Focal Contrast Enhancement Research Articles

Role of MRI in Diagnosis of Rapidly Progressive Cognitive Impairment- A Case Of Sporadic Creutzfeldt Jakob Disease (sCJD)

Abstract Background The role of neuroimaging in dementia extends beyond its traditional role of excluding neurosurgical lesions. Radiological findings may support the diagnosis of specific neurodegenerative disorders and aid differentiation from other neurodegenerative conditions e.g. CJD1. sCJD is a fatal neurodegenerative disease, which typically presents with neuropsychiatric symptoms, myoclonus, cerebellar and pyramidal/extrapyramidal symptoms. The diagnosis often is quite difficult, requiring high degree of suspicion, advanced neuroimaging and biochemical markers. Confirmation is only possible via brain biopsy. The condition can result in rapidly progressive dementia/neurocognitive decline and death, usually within a year from onset. Methods We report a case of a rapidly progressive dementia in a 79-year-old male who was diagnosed with probable sCJD, after having an MRI which picked up suspicious characteristic findings leading to the eventual diagnosis confirmation via further workup. The patient presented with acute rapid progressive forgetfulness and functional decline of 2 weeks with initial workup unyielding. Results A contrast MRI Brain eventually showed focal increased T2 and flair signals in the right parafalcine and parietal cortex demonstrating mild fluid restriction without any focal contrast enhancement, a pattern highly suspicious for sCJD. A CSF sample subsequently revealed RT-QulC positivity confirming the diagnosis. The patient eventually passed away despite ward-based care after 4 weeks of admission due to rapid neurological decline. A brain biopsy was not deemed feasible given patient’s condition. Conclusion The case highlights a relatively atypical symptomology of sCJD and, highlights the role of MRI imaging in assessment and diagnosis of dementias and specifically in sCJD. Increasing use of advanced neuroimaging may aid in diagnosis of specific/rare causes of rapidly progressive dementias. Reference Vitali, P., Maccagnano, E., Caverzasi, E., Henry, R.G., Haman, A., Torres-Chae, C., Johnson, D.Y., Miller, B.L. and Geschwind, M.D., 2011. Diffusion-weighted MRI hyperintensity patterns differentiate CJD from other rapid dementias. Neurology, 76(20), pp. 1711-1719.

Locoregional staging of breast cancer: contrast-enhanced mammography versus breast magnetic resonance imaging

PurposeBreast cancer diagnosis often involves assessing the locoregional spread of the disease through MRI, as multicentricity, multifocality and/or bilaterality are increasingly common. Contrast-enhanced mammography (CEM) is emerging as a potential alternative method. This study compares the performance of CEM and MRI in preoperative staging of women with confirmed breast carcinoma. Patients were also asked to fill in a satisfaction questionnaire to rate their comfort level with each investigation.MethodsFrom May 1st, 2021 to May 1st, 2022, we enrolled 70 women with confirmed breast carcinoma who were candidates for surgery. For pre-operative locoregional staging, all patients underwent CEM and MRI examination, which two radiologists evaluated blindly. We further investigated all suspicious locations for disease spread, identified by both CEM and MRI, with a second-look ultrasound (US) and eventual histological examination.ResultsIn our study cohort, MRI and CEM identified 114 and 102 areas of focal contrast enhancement, respectively. A true discrepancy between MRI and CEM occurred in 9 out of 70 patients examined. MRI reported 8 additional lesions that proved to be false positives on second-look US in 6 patients, while it identified 4 lesions that were not detected by CEM and were pathological (true positives) in 3 patients.ConclusionsCEM showed results comparable to MRI in the staging of breast cancer in our study population, with a high rate of patient acceptability.

RADT-48. RADIATION MYELOPATHY AFTER STANDARD DOSE RADIATION: POTENTIAL RADIATION SENSITIZING EFFECTS OF SYSTEMIC THERAPY

Abstract Radiation myelopathy is an uncommon adverse effect of spinal irradiation. It is especially rare following conventional palliative fractionation regimens for vertebral metastasis. We present two cases of delayed thoracic radiation myelopathy in patients with breast cancer. In both cases, palliative radiation of 30 Gy in ten fractions was administered to thoracic spinal metastases. Neither patient received concurrent systemic therapy. One began trastuzumab emtansine nine days after finishing radiation and the other started palbociclib three months afterwards, which was switched to capecitabine at disease progression. Each patient developed progressive leg weakness and numbness, ten months following radiation and eleven months following radiation (four weeks following capecitabine), respectively. MRI showed edema and focal contrast enhancement within the radiation field in each. Both were treated with high-dose corticosteroid, vitamin E, and pentoxifylline. Neither patient returned to their neurologic baseline and remain with significant deficits, requiring assistance with ambulation. Serial MRIs revealed eventual reduction in cord enhancement and edema, followed by cord atrophy in one case. The cumulative radiation dose of 30 Gy is typically within the safety tolerance of the spinal cord, so it is speculative if post-radiation systemic therapy played a role in the development of myelopathy. The timing is particularly convincing in the patient who developed symptoms within four weeks of starting capecitabine. These cases are similar to recently reported instances of radiation myelopathy after standard dose spinal radiation followed by chemotherapy or immunotherapy. Recognition of the potential radiation sensitizing effects of systemic therapy should lead clinicians to consider radiation myelopathy early if spinal cord symptoms develop and to consider the timing of initiation of potential sensitizing drugs when spinal radiation has been given.

Anti–Gamma Aminobutyric Acid B Autoimmune Encephalitis in an Indian Child with Early-Onset Seizures, Neurodegeneration, and Brain Calcification due to NRROS Variation: The First Reported Case Worldwide

AbstractA 1.5-year-old boy presented to us with a history of normal growth and developmental parameters until 6 months of age. However, at 7 months of age, he developed multiple types of seizures consisting initially of complex febrile seizures, followed by afebrile seizures. Multifocal clonic, generalized tonic–clonic, and myoclonic (multifocal and generalized) were the evolving seizure types. He had truncal hypotonia, but his appendicular hypotonia progressed to hypertonia over the next few months and further to decorticate posturing. Brain magnetic resonance imaging (MRI) showed generalized atrophy, predominantly frontotemporal, without any focal signal abnormalities or contrast enhancement. Computed tomography (CT) showed speckled calcification in subcortical white matter. Electroencephalogram showed bilateral frontotemporal epileptiform discharges with secondary generalization. His cerebrospinal fluid had normal cytology and biochemical results but was positive for anti–gamma aminobutyric acid B antibodies. Whole exome sequencing showed likely pathogenic, novel autosomal recessive homozygous variation of NRROS gene on chromosome 3 [c.1487G > A (p.Trp496Ter)], which impairs the functioning of anti-inflammatory cytokine transforming growth factor beta, resulting in a proinflammatory state within the central nervous system and thereby promoting autoimmune encephalitis. Parental Sanger sequencing validated the variation in both his parents. He was treated with both pulse methylprednisolone (30 mg/kg/day for 5 days) and intravenous immunoglobulin (2 g/kg), followed by slowly tapering of oral prednisolone and monthly intravenous immunoglobulin infusion (1 g/kg). There was significant reduction in seizure frequency and disappearance of epileptiform discharges from the electroencephalogram. However, the motor and cognitive improvement did not occur, and he had microcephaly and growth failure at the last follow-up. This is the 11th case report of neurodegeneration associated with NRROS gene variations, but the first report of autoimmune encephalitis being triggered by the variation in a child.

Diagnostic controversies in recurrent painful ophthalmoplegic neuropathy: single case report with a systematic review

BackgroundOphthalmoplegic migraine, renamed "Recurrent Painful Ophthalmoplegic Neuropathy" (RPON) in 2013 by the International Headache Society is a rare neurologic disorder characterized by recurrent attacks of ophthalmoplegia associated to ipsilateral headache. The etiology is still unknown. Typical magnetic resonance imaging findings show a focal nerve thickening and contrast enhancement. In the majority of cases, there is a full recovery within days or weeks. There is no evidence supporting a specific treatment. The review defines the characteristics of the recurrent painful ophthalmoplegic neuropathy in patients within 2 years of age underlying the importance of the role of magnetic resonance imaging even in presence of the first attack. Thus, an emblematic case report is presented.Case presentationThe authors present a case of third cranial nerve paresis in a 17-month-old male child, presenting a neuroradiological pattern highly suggestive of schwannoma, aneurism or recurrent painful ophthalmoplegic neuropathy. Thus, a review of the literature with the pediatric casuistry of recurrent painful ophthalmoplegic neuropathy occurred within 2 years of age focusing on diagnostic considerations is presented. The authors highlight the importance to consider recurrent painful ophthalmoplegic neuropathy in presence of magnetic resonance imaging findings and clinical symptoms referable to aneurysm or schwannoma. Thus, the review defines the characteristics and the neuroradiological findings at the first RPON attack occurred under 2 years of age.ConclusionAlthough two attacks are necessary, the review strongly suggests to consider recurrent painful ophthalmoplegic neuropathy even at the first attack, in presence of described characteristics and the aforementioned magnetic resonance imaging findings.

Clinico-radiological characteristics of anti-myelin oligodendrocyte glycoprotein antibody-associated autoimmune encephalitis in children.

To investigate the clinical characteristics and prevalence of paediatric anti-myelin oligodendrocyte glycoprotein (MOG) antibody-associated autoimmune encephalitis. A total of 94 paediatric patients (46 males, 48 females, median age 9 years 5 months, range: 8 months-17 years 8 months) with autoimmune encephalitis were recruited at Seoul National University Children's Hospital. We evaluated autoantibody status and identified patients with anti-MOG antibody-associated autoimmune encephalitis. Retrospective reviews of medical records were performed to describe clinical presentations, laboratory findings, treatments, and outcomes. Eight patients (five males, three females, median age 11 years 9 months) with anti-MOG antibody-associated encephalitis were identified (8.5% of those with autoimmune encephalitis), one of whom was copositive for anti-N-methyl-d-aspartate receptor (NMDAR) antibodies. Anti-NMDAR antibodies were identified in 23 patients (23 out of 94, 24.5%). Unilateral or bilateral cortical involvement was identified in five patients. Focal contrast enhancement was also identified in three of the five patients with cortical lesions. All patients showed favourable response to immunotherapy with a Modified Rankin Scale ≤2 at the last follow-up. Relapse was found in one patient and clinico-radiological remission was achieved with cyclic intravenous immunoglobulin therapy. Anti-MOG antibody-associated encephalitis accounts for a significant proportion of clinically defined paediatric patients with autoimmune encephalitis. Anti-MOG antibody-associated encephalitis should be included in the clinical spectrum of anti-MOG-associated diseases.

Clinical Implications of Initial Focal Contrast Enhancement Accompanying Acute Type B Intramural Hematoma

Clinical Implications of Initial Focal Contrast Enhancement Accompanying Acute Type B Intramural Hematoma

Primary Extranodal Follicular T-Cell Lymphoma and Ductal Breast Carcinoma Diagnosed by a Magnetic Resonance Imaging-Guided Vacuum-Assisted Biopsy: A Case Report

Patient: Female, 62-year-oldFinal Diagnosis: Primary non-Hodgkin lymphoma and ductal carcinoma in-situSymptoms: No specific symptomsMedication:—Clinical Procedure: —Specialty: RadiologyObjective:Rare co-existance of disease or pathologyBackground:Extranodal non-Hodgkin’s lymphomas (NHLs) are most commonly low-grade B-cell lymphomas, and the breast is not an usual site for this condition. This case report describes a 62-year-old woman with a primary NHL and ductal carcinoma in situ (DCIS) of the left breast diagnosed by a magnetic resonance imaging (MRI)-guided vacuum-assisted breast biopsy. The simultaneous diagnosis of breast cancer and NHL is rare, with few cases described in the literature. Primary breast lymphomas account only for 0.04% to 0.5% of breast malignancies.Case Report:In November 2016, a 62-year-old woman was treated for a peripheral T-cell lymphoma (follicular helper T-cell phenotype) of the left upper central breast; later she underwent periodic breast imaging follow-ups. In October 2018, MRI revealed a focal 33-mm non-mass contrast enhancement (according to the Breast Imaging Reporting and Data System –MRI lexicon of the American College of Radiology) in the lower external quadrant of the left breast. Neither mammography nor ultrasonography demonstrated any suspicious features. The multidisciplinary medical team performed an MRI-guided vacuum-assisted breast biopsy and the histological analysis confirmed the diagnosis of a DCIS. Subsequently, she underwent surgery resulting in eradication of the disease and has had regular follow-ups, including mammography, ultrasonography, and MRI.Conclusions:This is a rare case of both a primary NHL of the breast and DCIS, which was detected only by MRI. It highlights the role of an MRI-guided vacuum-assisted breast biopsy, which allows an accurate and economic diagnosis in case of suspicious findings on MRI. We recommend the use of MRI in follow-ups for patients with previous breast lymphomas (high-risk patients).

Retrospective analysis of multiparametric MRI to predict complete pathologic response after neo-adjuvant chemotherapy for muscle invasive bladder cancer.

e16535 Background: Muscle invasive bladder cancer (MIBC) is a life-threatening disease. Treatment is multimodal combining neo-adjuvant cisplatin-based chemotherapy (NAC) and radical cystectomy (RC). Accurately predicting complete pathologic response (pCR) using multiparametric MRI (mpMRI) could impact peri-operatively treatment. Methods: MIBC patients receiving NAC were evaluated at our institution with mpMRI before, after 2 cycles and after 4 cycles of dose dense MVAC (ddMVAC). Response after 4 cycles was retrospectively assessed using the method earlier described by Necchi et al with blinding of the readers for the pathological result of RC. Two radiologists independently evaluated 3 questions: residual disease at T1/T2-weighted images, presence of spots of restrictive diffusion within the bladder wall on diffusion-weighted imaging, and presence of focal contrast enhancement in the bladder wall on dynamic contrast enhanced images. Radiographic complete response (rCR) was defined as “No” on all three questions. Results: A total of 46 patients were identified having received ddMVAC for urothelial MIBC. Six patients did not undergo RC after NAC and were excluded from this analysis. Eleven out of 40 (28%) patients showed a complete pathologic response (ypT0). Baseline characteristics were similar compared to non-complete pathologic responders ( > ypT0), with the exception of hydronephrosis (9% in ypT0 vs. 52% in > ypT0). mpMRI questions could be assessed in 37 of 40 patients (93%). rCR was seen in 5 patients and was significantly associated with pCR (1-sided p value 0.021). Although sensitivity was low (36%), specificity was very high (96%) of this 3-step assessment. Positive likelihood ratio was 9.45, negative likelihood ratio 0.66. Concordance of assessment was very high. Conclusions: Using the 3-step imaging approach of Necchi et al, mpMRI can predict pCR after neo-adjuvant cisplatin-based chemotherapy with high specificity but low sensitivity. mpMRI should be included in future trials of multimodal management of MIBC and is an important predictive asset in routine clinical management.[Table: see text]

CENTRAL MYELIN AND DEMYELINATION

INTRODUCTION:CNS demyelinating disorders are common neurological illness that affects the brain. This causes signicant disability and mortality if not properly identied and treated. CNS demyelinating diseases includes multiple sclerosis, acute disseminated encephalomyelitis, NMO spectrum disorders and transverse myelits. Here we are reporting a case of central demyelination. CASE SUMMARY: 44yrs female admitted with subacute onset of weakness of all four limbs, more on left side and sensory disturbance in the form of numbness and tingling in all four limbs, more on left side, associated with urinary urgency and constipation. Patient had right optic neuritis 3 years back. General examination was normal. Vital signs were stable. On nervous system examination, patient had decresed visual acuity in right eye, with relative afferent pupillary defect and impaired red green colour vision, fundus was normal. Motor system examination, Patient had normal bulk, increased tone, brisk deep tendon reexes, power of 4 on right and 4- on left side with extensor planter on both sides. Patient had decresed sensation below C5 spinal cord level, predominantly joint position and vibration sense with positive lhermitte's sign. Other systems were normal. Routine lab investigation ,ECG,CXR were normal. CSF analysis revealed elevated protein of 72mg. MRI BRAIN WITH WHOLE SPINE SCREENING(contrast) revealed Multiple smallT2/FLAIR hyperintense lesion in bilateral juxta cortical, subcortical(involving the temporal lobes and callososeptal interface), subtle patchy enhancement in the left peritrigonal lesion and in body of corpus callosum. Mild atrophy of cervical cord from C3 to C5 with T2 hyperintense involving both hemicord from C2 to C5 level, focal Hyperintensity subtle contrast enhancement in Dorsal cord at the level of D11, Features suggestive of demyelination likely Multiple Sclerosis.

Multiple sclerosis with intractable vomiting and atypical area postrema lesion

BackgroundArea postrema syndrome is considered as one of the most typical presentations of neuromyelitis optica spectrum disorders (NMOSDs) (Wingerchuk et al., 2015). The involvement of area postrema is rarely seen in multiple sclerosis (MS). We are here report a case of a young woman with multiple sclerosis, presented with intractable vomiting, and her MRI brain showed acute T2 hyperintense signal over the area postrema. Case presentationA 36-year-old Asian woman who is known to have schizophrenia and multiple sclerosis since 2012. She was noted to have spastic gait, and the MRI brain in 2012 showed multiple perpendicular periventricular T2 lesions suggestive of multiple sclerosis (MS). However, she defaulted her neurologist follow-up and was not on any treatment for MS. She was admitted in 2016 with intractable vomiting, and her MRI brain showed T2 hyperintense signal over area postrema with focal contrast enhancement. Her MRI cervical spine was normal. The visual evoked potential study showed bilateral prolonged P100 latencies. Oligoclonal bands were detected in her CSF analysis. Both the serum aquaporin-4 IgG (AQP4 IgGs) antibody and myelin oligodendrocyte glycoprotein (MOG-IgGs) were negative. Her intractable vomiting resolved after a short course of intravenous methylprednisolone. She was treated as MS with interferon-beta 1a. She has been in remission since 2016, and her functional status also improved from the expanded disability status scale (EDSS) of 2.0 (in 2016) to 1.0 (in 2020). ConclusionWe proposed that although area postrema lesion is typically seen in NMOSDs, it may also be seen in MS. Current MRI criteria for MS and NMOSDs are not sufficiently specific, and the diagnostic criteria should only be used in the appropriate clinical context.

Leptomeningeal Contrast Enhancement Is Related to Focal Cortical Thinning in Relapsing-Remitting Multiple Sclerosis: A Cross-Sectional MRI Study.

Leptomeningeal inflammation is associated with the development of global cortical gray matter atrophy in multiple sclerosis. However, its association with localized loss of tissue remains unclear. The purpose of this study was to evaluate the relationship between leptomeningeal contrast enhancement, a putative marker of leptomeningeal inflammation, and focal cortical thinning in MS. Forty-three patients with relapsing-remitting MS and 15 with secondary-progressive MS were imaged on a 3T scanner. Cortical reconstruction was performed with FreeSurfer. Leptomeningeal contrast-enhancement foci were visually identified on 3D-FLAIR postcontrast images and confirmed using subtraction imaging. Leptomeningeal contrast-enhancement foci were mapped onto the cortex, and ROIs were obtained by dilating along the surface multiple times (n = 5, 10, 15, 20, 25, 30, 35, 40). Resulting ROIs were then mapped onto the homologous region of the contralateral hemisphere. Paired t tests compared the thickness of the cortex surrounding individual leptomeningeal contrast-enhancement foci and the corresponding contralateral region. Results were corrected for the false discovery rate. Differences between ipsilateral and contralateral ROIs progressively decreased with larger ROIs, but no significant effects were detected when considering the entire MS sample. In patients with relapsing-remitting MS only, significantly reduced cortical thickness was found for 5 dilations (-8.53%, corrected P = .04) and 10 dilations (-5.20%, corrected P = .044). Focal leptomeningeal contrast enhancement is associated with reduced thickness of the surrounding cortex in patients with relapsing-remitting MS, but not in those with secondary-progressive MS. Our results suggest that pathology associated with the presence of leptomeningeal contrast-enhancement foci has a stronger, localized effect on cortical tissue loss earlier in the disease.

Massive enlargement of all peripheral nerves

A 29 year old woman presented with three months of intermittent but increasing bifacial paraesthesia. On close questioning she reported five years of mild intermittent paraesthesiae in both hands and very mild proximal upper limb weakness. Examination revealed a small area of pinprick loss over the left cheek as the only abnormality. Nerve conduction study of both upper limbs demonstrated symmetric distal sensory and motor abnormalities strongly suggestive of demyelination (small or absent sensory potentials and markedly prolonged distal motor latencies). The remainder of extensive neurophysiologic assessment was normal. MRI brain and whole spine revealed massive symmetric enlargement of multiple cranial nerves, nerve roots, brachial and lumbosacral plexuses (Fig. 1A–F), without contrast enhancement. CSF showed albuminocytologic dissociation (protein 1.48 g/L) and extensive blood tests and whole body PET scan were normal. The most likely diagnosis isA.SarcoidosisB.Charcot Marie Tooth (CMT)C.Chronic Inflammatory Demyelinating Polyradiculopathy (CIDP)D.Leprosy CIDP was diagnosed based on these neurophysiologic, CSF and MRI findings and intravenous immunoglobulin (ivIg) was eventually commenced due to increasing symptoms. One year following presentation the patient was largely asymptomatic on maintenance monthly ivIg, with significant improvement in neurophysiologic parameters but no change in MRI features. The striking feature of this case was the extreme discordance between a mild clinical presentation of CIDP and the MRI findings of widespread and massive hypertrophy of multiple cranial nerves, nerve roots and plexuses. This point is highlighted by noting that the dramatic lumbar root and plexus MRI abnormalities were associated with absolutely no symptoms, signs or neurophysiological abnormalities. Massive peripheral nerve enlargement has a relatively limited differential diagnosis. Congenital causes, such as Charcot-Marie-Tooth or Neurofibromatosis; and acquired pathology, in particular leparomatous disease or an infiltrative process such as systemic amyloidosis, seemed unlikely given demyelinating neurophysiology that was clearly reversible with ivIg. Massive nerve root thickening on MRI has been reported in inflammatory conditions. The most common of these being CIDP, but to our knowledge this is the first report of massive cranial nerve enlargement in the condition [1Duggins A.J. McLeod J.G. Pollard J.D. et al.Spinal root and plexus hypertrophy in chronic inflammatory demyelinating polyneuropathy.Brain. 1999; 122: 1383-1390Crossref PubMed Scopus (193) Google Scholar, 2Adachi Y. Sato N. Okamoto T. et al.Brachial and lumbar plexuses in chronic inflammatory demyelinating polyradiculoneuropathy: MRI assessment including apparent diffusion coefficient.Neuroradiology. 2010; 53 ([published Online First: Epub Date]): 3-11https://doi.org/10.1007/s00234-010-0684-7Crossref PubMed Scopus (69) Google Scholar, 3Shibuya K. Sugiyama A. S-i Ito et al.Reconstruction magnetic resonance neurography in chronic inflammatory demyelinating polyneuropathy.Ann Neurol. 2015; 77 ([published Online First: Epub Date]): 333-337https://doi.org/10.1002/ana.24314Crossref PubMed Scopus (85) Google Scholar]. The initial report correlated massive root hypertrophy with a long and disabling disease course [[1]Duggins A.J. McLeod J.G. Pollard J.D. et al.Spinal root and plexus hypertrophy in chronic inflammatory demyelinating polyneuropathy.Brain. 1999; 122: 1383-1390Crossref PubMed Scopus (193) Google Scholar]. However subsequent case series have not found any definite correlation between nerve hypertrophy and disease severity or duration [2Adachi Y. Sato N. Okamoto T. et al.Brachial and lumbar plexuses in chronic inflammatory demyelinating polyradiculoneuropathy: MRI assessment including apparent diffusion coefficient.Neuroradiology. 2010; 53 ([published Online First: Epub Date]): 3-11https://doi.org/10.1007/s00234-010-0684-7Crossref PubMed Scopus (69) Google Scholar, 3Shibuya K. Sugiyama A. S-i Ito et al.Reconstruction magnetic resonance neurography in chronic inflammatory demyelinating polyneuropathy.Ann Neurol. 2015; 77 ([published Online First: Epub Date]): 333-337https://doi.org/10.1002/ana.24314Crossref PubMed Scopus (85) Google Scholar]. It is possible that MRI features differ between the various subtypes of CIDP and focal demyelination on neurophysiological testing of peripheral nerves has been associated with focal reversible contrast enhancement [3Shibuya K. Sugiyama A. S-i Ito et al.Reconstruction magnetic resonance neurography in chronic inflammatory demyelinating polyneuropathy.Ann Neurol. 2015; 77 ([published Online First: Epub Date]): 333-337https://doi.org/10.1002/ana.24314Crossref PubMed Scopus (85) Google Scholar, 4Kuwabara S. Nakajima M. Matsuda S. Hattori T. Magnetic resonance imaging at the demyelinative foci in chronic inflammatory demyelinating polyneuropathy.Neurology. 1997; 48 ([published Online First: Epub Date]): 874-877https://doi.org/10.1212/wnl.48.4.874Crossref PubMed Google Scholar]. However in routine practice MRI abnormalities simply provide supportive evidence of CIDP without specific management implications. The remarkable findings in our case suggest that massive hypertrophy can occur with very mild clinical disease. We propose that recurrent or chronic subclinical inflammation, demyelination and repair can lead to massive hypertrophy, almost as an oyster lays down layers of a pearl. Dr Fong was involved in the management and care of the patient and preparation and drafting of the manuscript. No disclosures relevant to this study are reported. Dr Masters was involved in the management and care of the patient and preparation and drafting of the manuscript and figures. No disclosures relevant to this study are reported. Dr Watson was involved in the management and care of the patient and preparation and drafting of the manuscript. No disclosures relevant to this study are reported.

Utilizing 18F-fluoroethyltyrosine (FET) positron emission tomography (PET) to define suspected nonenhancing tumor for radiation therapy planning of glioblastoma

AimThe authors sought to evaluate the impact of 18F-fluoroethyltyrosine (FET) positron emission tomography (PET) on radiation therapy planning for patients diagnosed with glioblastoma (GBM) and the presence of suspected nonenhancing tumors compared with standard magnetic resonance imaging (MRI). Methods and materialsPatients with GBM and contrast-enhanced MRI scans showing regions suspicious of nonenhancing tumor underwent postoperative FET-PET before commencing radiation therapy. Two clinical target volumes (CTVs) were created using pre- and postoperative MRI: MRI fluid-attenuated inversion recovery (FLAIR) sequences (CTVFLAIR) and MRI contrast sequences with an expansion on the surgical cavity (CTVSx). FET-PET was used to create biological tumor volumes (BTVs) by encompassing FET-avid regions, forming BTVFLAIR and BTVSx. Volumetric analyses were conducted between CTVs and respective BTVs using Wilcoxon signed-rank tests. The volume increase with addition of FET was analyzed with respect to BTVFLAIR and BTVSx. Presence of focal gadolinium contrast enhancement within previously nonenhancing tumor or within the FET-avid region was noted on MRI scans at 1 and 3 months after radiation therapy. ResultsTwenty-six patients were identified retrospectively from our database, of whom 24 had demonstrable FET uptake. The median CTVFLAIR, CTVSx, BTVFLAIR, and BTVSx were 57.1 mL (range, 1.1-217.4), 83.6 mL (range, 27.2-275.8), 62.8 mL (range, 1.1-307.3), and 94.7 mL (range, 27.2-285.5), respectively. When FET-PET was used, there was a mean increase in volume of 26.8% from CTVFLAIR to BTVFLAIR and 20.6% from CTVSx to BTVSx. A statistically significant difference was noted on Wilcoxon signed-rank test when assessing volumetric change between CTVFLAIR and BTVFLAIR (P < .0001) and CTVSx and BTVSx (P < .0001). Six of 24 patients (25%) with FET avidity before radiation therapy showed focal gadolinium enhancement within the radiation therapy portal. ConclusionsFET-PET may help improve delineation of GBM in cases with a suspected nonenhancing component. This may result in improved radiation therapy target delineation and reduce the risk of potential geographical miss. SummaryWe investigated the impact of 18F-fluoroethyltyrosine (FET) positron emission tomography (PET) on radiation therapy planning for patients diagnosed with glioblastoma (GBM) and a suspected nonenhancing tumor compared with standard magnetic resonance imaging. We performed volumetric analyses between clinical target volumes and respective biological target volumes using Wilcoxon signed-rank tests. FET-PET may help improve delineation of GBM in cases with a suspected nonenhancing component and reduce the risk of potential geographical miss.

Peripheral zone lesions of intermediary risk in multiparametric prostate MRI: Frequency and validation of the PI-RADSv2 risk stratification algorithm based on focal contrast enhancement

PurposeTo validate the risk stratification algorithm of the Prostate Imaging Reporting and Data System (PI-RADSv2) for intermediary risk lesions (PI-RADSv2 category 3) in the peripheral zone based on focal contrast enhancement and to compare cancer rates in category 3, upgraded category 4 and category 4 based on markedly low ADC value. Materials and methodsWe retrospectively analyze 172 consecutive patients undergoing prostate MRI with 315 histopathologically verified lesions. We select all lesions either assigned category 3 or category 4 in the peripheral zone for further analysis. We compare cancer rates with the two-sided chi-squared test. To determine inter-observer agreement about contrast enhancement two blinded radiologists evaluate the subset of category 3 lesions based on the diffusion weighted sequence. ResultsThe frequency of peripheral PI-RADS 3, upgraded PI-RADS 4 and PI-RADS 4 lesions based on markedly low ADC value is 10.8%, 10.8% and 20.3%, respectively. Cancer rates (significant cancer only) in these subgroups are 8.8% (3/34), 23.5% (8/34) and 40.6% (26/64), P < 0.01. Inter-observer agreement is moderate for evaluation of contrast enhancement with kappa values between 0.46 and 0.5. ConclusionWe demonstrate a trend of increasing cancer rate from PI-RADSv2 category 3 to upgraded category 4 to category 4 based on markedly low ADC value. Peripheral lesions of intermediary risk in the diffusion weighted sequence account for 21.6% of all prostate lesions encountered. Since it is likely that patient management recommendations will be linked to assessment categories in future versions of PI-RADS, cancer rates in upgraded category 4 and category 4 based on markedly low ADC values should be in a similar range. We conclude that in future studies of PI-RADSv2 upgraded category 4 and category 4 based on markedly low ADC value should be reported separately to generate a database for meta-analysis of cancer rates.

Early-Stage Glioblastomas: MR Imaging-Based Classification and Imaging Evidence of Progressive Growth.

The serial imaging changes describing the growth of glioblastomas from small to large tumors are seldom reported. Our aim was to classify the imaging patterns of early-stage glioblastomas and to define the order of appearance of different imaging patterns that occur during the growth of small glioblastomas. Medical records and preoperative MR imaging studies of patients diagnosed with glioblastoma between 2006 and 2013 were reviewed. Patients were included if their MR imaging studies showed early-stage glioblastomas, defined as small MR imaging lesions detected early in the course of the disease, demonstrating abnormal signal intensity but the absence of classic imaging findings of glioblastoma. Each lesion was reviewed by 2 neuroradiologists independently for location, signal intensity, involvement of GM and/or WM, and contrast-enhancement pattern on MR imaging. Twenty-six patients with 31 preoperative MR imaging studies met the inclusion criteria. Early-stage glioblastomas were classified into 3 types and were all hyperintense on FLAIR/T2-weighted images. Type I lesions predominantly involved cortical GM (n = 3). Type II (n = 12) and III (n = 16) lesions involved both cortical GM and subcortical WM. Focal contrast enhancement was present only in type III lesions at the gray-white junction. Interobserver agreement was excellent (κ = 0.95; P < .001) for lesion-type classification. Transformations of lesions from type I to type II and type II to type III were observed on follow-up MR imaging studies. The early-stage glioblastomas of 16 patients were pathologically confirmed after imaging progression to classic glioblastoma. Cortical lesions may be the earliest MR imaging-detectable abnormality in some human glioblastomas. These cortical tumors may progress to involve WM.

Emerging Concepts in Intramural Hematoma Imaging.

Intramural hematoma (IMH) is included in the spectrum of acute aortic syndrome and appears as an area of hyperattenuating crescentic thickening in the aortic wall that is best seen at nonenhanced computed tomography. IMH is historically believed to originate from ruptured vasa vasorum in the aortic media without an intimal tear, but there are reports of small intimomedial tears identified prospectively at imaging or found at surgery in some cases of IMH. These reports have blurred the distinction between aortic dissection and IMH and raise questions about what truly distinguishes the entities that compose acute aortic syndrome. The pathophysiology of these subgroups and the controversies surrounding their differentiation are discussed. The natural history of IMH is highly variable; it may resolve or progress to aneurysm, dissection, or rupture. The authors review various imaging prognostic factors that should be reported by the radiologist, including Stanford classification, maximum aortic diameter, maximum IMH thickness, focal contrast enhancement (including ulcerlike projection and intramural blood pool), and pleural or pericardial effusion. Medical (nonsurgical) versus surgical treatment strategies depend primarily on the Stanford classification, although more recent studies of Asian cohorts report success of initial medical treatment in patients with Stanford type A IMH, with timed (delayed) surgery for patients who develop complications. Understanding the imaging appearance and prognostic factors of IMH helps the radiologist and surgeon identify patients at greatest risk for complications to ensure appropriate treatment and improve patient outcomes. (©)RSNA, 2016.

Early Stage of Hyperintense Acute Reperfusion Marker on Contrast-Enhanced FLAIR Images in Patients With Acute Stroke.

Hyperintense acute reperfusion marker (HARM) is defined as delayed CSF enhancement on FLAIR images in patients with acute stroke and was observed in follow-up FLAIR images after initial MRI with contrast material administration. We hypothesized that different imaging findings of HARM could be present depending on the timing of FLAIR imaging after contrast material administration. Of 218 consecutive patients with acute stroke or transient ischemic attack over 3 months, 12 with linear contrast enhancement on the cortical surface in initial FLAIR images underwent serial FLAIR imaging. Initial FLAIR images were obtained 5 minutes after contrast material administration, and follow-up unenhanced FLAIR images were obtained 2.5-29 hours (mean, 13.2 hours) after initial FLAIR imaging. The enhancement patterns between initial and follow-up FLAIR images were compared. In all 12 patients, initial contrast-enhanced FLAIR images showed focal or multifocal linear contrast enhancement along the cortical surface near acute infarctions. On follow-up unenhanced FLAIR images, initial cortical enhancement spread diffusely and filled the subarachnoid space. HARM in acute stroke can be detected earlier than previously reported on initial contrast-enhanced FLAIR images. Different imaging findings of HARM depend on the timing of FLAIR imaging after contrast material administration.

Giant Extrapleural Solitary Fibrous Tumor of the Thigh.

Giant Extrapleural Solitary Fibrous Tumor of the Thigh.

Longitudinally extensive transverse myelitis: a rare association with common variable immunodeficiency

Transverse myelitis requires careful investigation, as although many causes respond acutely to immunomodulation, the longer term management depends upon its precise cause. We describe a patient presenting acutely with a...