-sectional imaging and invasive biopsy or surgical excision in favour of regular non-invasive follow-up ultrasound scans until the lesion involutes in late childhood.

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.

Unruptured giant lateral thoracic meningocele: extremely rare cause of cerebrospinal fluid (CSF) hypotension in neurofibromatosis type 1.

Neurofibromatosis type 1 (NF1) is a multisystem neurocutaneous disorder. Scoliosis and dural ectasia are features of the associated mesodermal dysplasia. Lateral thoracic meningoceles can develop in NF1 and progressively enlarge due to cerebrospinal fluid (CSF) pulsations. Large meningoceles can cause compressive symptoms in the thorax. We are reporting a case of a NF1 presenting with acute onset respiratory distress, who also had chronic orthostatic headaches. CT chest showed unruptured enlarging bilateral lateral thoracic meningoceles causing lung compression. MRI of the brain and spine showed features of CSF hypotension, explaining the headaches. CSF hypotension with unruptured meningoceles is extremely rare. Management of the condition is challenging since surgical removal is prone to complications due to underlying mesodermal abnormalities. Cystoperitoneal shunting to relieve lung compression may worsen CSF hypotension. A shunt with a programmable valve allowed controlled drainage and successfully relieved lung compression without worsening of orthostatic headaches in our case.

Recurrent sporadic malignant triton tumor in the carotid sheath in the absence of neurofibromatosis.

Malignant Triton Tumors (MTTs) are a rare and aggressive subtype of malignant peripheral nerve sheath tumors (MPNSTs), often associated with neurofibromatosis type 1. This case report describes a unique instance of recurrent sporadic MTT within the carotid sheath in a 33-year-old male without any personal or familial history of neurofibromatosis. The patient initially presented with a biopsy-confirmed MTT in the right neck, involving the carotid body and brachial plexus, and underwent partial resection, radiation therapy, and chemotherapy. Six months later, the patient presented with recurrent MTT, and subsequently underwent radical tumor resection, segmental right carotid artery resection, and deep femoral vein interposition. Recovery was complicated by hematoma formation, and the patient developed vocal fold paralysis and a left vocal fold cyst, necessitating further surgeries. Yearly follow-ups for 8 years revealed no recurrence. This case emphasizes the importance of comprehensive patient evaluation, including clinical history, imaging, and biopsy findings, for accurate diagnosis and prompt surgical intervention in managing such rare and aggressive tumors. Further research is needed to identify novel therapies and improve survival rates for patients with MTTs.

Synchronous Ectopic Thyroid Gland and Ectopic Parathyroid Adenoma on 99mTc-Sestamibi Scintigraphy and Correlative Imaging.

99mTc-sestamibi scintigraphy localizes parathyroid adenoma as a persistent focus of uptake on delayed images, whereas thyroid glands in normal or ectopic locations are seen on only early images and wash out on delayed images. We report a case of absence of eutopic neck thyroid activity and synchronous ectopic lingual thyroid and mediastinal parathyroid adenoma on scintigraphy confirmed with CT.

Practical microscopic neuroanatomy of the limbic system and basal forebrain identifiable on clinical 3T MRI.

Microscopic neuroanatomy of limbic system and basal forebrain on MRI is complex and is a terra incognita for many radiologists, clinicians, and neuroscientists. Interestingly, most of the important structures/at least anatomical regions containing these structures demonstrable on cadaveric and surgical dissections can be identified on clinical MRI, with 3T being much better than 1.5T. This article teaches the practical MRI identification of these structures which will greatly help in evaluating complex ailments like temporal lobe epilepsy, Alzheimer dementia, and other neuropsychiatric disorders. This knowledge will also aid in accurate reporting of tumor spread along the white matter fasciculi in the temporal stem/basal forebrain region. Limbic system includes the mesial temporal structures and their connections, piriform cortex including "area tempestas," and the septal area comprising of subcallosal area and paraterminal gyrus. Basal forebrain includes structures like substantia innominata with basal nucleus of Meynert, diagonal gyrus/diagonal band of Broca, and nucleus accumbens lying in between the anterior perforated substance inferiorly and the anterior commissure superiorly.

Case 302: Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids.

HISTORY: Part one of this case appeared 4 months previously and may contain larger images. A 21-year-old immunocompetent man who was a long-term resident of Qatar presented to the emergency department with recurrent episodes of unprovoked generalized tonic-clonic seizures lasting 2-3 minutes that spontaneously resolved and were associated with postictal confusion. The patient also had progressive mild diplopia, intermittent dizziness, and numbness in the left arm over the course of 3 months. The patient did not have any other systemic symptoms or chronic medical diseases. He did not have any history of intake of illicit drugs, supplements, or regular medications; he had not received any recent vaccinations; and he had not undergone any surgical procedures. He had no history of travel. At presentation, vital signs were normal. Neurologic examination showed mild left homonymous hemianopia, normal gait with no cerebellar signs, and preserved sensations, power, tone, and reflexes in all four limbs. An electroencephalogram showed no epileptiform discharges. Chest CT and extensive laboratory work-up, including viral, fungal, bacterial, and parasite work-up, thyroid function tests, and immunologic blood tests yielded normal results. Those included normal complete and differential blood counts and normal serum chemistry. Serum analysis was negative for antinuclear antibody, Sjögren syndrome antigens A and B, cytoplasmic antineutrophil cvtoplasmic antibody, and paraneoplastic profile. Serum evaluation was also negative for human immunodeficiency virus type 1 and type 2 RNA, and Brucella, Schistosoma, and toxoplasma antibodies. Venereal Disease Research Laboratory (VDRL) and rapid plasma regain (RPR) test results were negative. Cerebrospinal fluid (CSF) analysis revealed clear fluid and normal pressure and biochemistry, except for elevated protein concentration (0.48 g/L) (normal range, 0.15-0.45 g/L). There were 43 leukocytes/µL (99% lymphocytes) (normal range, 0-5 leukocytes/µL; lymphocytes range, 40%-80%), with no atypical or malignant cells. CSF Gram staining, acid-fast staining, cryptococcal antigen, varicella-zoster virus polymerase chain reaction (PCR), herpes simplex virus PCR, VDRL, and RPR test results were negative. CSF cultures did not show any evidence of growth of bacteria, fungi, or acid-fast bacillus. CSF flow cytometry did not show a monoclonal lymphoid population. No CSF oligoclonal bands were detected. Conventional brain MRI with intravenous administration of contrast material and perfusion study were performed and included different sequences (Figs 1-3).

Loss of corneal nerves and brain volume in mild cognitive impairment and dementia.

Introduction: This study compared the capability of corneal confocal microscopy (CCM) with magnetic resonance imaging (MRI) brain volumetry for the diagnosis of mild cognitive impairment (MCI) and dementia. Methods: In this cross-sectional study, participants with no cognitive impairment (NCI), MCI, and dementia underwent assessment of Montreal Cognitive Assessment (MoCA), MRI brain volumetry, and CCM. Results: Two hundred eight participants with NCI (n = 42), MCI (n = 98), and dementia (n = 68) of comparable age and gender were studied. For MCI, the area under the curve (AUC) of CCM (76% to 81%), was higher than brain volumetry (52% to 70%). For dementia, the AUC of CCM (77% to 85%), was comparable to brain volumetry (69% to 93%). Corneal nerve fiber density, length, branch density, whole brain, hippocampus, cortical gray matter, thalamus, amygdala, and ventricle volumes were associated with cognitive impairment after adjustment for confounders (All P's < .01). Discussion: The diagnostic capability of CCM compared to brain volumetry is higher for identifying MCI and comparable for dementia, and abnormalities in both modalities are associated with cognitive impairment.

Case 302.

History A 21-year-old immunocompetent man who was a long-term resident of Qatar presented to the emergency department with recurrent episodes of unprovoked generalized tonic-clonic seizures lasting 2-3 minutes that spontaneously resolved and were associated with postictal confusion. The patient also had progressive mild diplopia, intermittent dizziness, and numbness in the left arm over the course of 3 months. The patient did not have any other systemic symptoms or chronic medical diseases. He did not have any history of intake of illicit drugs, supplements, or regular medications; he had not received any recent vaccinations; and he had not undergone any surgical procedures. He had no history of travel. At presentation, vital signs were normal. Neurologic examination showed mild left homonymous hemianopia, normal gait with no cerebellar signs, and preserved sensations, power, tone, and reflexes in all four limbs. An electroencephalogram showed no epileptiform discharges. Chest CT and extensive laboratory work-up, including viral, fungal, bacterial, and parasite work-up, thyroid function tests, and immunologic blood tests yielded normal results. Those included normal complete and differential blood counts and normal serum chemistry. Serum analysis was negative for antinuclear antibody, Sjögren syndrome antigens A and B, cytoplasmic antineutrophil cvtoplasmic antibody, and paraneoplastic profile. Serum evaluation was also negative for human immunodeficiency virus type 1 and type 2 RNA, and Brucella, Schistosoma, and toxoplasma antibodies. Venereal Disease Research Laboratory (VDRL) and rapid plasma regain (RPR) test results were negative. Cerebrospinal fluid (CSF) analysis revealed clear fluid and normal pressure and biochemistry, except for elevated protein concentration (0.48 g/L) (normal range, 0.15-0.45 g/L). There were 43 leukocytes/µL (99% lymphocytes) (normal range, 0-5 leukocytes/µL; lymphocytes range, 40%-80%), with no atypical or malignant cells. CSF Gram staining, acid-fast staining, cryptococcal antigen, varicella-zoster virus polymerase chain reaction (PCR), herpes simplex virus PCR, VDRL, and RPR test results were negative. CSF cultures did not show any evidence of growth of bacteria, fungi, or acid-fast bacillus. CSF flow cytometry did not show a monoclonal lymphoid population. No CSF oligoclonal bands were detected. Conventional brain MRI with intravenous administration of contrast material and a perfusion study were performed and included different sequences (Figs 1-3).

Autoimmune encephalitis and seizures, cerebrospinal fluid, imaging, and EEG findings: a case series.

Antibody-mediated encephalitides constitute a group of inflammatory brain diseases characterized by prominent neuropsychiatric symptoms and are associated with antibodies against neuronal cell-surface proteins, ion channels, or receptors. The diagnosis and management of autoimmune encephalitis include evaluation of the clinical presentation, brain imaging, cerebrospinal fluid (CSF) findings, antibody detection, and electroencephalography (EEG) findings. This is a retrospective study of adults 18 years or older with autoimmune encephalitis due to antibodies against membrane surface antigens as well as anti-glutamic acid decarboxylase (anti-GAD) antibodies. The electronic medical record was reviewed for demographic data, clinical data, laboratory results, EEG, and imaging findings. Antibody screening was requested for 341 patients between May 2014 and December 2019. Antibody screening was positive in 37 patients presenting with seizures and/or encephalopathy. Of these, 10 patients tested positive for antibodies against neuronal surface antigens or anti-GAD antibodies-2 patients had anti-GAD antibody encephalitis, 5 had anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis, and 3 had anti-leucine-rich glioma-inactivated 1 (anti-LGI1) encephalitis. Demographics, clinical presentation, EEG, imaging, and CSF findings are reported. Autoimmune encephalitides are a diverse group of disorders with a few common clinical features and MRI findings. MRI, EEG, and CSF findings can be normal or show nonspecific findings in autoimmune encephalitis. Therefore, early diagnosis of these disorders requires a high level of suspicion to avoid delaying the diagnosis. Carefully looking for diagnostic clinical features (e.g., faciobrachial dystonic seizures in anti-LGI1 encephalitis), significant findings in MRI (e.g., limbic encephalitis), and some EEG patterns (e.g., extreme delta brush and generalized rhythmic delta activity in anti-NMDAR encephalitis) may help in early diagnosis.

Susceptibility-weighted Imaging in Neuroradiology: Practical Imaging Principles, Pearls and Pitfalls.

Susceptibility-weighted imaging (SWI) was one of the recent and helpful advancement in magnetic resonance imaging. Its utilization -provided valuable information for the radiologists in multiple fields, including neuroradiology. SWI was able to demonstrate cerebral paramagnetic and diamagnetic substances. Therefore, the applications of this imaging technique were diverse in research and clinical neuroradiology. This article reviewed the basic technical steps, various clinical applications of SWI, and potential limitations. The practicing radiologist needs to be oriented about using SWI and phase images in the right- and left-handed MRI systems to demonstrate different brain pathologies, including neurovascular diseases, traumatic brain injuries, brain tumors, infectious and inflammatory, and neurodegenerative diseases.

Rosai-Dorfman disease of cranial and spinal origin - A case series.

BACKGROUND: Rosai-Dorfman disease (RDD) is an idiopathic nonneoplastic lymphadenopathy disorder which is characterized by lymph node enlargement, but it may also presents primarily involving a variety of extranodal sites, including central nerves system and craniospinal axis. This study reports five cases of craniospinal RDD, with review of epidemiology, clinical presentation, imaging, and histopathological features with current management strategies. CASE DESCRIPTION: Five cases of RDD are diagnosed at Hamad General Hospital, Qatar, during 2013-2018. Two cases had dural-based cranial lesions with overlying cranial involvement while three cases were having extradural thoracic spine lesions. All cases underwent surgical intervention and confirmed by histopathology. CONCLUSION: Craniospinal RDD is a rare clinical presentation and poses significant diagnostic challenges preoperatively due to its similarity with other neoplastic or inflammatory diseases. Surgical option to remove compressive neural pathology provides a good clinical outcome with no recurrence in long-term follow-up.

Primary Hypoparathyroidism Mimicking Ankylosing Spondylitis in a Young Man with Fahr's Syndrome: A Case Report.

Patients with chronic idiopathic hypoparathyroidism may develop neurological complications, including calcification of the basal ganglia and other areas of the brain. In Fahr's syndrome, intracranial calcification is associated with an underlying disorder such as hypo or hyperparathyroidism. We report the case of a 37-year-old gentleman, with a history of bilateral cataract surgery and seizures, who presented with a new episode of seizure and was found to have severe hypocalcemia and bilateral symmetric intracranial calcification due to previously diagnosed primary hypoparathyroidism. He had symptoms and signs mimicking ankylosing spondylitis (AS), but with negative radiological and serological findings, not fitting into the diagnosis of axial spondyloarthropathies (SpA), as per standard criteria. Patients with long-standing idiopathic hypoparathyroidism can have severe calcification of soft tissues and bones, including vertebrae and paravertebral soft tissues, causing inflammatory back pain and stiffness. It is vital to report such cases as their occurrence is rare, and physicians should be aware of the possibility while evaluating patients with inflammatory back pain. Treatment in these cases is directed towards hypocalcemia and underlying primary pathology rather than spondyloarthropathy.

Corneal Nerve and Brain Imaging in Mild Cognitive Impairment and Dementia.

BACKGROUND: Visual rating of medial temporal lobe atrophy (MTA) is an accepted structural neuroimaging marker of Alzheimer's disease. Corneal confocal microscopy (CCM) is a non-invasive ophthalmic technique that detects neuronal loss in peripheral and central neurodegenerative disorders. OBJECTIVE: To determine the diagnostic accuracy of CCM for mild cognitive impairment (MCI) and dementia compared to medial temporal lobe atrophy (MTA) rating on MRI. METHODS: Subjects aged 60-85 with no cognitive impairment (NCI), MCI, and dementia based on the ICD-10 criteria were recruited. Subjects underwent cognitive screening, CCM, and MTA rating on MRI. RESULTS: 182 subjects with NCI (n = 36), MCI (n = 80), and dementia (n = 66), including AD (n = 19, 28.8%), VaD (n = 13, 19.7%), and mixed AD (n = 34, 51.5%) were studied. CCM showed a progressive reduction in corneal nerve fiber density (CNFD, fibers/mm2) (32.0±7.5 versus 24.5±9.6 and 20.8±9.3, p < 0.0001), branch density (CNBD, branches/mm2) (90.9±46.5 versus 59.3±35.7 and 53.9±38.7, p < 0.0001), and fiber length (CNFL, mm/mm2) (22.9±6.1 versus 17.2±6.5 and 15.8±7.4, p < 0.0001) in subjects with MCI and dementia compared to NCI. The area under the ROC curve (95% CI) for the diagnostic accuracy of CNFD, CNBD, CNFL compared to MTA-right and MTA-left for MCI was 78% (67-90%), 82% (72-92%), 86% (77-95%) versus 53% (36-69%) and 40% (25-55%), respectively, and for dementia it was 85% (76-94%), 84% (75-93%), 85% (76-94%) versus 86% (76-96%) and 82% (72-92%), respectively. CONCLUSION: The diagnostic accuracy of CCM, a non-invasive ophthalmic biomarker of neurodegeneration, was high and comparable with MTA rating for dementia but was superior to MTA rating for MCI.

Mediastinal seminoma presenting as a neck mass falsely diagnosed as anaplastic thyroid carcinoma: A case report.

Germ cell tumors can occur in the mediastinum. They account for about 20% of tumors at this location. The majority are located in the anterior mediastinum and usually occur in young adult males. Extension of mediastinal germ cell tumors into the neck with mass formation is a very rare and unusual event. Herein, we report a case of a 34 year old male who presented with a progressively enlarging neck mass. Fine Needle Aspiration (FNA) was performed as initial evaluation and showed cellular smears comprising atypical large cells with prominent irregular nucleoli and moderate amount of cytoplasm with lymphocytic infiltrate and some epithelioid granulomas in the background. The mass was misdiagnosed initially on the cytology smears as anaplastic thyroid carcinoma. The subsequent tissue core biopsy showed sheets and nests of atypical cells admixed with ill-defined granulomatous inflammation. By immunohistochemistry, the tumor cells were immunoreactive with SALL4, PLAP and OCT3/4, compatible with malignant germ cell tumor, seminomatous type. It is very rare for patients with primary mediastinal seminoma to present initially with a neck mass. Fine Needle Aspiration (FNA) of this "neck mass" can lead to misinterpretation of findings due to similarities in cytological features between malignant germ cell tumors and other undifferentiated malignant neoplasms and the diagnosis, therefore, can be very challenging.

Functional magnetic resonance imaging of head and neck cancer: Performance and potential.

Functional magnetic resonance imaging (MRI) of tumors of the head and neck usually encompasses diffusion-weighted imaging (DWI) and intravenous (IV) contrast T1 dynamic perfusion imaging (DCE-MRI or PWI). Both techniques can characterize different tissues by probing into their microstructure, providing a novel approach in oncological imaging. In this pictorial review, we will cover the important technical aspects of DWI and PWI, the pathophysiological background and the current applications and potential of these functional MRI techniques in the imaging of head and neck cancer.

State of the art epilepsy imaging: an update.

Drug-resistant epilepsy is a chronic debilitating disorder, but many cases are potentially curable by surgery. The key to the successful epilepsy surgery with complete postoperative seizure-free status is precise localization of the epileptic zone that must be resected and the adjacent eloquent areas of gray and white matter that must be preserved to avoid neurological defects. This article reviews the current state of the art epilepsy imaging techniques facilitating successful epilepsy surgery. The overview of the imaging appearances of the most common epileptogenic etiologies that are amenable to surgical resection is also included in this review.

Reversible blindness in cryptococcal meningitis with normal intracranial pressure: case report and review of the literature.

Ocular complications in cryptococcal meningitis (CM) are commonly attributed to elevated intracranial pressure (ICP). We report a case of reversible vision loss complicating AIDS-related CM with a normal ICP. We review other cases of blindness in CM with normal ICP and the potential role of corticosteroids as treatment.

Congenital cystic neck masses: embryology and imaging appearances, with clinicopathological correlation.

Congenital cystic masses of the neck are uncommon and can present in any age group. Diagnosis of these lesions can be sometimes challenging. Many of these have characteristic locations and imaging findings. The most common of all congenital cystic neck masses is the thyroglossal duct cyst. The other congenital cystic neck masses are branchial cleft cyst, cystic hygroma (lymphangioma), cervical thymic and bronchogenic cysts, and the floor of the mouth lesions including dermoid and epidermoid cysts. In this review, we illustrate the common congenital cystic neck masses including embryology, clinical findings, imaging features, and histopathological findings.