Recurrent petit mal seizures in Erdheim-Chester disease mimicking an intra-axial brain tumor: illustrative case.

BACKGROUND: Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis characterized histologically by foamy histiocytes and Touton giant cells in a background of fibrosis. Bone pain with long bone osteosclerosis is highly specific for ECD. Central nervous system involvement is rare, although dural, hypothalamic, cerebellar, brainstem, and sellar region involvement has been described. OBSERVATIONS: A 59-year-old man with a history of ureteral obstruction, medically managed petit mal seizures, and a left temporal lesion followed with serial magnetic resonance imaging (MRI) presented with worsening seizure control. Repeat MRI identified bilateral amygdala region lesions. Gradual growth of the left temporal lesion over 1 year with increasing seizure frequency prompted resection. A non-Langerhans cell histiocytosis with a BRAF V600E mutation was identified on pathology. Imaging findings demonstrated retroperitoneal fibrosis and long bone osteosclerosis with increased fluorodeoxyglucose uptake that, together with the neuropathologic findings, were diagnostic of ECD. LESSONS: This case of biopsy-proven ECD is unique in that the singular symptom was seizures well controlled with medical management in the presence of similarly located bilateral anterior mesial temporal lobe lesions. Although ECD is rare intracranially, its variable imaging presentation, including the potential to mimic seizure-associated medial temporal lobe tumors, emphasizes the need for a wide differential diagnosis.

Lack of Clinical Utility of Labeled White Blood Cell Scintigraphy in Patients With Fever of Unknown Origin.

BACKGROUND: Labeled white blood cell scintigraphy (WBCS) has been used for over 40 years to localize an infection source in patients with fever of unknown origin (FUO). It continues to be in widespread use for such patients in modern times, despite the tremendous advances in modern radiological imaging and laboratory medicine. METHODS: We critically evaluated the clinical contribution of WBCS performed in 132 patients with FUO at 7 hospitals from mid-2015 to the end of 2019. For each patient, all radiographic and laboratory results and all electronic clinical notes were carefully evaluated as many days before and after the scan as necessary to arrive at a final diagnosis. RESULTS: Although 50 WBCS (38%) showed positive findings, the majority of these were false positive (FP). Of the 19 true-positive (TP) scans, most were already known or about to become known by tests already ordered at the time of the scan. Only 2 TP scans (1.5%) contributed to the final diagnosis, and these did so only indirectly. FP scans led to 7 unnecessary procedures. CONCLUSIONS: In FUO patients for whom an infection source is not discovered following an appropriate radiographic and laboratory workup, WBCS is not a useful procedure.

Two different 18F-FDG brain PET metabolic patterns in autoimmune limbic encephalitis.

PURPOSE: Autoimmune limbic encephalitis (ALE) is a severe, but treatable, neuropsychiatric disorder that is difficult to diagnose clinically. With the goal of improving diagnosis of this disorder, we retrospectively evaluated the cerebral FDG PET pattern in a group of patients with ALE. MATERIALS AND METHODS: Nine adult patients with subacute cognitive decline were eventually diagnosed with ALE based on clinical presentation, cerebrospinal fluid inflammatory markers, and response to immunosuppressive therapy. All patients received FDG PET brain scanning during their diagnostic evaluation, which were retrospectively reviewed for this study. RESULTS: Our patients' scans fell into 2 readily separable patterns. Five younger patients had a mixed metabolic pattern most easily recognized by pronounced occipital hypometabolism, accentuated by hypermetabolism in the temporal and orbitofrontal cortex. Other, milder findings were also present. Once this unusual pattern was established as corresponding to ALE, it helped lead to the correct clinical diagnosis in the last 2 patients. Four older patients had scans that closely resembled diffuse neurodegenerative disease. CONCLUSIONS: We found 2 different PET scan patterns in patients with ALE. One is an easily recognizable mixture of hyper- and hypometabolism that has also been described in a few recent case reports and is potentially specific for ALE. The other is indistinguishable from neurodegenerative disease. We propose that awareness of these patterns may contribute to the diagnosis of this elusive, but treatable, neurologic disorder.

Diagnostic nuclear medicine in the ED.

Although the decision to use nuclear medicine (NM) modalities in the acute care setting is limited by several factors, there are instances in which the use of NM techniques can provide elegant and efficient solutions to otherwise expensive and resource consuming situations. Herein, we describe the indications and NM techniques used for the evaluation of low-risk patients with chest pain, suspected pulmonary embolus, acute cholecystitis, gastrointestinal bleeding, acute scrotum, and the radiographically occult fracture.

Developing measurement indices to enhance protection and resilience of critical infrastructure and key resources.

The US Department of Homeland Security (DHS) is developing indices to better assist in the risk management of critical infrastructures. The first of these indices is the Protective Measures Index - a quantitative index that measures overall protection across component categories: physical security, security management, security force, information sharing, protective measures and dependencies. The Protective Measures Index, which can also be recalculated as the Vulnerability Index, is a way to compare differing protective measures (eg fence versus security training). The second of these indices is the Resilience Index, which assesses a site's resilience and consists of three primary components: robustness, resourcefulness and recovery. The third index is the Criticality Index, which assesses the importance of a facility. The Criticality Index includes economic, human, governance and mass evacuation impacts. The Protective Measures Index, Resilience Index and Criticality Index are being developed as part of the Enhanced Critical Infrastructure Protection initiative that DHS protective security advisers implement across the nation at critical facilities. This paper describes two core themes: determination of the vulnerability, resilience and criticality of a facility and comparison of the indices at different facilities.

Exploratory study of 99mTc-EC20 imaging for identifying patients with folate receptor-positive solid tumors.

UNLABELLED: 99mTc-EC20 is a folate receptor (FR)-targeted imaging agent consisting of the vitamin folate conjugated to 99mTc. FR is expressed on a variety of epithelial cancers, with advanced cancers often expressing FR at significantly higher levels than earlier stages of the disease. The goals of this pilot study were to determine the percentages of various solid tumors that accumulate 99mTc-EC20 in vivo and to correlate 99mTc-EC20 uptake with immunohistochemistry (IHC) analysis of FR expression in available biopsied tumor tissue. METHODS: A total of 154 patients with proven or suspected cancer and at least one lesion of > or =1.5 cm underwent imaging with 99mTc-EC20. The majority of these patients (77%) had a diagnosis of renal cell carcinoma. The remaining patients had a variety of other solid tumors. Whole-body planar images were obtained 1-2 h after injection, followed by SPECT of the region containing index lesions. The uptake of 99mTc-EC20 in tumors was scored as no uptake, mild uptake, or marked uptake. The resultant 99mTc-EC20 data were analyzed for correlation with the expression of the alpha-isoform of FR, as determined by IHC analysis, in tissue available from prior or subsequent surgery or biopsy. RESULTS: The administration of 99mTc-EC20 was well tolerated. Tumors with increased 99mTc-EC20 uptake were identified in 68% of patients, and IHC results were positive for the expression of the alpha-isoform of FR in 67% of patients. The agreement between methods was 61% overall (kappa = 0.096; 95% confidence interval = -0.085 to 0.277), with 72% agreement of positive results and 38% agreement of negative results. CONCLUSION: In vivo imaging with 99mTc-EC20 identified approximately two thirds of patients as having FR-positive tumors. Agreement between imaging and in vitro IHC was poor but was potentially confounded by a lack of correlation between the time of tissue sampling and the time of 99mTc-EC20 imaging, the heterogeneous expression of FR in metastatic lesions from the same patient, and the inability to detect the beta-isoform of FR by IHC. This pilot study of 99mTc-EC20 scintigraphy indicates that the agent is safe and well tolerated and that this noninvasive procedure may have utility in selecting patients likely to benefit from FR-targeted therapy.

Imaging of lymph flow in breast cancer patients after microdose administration of a near-infrared fluorophore: feasibility study.

PURPOSE: To prospectively demonstrate the feasibility of using indocyanine green, a near-infrared (NIR) fluorophore at the minimum dose needed for noninvasive optical imaging of lymph nodes (LNs) in breast cancer patients undergoing sentinel lymph node mapping (SLNM). MATERIALS AND METHODS: Informed consent was obtained from 24 women (age range, 30-85 years) who received intradermal subcutaneous injections of 0.31-100 microg indocyanine green in the breast in this IRB-approved, HIPAA-compliant, dose escalation study to find the minimum microdose for imaging. The breast, axilla, and sternum were illuminated with NIR light and the fluorescence generated in the tissue was collected with an NIR-sensitive intensified charged-coupled device. Lymphoscintigraphy was also performed. Resected LNs were evaluated for the presence of radioactivity, blue dye accumulation, and fluorescence. The associations between the resected LNs that were fluorescent and (a) the time elapsed between NIR fluorophore administration and resection and (b) the dosage of NIR fluorophores were tested with the Spearman rank and Pearson product moment correlation tests, respectively. RESULTS: Lymph imaging consistently failed with indocyanine green microdosages between 0.31 and 0.77 microg. When indocyanine green dosages were 10 microg or higher, lymph drainage pathways from the injection site to LNs were imaged in eight of nine women; lymph propulsion was observed in seven of those eight. When propulsion in the breast and axilla regions was present, the mean apparent velocities ranged from 0.08 to 0.32 cm/sec, the time elapsed between "packets" of propelled fluid varied from 14 to 92 seconds. In patients who received 10 microg of indocyanine green or more, a weak negative correlation between the fluorescence status of resected LNs and the time between NIR fluorophore administration and LN resection was found. No statistical association was found between the fluorescence status of resected LNs and the dose of NIR fluorophore. CONCLUSION: NIR fluorescence imaging of lymph function and LNs is feasible in humans at microdoses that would be needed for future molecular imaging of cancer-positive LNs.

PET imaging of cerebral and pulmonary Nocardia infection.

We report the case of a 69-year-old man who was suspected to have lung cancer with a single metastasis to the brain. Initial workup for neurologic and pulmonary symptoms demonstrated a ring-enhancing lesion in his right frontal lobe on MRI and a lung mass on CT. An F-18 fluorodeoxyglucose positron emission tomography (FDG PET) scan demonstrated marked glucose hypermetabolism in the lung and brain lesions with maximal standard uptake values (SUV) in both lesions of approximately 11. Biopsy of the brain mass revealed an abscess and cultures grew Nocardia. He was treated for nocardiosis, and a repeat CT of the chest in 2 months and MRI of the brain in 5 months showed nearly complete resolution of the lesions. Currently, there are few reported cases of PET evaluation of brain abscesses, particularly Nocardia. We discuss the appearance of brain infections on FDG PET scans in immunocompetent and immunocompromised patients.

Hyperscanning: simultaneous fMRI during linked social interactions.

"Plain question and plain answer make the shortest road out of most perplexities." Mark Twain-Life on the Mississippi. A new methodology for the measurement of the neural substrates of human social interaction is described. This technology, termed "Hyperscan," embodies both the hardware and the software necessary to link magnetic resonance scanners through the internet. Hyperscanning allows for the performance of human behavioral experiments in which participants can interact with each other while functional MRI is acquired in synchrony with the behavioral interactions. Data are presented from a simple game of deception between pairs of subjects. Because people may interact both asymmetrically and asynchronously, both the design and the analysis must accommodate this added complexity. Several potential approaches are described.