Diffuse Pachymeningeal Enhancement Research Articles

Spinal cord compression by cystic IgG4-related spinal pachymeningitis mimicking neurocysticercosis: a case report

BackgroundTo report a case of IgG4-related pachymeningitis presenting with cystic lesions mimicking neurocysticercosis.Case presentationA 40-year-old female patient with tetraparesis, dysphagia and dysphonia was evaluated with clinical examination, magnetic resonance imaging, and meningeal biopsy. Magnetic resonance imaging (MRI) revealed diffuse pachymeningeal enhancement involving the cranial, cervical, thoracic, and lumbar segments with spinal cord compression and cystic lesions. CSF immunology was initially positive for cysticercus cellulosae. After disease progression a meningeal biopsy was compatible with IgG4 related disease. The patient had partial response to rituximab and needed multiple surgical procedures for spinal cord decompression and CSF shunting.ConclusionsThis case highlights the possibility of IgG4-related disease in patients with diffuse pachymeningitis causing spinal cord compression, even with cystic lesions on MRI. Diagnosis of IgG4-related pachymeningitis is paramount due to the possibility of treatment response to immunotherapy, particularly to anti-CD20 agents.

Spontaneous intracranial hypotension: Exploring the viability of non-contrast FLAIR as a substitute for contrast-enhanced T1WI in assessing pachymeningeal thickening.

To avoid contrast administration in spontaneous intracranial hypotension (SIH), some studies suggest accepting diffuse pachymeningeal hyperintensity (DPMH) on non-contrast fluid-attenuated inversion recovery (FLAIR) as an equivalent sign to diffuse pachymeningeal enhancement (DPME) on contrast-enhanced T1WI (T1ce), despite lacking thoroughperformance metrics. This study aimed to comprehensively explore its feasibility. In this single-center retrospective study, between April 2021 and November 2023, brain MRI examinations of 43 patients clinically diagnosed with SIH were assessed using 1.5 and 3.0 Tesla MRI scanners. Two radiologists independentlyassessed the presence or absence of DPMH on FLAIR and DPME on T1ce, with T1ce serving as a gold-standard for pachymeningeal thickening. The contribution of the subdural fluid collections to DPMH was investigated with quantitative measurements. Using Cohen's kappa statistics, interobserver agreement was assessed. In 39 out of 43 patients (90.7%), pachymeningeal thickening was observed on T1ce. FLAIR sequence produced an accuracy, sensitivity, specificity, positive predictive value, and negative predictive value of 72.1%, 71.8%, 75.0%, 96.6%, and 21.4% respectively, for determining pachymeningeal thickening. FLAIR identified pachymeningeal thickening in 28 cases; however, among these, 21 cases (75%) revealed that the pachymeningeal hyperintense signal was influenced by subdural fluid collections. False-negative rate for FLAIR was 28.2% (11/39). The lack of complete correlation between FLAIR and T1ce in identifying pachymeningeal thickening highlights the need for caution in removing contrast agent administration from the MRI protocol of SIH patients, as it reveals a major criterion (i.e., pachymeningeal enhancement) of Bern score.

Factors to predict recurrence after epidural blood patch in patients with spontaneous intracranial hypotension.

This study aimed to identify predictors for the recurrence of spontaneous intracranial hypotension (SIH) after epidural blood patch (EBP). Epidural blood patch is the main treatment option for SIH; however, the characteristics of patients who experience relapse after successful EBP treatment for SIH remain understudied. In this exploratory, retrospective, case-control study, we included 19 patients with SIH recurrence after EBP and 36 age- and sex-matched patients without recurrence from a single tertiary medical institution. We analyzed clinical characteristics, neuroimaging findings, and volume changes in intracranial structures after EBP treatment. Machine learning methods were utilized to predict the recurrence of SIH after EBP treatment. There were no significant differences in clinical features between the recurrence and no-recurrence groups. Among brain magnetic resonance imaging signs, diffuse pachymeningeal enhancement and cerebral venous dilatation were more prominent in the recurrence group than no-recurrence group after EBP (14/19 [73%] vs. eight of 36 [22%] patients, p = 0.001; 11/19 [57%] vs. seven of 36 [19%] patients, p = 0.010, respectively). The midbrain-pons angle decreased in the recurrence group compared to the no-recurrence group after EBP, at a mean (standard deviation [SD]) of -12.0 [16.7] vs. +1.8[18.3]° (p = 0.048). In volumetric analysis, volume changes after EBP were smaller in the recurrence group than in the no-recurrence group in intracranial cerebrospinal fluid (mean [SD] -11.6 [15.3] vs. +4.8 [17.1] mL, p = 0.001) and ventricles (mean [SD] +1.0 [2.0] vs. +2.0 [2.5] mL, p = 0.003). Notably, the random forest classifier indicated that the model constructed with brain volumetry was more accurate in discriminating SIH recurrence (area under the curve = 0.80 vs. 0.52). Our study suggests that volumetric analysis of intracranial structures may aid in predicting recurrence after EBP treatment in patients with SIH.

Spontaneous intracranial hypotension and bilateral subdural hematoma in the spectrum of antiphospholipid syndrome and systemic lupus erythematosus: a challenging clinical encounter

Spontaneous intracranial hypotension (SIH) linked to the leakage of cerebrospinal fluid typically manifests as positional headaches. The connection between connective tissue disorders and SIH is widely recognized. Nevertheless, instances of SIH related to systemic lupus erythematosus are infrequently reported. We report a 42-year-old female with systemic lupus erythematosus and antiphospholipid syndrome who presented with severe orthostatic headaches. Imaging revealed bilateral subdural hematomas and diffuse pachymeningeal enhancement. Conservative treatment was administered, and the patient showed improvement with resolution of symptoms. It is important to note that not all imaging studies consistently reveal all the characteristic features of SIH. Due to these diagnostic limitations, it is crucial for clinicians to consider a combination of patient history, clinical evaluation and imaging findings meticulously.

Gauging the pressure: The clinical dilemma with Chiari! (P11-4.004)

<h3>Objective:</h3> N/A <h3>Background:</h3> Differentiating between Chiari Malformation I (CMI) and acquired tonsillar herniation due to spontaneous or iatrogenic intracranial hypotension (IH) can be challenging. We describe a case of IH misdiagnosed as CM1 to emphasize several distinct clinical signs that can prevent misdiagnosis. <h3>Design/Methods:</h3> <h3>Case Description:</h3> A 36-year-old multigravida female with history of multiple epidural analgesia during vaginal deliveries presented with 6 months of constant, poorly localized headaches. Fundoscopic exam revealed bilateral mild inferior disc edema without vessel obscuration. Contrasted magnetic resonance imaging (MRI) showed diffuse pachymeningeal enhancement with a 7mm downward herniation of the cerebellar tonsils but no syrinx in the upper cervical cord. The patient was thought to have CM1, and she was treated with supportive care. After 2 years, the patient again presented with severe intractable headaches and vomiting 7 weeks following epidural for vaginal delivery. Repeat MRI brain showed a 23 mm downward herniation of the cerebellar tonsils associated with severe effacement of the basilar and prepontine cisterns and diffuse sulcal effacement. MRI cervical spine showed a large cervical syrinx at this time. MRI thoracic and lumbar spine showed an epidural cerebrospinal fluid (CSF) collection extending from T1 to L3 levels with diffuse cord edema, confirming CSF leak. Given high risk of complications, blood patch was not attempted, and the patient ultimately underwent suboccipital decompressive craniotomy. <h3>Results:</h3> N/A <h3>Conclusions:</h3> This case highlights the importance of considering acquired causes of cerebellar tonsillar herniation in symptomatic adults. Headache description, chronicity, and presence/absence of syrinx cannot differentiate CMI from IH. Pachymeningeal enhancement, subdural hygromas, pituitary enlargement, distention of cerebral venous sinuses, and effacement of subarachnoid spaces along with flattening of pons are more indicative of IH. Evidence of an extradural CSF collection in the spinal cord or a CSF leak on computerized tomography myelogram are diagnostic of IH. <b>Disclosure:</b> Miss Phillips has nothing to disclose. Dr. Mannyam has nothing to disclose. Dr. Sharma has nothing to disclose.

Spontaneous intracranial hypotension syndrome presented with sixth cranial nerve palsy: a case report

Introduction Spontaneous intracranial hypotension (SIH) is a secondary cause of headache often misdiagnosed and underdiagnosed disorder. The main presentation is orthostatic headache. An uncommon symptom is cranial nerve palsy. We described a case of SIH that presented to our service with sixth cranial nerve palsy. Case Report Female, 39 years old, no relevant medical past or history of trauma, awoke due intense holocranial headache, associated with vomiting and photophobia, worsening in orthostasis. In the general emergency department, she was release with prescription of analgesics and topiramate, without resolution. After a week, the patient reported diplopia and was referred to our service, a tertiary center. In admission, she kept an intense orthostatic headache and in neurologic examination she presented right sixth nerve palsy, no other deficits. Laboratorial tests were normal, including metabolic and infectious marks. Cerebrospinal fluid (CSF) opening pressure was low (50 mmH2O) and analysis showed elevated protein levels (158mg/dl). MRI showed diffuse pachymeningeal enhancement and venous sinus distension, mainly in superior sagittal venous sinus. CT myelogram detected a spinal CSF leak in transition C1-C2. Initially, performed hydration and bed rest, while patient with partial improvement of headache. An epidural blood patch was then performed using 15ml of autologous blood with compatible contrast and lidocaine. There was complete resolution of pain after the procedure and four weeks later complete resolution of right sixth nerve palsy. Discussion According to International Classification of Headaches Disorders, third edition (ICHD-3), the headache attributed to SIH is descripted as a headache has developed in temporal relation to low CSF pressure (&lt;60mm H2O) and/or evidence of CSF leakage on imaging. (To see the complete abstract, please, check out the PDF).

DIFFUSE LEPTOMENINGEAL GLIONEURONAL TUMOR: A RARE CAUSE OF MALIGNANT PLEURAL EFFUSION

DIFFUSE LEPTOMENINGEAL GLIONEURONAL TUMOR: A RARE CAUSE OF MALIGNANT PLEURAL EFFUSION

Neurology of Rheumatic Diseases

This review examines the neurologic complications of several rheumatic diseases, including rheumatoid arthritis, seronegative spondyloarthritis, systemic lupus erythematosus, scleroderma and related diseases, vasculitis, spinal osteoarthritis and spondylosis, and primary Sjögren syndrome. Tables describe the categories of neurologic complications of lupus, the prevalence of neurologic manifestations among patients with lupus, distribution of nerve involvement among patients with mononeuritis multiplex caused by microscopic polyangiitis, sensitivity of combined superficial peroneal nerve and peroneus brevis muscle in patients with suspected vasculitis, sites of central nervous system involvement among patients with parenchymal neuro-Behçet syndrome, and localization of cervical radiculopathy. Figures include a radiograph of a patient with rheumatoid arthritis, magnetic resonance imaging showing diffuse pachymeningeal enhancement and the cervical patient with lupus, and photographs of a muscle biopsy of a patient with vasculitis nephropathy and anterior ischemic optic neuropathy secondary to temporal arteritis. This review contains 5 figures, 6 tables, and 77 references Keywords: Rheumatoid arthritis, spondyloarthritis, systemic lupus erythematosus, scleroderma, vasculitis, spinal osteoarthritis, spondylosis, Sjögren syndrome, neurologic deficit, radiculopathy, Sciatica

Magnetic resonance imaging findings of intracranial hypotension.

This study aims to evaluate the differences in the sizes and configurations of various structures on brain MRIs of patients with intracranial hypotension (ICH) compared to normal individuals. The present study consisted of two study groups as 21 patients with intracranial hypotension and 21 healthy individuals. Cranial MRI findings of patients with intracranial hypotension were compared retrospectively with MRI findings of patients without any pathology. Pachymeningeal enhancement, mamillopontine distance, venous sinus diameters, transverse and straight sinus distension, pituitary gland enlargement, tonsillar herniation, bleeding (subdural, epidural), pontomesencephalic angle, lateral ventricular angle, and pituitary infundibular angle were evaluated on MRI. Intracranial hypotension developed spontaneously in 6 cases and secondary in 15 patients. Diffuse pachymeningeal enhancement was observed in all intracranial hypotension cases. In addition, transverse sinus distension was observed in 19 cases, straight sinus distension in 17 cases, subdural effusion in 7 cases, spinal epidural effusion in 3 cases, tonsillar herniation in 2 cases, and thrombosis in dural sinuses in 2 cases. The intracranial hypotension group vs control group had dominant transverse sinus diameter 10 ± 1.75 vs 7.52 ± 1.2mm, straight sinus diameter 4.76 ± 0.92 vs 3.69 ± 0.57mm, superior sagittal sinus diameter 8.35 ± 1.57 vs 6.37 ± 0.71mm, pontomesencephalic angle 46.67 ± 9.73° vs 56.27° ± 8.9°, mamillopontine distance 5.83 ± 1.5 vs 6.85 ± 1.1mm, lateral ventricular angle 131.13° ± 6.17° vs 135.19° ± 5.28°, pituitary infundibular angle 44.42° ± 12.09° vs 63.3° ± 11.56°, and pituitary gland height 8.5 ± 1.83 vs 5.5 ± 1.27mm, respectively. In cases with clinically suspected intracranial hypotension, MRI findings may contribute to the diagnosis of intracranial hypotension with quantitative evaluations.

The time sequence of brain MRI findings in spontaneous intracranial hypotension.

To investigate the time sequence of brain magnetic resonance imaging findings of spontaneous intracranial hypotension. We retrospectively reviewed the medical records and brain magnetic resonance imaging findings of consecutive patients with spontaneous intracranial hypotension hospitalized between January 2007 and December 2017. Patients were divided into quartiles based on intervals between initial spontaneous intracranial hypotension symptom onset and brain magnetic resonance imaging scan. Six categorical and five continuous brain magnetic resonance imaging findings were assessed, including venous distension sign, enlarged pituitary gland, diffuse pachymeningeal enhancement, mid-brain pons deformity, subdural fluid collection, flattening of pons, midbrain-pons angle, descent of cerebral aqueduct, mamillopontine distance, distance of suprasellar cistern, and distance of prepontine cistern. In addition, we also calculated the neuroimaging scores with a score ≥5 classified as 'high probability of spontaneous intracranial hypotension' and a score ≥3 as 'intermediate-to-high probability.' Then, we analyzed the linkage between the onset-neuroimaging interval and brain magnetic resonance imaging findings, as well as different neuroimaging scores. A total of 173 patients (57 males and 116 females) were included in the analysis, and the range of onset-neuroimaging interval was 1 to 89 days (median [interquartile range] = 17 [7 to 30 days]). We divided the patients into quartiles based on their onset-neuroimaging interval (the first quartile: 0-6 days; the second quartile: 7-16 days; the third quartile: 17-29 days; the fourth quartile: ≥30 days). Among brain magnetic resonance imaging findings, the incidence of venous distension sign was high (>75%), with no difference among quartiles (p = 0.876). The incidence of diffuse pachymeningeal enhancement (p = 0.001), severe midbrain-pons deformity (p = 0.001), and subdural fluid collection (<0.001) followed a significant stepwise increase from the first quartile to fourth quartile. Patients with shorter onset-neuroimaging intervals were less likely to have neuroimaging scores ≥5 (<17 vs. ≥17 days: 72.9% vs. 86.4%; odds ratio = 2.3 [95% CI 1.1-5.1], p = 0.028), but not neuroimaging scores ≥3 (<17 vs. ≥17 days: 92.9% vs. 92.0%, p = 0.824). The emergence of brain magnetic resonance imaging findings of spontaneous intracranial hypotension depended on disease duration and appeared sequentially. When using brain magnetic resonance imaging findings or neuroimaging scores for diagnostic purposes, the onset-neuroimaging interval should be considered.

Comparisons of clinical characteristics, brain MRI findings, and responses to epidural blood patch between spontaneous intracranial hypotension and post-dural puncture headache: retrospective study

BackgroundSpontaneous intracranial hypotension and post-dural puncture headache are both caused by a loss of cerebrospinal fluid but present with different pathogeneses. We compared these two conditions concerning their clinical characteristics, brain imaging findings, and responses to epidural blood patch treatment.MethodsWe retrospectively reviewed the records of patients with intracranial hypotension admitted to the Neurology ward of the Pusan National University Hospital between January 1, 2011, and December 31, 2019, and collected information regarding age, sex, disease duration, hospital course, headache intensity, time to the appearance of a headache after sitting, associated phenomena (nausea, vomiting, auditory symptoms, dizziness), number of epidural blood patch treatments, and prognosis. The brain MRI signs of intracranial hypotension were recorded, including three qualitative signs (diffuse pachymeningeal enhancement, venous distention of the lateral sinus, subdural fluid collection), and six quantitative signs (pituitary height, suprasellar cistern, prepontine cistern, mamillopontine distance, the midbrain-pons angle, and the angle between the vein of Galen and the straight sinus).ResultsA total of 105 patients (61 spontaneous intracranial hypotension patients and 44 post-dural puncture headache patients) who met the inclusion criteria were reviewed. More patients with spontaneous intracranial hypotension required epidural blood patch treatment than those with post-dural puncture headache (70.5% (43/61) vs. 45.5% (20/44); p = 0.01) and the spontaneous intracranial hypotension group included a higher proportion of patients who underwent epidural blood patch treatment more than once (37.7% (23/61) vs. 13.6% (6/44); p = 0.007). Brain MRI showed signs of intracranial hypotension in both groups, although the angle between the vein of Galen and the straight sinus was greater in the post-dural puncture headache group (median [95% Confidence Interval]: 85° [68°-79°] vs. 74° [76°-96°], p = 0.02).ConclusionsPatients with spontaneous intracranial hypotension received more epidural blood patch treatments and more often needed multiple epidural blood patch treatments. Although both groups showed similar brain MRI findings, the angle between the vein of Galen and the straight sinus differed significantly between the groups.

Clinical Presentation, Investigation Findings, and Treatment Outcomes of Spontaneous Intracranial Hypotension Syndrome

Spontaneous intracranial hypotension (SIH) is a highly disabling but often misdiagnosed disorder. The best management options for patients with SIH are still uncertain. To provide an objective summary of the available evidence on the clinical presentation, investigations findings, and treatment outcomes for SIH. Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) reporting guideline-compliant systematic review and meta-analysis of the literature on SIH. Three databases were searched from inception to April 30, 2020: PubMed/MEDLINE, Embase, and Cochrane. The following search terms were used in each database: spontaneous intracranial hypotension, low CSF syndrome, low CSF pressure syndrome, low CSF volume syndrome, intracranial hypotension, low CSF pressure, low CSF volume, CSF hypovolemia, CSF hypovolaemia, spontaneous spinal CSF leak, spinal CSF leak, and CSF leak syndrome. Original studies in English language reporting 10 or more patients with SIH were selected by consensus. Data on clinical presentation, investigations findings, and treatment outcomes were collected and summarized by multiple observers. Random-effect meta-analyses were used to calculate pooled estimates of means and proportions. The predetermined main outcomes were the pooled estimate proportions of symptoms of SIH, imaging findings (brain and spinal imaging), and treatment outcomes (conservative, epidural blood patches, and surgical). Of 6878 articles, 144 met the selection criteria and reported on average 53 patients with SIH each (range, 10-568 patients). The most common symptoms were orthostatic headache (92% [95% CI, 87%-96%]), nausea (54% [95% CI, 46%-62%]), and neck pain/stiffness (43% [95% CI, 32%-53%]). Brain magnetic resonance imaging was the most sensitive investigation, with diffuse pachymeningeal enhancement identified in 73% (95% CI, 67%-80%) of patients. Brain magnetic resonance imaging findings were normal in 19% (95% CI, 13%-24%) of patients. Spinal neuroimaging identified extradural cerebrospinal fluid in 48% to 76% of patients. Digital subtraction myelography and magnetic resonance myelography with intrathecal gadolinium had high sensitivity in identifying the exact leak site. Lumbar puncture opening pressures were low, normal (60-200 mm H2O), and high in 67% (95% CI, 54%-80%), 32% (95% CI, 20%-44%), and 3% (95% CI, 1%-6%), respectively. Conservative treatment was effective in 28% (95% CI, 18%-37%) of patients and a single epidural blood patch was successful in 64% (95% CI, 56%-72%). Large epidural blood patches (>20 mL) had better success rates than small epidural blood patches (77% [95% CI, 63%-91%] and 66% [95% CI, 55%-77%], respectively). Spontaneous intracranial hypotension should not be excluded on the basis of a nonorthostatic headache, normal neuroimaging findings, or normal lumbar puncture opening pressure. Despite the heterogeneous nature of the studies available in the literature and the lack of controlled interventional studies, this systematic review offers a comprehensive and objective summary of the evidence on SIH that could be useful in guiding clinical practice and future research.

Myeloid Sarcoma Involving the Central Nervous System Without Evidence of Preexisting Hematologic Disease: A Case Report

Abstract Introduction/Objective Myeloid sarcoma within the central nervous system is a rare presentation of an infiltrative mass of immature myeloid cells. It is typically found in association with myeloid dysplastic syndrome, myeloproliferative disorder, or acute myeloid leukemia. Rarely, it can occur in the absence of these diseases. Methods We present the case of 68-year-old female with worsening nausea and headaches. Brain MRI showed diffuse pachymeningeal enhancement with numerous nodular enhancing extra-axial masses overlying both cerebral hemispheres. Results Open biopsy of these masses showed gelatinous, hemorrhagic, brown tissue measuring 3.0 x 2.0 x 0.7 cm. Histologically, the specimen shows a diffuse polymorphous atypical myeloid proliferation consisting of all three cell lineages. There are nodules composed predominantly of large blastoid cells with 1 to 2 prominent oblong nucleoli, consistent with erythroblasts and early erythroid precursors. In between the nodules there are myeloid cells in different stages of maturation, including numerous eosinophilic precursors. Scattered megakaryocytes are also identified. Immunohistochemistry shows positivity for E-cadherin, CD235a and spectrin which highlight the immature erythroid cells. MPO, muramidase, and CD33 are positive in myeloid elements. Eosinophilic peroxidase highlights numerous eosinophilic precursors. CD34 is positive in the endothelial cells and very rare hematopoietic cells. CD20 and PAX 5 highlights B-cells, CD3 highlights T-cells, and CD4 highlights monocytic precursors, monocytes, histiocytes and a subset of T-cells. Conclusion Additional work up at the time of biopsy including FLOW cytometry and bone marrow biopsies showed no evidence of hematologic pathology. In summary the histologic and immunohistochemical findings demonstrate a tumor composed of proliferating immature myeloid cells most consistent with myeloid sarcoma without any evidence of hematological disease.

Spontaneous Intracranial Hypotension: Atypical Radiologic Appearances, Imaging Mimickers, and Clinical Look-Alikes.

Spontaneous intracranial hypotension is a condition characterized by low CSF volume secondary to leakage through a dural defect with no identifiable cause. Patients classically present with orthostatic headaches, but this symptom is not specific to spontaneous intracranial hypotension, and initial misdiagnosis is common. The most prominent features of spontaneous intracranial hypotension on intracranial MR imaging include "brain sag" and diffuse pachymeningeal enhancement, but these characteristics can be seen in several other conditions. Understanding the clinical and imaging features of spontaneous intracranial hypotension and its mimickers will lead to more prompt and accurate diagnoses. Here we discuss conditions that mimic the radiologic and clinical presentation of spontaneous intracranial hypotension as well as other disorders that CSF leaks can imitate.

Head Injury - Soccer

HISTORY: A 16 year-old male presented to an outpatient sports medicine clinic one month after a head-to-head collision during a soccer match with concern for concussion. There was no loss of consciousness, and he continued to play the rest of the game with a mild headache. He felt normal and asymptomatic that evening and was able to complete homework. The following morning he felt tired, but was able to perform adequately at school. Over the coming weeks, he noticed progressive worsening of his cognitive symptoms and tiredness. His athletic trainer became concerned for a concussion and held him from practice. One week prior to presentation he noted midline lower lip numbness, teeth pain while chewing, hearing his pulse in his left ear, and poor sleep due to sweatiness. He also felt progressive lethargy and difficulty concentrating and missed the last three days of school due to these symptoms. His medical history was significant only for a recently diagnosed inguinal hernia. PHYSICAL EXAMINATION: Mild cognitive deficits in attention and memory, impaired balance on BESS, normal motor strength. Cranial nerve exam showed ptosis of the left eye, mild left facial nerve palsy, decreased hearing in the left ear, and altered sensation to light touch over the middle lower lip. There was no focal tenderness or deformity of the skull or scalp. DIFFERENTIAL DIAGNOSIS: Mild traumatic brain injury Intracranial hemorrhage Cerebral mass Bell’s palsy TESTS AND RESULTS: MRI Brain with and without contrast: Asymmetric enhancement of the left 7th cranial nerve, asymmetric nodular enhancement along the left 5th nerve with enhancement of muscles of mastication, diffuse bilateral pachymeningeal enhancement. Complete Blood Count: WBC 19k, platelets 80, hematocrit 30.4 CT Chest, Abdomen, Pelvis: Large abdominal soft-tissue mass herniating through the inguinal canal, most consistent with a lymphoma CSF Cytology: Enlarged B-cells most consistent with Burkitt lymphoma. FINAL DIAGNOSIS: Stage IV Burkitt lymphoma, with primary lesion in abdomen, and perimeningeal spread, causing cranial nerve V and VII palsies. TREATMENT AND OUTCOMES: 1. Admitted for prompt initiation of chemotherapy. 2. Cranial nerve symptoms resolved with chemotherapy and steroids. 3. After multiple rounds of chemotherapy, there is no evidence of residual lymphoma.

Head Injury - Soccer

HISTORY: A 16 year-old male presented to an outpatient sports medicine clinic one month after a head-to-head collision during a soccer match with concern for concussion. There was no loss of consciousness, and he continued to play the rest of the game with a mild headache. He felt normal and asymptomatic that evening and was able to complete homework. The following morning he felt tired, but was able to perform adequately at school. Over the coming weeks, he noticed progressive worsening of his cognitive symptoms and tiredness. His athletic trainer became concerned for a concussion and held him from practice. One week prior to presentation he noted midline lower lip numbness, teeth pain while chewing, hearing his pulse in his left ear, and poor sleep due to sweatiness. He also felt progressive lethargy and difficulty concentrating and missed the last three days of school due to these symptoms. His medical history was significant only for a recently diagnosed inguinal hernia. PHYSICAL EXAMINATION: Mild cognitive deficits in attention and memory, impaired balance on BESS, normal motor strength. Cranial nerve exam showed ptosis of the left eye, mild left facial nerve palsy, decreased hearing in the left ear, and altered sensation to light touch over the middle lower lip. There was no focal tenderness or deformity of the skull or scalp. DIFFERENTIAL DIAGNOSIS: Mild traumatic brain injury Intracranial hemorrhage Cerebral mass Bell’s palsy TESTS AND RESULTS: MRI Brain with and without contrast: Asymmetric enhancement of the left 7th cranial nerve, asymmetric nodular enhancement along the left 5th nerve with enhancement of muscles of mastication, diffuse bilateral pachymeningeal enhancement. Complete Blood Count: WBC 19k, platelets 80, hematocrit 30.4 CT Chest, Abdomen, Pelvis: Large abdominal soft-tissue mass herniating through the inguinal canal, most consistent with a lymphoma CSF Cytology: Enlarged B-cells most consistent with Burkitt lymphoma. FINAL DIAGNOSIS: Stage IV Burkitt lymphoma, with primary lesion in abdomen, and perimeningeal spread, causing cranial nerve V and VII palsies. TREATMENT AND OUTCOMES: 1. Admitted for prompt initiation of chemotherapy. 2. Cranial nerve symptoms resolved with chemotherapy and steroids. 3. After multiple rounds of chemotherapy, there is no evidence of residual lymphoma.

Management of spontaneous intracranial hypotension: a series of 31 cases over 15-years with a challenging outlier

Spontaneous intracranial hypotension (SIH) has been classified as a triad of postural headache, low CSF opening pressure (below 60mmH20) and diffuse pachymeningeal gadolinium enhancement on MRI. SIH is due to a non-iatrogenic defect in the dura somewhere along the neuraxis (usually in the spine). The resultant leak depressurizes the system and undermines the buoyancy-providing quality of CSF. In many cases the site of leak is not identified. Epidural blood patch (EBP) is a well-established treatment of SIH but is not always effective. We retrospectively analysed thirty-one cases of SIH who required at least one EBP. They were managed over a fifteen-year period at a single institution. EBP resulted in a significant improvement in headache for 77% of patients. We report in more detail on a particularly challenging outlier where severe SIH resulted in coma. Serial epidural blood patches, burr hole evacuation of chronic subdural haematomata, and infusion of 0.9% NaCl via an intradural spinal catheter were all required to enable long-term recovery.

A clinical analysis on 40 cases of spontaneous intracranial hypotension syndrome

Objective: To investigate clinical and imaging features of 40 patients with spontaneous intracranial hypotension (SIH).Methods: 40 cases of spontaneous intracranial hypotension (SIH) diagnosed in our hospital from June 2013 to September 2017 were collected and retrospectively analyzed.Results: In our study, the male to female ratio was 2:3. The average age of onset was 43.0 ± 15.0 years. There were 12 (30.0%) patients with clear incentives, mostly catching cold. The average length of hospital stay was 11.2 ± 6.3 days. All the patients showed orthostatic headaches, 62.5% patients with nausea or vomiting, 40.0% patients with neck stiffness, 17.5% patients with dizziness and vertigo, 10.0% patients with numbness and weakness of limbs, 5% patients with neck discomfort, and 2.5% patients with visual symptoms (visual impairment, photophobia, diplopia). 24 patients underwent CT scans which showed no abnormalities in 20 cases (83.3%), subdural fluid accumulation in 3 cases (12.5%), and subdural haematoma in 1 case (2.5%). Cranial contrast-enhanced MR scans showed diffuse pachymeningeal enhancement (95.83%, 23/24), signs of pituitary hyperaemia in 5 cases (20.8%), subdural fluid accumulation and subdural hematoma in 4 cases (16.7%), sagging of the brain in 3 cases (12.5%), and engorgement of venous structures in 1 case (4.1%). Six patients underwent plain and contrast-enhanced spinal MR scans which showed varying degrees of dural thickening and enhanced performance in all the patients. 92.5% (37/40) of patients had cerebrospinal fluid pressure <60 mmH2O on lumbar puncture. 97.5% of patients underwent conservative treatment with drugs and had a good outcome.Conclusion: Orthostatic headache and cranial MRI diffuse pachymeningeal enhancement are characteristic features of SIH. Cranial contrast-enhanced MR scan is recognized as the first and non-invasive investigation in the diagnosis of SIH. Most patients had cerebrospinal fluid pressure <60 mmH2O. The vast majority of patients improved with fluid replacement.

Cerebrospinal fluid leak causing spontaneous intracranial hypotension

Spontaneous intracranial hypotension (SIH) is one of the important causes of headache in young and middle-aged and is commonly misdiagnosed in the initial stages. SIH generally results from a spontaneous cerebrospinal fluid (CSF) leak usually in the spinal region subsequently leading to headache, usually postural in nature. SIH is characterized by a triad of postural headache, diffuse pachymeningeal enhancement diagnosed on magnetic resonance imaging, and low CSF opening pressure of

Intracranial hypotension mimicking chronic progressive external ophthalmoplegia

ABSTRACTIntracranial hypotension (ICH) is characterized by low cerebrospinal fluid pressure, postural headaches, and diffuse pachymeningeal enhancement on magnetic resonance imaging (MRI). A variety of ophthalmoparetic manifestations have been reported in the context of the ICH. The authors describe an unusual case of a 64-year-old woman who presented with rapid onset of headaches, bilateral upper-lid ptosis, and blurring of vision within 4 days after sustaining a trivial head injury. She was noted to have bilateral symmetrical ophthalmoplegia and ptosis-simulating chronic progressive external ophthalmoplegia. MRI revealed characteristic features of ICH. Subsequent autologous epidural patch therapy led to resolution of the headache and imaging findings; however, her ptosis and motility disorder persisted. Despite existing therapeutic measures for ICH, irreversible cranial nerve damage may ensue due to significant cerebral decent or ischemic injury.