Dural Thickening Research Articles

Variant sella morphology and pituitary gland height in adult patients with Chiari II malformation: potential pitfall in MRI evaluation

PurposeTo systematically evaluate the sella morphology and pituitary gland height on brain MRI of Chiari II malformation (C2M) patients to understand the observed high incidence of apparent enlargement of the pituitary gland. MethodsBrain MRIs of C2M patients at a single tertiary care adult institution were retrospectively reviewed. We also evaluated two age and gender-matched control groups-patients with normal brain MRI (C1 group) and chronic ventricular shunts (C2 group). The heights of tuberculum sella, dorsum sella, and pituitary gland were measured and compared. The presence or absence of dural thickening was noted. Results21 patients were included in each group. In C2M group, a pituitary adenoma was suggested on 24% of the MRIs. The dorsum sella was significantly smaller in the C2M group (4.8 mm) compared to both the C1 group (7.4 mm, p < 0.001) and the C2 group (7.1 mm, p < 0.001). The pituitary gland was also larger in the cranial-caudal dimension in C2M group (8.6 mm) as compared to both the C1 group (6.6 mm, p < 0.01) and the C2 group (6.0 mm, p < 0.001). One C2M patient with a pituitary gland<10 mm was suggested to have a pituitary adenoma on outside MRI, although a normal pituitary gland was seen on pathology. ConclusionC2M patients have shallow sella which can accentuate the pituitary gland height. The mean pituitary height was larger in C2M patients, but no functional pituitary pathology was present. It is important to consider sella morphology when evaluating the pituitary gland of C2M patients to avoid unnecessary medical and surgical interventions.

RARE-52. AN EXTRA-VENTRICULAR NEUROCYTOMA PRESENTING WITH DIFFUSE LEPTOMENINGEAL DISEASE WITH OPTIC NEURITIS, A CASE REPORT

Abstract INTRODUCTION Neurocytoma is classically a rare intraventricular tumor that affects young adults. Due to its location, it usually presents with obstructive hydrocephalus and typically has a favorable prognosis after adequate surgical intervention. CASE: In this report, we describe the case of a 19-year-old man who developed progressive decreased visual acuity and headaches that led to a diagnosed of hydrocephalus and a ventriculoperitoneal shunt (VPS) placement. Work up at presentation revealed a thoracic spinal cord tumor which was completed resected and pathology was consistent with extra-ventricular neurocytoma. With no further intervention, he experienced signs of worsening intracranial pressure (ICP) and visual acuity, becoming steroid-dependent. Repeat imaging 3 months later showed early signs of leptomeningeal disease (LMD), optic nerve enhancement, and diffuse dural thickening. A dural biopsy was done and read as nonspecific inflammation. He then came to our center for a second opinion. We reviewed pathology from original tumor and confirmed extra-ventricular neurocytoma. We, however, read the dural biopsy as an organizing subdural hematoma. We analyzed his cerebrospinal fluid (CSF) and cytology showed neurocytoma cells, linking his LMD to initial diagnosis. He went on to receive photon craniospinal radiation (CSI) with clinical improvement and decreased steroid requirements. Most recent imaging showed improving LMD. DISCUSSION Extra-ventricular neurocytoma is an extremely rare disease with very few cases reported in the literature and our case is unique due to the severe leptomeningeal involvement affecting the optic nerves and the presence of neurocytoma cells on the CSF. There is no consensus on how to best treat these cases, however, our patient improved after CSI.

Spontaneous Intracranial Hypotension Complicated by Cerebral Venous Thrombosis Relieved by Epidural Blood Patch

Spontaneous intracranial hypotension (SIH) is characterized by orthostatic headache, diffuse dural thickening, and enhancement in magnetic resonance imaging. Cerebral venous thrombosis (CVT) has been reported to be a rare complication of SIH. There is no consensus in anticoagulation treatment of CVT secondarily caused by SIH. We report a female patient with SIH complicated by CVT and spontaneously regressed CVT not by anticoagulation but by epidural blood patch. Key Words: Spontaneous intracranial hypotension, Venous thrombosis, Epidural blood patch

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.

Imaging Appearances and Pathologic Characteristics of Spinal Epidural Meningioma.

Spinal epidural meningioma is an uncommon tumor. This study aimed to analyze the imaging and pathologic characteristics of this rare tumor. Fourteen confirmed cases of epidural meningioma were retrospectively reviewed, and imaging characteristics and pathologic findings were analyzed to identify the typical features. The mean age of the patients (4 men, 10 women) was 44.9 years. Twelve tumors were in the cervical spinal canal, and 2, in the thoracic spinal canal. There were 9 en plaque meningiomas, 4 dumbbell-shaped meningiomas, and 1 fusiform/ovoid meningioma. The epidural meningiomas extended over 2-5 spinal segments (mean, 3.2 spinal segments). A soft epidural mass was seen in 12/14 (86%) patients. Dural calcification was seen in 8/14 (57%) tumors. Tumor caused intervertebral foramen enlargement in 10/14 (71%) patients and adhered to the nerve roots in 11/14 (79%) patients. Intradural invasion was seen in 8/14 (57%) patients. The dural tail sign was present in 13/14 (93%) tumors on contrast-enhanced T1WI. Regarding pathologic type, 10 of 14 (71%) were psammomatous, 2 of 14 (14%) were meningothelial, 1 of 14 (7%) was angiomatous, and 1 of 14 (7%) was transitional. During follow-up (mean follow-up, 73.4 months; range, 4-192 months), 7 patients had recurrence. Recurrences were between 4 and 192 months after the operation. Epidural meningioma has 3 different growth patterns. Dural thickening, calcification, invasion, and epidural mass formation are characteristic features of epidural meningioma. Regular follow-up imaging is required to detect recurrence.

Pachymeningitis in granulomatosis with polyangiitis: case series with earlier onset in younger patients and literature review.

The objective of this study is to describe the characteristics of patients with pachymeningitis (PM) in granulomatosis with polyangiitis (GPA) from Latin America, including three young patients. This is a retrospective case series. Patients were classified according to the ACR criteria, the 2012 Chapel Hill Consensus Conference Nomenclature and the EMA algorithm. Demographic, clinical, serological, and neuroimaging characteristics are described. Thirteen patients (nine females, four males) were identified. Mean age±SD of PM diagnosis was 35.5±20.4years (median 48, range 8-71years). Mean time±SD between GPA first symptom and PM diagnosis was 59.8±70.1months (median 48, range 2-252months). An important difference between children and adults was the median time elapsed between first GPA symptoms and PM diagnosis (range 2-4months vs 5-252months, respectively). Chronic headache was present in all, followed by intracranial hypertension (n=5), single cranial nerve palsy and orbital mass (n=4), seizures (n=3), cavernous sinus syndrome and multiple cranial nerve palsies (n=2), and meningism and cerebellar syndrome (n=1 each). At time of PM diagnosis, mean BVAS/WG (Birmingham Vasculitis Activity Score for Wegener's granulomatosis) was 4±2.4 and mean VDI of 2±1.6, mostly due to ENT damage. Gadolinium-enhanced brain MRI showed dural thickening in 12 patients and leptomeningeal enhancement in one. All received a combination of glucocorticoids plus immunosuppressants, rituximab being used favourably in one refractory case. Improvement was observed in 12 patients. Chronic headache should lead to suspect PM. PM predominates in localised GPA. Children may present it earlier in the disease course than adults. Treatment is non-standardised and remains difficult.

P.108 Diffuse large B-cell Lymphoma secondary to iatrogenic immunosuppression with unusual MRI findings and literature review

Background: Immunosuppressive therapy is a risk factor for lymphoproliferative disorders. We present a case of primary CNS B-cell lymphoma in the setting of iatrogenic immunosuppression from azathioprine usage. A literature review is provided. Methods: Case report Results: 64-year-old male presents with several weeks of cognitive decline, impaired speech, and headache with a history of ulcerative colitis (on azathioprine and 5-ASA) with no radiological evidence of systemic malignancy. MR showed left frontal extra-axial mass (4.0 x 2.4 x 4.0 cm) with heterogeneous enhancement of a solid component with local dural thickening. The enhancing mass had solid and cystic components. Radiological differential included dural metastasis, atypical meningioma or unusual intra-axial mass including GBM with some dural involvement. He underwent surgical resection, which showed a primary CNS lymphoma, diffuse large B-cell, CD 20 + and EBV +. Post-operatively his cognition improved. Azathioprine was stopped and 5-ASA was increased. He proceeded with MPVC (methotrexate, procarbazine, vincristine, and cytarabine) chemotherapy. Conclusions: Our case shows isolated extra-nodal CNS manifestation of lymphoma in the context of immunosuppressive medications with strikingly atypical MR findings leading to a pre-operative diagnostic dilemma. Treatment is challenging and needs to be individually tailored due to a need for stopping immunosuppressive agents in conjunction with CNS lymphoma treatment.

Dural Thickening of the Internal Auditory Canal in Patients With Spontaneous Intracranial Hypotension Syndrome.

This study aimed to assess the utility of dural thickening of the internal auditory canal (IAC) in patients with spontaneous intracranial hypotension (SIH) syndrome and determined the sensitivity and specificity of this image finding. Magnetic resonance images were evaluated for 22 cases of definite SIH and 16 cases of unlikely SIH. On contrast-enhanced magnetic resonance imaging, pachymeningeal enhancement and dural thickening of the IAC were assessed. Pachymeningeal enhancement was observed in 21 of 22 patients in the definite SIH group and 1 of 16 patients in the unlikely SIH group (sensitivity, 95.5%; specificity, 93.8%). Dural thickening of the IAC was observed in 15 of 22 patients in the definite SIH group and 0 of 16 patients in the unlikely SIH group (sensitivity, 68.2%; specificity, 100%). Dural thickening of the IAC showed 100% specificity for SIH syndrome and can increase the accuracy of diagnosis of SIH syndrome.

A quantitative study of intracranial hypotensive syndrome by magnetic resonance

ObjectivesThe study aims to investigate the magnetic resonance imaging (MRI) findings of intracranial hypotension syndrome (IHS) and the change of quantitative indicators, so as to yield a deeper understanding of the disease. Patients and methodsThe clinical data and MRI findings of 26 cases of IHS which were confirmed by lumbar puncture were retrospectively analyzed. Two physicians evaluated the MRI findings including thickening and enhancement of dural, pituitary enlargement, subdural effusion (hematocele), venous engorgement and brain sagging, and measured the quantitative indicators including mamillopontine distance and pontomesencephalic angle. The consistency between the two results of the physicians was assessed by Kappa consistency test. The differences of mamillopontine distance and pontomesencephalic angle between the patient group and the control group were determined by paired t-test. The diagnostic efficiency of mamillopontine distance and pontomesencephalic angle was assessed by area under the ROC curve, and their best diagnostic thresholds were also determined, respectively. Age- and sex-matched healthy volunteers controls (n=26) were recruited and served as the control group. ResultsAll of the 26 patients suffered from the characterized by orthostatic headache of IHS. The clinical evaluations of dural thickening and enhancement, pituitary enlargement, subdural effusion (hematocele), venous engorgement by the two physicians showed excellent agreements (κ=0.808, 1 and 0.906, P<0.01), and the clinical evaluations of brain sagging showed medium agreements (κ=0.606, P<0.01). The mamillopontine distance and pontomesencephalic angle of the patient group were 5.4±1.6mm and 47.8±8.7°, respectively, which were obviously less than those of the control group (6.9±1.1mm and 61.0±6.1°, respectively), and the differences were statistically significant (t=−4.563, P<0.01; t=−.329, P<0.01). The area under ROC curve of mamillopontine distance and pontomesencephalic angle were 0.774 and 0.908, respectively, and the diagnostic value of pontomesencephalic angle was higher than that of the mamillopontine distance. The sensitivity and specificity were 73.1% and 73.1%, respectively, when diagnostic threshold of mamillopontine distance was 6.4mm. The sensitivity and specificity were 76.9% and 96.2%, when diagnostic threshold of pontomesencephalic angle was 51.7°. ConclusionThe MRI findings presented characteristic features of IHS. The quantitative indicators including mamillopontine distance and pontomesencephalic angle were helpful for clinical diagnosis of subjective findings of IHS.

Progressive headache and lethargy in a middle-age female patient: answer

D. Metastasis Following MRI and pre-operative workup, the patient underwent an endoscopic transnasal/transsphenoidal biopsy of the sella lesion with formal histopathology demonstrating malignant tumour cells which were strongly CK7 and thyroid transcription factor-1 positive and GCDFP-15, PR, HER2, CK20, CDX-2 and WT1 negative consistent with a metastasis from a lung primary cancer. The patient underwent staging CT scanning, which demonstrated a right upper lobe lung lesion associated with right hilar adenopathy and a bulky adrenal gland. The patient has commenced stereotactic radiosurgery for the pituitary metastasis and is currently on hormone replacement. Pituitary metastasis is uncommon, comprising of 0.14 to 28.1% of all brain metastases in an autopsy series [[1]Nelson P.B. Robinson A.G. Martinez A.J. Metastatic tumor of the pituitary gland.Neurosurgery. 1987; 21: 941-944Crossref PubMed Scopus (64) Google Scholar]. The most common primary disease is breast cancer in women and lung cancer in men [[2]Rajput R. Bhansali A. Dutta P. et al.Pituitary metastasis masquerading as non-functioning pituitary adenoma in a woman with adenocarcinoma lung.Pituitary. 2006; 9: 155-157Crossref PubMed Scopus (20) Google Scholar], however, it is rare that a pituitary metastasis is the first manifestation of disease [[3]Ruelle A. Palladino M. Andrioli G.C. Pituitary metastases as presenting lesions of malignancy.J Neurosurg Sci. 1992; 36: 51-54PubMed Google Scholar]. Patients present with visual disturbance, particularly hemianopia, which can be explained by suprasellar extension of the tumour compressing the optic apparatus. Other cranial nerve deficits are less common but can be seen with tumour extension to the parasellar region and cavernous sinus. In a case series by McCormack et al. 70% of patients presented with diabetes insipidus. This can be attributed to posterior pituitary involvement [[4]McCormick P.C. Post K.D. Kandji A.D. et al.Metastatic carcinoma to the pituitary gland.Br J Neurosurg. 1989; 3: 71-79Crossref PubMed Scopus (166) Google Scholar]. In comparison, in the more common pituitary adenoma, diabetes insipidus is reported in only 1% of cases [[5]Max M.B. Deck M.D. Rottenberg D.A. Pituitary metastasis: incidence in cancer patients and clinical differentiation from pituitary adenoma.Neurology. 1981; 31: 998-1002Crossref PubMed Google Scholar]. This also suggests the importance of the inferior hypophyseal artery as the blood supply to the posterior pituitary from the systemic circulation. MRI is a powerful diagnostic tool to determine size and location of pituitary tumours, however, there are no single or collection of hallmark radiological features that can accurately determine tumour types that can be found in the sellar as metastases [[6]Koshimoto Y. Maeda M. Nakiki H. et al.MR of pituitary metastasis in a patient with diabetes insipidus.AJNR Am J Neuroradiol. 1995; 16: 971-974PubMed Google Scholar]. The features that are atypical in this case include the relatively small/normal sized sella and the involvement of the pituitary stalk. Other features that may be helpful to distinguish a macroadenoma from a metastasis can also include irregular edges, dural thickening, bony destruction and invasion rather than remodeling of the sella [7Rupp D. Molitch M. Pituitary stalk lesions.Curr Opin Endocrinol Diabetes Obes. 2008; 15: 339-345Crossref PubMed Scopus (39) Google Scholar, 8Hamilton B.E. Salzman K.L. Osborn A.G. Anatomic and pathologic spectrum of pituitary infundibulum lesions.AJR Am J Roentgenol. 2007; 188: W223-32Crossref PubMed Scopus (59) Google Scholar]. A high T1 signal spot of the posterior pituitary is demonstrated in a normal patient, however, a loss of this radiological feature may indicate tumour infiltration into the infundibulum as well as the posterior pituitary [[9]Cote M. Salzman K.L. Sorour M. et al.Normal dimensions of the posterior pituitary bright spot on magnetic resonance imaging.J Neurosurg. 2014; 120: 357-362Crossref PubMed Scopus (48) Google Scholar]. Pituitary metastasis presenting as the first manifestation of remote metastatic disease is rare, however, should be considered in patients presenting with diabetes insipidus. Progressive headache and lethargy in a middle-age female patient: questionJournal of Clinical NeuroscienceVol. 26PreviewA 63-year-old woman with a background of depression, uterine fibroids, fibrocystic disease of the breast and smoking presented with progressive intermittent severe headaches and lethargy associated with blurred vision and loss of peripheral vision. On examination, the patient had a bi-temporal hemianopia on confrontation, which was confirmed with formal Humphrey field testing (Fig. 1) along with a visual acuity on Snellen chart of 6/6 and 6/7.5 and Ishihara plate tests of 7/13 and 3/13 in the right and left eye, respectively. Full-Text PDF

The successful treatment of a recurrent intracranial, dural-based plasmacytoma with lenalidomide

A 75 year old woman presented in October of 2008 with new onset symptoms of headaches, difficulty with penmanship, forgetfulness and vertigo. Her past medical history includes sarcoidosis and osteoporosis. As a result of the sarcoidosis, she has chronic interstitial pulmonary fibrosis but refused treatment with chronic steroids. The physical exam was unremarkable and her Karnofsky performance status was 80. She was evaluated for vestibular abnormalities and a magnetic resonance imaging (MRI) with and without gadolinium was obtained. This demonstrated an enhancing extra-axial, dural-based mass in the high biparietal region, measuring 3.0 cm 9 6.6 cm in the axial plane and encasing the superior sagittal sinus. This was initially thought to represent a meningioma. She underwent a stereotactic biopsy of the mass. The Haematoxylin & Eosin (HE whereas the majority of cells were positive for EMA, and all were strongly positive for MUM-1 (Fig. 1b–d). IHC stains were strongly positive for kappa light chains and negative for lambda chains demonstrating the clonality of the plasma cells in the specimen (Fig. 1e, f). Both serum and urine immunofixation showed no evidence of a monoclonal protein. Serum free light chain ratio was also normal. The blood counts, serum creatinine and calcium are normal. A bone marrow biopsy revealed no evidence of a plasma cell dyscrasia and a skeletal survey showed no destructive lytic lesions, thus effectively ruling out multiple myeloma. Due to the solitary location of the tumor, she was treated with image-modulated radiation therapy (IMRT) in November of 2008, receiving a total of 4,500 cGy. Following this treatment, her MRI normalized and her symptoms improved including the resolution of her headaches. In August 2009, a repeat MRI revealed a new area of dural thickening in a region that had not previously received radiotherapy. She received a second course of IMRT of 4,500 cGy in 25 fractions in September of 2009. A post-treatment MRI showed another complete response. In June 2010, an MRI revealed a new, third homogeneously enhancing lesion measuring approximately 5.3 cm in cranial-caudal dimension (Fig. 2a). Due to the extent of prior radiation therapy and her advanced age, alternatives to radiotherapy were considered in an attempt to avoid excessive neurotoxicity. Given the dural nature of the lesion, it was thought that systemic therapy could access and treat the lesion. A previously published study evaluated the use of low dose dexamethasone combined with lenalidomide (Revlimid ; Celgene Corp., Summit, NJ) for multiple myeloma, and demonstrated an improved time to progression and overall C. E. Devoe (&) Monter Cancer Center, Hofstra North Shore-LIJ School of Medicine, North Shore-Long Island Jewish Health System, 450 Lakeville Road, Lake Success, NY 11042, USA e-mail: CDevoe@NSHS.edu

IgG4-related Disease Presenting as Dural Thickening — A Rare Cause of Myelopathy

Clinical presentation a 58-year-old woman presented with pain in the periscapular region followed by numbness in the feet which extended up the torso over several days. the patient had no history of trauma, or bleeding disorders. She was not anticoagulated. She had no other systemic symptoms. Magnetic resonance imaging (Mri) with gadolinium demonstrated a circumferential epidural-dural lesion from C7 to t6 causing spinal cord compression (figure 1). this

Alternative treatment of intracranial hypotension presenting as postdural puncture headaches using epidural fibrin glue patches: two case reports

Introduction: Intracranial hypotension is a neurologic syndrome characterized by orthostatic headaches and, radiographically, by dural thickening and enhancement as well as subdural collections. Several of etiologies exist, including surgical dural violations, lumbar puncture, or spontaneous cerebrospinal fluid leak. Current management includes conservative management consisting of bed rest, caffeine, and hydration. When conservative management fails, open surgical or percutaneous options are considered. Currently, the gold standard in percutaneous management of intracranial hypotension involves the epidural injection of autologous blood. Recently, some therapies for intracranial hypotension have employed the use of epidural fibrin glue. Case Presentation: Two cases of patients with persistent postdural puncture headaches are presented. Epidural fibrin glue injection alleviated the orthostatic headaches of two patients with intracranial hypotension. Conclusion: Although consideration must be afforded for the potential risks of viral transmission and aseptic meningitis, the utilization of epidural fibrin glue injection as an alternative or adjunct to the epidural blood patch in the treatment of intracranial hypotension should be further investigated.

Magnetic Resonance Imaging Findings of Pediatric Neurobrucellosis

Brucellosis is an endemic disease often observed in the Mediterranean and Middle East regions. Systemic brucellosis is the most frequent clinical form of this infection; however, hematogenic spread may result in the focal form of the disease. Neurobrucellosis is a rare disease seen in 0, 5-25 % of the adults with systemic brucellosis. 0,8 % of the children affected by systemic brucellosis are reported to have neurological complications. A 16 year old female applied with the complaints of headache, vomiting, bilateral hip and knee pain, and inability to walk. Physical and laboratory examination, brain computed tomography (CT) brain and cervical spinal MRI were carried out as well as the magnetic resonance spectroscopy (MRS) of the lesion areas in the brain. On MRI hydrocephaly in 3rd, 4th and lateral ventricles, atrophic dilatation in bilateral hemispheric cortical sulcuses were seen. On T2W and FLAIR images, hyperintense focal nodular lesions not accompanied by pathological contrast enhancement were detected on parietal subcortical white matter and the periventricular deep white matter. Dural thickening and contrast enhancement on the bilateral parietal region were observed. On cervical spinal MRI, leptomeningeal enhancing was at the level of C1-C7. On MRS applied to lesions in brain (TE 136 and 31 ms), lactate peak at 1.3 ppm was observed. In the differential diagnosis of central nervous system diseases in children living in endemic regions, neurobrucellosis should be kept in mind, though observed rarely. In these cases, the neurological system involvement that cannot be demonstrated via CT, can be shown with MRI effectively.

Idiopathic Pachymeningitis Presenting with Progressive Sensorineural Hearing Loss, Tinnitus and Confusion

The patient was a 74-year-old female who presented with progressive left hearing loss, nonpulsatile tinnitus, and confusion. She denied headache, visual symptoms, and vertigo. She had a normal neurotologic examination. Pure tone audiometry showed left low frequency sensorineural hearing loss (SNHL) and a bilateral symmetric high frequency SNHL, with word recognition of 100% right, 46% left (Fig. 1). Serologic studies revealed a nonspecific monoclonal gammopathy. Other serologic tests were negative, including CBC, ANA, Lyme titer, syphilis, angiotensin converting enzyme, anti-double stranded DNA and ANCA antibodies. Cerebrospinal fluid testing revealed normal cytology with a mild increase in protein. Computed tomography of the abdomen and chest was normal, as was bone marrow biopsy. Magnetic resonance imaging (MRI) demonstrated diffuse smooth dural thickening and enhancement consistent with idiopathic hypertrophic pachymeningitis (IHP) (Fig. 2). Figure 1 Pure tone audiograms from 2009 through 2012, demonstrating progressive hearing loss in the left (A) and right (B) ears. At baseline (solid black line), the patient had a left 30 dB SRT and 100% WR, and a right SRT of 20 dB and WR 100%. After onset of ... Figure 2 Axial T1-weighted contrast-enhanced MRI of brain from prior to treatment (A) demonstrating initial enhancement of dura of the internal auditory canals left > right (arrowheads) prior to treatment. Following pulse steroid treatment (B), a significant ... The patient was initially treated with oral prednisone with minimal improvement. She then received IV methylprednisolone infusions every two weeks for two months with improvement in left hearing, tinnitus, mental status, and dural enhancement on MRI. Six months following initial presentation, she showed no further progression of her symptoms while off steroid therapy.

Intracranial Extramedullary Hematopoiesis: A Rare Cause of Headaches

Ectopic bone marrow production, known as extramedullary hematopoiesis, may result in symptoms due to compression on normal structures. We present the multimodality imaging findings and subsequent management of a rare case of symptomatic extramedullary hematopoiesis within the calvarium. Case report. A 54-year-old male with a history of myelofibrosis and no previous diagnosis of a headache disorder presented to the emergency department with worsening severe bilateral headaches. A nonenhanced CT of the brain was performed and diffuse extra-axial nodular hyperdensities were visualized. MRI of the brain demonstrated diffuse extra-axial avidly enhancing nodular masses, dural thickening and marked susceptibility. No paravertebral masses, typical for extramedullary hematopoiesis, were present in the chest or abdomen. Although the clinical team considered a biopsy to confirm the diagnosis, we suggested a noninvasive confirmatory test. The subsequent Tc99m sulfur colloid scan corroborated the diagnosis. The patient was then referred to radiation oncology for treatment. In summary, extramedullary hematopoiesis is a hematologic compensatory disorder that rarely occurs within the CNS and may cause neurological compromise due to compression on underlying structures. The diagnosis can be made with noninvasive imaging and treated with low dose radiation therapy.

Back to the scanner: expected and unexpected imaging findings following spinal puncture and access

Spinal canal procedures to withdraw fluid or introduce drugs are common minimally invasive techniques after which patients may experience new symptoms causing presentation to the emergency department. CT or MR imaging as part of the diagnostic workup may reveal expected or unexpected findings which may explain these symptoms. Small foci of gas within the spinal canal or intracranial compartment are common but are usually self-limited. Postlumbar puncture headache, presumed to be due to intracranial hypotension, may manifest on brain MR as dural thickening and low-lying cerebellar tonsils. Treatment with a blood patch is sometimes required for persistent symptoms. Spinal subarachnoid hemorrhage may be a devastating complication of dural puncture and may result in pain and paraplegia. In this review, technical details of these procedures are described and examples of common and uncommon postprocedure appearances on imaging are presented.

Intracranial mass‐forming lesion associated with dural thickening and hypophysitis

Hiromi Kanno, Mishie Tanino, Kentaro Watanabe, Yoshimaru Ozaki, Tamio Itoh, Taichi Kimura, Hiroshi Nishihara, Tomoo Itoh, Takuhito Narita, Kazuo Nagashima and Shinya Tanaka Laboratory of Cancer Research, Department of Pathology, Laboratory of Translational Pathology, Hokkaido University School of Medicine, Department of Neurosurgery, Nakamura Memorial Hospital, Department of Diagnostic Pathology, Kobe University Hospital, Kobe, Department of Neurosurgery, Seiwa Memorial Hospital, Department of Pathology, Sapporo Higashi-Tokushukai Hospital, Sapporo, Japan

Lentiform fork sign in a child with dialysis disequilibrium syndrome: A transient MRI pattern which emphasizes neurologic consequence of metabolic acidosis

A rare but important complication related to otherwise uneventful brain and spine surgery is becoming more recognized and more frequently reported in the medical literature. This has been variably labeled as pseudohypoxic brain swelling or postoperative hypotension-associated venous congestion. This poorly understood condition occurs in the setting of surgical intervention and is thought to be related to cerebrospinal fluid leak or evacuation, decreased intracranial pressure, and subsequent development of deep venous congestion affecting the basal ganglia, thalami, and cerebellum. Clinically, patients may have global neurologic deficit and outcomes range from full recovery to vegetative state or death. The imaging correlate includes atypical edema, infarction, or hemorrhage and can overlap the appearance of diffuse hypoxic injury, for which this condition can be mistaken both clinically and radiologically. Although this deep brain tissue edema can be associated with other signs of cerebrospinal fluid hypotension such as dural thickening, brain sagging, and cerebellar herniation, it can be isolated, making the diagnosis challenging.We present 2 cases of unexpected clinical deterioration occurring in patients with otherwise uncomplicated neurosurgery, 1 with craniotomy and the other with lumbar spine intervention. Both patients exhibit similar appearing edema in the deep gray structures on postoperative magnetic resonance imaging scans. In addition to reviewing the prior literature and imaging findings, we evaluate the imaging findings to determine if there are unique features or signatures that might allow differentiation of PHBS from hypoxic-ischemic encephalopathy.The lentiform rim sign can be helpful for differentiation of pseudohypoxic brain swelling versus hypoxic-ischemic encephalopathy.

E-065 Diagnosis and management of intracranial hypotension: a case review

PurposeHeadache remains one of the top three most common indications for neurologic imaging. Intracranial hypotension is one causative etiology of headache, which although rare, carries considerable morbidity and potential mortality....