Reactive hemophagocytic lymphohistiocytosis: Epidemiological, clinico-biological and etiological profile.

Hemophagocytic lymphohistiocytosis (HLH) is an hyperinflammatory state resulting from increased secretion of proinflammatory cytokines, which are responsible for clinical, biological and cytological manifestations. OBJECTIVE: The aim of our study is to describe the epidemiological, clinical, biological, etiological and evolutionary profile of HLH in Tunisia. METHODS: A retrospective study that involved patients, with images of hemophagocytosis in myelograms analyzed at the laboratory of biological hematology of the University Hospital "Hédi-Chaker" of Sfax-Tunisia, followed at these departments: hematology, internal medicine, department of infectious-diseases and department of gastroenterology, (June2017- May2021). First, we identified all patients with hemophagocytosis images. Secondly, we selected the patients who fulfilled the diagnostic criteria of the HLH-2004-score. RESULTS: Nineteen patients were included in this study. Nine men and 10 women with a mean age of 37.95 years. Fever was present in all patients. Organomegaly was described in 74% of cases. The most frequent cytopenia was anemia (100%). Hypertriglyceridemia was noted in 79% of cases and hyperferritinemia (> 500 ng/mL) was ubiquitous. In myelogram, 68% of patients had slides showing numerous or very numerous images of hemophagocytosis. The infectious pathology was the most common cause of HLH (42%). No cause was found in 10% of cases. The corticosteroid therapy at a dose of 1 mg/kg/day was prescribed in 89% of our patients. The overall evolution was favorable in 58% of cases. The mortality was not associated with the causal pathology (p=0.218). CONCLUSION: Secondary HLH is likely to be under-recognized, which contributes to its high morbidity and mortality. Early recognition is crucial for any reasonable attempt at curative therapy.

Cerebrospinal Fluid Leaks: Challenges in Localizing Spontaneous Spinal Leak Sites and Minimally Invasive Treatment.

The confirmation of cerebrospinal fluid (CSF) leaks in the setting of spontaneous intracranial hypotension (SIH) by imaging involves a growing toolset of multimodal advanced spinal and skull base imaging techniques, for which exists a unique set of challenges for each CSF leak type. Furthermore, the repertoire of minimally invasive CSF leak treatment beyond nontargeted epidural blood patch administration has grown widely, with varied practices across institutions. This review describes current diagnostic imaging and treatment modalities as they apply to the challenges of CSF leak localization and management.

[An expanding spleen]. / Une rate qui s'dilate.

A 24 years-old woman was admitted to hospital for an epistaxis that had not resolved. Biological results show bi-cytopenias (anemia and thrombopenia). Clinically she presents a fourth grade splenomegaly. To explore these cytopenias, a myelogram is performed.

Intrathecal injection of human placental mesenchymal stem cells derived exosomes significantly improves functional recovery in spinal cord injured rats.

BACKGROUND: Spinal cord injury (SCI) due to lack of restoration of damaged neuronal cells is associated with sensorimotor impairment. This study was focused on using the human placental mesenchymal stem cells- exosome (HPMSCs- Exosomes) in an animal model of severe SCI under myelogram procedure. METHODS AND RESULTS: Intrathecal injection of exosomes was performed in the acute phase of SCI in female rats. The improved functional recovery of the animals was followed for 6 weeks in control (saline, n = 6) and HPMSCs- EXO (HPMSCs-Exosomes, n = 6) groups. Pathological changes and glial scar size were evaluated. The Immunohistochemistry (IHC) of GFAP and NF200 factors as well as the apoptosis assay was investigated in the tissue samples from the injury site. The results demonstrated that HPMSCs-exosomes can improve motor function by attenuating apoptosis of neurons at the injury site, decreasing GFAP expression and increasing NF200 in the HPMSCs-EXO group. Also, HPMSCs-exosomes by preventing the formation of cavities causes preservation of tissue in SCI rats. CONCLUSIONS: These findings demonstrate the effectiveness of HPMSC-Exosomes as a therapeutic method to improve functional recovery, reduce pathological changes associated with injury, and prevent chronicity after SCI. The neuroprotective and anti-apoptotic potential of HPMSCs- Exosomes may be a promising therapeutic approach for SCI. Another result was the importance of intrathecal injection of exosomes in the acute phase, which accelerated the healing process. Furthermore, the myelogram can be a feasible and suitable method to confirm the accuracy of intrathecal injection and examine the subarachnoid space in the laboratory animals.

CSF-Venous Fistula of the Clival Skull Base: A Unique Case Study and Literature Review.

An adolescent male presented with orthostatic headaches following head trauma. MRI showed cerebellar tonsil displacement and a bony defect in the clival skull base. Digital subtraction myelography (DSM) confirmed a cerebrospinal fluid-venous fistula (CVF). This was repaired endoscopically. CVFs cause uncontrolled flow of CSF into the venous system resulting in symptoms of intracranial hypotension. They're often difficult to identify on initial imaging. This is the first reported CVF originating in the central skull base, and the first treated via endoscopic trans-nasal approach. CVFs may elude initial imaging, making DSM crucial for unexplained spontaneous intracranial hypotension. Laryngoscope, 134:645-647, 2024.

Direct pseudomeningocele contrast injection for spinal CSF leak localization.

CT myelography has been traditionally used to evaluate post-operative paraspinal fluid collections to discern CSF leaking into a pseudomeningocele versus a contained seroma. Rather than performing a lumbar puncture and injecting intrathecal contrast for myelography, we present the first report of direct contrast injection into a post-operative paraspinal pseudomeningocele for CSF leak confirmation and localization. This is a simple procedure that has several advantages over a conventional CT myelogram for the evaluation of post-operative paraspinal fluid collections.

An Uncommon Case of Multiple, Recurrent Cerebrospinal Fluid Venous Fistulas.

We present a clinical case to discuss the use of computed tomography (CT) spine myelograms alongside a complete history to diagnose multiple cerebrospinal fluid (CSF) venous fistulas (CVFs). The goal of this study is to familiarize clinicians with this challenging diagnosis and the utility of these studies in localizing leaks. A 63-year-old male patient with a history of cervical spinal stenosis, intermittent double vision, and sinus pressure managed with intermittent steroids presented to the clinic. He provided a detailed timeline of his previous symptoms and previous workups leading to the suspicion of intracranial hypotension due to CSF leak vs. CVF. Our workup, including magnetic resonance imaging (MRI) of the cervical spine and lumbar puncture (LP), was conducted. A CT thoracic spine myelogram was completed to localize the fistula site which was followed by the embolization of the fistula. The patient revealed complete resolution of his symptoms confirmed by imaging done one week postoperatively. This was a difficult case complicated by chronic misdiagnosis and confounding factors. CVFs were first described less than a decade ago; however, they are an extremely important cause of spontaneous intracranial hypotension. CVFs can be challenging to detect on conventional anatomical imaging like MRI. Thus, CT myelogram studies and a thorough history are crucial for accurate diagnosis. It is essential that clinicians, including ophthalmologists, learn to recognize CVFs as a potential cause of intracranial hypotension and become familiar with this diagnosis and its workup in the hopes that, unlike this case, the diagnosis and resolution of patients' life-altering symptoms are not delayed.

Utility of bone marrow examination in retinoblastoma and their correlation with hematological features.

Retinoblastoma makes up about 3% of all childhood malignancies. The frequency of metastatic retinoblastoma ranges from 4.8 to 11%. Assessing the bone marrow status of newly diagnosed patients is crucial because of the advantages of autologous bone marrow transplants for high-risk patients. This study aimed to determine the utility of bone marrow examination in cases of retinoblastoma and its correlation with hematological findings. This retrospective study was conducted at the Department of Pathology, King George's Medical University, Lucknow, India. A total of 34 cases of retinoblastoma with bone marrow examination were included in the study. Bone marrow infiltration was present in 17.65% (6/34) cases of retinoblastoma. Bone marrow aspirate myelogram showed that marrow metastasis in retinoblastoma was significantly linked with a reduced percentage of total myeloid cells (p=0.001) and segmented cells (p=0.006). The present study demonstrated that 15% (3/20) of retinoblastoma patients previously classified as nonmetastatic before bone marrow examination (stages I to III based on histology, imaging, and bone scan) had bone marrow metastases following bone marrow examination and were upgraded to stage IV. To conclude, a diligent and exhaustive search for metastatic cells in bone marrow is advised if the myelogram shows a reduced percentage of total myeloid and segmented cells. All stage II and stage III cases of retinoblastoma must undergo bone marrow examination for early metastasis detection, as it may result in an upgrade to stage IV disease, impacting the prognosis and necessitating distinct treatment modalities.

Exceptional Manifestation of Multiple Myeloma.

Despite being a rare occurrence, multiple myeloma (MM) has been reported as an alternative cause of pleurisy, with approximately 50 documented cases in the literature so far. In this case report, we present the clinical scenario of a patient who sought medical attention due to symptoms of dyspnea, chest pain, and weight loss. Through a comprehensive diagnostic evaluation, it was determined that the patient's pleural involvement was attributable to MM, a hematological malignancy. This case highlights the importance of considering MM as a potential etiology in patients presenting with pleural manifestations, even in settings where tuberculosis is the prevailing cause.

Updated ultrafast dynamic computed tomography myelography technique for cerebrospinal fluid leaks.

We present an updated ultrafast dynamic computed tomography myelography technique that can be used for the localization of spontaneous spinal cerebrospinal fluid leaks in the setting of spontaneous intracranial hypotension. This has over twice the temporal resolution of previously described techniques at the same radiation dose output.

Outcome of epidural blood patch for imaging-negative spontaneous intracranial hypotension.

BACKGROUND: Spontaneous intracranial hypotension is diagnosed by an abnormal finding in brain MRI, spinal imaging, or lumbar puncture. However, the sensitivity of each test is low. We investigated whether patients with suspected spontaneous intracranial hypotension and negative imaging findings would respond to epidural blood patch. METHODS: We prospectively recruited patients with new-onset orthostatic headache admitted at the Samsung Medical Center from January 2017 to July 2021. In patients without abnormal imaging findings and no history of prior epidural blood patch, treatment outcome-defined as both 50% response in maximal headache intensity and improvement of orthostatic component-was collected at discharge and three months after epidural blood patch. RESULTS: We included 21 treatment-naïve patients with orthostatic headache and negative brain and spinal imaging results who received epidural blood patch. After epidural blood patch (mean 1.3 times, range 1-3), 14 (66.7%) and 19 (90.5%) patients achieved both 50% response and improvement of orthostatic component at discharge and three months post-treatment, respectively. Additionally, complete remission was reported in 11 (52.4%) patients at three-month follow-up, while most of the remaining patients had only mild headaches. Among nine (42.9%) patients who underwent lumbar puncture, none had an abnormally low opening pressure (median 13.8 cm H2O, range 9.2-21.5). CONCLUSION: Given the high responder rates of epidural blood patch in our study, empirical epidural blood patch should be considered to treat new-onset orthostatic headache, even when brain and spinal imaging are negative. The necessity of lumbar puncture is questionable considering the high response rate of epidural blood patch and low rate of "low pressure."

Morphological changes in nerve rootlets in patients with cervical radiculopathy assessed using computed tomography myelogram.

Background: There are only a few reports that utilize computed tomography myelogram (CTM) to define cervical nerve rootlet compromise in patients with radiculopathy. Here, we assessed the characteristic morphological changes in nerve rootlets in 32 cases with cervical radiculopathy by analyzing CTMs warranting anterior foraminotomy. Methods: CTMs were analyzed to elucidate morphological changes in nerve rootlets in 31 patients/32 cases with cervical radiculopathy. Notably, three of the four neurosurgeons evaluating these CTM had to agree on the presence of "significant" changes consisting of contrast-enhanced defects near the entrance of the intervertebral foramen on axial sections. Results: Our findings included; posterior deviation of the ventral rootlet on the sagittal section (63%), ventral rootlet meandering or horizontalization (52%), and dorsal rootlet meandering (39%) on the coronal section. Furthermore, 16% of cervical nerve rootlets may exhibit an abnormal course proximal to the compression site (i.e., the definition of the "rootlet stagnation sign"). Conclusion: CTM better defines the anatomy, pathology, and radiology impacting the anterior or posterior cervical roots in patients presenting with cervical radiculopathy.

Pantopaque: Does It Still Migrate With Gravity After 30 Years?

Pantopaque was an oil-based positive contrast media used in central nervous system imaging before the use of water-soluble contrast agents. It is no longer used due to side effects, including arachnoiditis. Prior studies have indicated that remnants of pantopaque can be seen in modern radiographic imaging, including CT and MRI. With its use obsolete, these remnants have been increasingly mislabeled from "tumor" to "shot gun pellets". An understanding of this historic modality will usually lead to the correct diagnosis.

T2-Sampling Perfection With Application-Optimized Contrasts by Using Flip Angle Evolution (SPACE) Protocol MRI: A Safe, Minimally Invasive Screening Tool for Spinal CSF Leak Causing Spontaneous Intracranial Hypotension.

Spontaneous intracranial hypotension (SIH) due to a spinal cerebrospinal fluid (CSF) leak is secondary cause of headache with potentially devastating consequences. Its diagnosis is complicated owing to the lack of a reasonable, minimally invasive screening test. This results in many patients remaining undiagnosed for years after the headache onset. Current testing approaches are either overly invasive, namely the CSF infusion protocol or both invasive and insensitive viz. lumbar puncture (LP) with an opening pressure (OP) or computed tomography myelogram (CTM). These diagnostic methods are frequently employed in a clinical setting since they require access to the thecal space; they unfortunately have a dearth of sensitivity. CTM will not document a leak if it is intermittent or very slow and in the setting of a spinal CSF leak, the OP on LP may be high, low, or normal. A potential remedy for this state is the T2-sampling perfection with application-optimized contrasts by using flip angle evolution (SPACE) protocol spinal magnetic resonance imaging (MRI). We present two cases that demonstrate its potential value as a screening tool. It is well known for its high sensitivity for identifying spinal pathology and is minimally invasive, making it a good choice for a screening modality when diagnosing possible SIH cases.

Dorsal arachnoid web: A rare clinical entity.

A dorsal arachnoid web is a rare entity involving abnormal formation of arachnoid mater into a dense web which may result in ventral herniation of the spinal cord and significant neurologic symptoms. Back pain or a compressive myelopathy are two of the most common presenting symptoms. CT myelogram or MRI can aid in the diagnosis of arachnoid web, and management of includes surgical and conservative approaches. Herein, we describe two interesting cases of arachnoid webs highlighting the diagnostic approach and differences in the approach to management of each case.

[A rare case of non-secretory multiple myeloma]. / Un cas rare de myélome multiple non-sécrétant.

An 88-year-old man is sent to hospital because of a rapid worthening of his general state. A myelogram is obtained following the discovery of hypercalcemia associated with a diminished glomerular filtration rate and the discovery of osteolytic lesions, showing a massive proliferation of plasma cells. The lack of plasma and urine detection of both monoclonal antibodies and immunoglobulin light chains, as well as an immature plasma cell phenotype, suggest the diagnosis of non-secretory multiple myeloma. This article aims to describe the atypical clinical presentation of a rare case of multiple myeloma.

When does CT myelography add value beyond MRI for lumbar degenerative disease?

BACKGROUND CONTEXT: In patients with lumbar spinal stenosis, it is crucial for clinicians to identify all symptomatic levels. Prior studies have demonstrated that CT myelography has a greater sensitivity in revealing stenosis (94.4%) compared to MRI (75.9%). However, this is an invasive test that should be used judiciously. No study has identified subgroups of patients that do or do not benefit from this additional invasive testing. PURPOSE: The objective of this study was to identify subgroups of patients with lumbar stenosis for whom CT myelogram could be expected to provide additional information following an MRI scan. STUDY DESIGN: Retrospective chart review. PATIENT SAMPLE: Consecutive series of patients with lumbar degenerative disease seen at a single multisurgeon tertiary spine center. OUTCOME MEASURES: Degree of stenosis on MRI or CT myelo. METHODS: Medical records were reviewed to collect standard demographic and surgical data and patient diagnoses. MRI and CT myelo obtained within 6 months of each other in patients >45 years old with a diagnosis of central stenosis, spondylolisthesis or degenerative scoliosis were reviewed. Each lumbar level was recorded as mild, moderate, or severe based on the radiologist's report. Fisher exact test was performed with change in recorded severity of stenosis from MRI to CT myelo as the primary outcome of interest. RESULTS: Of 269 patients, 207 (80%) had at least one level of moderate or severe central stenosis on MRI and 62 had mild or no stenosis on MRI. Of the 207, 139 (67%) had multilevel stenosis and 68 (33%) had single level stenosis. CT myelo identified a greater proportion of additional stenotic levels in patients with multilevel stenosis (80/139, 58%) compared to patients with single-level stenosis (27/68, 40%, p=.018). In 62 patients with a clinical diagnosis of lumbar stenosis but no moderate to severe stenosis on MRI, CT myelogram identified three additional stenotic levels (3/65, 5%, p=.836). CONCLUSIONS: CT myelography is not as useful in providing additional information in patients with no stenosis or single level stenosis as compared to patients with multilevel stenosis.

Iatrogenic CSF Leak from CT Myelogram Leading to Cauda Equina Compression: A Case Report.

Introduction: Although magnetic resonance imaging (MRI) is the preferred imaging modality for diagnosing cauda equina syndrome (CES), computerized tomography (CT) myelogram may be used in patients who are unable to undergo MRI. When inserting the needle for CT myelogram, there is a risk of cerebrospinal fluid (CSF) leak, which theoretically could lead to CES. To the best of our knowledge, there are no reports of CT myelogram resulting in cauda equina compression. Case Report: We report the case of a 38-year-old man who underwent surgical decompression for CES and developed an iatrogenic CSF leak from a pre-operative CT myelogram causing recurrent thecal sac compression requiring repeat surgery and dural repair. Conclusion: Although CT myelogram may be used to aid in the diagnosis of CES, consideration should be given to the potential risk for causing a CSF leak and resultant thecal sac compression.

Fully automated rigid image registration versus human registration in postoperative spine stereotactic body radiation therapy: a multicenter non-inferiority study.

To confirm the fully automated rigid image registration (A-RIR) accuracy in postoperative spine stereotactic body radiation therapy (SBRT), we conducted a multicenter non-inferiority study compared to the human rigid image registration (H-RIR). Twenty-eight metastatic cancer patients who underwent postoperative spine SBRT are enrolled-image registration (IR) of planning computed tomography (CT) and CT-myelogram for delineating the spinal cord. The adopted A-RIR workflow is a contour-focused algorithm performing a rigid registration by maximizing normalized mutual information (NMI) restricted to the data contained within the automatically extracted contour. Three radiation oncologists (ROs) from multicenters were prompted to review two blinded registrations and choose one for clinical use. Indistinguishable cases were allowed to vote equivalent, counted A-RIR side. A-RIR is considered non-inferior to H-RIR if the lower limit of the 95% confidence interval (CI) of A-RIR preferable/equivalent is greater than 0.45. We also evaluated the NMI improvement from the baseline and the translational/rotational errors between A-RIR and H-RIR. The A-RIR preferable/equivalent was selected in 21 patients (0.75, 95% CI: 0.55-0.89), demonstrating non-inferiority to H-RIR. The A-RIR's NMI improvement was greater than H-RIR in 24 patients: the mean value ± SD was 0.225 ± 0.115 in A-RIR and 0.196 ± 0.114 in H-RIR (P < 0.001). The absolute translational error was 0.38 ± 0.31 mm. The rotational error was -0.03 ± 0.20, 0.05 ± 0.19, -0.04 ± 0.20 degrees in axial, coronal, and sagittal planes (range: -0.66-0.52). In conclusion, A-RIR shows non-inferior to H-RIR in CT and CT-myelogram registration for postoperative spine SBRT planning.

Diagnostic Evaluation and Cervical Spine Surgery in the Setting of a Cardiac Left Ventricular Assist Device: Challenges and a Case Illustration.

Due to incompatibility with magnetic resonance imaging, patients with left ventricular assist devices (LVADs) presenting with pathologies of the spinal soft tissues or neural elements represent diagnostically complex cases. We present a case of a patient undergoing a CT (computed tomography) myelogram and subsequent successful cervical posterior laminectomy. A C1-C2 lateral puncture approach CT myelogram revealed nearly a complete block of contrast movement at the level of the C2-C3 vertebrae concerning a compressive etiology. The cervical lateral approach was chosen based on patient symptomology and concern that contrast dye injected in the lumbar spine would not travel to the region of interest due to altered CSF pulsatility caused by the LVAD device. A C3-C7 posterior laminectomy was then successfully performed. Intra-operatively, however, there was no sign of a compressive lesion, and ultrasound confirmed a decompressed spinal cord. This case highlights the diagnostic challenges of pre-operative evaluation in patients with LVADs in which the efficacy of performing CT myelograms is also questionable due to potential alterations in cerebrospinal fluid movement due to variations in arterial pulsatility due to LVAD physiology.