SMART Syndrome: A Case Report

Introduction: Stroke-like migraine attacks after radiation therapy (SMART) syndrome, is a late complication of brain radiotherapy. (1) Symptoms are commonly subacute in onset and involve migraine type of headache, seizures, focal neurologic deficits.(2) . MRI findings are usually unilateral and posterior predominant cortical-subcortical hyperintensity, swelling and prominent gyriform (cortical and leptomeningeal) gadolinum enhancement in the areas of brain that underwent irradiation with or without diffusion restriction.(1) There is no standard treatment protocol of SMART syndrome. Antiepileptics and corticosteroids are commonly used drugs.Case Report: A 65 years old woman diagnosed with breast cancer with brain metastases and treated with more than 50 Gy brain radiotherapy. A couple of months later patient presented with acute right sided weakness and numbness, episodic myoclonic jerking of the right arm and leg and gait instability. MRI and magnetic resonance angiography of the brain with gadolinium revealed left parietooccipital cortical diffusion restriction and accompanying dilatation of the left posterior cerebral artery as new findings. Computed tomography perfusion revealed increased perfussion in the affected area. The patient was diagnosed with SMART syndrome.Management & Outcome: The patient was treated with dexamethasone (16 mg/day) and anticonvulsant therapy. Myoclonic seizures had almost completely remitted. However, her cognitive impairment persisted, then the patient arrested because of aspiration a month later.Discussion: Besides confirming SMART syndrome, diagnostic investigations is also important to exclude other etiologies. Posterior reversible encephalopathy syndrome, post-ictal changes, meningoencephalitis and cerebrovascular diseases are radiological differential diagnosis which should be considered.(3) Proper and early diagnosis of SMART syndrome is significant to refrain unneccessary aggressive approaches and for appropriate treatment to prevent sequela lesions.

5-Fluorouracil Neurotoxicity in the Absence of Dihydropyrimidine Dehydrogenase Deficiency Case Report

5 fluorouracil (5-FU)-related neurotoxicity is a rare and severe complication of 5-FU administration. Dihydropyrimidine dehydrogenase (DPD) deficiency is associated with an increased risk of serious adverse reactions due to its role in 5-FU metabolism. We report a case of acute reversible neurotoxicity with global areas of diffusion restriction in a patient with colorectal adenocarcinoma being treated with leucovorin calcium, 5-fluorouracil, and oxaliplatin (FOLFOX) without DPD deficiency following uridine triacetate administration.

Atypical pituitary abscess lacking rim enhancement and diffusion restriction with an unusual organism, Moraxella catarrhalis: A case report and review of the literature

Background: Pituitary abscess (PA) can be fatal if diagnosed late. Rim enhancement is a typical radiological finding of PA on postgadolinium T1-weighted magnetic resonance imaging (MRI). Diffusion-weighted imaging is helpful in distinguishing PA from other sellar cystic lesions. Herein, we report the first atypical case of PA showing neither rim enhancement nor diffusion restriction with an unusual organism, Moraxella catarrhalis. Case Description: A 77-year-old woman presented with headache, polyuria, polydipsia, and fatigue for a month before presenting to a local hospital. MRI showed pituitary enlargement with contrast enhancement. She had neither fever nor visual deficits and was followed up with hormonal replacement. Six months later, she complained of visual impairment, and MRI showed further pituitary enlargement with a thickened stalk compressing the optic chiasma. Neither rim enhancement nor diffusion restriction was observed. Endoscopic endonasal transsphenoidal surgery was performed based on the radiological diagnosis of lymphocytic hypophysitis or pituitary tumors. A thick, creamy yellow pus was drained from the sellar lesion. Intraoperative rapid histopathological findings revealed polymorphonuclear leukocytes infiltrating the pituitary gland. PA was diagnosed, and irrigation and open drainage of the abscess was performed. Bacterial culture of the pus detected M. catarrhalis by mass spectrometer, confirming the diagnosis. She underwent appropriate antibiotic administration, and her visual deficits improved. Conclusion: We report the first atypical case of PA showing neither rim enhancement nor diffusion restriction with M. catarrhalis. Even if preoperative findings are not suggestive of PA, it should be considered as a differential diagnosis. Intraoperative rapid histopathological findings are useful for accurately diagnosing PA and initiating appropriate surgical treatment.

DIAGNOSTIC ROLE OF DIFFUSION-WEIGHTED IMAGE OF THE LIVER WITH MAGNETIC RESONANCE IMAGING IN PREDICTING ABSTINENCE DISORDERS IN PATIENTS WITH ALCOHOLIC LIVER DISEASE

Objective. To assess the diagnostic role of diffusion-weighted images of the liver with magnetic resonance imaging in predicting abstinence disorders in patients with alcoholic liver disease. Methods. A total of 122 patients with ALD aged 48±5.4 years were examined. The survey algorithm we used included: performing liver DWI with MRI (n=122) with b-value values of 100/600/1000, ultrasound of abdominal organs with clinical elastography – 97 (80%) patients. Trepan liver biopsy was chosen as a reference method (n=64). Results. The patients were monitored for 2.5 years. The terms of follow-up were selected individually, depending on the results of clinical and laboratory research methods. A high correlation was established (r=0.879), when comparing clinical elastography and quantitative indicators of DWI of the liver, at admission and during dynamic observation of patients, also at the middle level, the data obtained correlated with the results of trephine biopsy of the liver (r=0.721). After 3 months, 6 (15%) of 40 patients showed normalization of biochemical blood test parameters with no diffusion restriction according to the results of DWI of the liver. Based on the results obtained, a high correlation was noted between changes in the biochemical blood test and MRI data in the DWI mode. After 9 months of follow-up, according to DWI data, 34 patients showed persistence of cytolysis syndrome and limited diffusion on DWI of the liver. After collecting an additional history and clarifying the details of the lifestyle of the patients' relatives, it was found that these patients continued to consume alcoholic beverages against the background of the received treatment, which was manifested by the presence of diffusion restriction on MRI in the DWI mode, which was a magnetic resonance sign of the presence of inflammatory processes in the structure of the parenchyma liver. After 12 months, positive dynamics – the absence of diffusion restriction according to the results of DWI of the liver was noted in 34 patients, which indicates the effectiveness of using the qualitative characteristics of DWI of the liver to assess the violation of the abstinence regimen (AUROC=0.906 (95% CI 0.872-0.916)). But in 16 (13%) patients from this group, changes in the biochemical blood test were noted, but no diffusion limitation was noted according to the DWI of the liver. Patients (n=16) underwent a correction of the received treatment – after 1 month there was a positive trend. There was a correlation of quantitative parameters of DWI of the liver with clinical forms of ALD, regardless of the presence or absence of diffusion restriction (r=0.936). Next, we assessed the prognostic and diagnostic significance of the developed criteria for DWI of the liver for patients with ALD on admission. The results of the study indicated the effectiveness of using the diagnostic and prognostic model of MRI in the DWI mode for patients with ALD on admission and in dynamic observation. Conclusions. 1. A high correlation was found between the quantitative parameters of DWI of the liver and clinical elastography (r=0.879) at admission and follow-up. Average correlation relationship of DWI of the liver with the results of trephine biopsy of the liver in patients with ALD on admission and follow-up (r=0.721). There was a high correlation between the results of DWI of the liver on MRI with the data of clinical and laboratory parameters in dynamic observation of patients with ALD: no diffusion limitation – positive (r=0.887); yes – negative (r=0.887). The high prognostic and diagnostic value of DWI of the liver in assessing the violation of the abstinence regimen in patients with ALD was established (AUROC=0.906 (95% CI 0.872-0.916)). Prognostic and diagnostic criteria for liver DWI on MRI in patients with ALD at admission: qualitative characteristic – AUROC=0.846 (95% CI 0.811-0.862), quantitative characteristic – AUROC=0.909 (95% CI 0.879-0.912); with dynamic observation: qualitative characteristic – AUROC=0.949 (95% CI 0.907-0.965), quantitative characteristic – AUROC=0.917 (95% CI 0.876-0.932).

Sneddon’s Syndrome as rare and underdiagnosed cause of stroke: about two cases / A Síndrome de Sneddon como causa rara e subdiagnosticada de AVC: cerca de dois casos

Case 1. Female, 27 years old, with migraine with aura. Use of combined contraceptive therapy. She presented visual and cutaneous complaints for eight months, using prednisone 60mg/day due to the hypothesis of systemic vasculitis. On examination, right temporal hemianopsia, normal fundus of the eye, Raynaud ‘s phenomenon with necrosis stitches in fingers and livedo were noted. Brain MRI with diffusion restriction in the left occipital lobe, hypersignal foci in the parietal lobe, and head of the left caudate nucleus. Reticular Livedo (RL) compatible biopsy. Anticardiolipin IgM (13.3 U/mL), ESR (47mm/h in the 1st hour and 52mm/h in the 2nd hour), and VDRL 1:8 reagents. Normal CSF. Initially treated with warfarin, she has been using rivaroxaban for two years, remaining asymptomatic. Case 2. Female, 39 years old, with a report of sudden dysarthria and right hemiparesis, with a previous episode of vertigo. A lumbosacral spine MRI was performed, presenting no alterations. Normal CSF. Antiphospholipid antibodies: Beta-2-glycoprotein I IgM 21 UR/mL and IgG 9 UR/mL, lupus anticoagulant 0.98, anticardiolipin IgG 9.4 GPL and IgM 31 MPL. Cranial MRI showed old ischemic gaps and hyperintense areas on T2/FLAIR with diffusion restriction affecting the left paramedian portion of the pons, related to acute stroke. Presence of livedo in the distal region of the limbs. In current use of warfarin 7.5mg/day, presenting remission of symptoms. Discussion: Sneddon Syndrome (SS) is a non-inflammatory thrombotic vasculopathy characterized by the concomitant presence of livedo reticularis and stroke. Neurological manifestations are present in up to 77% of cases. It is more prevalent in women between 20 and 42 years old. Treatment is aimed at preventing potentially serious, disabling, and even fatal new vascular events. Final Comments: SS is a rare syndrome that probably results from a series of acquired or congenital hemostatic abnormalities that preferentially involve the cutaneous and cerebral vascular beds. Although it has no specific biomarker, there are reports associating SS with antiphospholipid antibodies and VDRL reactor. Clinical presentation, laboratory findings and skin biopsy associated with an excellent response to anticoagulant treatment were fundamental in the diagnostic elucidation.

Giant tumefactive perivascular spaces in a pediatric patient: A rare radiological entity

Background: Giant tumefactive perivascular spaces (TPVS) are radiological rarities and may mimic other neurological structural lesions. Fewer than 80 cases have been reported in the literature with even fewer in the pediatric population. Case Description: The authors present an image report showcasing a 3-year-old boy presenting with uncontrolled seizures despite multiple anti-epileptic medications. His magnetic resonance imaging showed multiple, non-contrast enhancing cyst clusters within the left parieto-occipital region that was hyperintense on T2-weighted imaging, and isointense to cerebrospinal fluid. Due to a characteristic absence of perilesional edema seen on fluid-attenuated inversion recovery imaging or diffusion restriction on diffusion-weighted imaging (DWI) sequences, this was diagnosed as a giant TPVS. Conclusion: Accurate diagnosis of these rare radiological entities is based on pathognomonic findings that can help prevent unnecessary surgery and guide management for patients, particularly in the pediatric population as seen in our case.

USE OF RADIOMIC MARKERS IN PREOPERATIVE DETECTION OF EXTRA-AXIAL TUMOR GRADE

Backgraund: Extra-axial tumors are one of the tumor groups which are difficult for primary differential diagnostics. Detection and standardization of radiomic markers is one of the main problems of our time. Aim: To detect radiomic markers for preoperative assessment of extra-axial tumor grade. Materials and methods: Retrospective analysis of MRI (1.5T) data of 156 patients with extra-axial tumors. The patients were divided into 2 groups: Group 1(n=106) with perifocal changes, Group 2 (n=50) extra-axial tumor without perifocal changes. Diffusion and perfusion sequences were included in the scanning protocol. The areas of interest were (1) the lesion and (2) the area of perifocal changes. Measurements were made from the lesion and the area of perifocal changes on ACD and DSE maps, DCE was analyzed. Results: The maximum lesion size in Group 1 was 2.2cm (1.4; 4.3), in Group 2 - 1.2cm (0.9; 3.5). In Group 1 diffusion restriction from the lesion was detected in 42 patients (39.6%), in Group 2 in 7 patients (14%). The maximum size of perifocal changes in Group 1 was 2.85cm (1.5; 4.7). Diffusion restriction was detected in 52 cases (49.1%). In patients of Group 2 with verified meningioma multivariate linear regression analysis showed that the maximum size of the lesion demonstrated a 3.3-time increase of rCBF from the area of perifocal changes (coef. 3.3 CI 1.27; 5.28) p = 0.003, however, it demonstrated a 4-time decrease of rCBF (coef. 4 CI -7.46;-0.71) p = 0.02. Conclusions: Perfusion and diffusion method combined with anatomical sequences show potential and can be used as radiomic markers for diagnostic assessment and treatment of extra-axial tumors. There is further potential in detecting radiomic functional markers from the area of perifocal changes.

NIMG-69. FREQUENCY OF TUMOR-RELATED VASCULATURE IN PRIMARY CNS LYMPHOMA ON ROUTINE MRI

Primary central nervous system lymphoma (PCNSL) is known to express angiogenic factors and can have spontaneous or biopsy-related hemorrhage. Radiographically, PCNSL is characterized by homogenous enhancement and diffusion restriction, as well as T2/FLAIR abnormalities. A higher frequency of tumor-related vessels has been reported in gliomas compared to brain metastases, but this has not been assessed in PCNSL. We used a clinical database of patients with PCNSL treated at the University of Washington and reviewed pretreatment MRIs for 48 patients treated between 2014-2021 for whom susceptibility sequences were available. Median age at diagnosis was 64 years (range 35-90 years). Patients were 27.1% male and 72.9% female. Each lesion was scored by at least two independent reviewers for the presence of a prominent vessel located either centrally or eccentrically, and for the presence of hemorrhage. Lesions that were scored differently by the two reviewers (n=26) were then scored by a third independent reviewer. In several cases the findings were felt to be indeterminate. Lesions were considered measurable if they were greater than 5 mm in two perpendicular planes, and a total of 93 measurable lesions were categorized. Prominent vessels were seen in 56/93 (60.2%) lesions, with 26/56 (46.4%) being central and 30/56 (55.4%) being eccentric. An additional 7/93 (7.5%) lesions were indeterminate. Hemorrhage was seen in 11/93 (11.8%) lesions. We conclude that prominent vessels can be seen in a majority of PCNSL lesions on standard MRI.