Duropathy as a rare motor neuron disease mimic: from bibrachial amyotrophy to infratentorial superficial siderosis.

BACKGROUND: Bibrachial amyotrophy associated with an extradural CSF collection and infratentorial superficial siderosis (SS) are rare conditions that may occasionally mimic ALS. Both disorders are assumed to be due to dural tears. CASE PRESENTATION: A 53-year-old man presented with a 7-year history of slowly progressive asymmetric bibrachial amyotrophy. Initially, a diagnosis of atypical motor neuron disease (MND) was made. At re-evaluation 11 years later, upper limb wasting and weakness had further progressed and were accompanied by sensorineural hearing loss. MRI of the brain and spine demonstrated extensive supra- and infratentorial SS (including the surface of the whole spinal cord) as well as a ventral longitudinal intraspinal fluid collection (VLISFC) extending along almost the entire thoracic spine. Osteodegenerative changes were observed at C5-C7 level, with osteophytes protruding posteriorly. The bony spurs at C6-C7 level were hypothesized to have lesioned the dura, causing a CSF leak and thus a VLISFC. Review of the MRI acquired at first evaluation showed that the VLISFC was already present at that time (actually beginning at C7 level), whereas the SS was not. 19 years after the onset of upper limb weakness, the patient additionally developed parkinsonism. Response to levodopa, brain scintigraphy with 123I-ioflupane and brain MRI with nigrosome 1 evaluation were consistent with idiopathic Parkinson's disease (PD). On the latest follow-up 21 years after symptom onset, the VLISFC was unchanged, as were upper arm weakness and wasting. CONCLUSIONS: Based on the long-term follow-up, we could establish that, while the evidence of the VLISFC was concomitant with the clinical presentation of upper limb amyotrophy and weakness, the radiological signs of SS appeared later. This suggests that SS was not per se the cause of the ALS-like clinical picture, but rather a long-term sequela of a dural leak. The latter was instead the causative lesion, giving rise to a VLISFC which compressed the cervical motor roots. Dural tears can actually cause several symptoms, and further studies are needed to elucidate the pathophysiological correlates of "duropathies". Finally, as iron metabolism has been implicated in PD, the co-occurrence of PD with SS deserves further investigation.

Resection vs. coagulation of dural attachment in patients with spinal meningioma: an updated systematic review and meta-analysis.

BACKGROUND: The Simpson grading scale assumes dural resection (grade I) is more effective against recurrence than coagulation (grade II). However, the results of recent studies have raised doubts about this effectiveness in spinal meningiomas. Therefore, we aimed to perform a meta-analysis comparing outcomes between Simpson grades I and II in spinal meningiomas. METHODS: According to the PRISMA statement, we systematically searched PubMed, EMBASE, and Web of Science for studies involving patients with spinal meningiomas who underwent Simpson grades I, II, III, or IV. Outcomes were radiological tumor recurrence, postoperative neurological deficits, and procedure-related complications. RESULTS: We included 54 studies with a total of 3334 patients. Simpson grades I, II, III, and IV were performed in 674 (20%), 2205 (66%), 254 (8%), and 201 (6%) patients, respectively. The follow-up ranged from 9 to 192 months, and 95.4% of all tumors were WHO grade 1. There was no difference in radiological tumor recurrence (OR 0.80, 95% CI: 0.46-1.36, P = 0.41; I2 = 0%), postoperative neurological deficits (OR 0.74, 95% CI: 0.32-1.75, P = 0.50; I2 = 0%) or procedure-related complications (OR 2.22, 95% CI: 0.80-6.13, P = 0.12; I2 = 3%) between Simpson grades I and II. Furthermore, no significant difference in postoperative neurological deficits or procedure-related complications was detected when comparing all Simpson's to each other. However, radiological tumor recurrences in Simpson I and II were significantly lower than in III and IV, with Simpson III outperforming IV (OR 0.19, 95% CI: 0.09-0.40, P < 0.01; I2 = 0%). CONCLUSION: Simpson grade I is not more effective than grade II in any outcome, although both are superior to III and IV in tumor recurrence. Our results might suggest that dural coagulation is preferable over resection when the latter carries a higher risk of complications.

Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue of the dura mimicking meningioma: a case report and literature review.

MALT lymphoma of the dura is a very rare type of low-grade B-cell lymphoma. Little more than 100 cases have been reported in the literature to date. We report a 43-year-old woman who was referred to hospital because of a series of three tonic-clonic seizures on the day of admission. Neurological examination revealed confusion and aphasia. Magnetic resonance imaging (MRI) showed a contrast-enhanced, broad-based lesion along the dura in the left parieto-occipital area. The suspicion of an en plaque meningioma was raised. The tumour invaded the brain parenchyma with visible extension into the brain sulci. There was a marked brain oedema surrounding the lesion and causing the midline shift 8 mm to the right. After stabilization of neurological condition (intravenous diuretics and steroids), the operation was performed. The diagnosis of dural MALT lymphoma was established. During the pathological examination, it was especially problematic to distinguish MALT lymphoma from follicular lymphoma, but the final diagnosis was MALT lymphoma. Surgical partial removal with additional R-CVP immunochemotherapy (rituximab, cyclophosphamide, vincristine and prednisone) resulted in complete remission. The follow-up period is 1 year. Our presented case of a MALT lymphoma highlights the fact that surgical partial removal with additional immunochemotherapy is an available option in these rare intracranial tumours.

Automatic segmentation of dura for quantitative analysis of lumbar stenosis: A deep learning study with 518 CT myelograms.

BACKGROUND: The diagnosis of lumbar spinal stenosis (LSS) can be challenging because radicular pain is not often present in the culprit-level localization. Accurate segmentation and quantitative analysis of the lumbar dura on radiographic images are key to the accurate differential diagnosis of LSS. The aim of this study is to develop an automatic dura-contouring tool for radiographic quantification on computed tomography myelogram (CTM) for patients with LSS. METHODS: A total of 518 CTM cases with or without lumbar stenosis were included in this study. A deep learning (DL) segmentation algorithm 3-dimensional (3D) U-Net was deployed. A total of 210 labeled cases were used to develop the dura-contouring tool, with the ratio of the training, independent testing, and external validation datasets being 150:30:30. The Dice score (DCS) was the primary measure to evaluate the segmentation performance of the 3D U-Net, which was subsequently developed as the dura-contouring tool to segment another unlabeled 308 CTM cases with LSS. Automatic masks of 446 slices on the stenotic levels were then meticulously reviewed and revised by human experts, and the cross-sectional area (CSA) of the dura was compared. RESULTS: The mean DCS of the 3D U-Net were 0.905 ± 0.080, 0.933 ± 0.018, and 0.928 ± 0.034 in the five-fold cross-validation, the independent testing, and the external validation datasets, respectively. The segmentation performance of the dura-contouring tool was also comparable to that of the second observer (the human expert). With the dura-contouring tool, only 59.0% (263/446) of the automatic masks of the stenotic slices needed to be revised. In the revised cases, there were no significant differences in the dura CSA between automatic masks and corresponding revised masks (p = 0.652). Additionally, a strong correlation of dura CSA was found between the automatic masks and corresponding revised masks (r = 0.805). CONCLUSIONS: A dura-contouring tool was developed that could automatically segment the dural sac on CTM, and it demonstrated high accuracy and generalization ability. Additionally, the dura-contouring tool has the potential to be applied in patients with LSS because it facilitates the quantification of the dural CSA on stenotic slices.

Quantitative measurement of dural ectasia: associations with clinical and genetic characteristics in Marfan syndrome.

PURPOSE: Dural ectasia (DE) may significantly impact Marfan syndrome (MFS) patients' quality of life due to chronic lower back pain, postural headache and urinary disorders. We aimed to evaluate the association of quantitative measurements of DE, and their evolution over time, with demographic, clinical and genetic characteristics in a cohort of MFS patients. METHODS: We retrospectively included 88 consecutive patients (39% females, mean age 37.1 ± 14.2 years) with genetically confirmed MFS who underwent at least one MRI or CT examination of the lumbosacral spine. Vertebral scalloping (VS) and dural sac ratio (DSR) were calculated from L3 to S3. Likely pathogenic or pathogenic FBN1 variants were categorized as either protein-truncating or in-frame. The latter were further classified according to their impact on the cysteine content of fibrillin-1. RESULTS: Higher values of the systemic score (revised Ghent criteria) were associated with greater DSR at lumbar (p < 0.001) and sacral (p = 0.021) levels. Patients with protein-truncating variants exhibited a greater annual increase in lumbar (p = 0.039) and sacral (p = 0.048) DSR. Mutations affecting fibrillin-1 cysteine content were linked to higher VS (p = 0.009) and DSR (p = 0.038) at S1, along with a faster increase in VS (p = 0.032) and DSR (p = 0.001) in the lumbar region. CONCLUSION: Our study shed further light on the relationship between genotype, dural pathology, and the overall clinical spectrum of MFS. The identification of protein-truncating variants and those impacting cysteine content may therefore suggest closer patient monitoring, in order to address potential complications associated with DE.

Comparative Gene-Expression Analysis of the Ligamentum Flavum of Patients with Lumbar Spinal Canal Stenosis: Comparison between the Dural and Dorsal Sides of the Thickened Ligamentum Flavum.

Thickening of the ligamentum flavum is the main factor in the development of lumbar spinal canal stenosis (LSCS). Although previous studies have reported factors related to ligamentum flavum thickening, its etiology has not been clarified. Furthermore, it is often difficult to set proper controls to investigate the pathologies of thickening due to differences in patient characteristics, such as age, sex, obesity, and comorbidities. This study aimed to elucidate the pathologies of ligamentum flavum thickening by comparing the dural and dorsal sides of the thickened ligamentum flavum in patients with LSCS. Ligamentum flavum samples were collected from 19 patients with LSCS. The samples were divided into the dural and dorsal sides. The dural side was used as a control to assess the pathologies occurring on the dorsal side. Elastic Masson staining was used to assess the elastic fibres. Gene expression levels were comprehensively assessed using quantitative reverse transcription polymerase chain reaction and DNA microarray analyses. Gene ontology analysis was used to identify biological processes associated with differentially expressed genes. The elastic fibres were significantly decreased on the dorsal side of the thickened ligamentum flavum. Genes related to fibrosis, inflammation, tissue repair, remodeling, and chondrometaplasia, such as COL1A2, COL3A1, COL5A1, TGFB1, VEGFA, TNFA, MMP2, COL10A1, and ADAMTS4, were highly expressed on the dorsal side of the thickened ligamentum flavum. The biological processes occurring on the dorsal side of the thickened ligamentum flavum were extracellular matrix organization, cell adhesion, extracellular matrix disassembly, and proteolysis.These are considered important pathologies of ligamentum flavum thickening.

A Subdural Dissection of Cerebrospinal Fluid Causing Cauda Equina Centralization After Durotomy: A Case Report.

CASE: A 61-year-old woman with recurrent left L5 radiculopathy underwent revision L4-5 decompression complicated by incidental durotomy requiring primary repair. Postoperative course was complicated by wound drainage and headache. Repeat magnetic resonance imaging demonstrated cerebrospinal fluid dissecting a plane deep to the dura mater but superficial to the arachnoid, with the collection compressing the cauda equina in an atypical horizontal and linear fashion. Nonoperative treatment was ineffective, and she required revision decompression and dural repair. CONCLUSION: Spine surgeons should recognize this finding on postoperative imaging as a potential sign of an incomplete dural repair necessitating return to the operating room.

Occipital Sinus-Sparing Linear Paramedian Dural Incision: A Technical Note and Case Series for Median Suboccipital Approach.

BACKGROUND: Durotomies, traditionally used during the midline suboccipital approach, involve sacrificing the occipital sinus (OS) with consequent shrinking of the dura, risk of venous complications, difficulty performing watertight closure, and a higher rate of postoperative cerebrospinal fluid (CSF) leaks. The present technical note describes the OS-sparing linear paramedian dural incision, which leads to a decrease in the risk of complications during the median suboccipital approach in our case series. METHODS: The OS-sparing linear incision technique involves a dural incision placed 1 cm lateral to the OS. The angle of view of the microscope is frequently changed to overcome the narrowed exposure of the linear durotomy. Copious irrigation with saline prevents drying of the dura. A running watertight closure of the dura is performed. The overall results of 5 cases are reviewed. RESULTS: The cases were 3 tumors and 2 cavernomas. The OS was preserved in all 5, and no duraplasty was needed. The average dura closure time was 16.8 minutes. No CSF leak occurred, and no wound complications were observed. A gross total resection of the lesion was achieved in all the patients. The mean follow-up was 10.2 months, and there were no late complications related to the dura closure. CONCLUSIONS: In comparison to the types of durotomies conventionally used for the midline suboccipital approach, the OS-sparing linear paramedian dural incision entails lower risks of bleeding, venous complications, CSF leaks, and infections by avoiding duraplasty. Validation of this technical note on a larger patient cohort is needed.

Urokinase Plasminogen Activator Receptor: An Important Focal Player in Chronic Subdural Hematoma?

Chronic subdural hematoma (CSDH) development involves inflammatory, angiogenetic, and fibrinolytic mechanisms, several components of which are now unraveled through intensive research. The urokinase plasminogen activator receptor (uPAR) is part of the plasminogen activator system and possesses inflammatory, angiogenetic, and fibrinolytic capabilities. As a first, this study aims to identify uPAR in the hematoma fluid, hematoma membrane, dura mater, and systemic blood from patients with CSDH and, if present, to investigate if the uPAR level at the time of surgery may be a predictor for later developing recurrent CSDH. uPAR expression in the hematoma membrane and dura mater was analyzed using immunohistochemistry and presented as the H-score of the positive immunostaining. The uPAR levels in the hematoma fluid and systemic blood were determined using a multiplex antibody bead kit (Luminex). Samples were collected at the time of the first CSDH surgery, and in the case of recurrent CSDH within 90 days, the samples were again collected at reoperation. A comparison of uPAR expression between the hematoma membrane and dura mater, as well as uPAR levels in systemic blood and hematoma fluid, was performed using the Wilcoxon rank sum test. We included 112 patients, 26 of whom had recurrent CSDH. The median hematoma uPAR level was 22,125 (14,845-33,237) and significantly higher than the median systemic blood level of 789 pg/L (465-2,088) (p < 0.001). Similarly, the uPAR level of the hematoma membrane was 14.3 (7.54-44.8) and significantly higher than the dural uPAR level of 0.81 (0.3-1.98) (p < 0.001). For the first time, we identified uPAR in the subdural fluid, hematoma membrane, dura mater, and systemic blood from patients with CSDH. The high expression of uPAR in the subdural fluid and hematoma membrane indicates that the mechanisms of CSDH are predominantly in the subdural fluid collection and surrounding hematoma membrane.

Clinical Observation of Various Types of Idiopathic Hypertrophic Cranial Pachymeningitis.

BACKGROUND: To assist doctors in making better treatment decisions and improve patient prognosis, it is important to determine which therapy modalities are suitable for various forms of idiopathic hypertrophic cranial pachymeningitis (IHCP). METHODS: All cases were received from the hospital medical record system, and some follow-up information was gathered through telephone follow-up. RESULTS: A total of 26 patients, 14 men and 12 women, with ages ranging from 20 to 73 years and a mean of 47.42 years, were included in the research. Regular types were less likely to recur than irregular and nodular types, focal types were less likely to recur than diffuse types, and corticosteroid-refractory types were more likely to recur than corticosteroid-sensitive types. CONCLUSIONS: The extent and shape of the lesion and susceptibility to corticosteroids are potential factors that could influence recurrence. Futhermore, this paper also proposes the fibroblasts as a new therapeutic target which may improve the quality of prognostic survival of patients.

Peptidomics insights: neutrophil extracellular traps (NETs) related to the chronic subdural hemorrhage.

Chronic subdural hemorrhage (CSDH) refers to a hematoma with an envelope between the dura mater and the arachnoid membrane and is more common among the elderly. It was reported that the dura mater, which is highly vascularized with capillary beds, precapillary arterioles and postcapillary venules play an important role in the protection of the central nervous system (CNS). Numerous evidences suggests that peptides play an important role in neuroprotection of CNS. However, whether dura mater derived endogenous peptides participate in the pathogenesis of CSDH remains undetermined. In the current study, the peptidomic profiles were performed in human dura of CSDH (three patients) and the relative control group (three non-CSDH samples) by LC-MS (liquid chromatography-mass spectrometry). The results suggested that a total of 569 peptides were differentially expressed in the dura matter of CSDH compared with relative controls, including 217 up-regulated peptides and 352 down-regulated peptides. Gene Ontology (GO) analysis demonstrated that the precursor proteins of those differentially expressed peptides were involved in the various biological processes. Interestingly, Kyoto Encyclopedia of Genes and Genomes (KEGG) analysis suggested that NETs participated in the pathogenies of CSDH. Further investigate showed that H3Cit was significantly elevated in the dural and hematoma membranes of patients with CSDH compared to patients without CSDH. Taken together, our results showed the differentially expressed peptides in human dura mater of CSDH and demonstrated that NETs formation in the dural and hematoma membranes might be involved in the pathogenesis of CSDH. It is worth noting that pharmacological inhibition of NETs may have potential therapeutic implications for CSDH.

A selected case series of idiopathic hypertrophic pachymeningitis in a single center: Pathological characteristics and case-oriented review.

Hypertrophic pachymeningitis (HP) is a relatively rare disease of the central nervous system characterized by local or diffuse fibrous thickening of the dura mater. At present, there is still insufficient research on the pathogenesis and treatment strategies of this disease. We reported a continuous case series of seven patients with idiopathic HP (IHP), and also details one case of immunoglobulin G4-related HP requiring surgical intervention. Early diagnosis and appropriate surgical intervention for IHP could prevent the progression of permanent neurological damage and spinal cord paraplegia.

Catastrophic Outcome Following Apparently Trivial Nondominant Transverse Sinus Injury During Resection of a Tentorial Meningioma: Case Report.

A 52-year-old man was admitted to our hospital with symptoms of raised intracranial pressure and cerebellar dysfunction caused by a medium-sized (4 cm in diameter) tentorial meningioma with an infratentorial extension. Preoperative magnetic resonance imaging showed that the tumor indented and possibly partially invaded the adjacent junction of the nondominant transverse and sigmoid sinuses. The contralateral dominant transverse sinus was fully patent. Total surgical removal of the lesion was done through the left retrosigmoid approach. During dissection of the meningioma, some bleeding from the venous sinus was noted, which was easily controlled by packing with hemostatic materials. The initial postoperative period was unremarkable, but approximately 48 h after surgery, acute clinical deterioration caused by hemorrhagic venous infarction of the left cerebellar hemisphere and brain stem developed and necessitated urgent reoperation for the evacuation of hematoma and brain decompression. Thereafter, the patient remained in a prolonged coma with a severe neurological deficit. After several years of extensive neurorehabilitation, he was able to walk with support but had a tracheostomy, required a feeding tube, and voided with a urinary catheter. Such a catastrophic outcome after an apparently trivial nondominant transverse sinus injury during resection of a tentorial meningioma raises the question whether reconstruction of the sinus wall with preservation of its patency might have prevented this complication in our patient.

The role of the dura mater in cerebral metastases.

OBJECTIVE: The objective of this review was to describe the immunological changes that take place in the dura mater in response to metastatic disease that seeds the CNS. The authors hypothesized that the dura's anatomy and resident immune cell population play a role in enabling metastasis to the brain and leptomeninges. METHODS: An extensive literature search was conducted to identify evidence that supports the dura's participation in metastasis to the CNS. The authors' hypothesis was informed by a recent upsurge in studies that have investigated the dura's role in metastatic development, CNS infections, and autoimmunity. They reviewed this literature as well as the use of immunotherapy in treating brain metastases and how these therapies change the meningeal immune landscape to overcome and reverse tumor-promoting immunosuppression. RESULTS: Evidence suggests that the unique architecture and immune cell profile of the dura, compared with other immune compartments within the CNS, facilitate entry of metastatic tumor cells into the brain. Once these tumor cells penetrate the dural barrier, they propagate an immunosuppressive tumor microenvironment. Therefore, immunotherapy may serve to overcome this immunosuppressive environment and liberate proinflammatory immune cells in an effort to combat metastatic disease. CONCLUSIONS: Within the next few years, the authors expect the addition of several more scientific studies into the literature that further underscore the dura as a chief participant and neuroanatomical barrier in neuro-oncology.