Differential Diagnosis of Behavioral Variant and Semantic Variant of Frontotemporal Dementia Using Visual Rating Scales.

BACKGROUND: Frontotemporal dementia (FTD) represents the second most frequent early onset of dementia in people younger than 65 years. The main syndromes encompassed by the term FTD are behavioral variant of Frontotemporal dementia (bvFTD), non-fluent variant primary progressive aphasia (nfvPPA) and semantic variant (SD). AIMS: To assess the bvFTD and SD, which represent the most common subtypes of FTD, using visual rating scales. METHODS: Brain MRI exams of 77 patients either with bvFTD (n=43) or SD (n=34) were evaluated. The rating scales used were: Global cortical atrophy (GCA), Fazekas Scale: periventricular (PV) and white matter (WM) changes, Koedam rating scale and visual scales regarding specific cortical regions: dorsofrontal (DF), orbitofrontal (OF), anterior cingulate (AC), basal ganglia (BG), anterior- temporal (AT), insula, lateral-temporal (LT), entorhinal (ERC), perirhinal (PRC), anterior fusiform( AF), anterior hippocampus (AHIP) and posterior hippocampus (PHIP). Both Left (L) and Right (R) hemispheres were evaluated. RESULTS: R-OF (p=0.059), L-OF (p<0.0005), L-AT (p=0.047) and L-AHIP (p=0.007) have a statistically significant effect on the variable occurrence of SD compared to bvFTD. The indicators with the highest value of the area under the curve (AUC) were R-AC (0.829), L-OF (0.808), L-AC (0.791) and L-AF (0.778). Highest sensitivity was achieved by R-OF (97%) and L-AF (75%). Highest specificity was achieved by L-OF (95%), L-AT (91%) followed by R-AC (84%). Best combination of sensitivity and specificity was achieved by L-AF (74%-79%), L-OF (56%-95%) and R-OF (97%-42%). Best combination of PPV and NPV was achieved by L-OF (90%-73%), LAT (83%-72%) and R-AC (77%-77%). CONCLUSION: Visual rating scales can be a practical diagnostic tool in the characterization of patterns of atrophy in FTLD and may be used as an alternative to highly technical methods of quantification.

Eosinophilic Cholangitis--A Challenging Diagnosis of Benign Biliary Stricture: A Case Report.

When confronting a biliary stricture, both benign and malignant etiologies must be carefully considered as a variety of benign biliary strictures can masquerade as hilar cholangiocarcinoma (CCA). Therefore, patients could undergo a major surgery despite the possibility of a benign biliary disease. Approximately 15% to 24% of patients undergoing surgical resection for suspected biliary malignancy will have benign pathology. Eosinophilic cholangitis (EC) is a rare benign disorder of the biliary tract, which can cause obstructive jaundice and can pose a difficult diagnostic task. We present a rare case of a young woman who was referred to our hospital with obstructive painless jaundice due to a biliary stricture at the confluence of the hepatic bile ducts, with a provisional diagnosis of cholangiocarcinoma. Though, during her work up she was found to have EC, an extremely rare benign cause of biliary stricture, which is characterized by a dense eosinophilic infiltration of the biliary tree causing stricturing, fibrosis, and obstruction and which is reversible with short-term high-dose steroids. Despite its rarity, EC should be taken into consideration when imaging modalities demonstrate a biliary stricture, especially if preoperative diagnosis of malignancy cannot be made, in the setting of peripheral eosinophilia and the absence of cardinal symptoms of malignancy.

Immune reconstitution inflammatory syndrome mimicking progressive multifocal leucoencephalopathy in a multiple sclerosis patient treated with natalizumab: a case report and review of the literature.

Natalizumab (NTM) represents an effective drug for the treatment of relapsing-remitting multiple sclerosis (RRMS). Progressive multifocal leucoencephalopathy (PML) is a potential life-threatening complication of NTM treatment. A close follow-up and MRI monitoring of patients under NTM are required to avoid such devastating complications. The case of a 47-year-old woman with RRMS (EDSS 1.5) treated with NTM for 44 months is reported. The patient had a relapse with mild cerebellar symptomatology and visual complaints. MRI revealed a new area of abnormal signal intensity in the subcortical white matter of the right parietal lobe with mild peripheral enhancement. Visual fields showed scotomata mostly of the left eye. NTM was discontinued. JC virus (JCV) polymerase chain reaction (PCR) in cerebrospinal fluid was negative. The patient received IV corticosteroids for 5 days and then monthly for 2 months with subsequent clinical and MRI improvement. On month 4, she presented with a new relapse with severe ataxia, mild behavioral change, increase of cerebellar symptoms and internuclear opthalmoplegia (EDSS 3.5). MRI showed reappearance of the right parietal lobe lesion, with decreased size and less pronounced contrast enhancement. A new 2-cm lesion was noted in the left cerebellar hemisphere with a speckled pattern of contrast enhancement. JCV PCR was negative and the patient was treated with IV corticosteroids. On month 12, she demonstrated clinical and MRI improvement. Although initially PML was highly suspected in this patient, the clinical and MRI findings were supportive of the presence of immune reconstitution inflammatory syndrome (IRIS).

Complete traumatic main pancreatic duct disruption treated endoscopically: a case report.

INTRODUCTION: Pancreatic injury is uncommon and the management remains controversial. The integrity of the main pancreatic duct is considered the most important determinant for prognosis. CASE PRESENTATION: A 19-year-old Greek man was referred to our tertiary referral centre due to blunt abdominal trauma and an associated grade III pancreatic injury. He was haemodynamically stable and his initial treatment was conservative. Due to deterioration in his clinical symptomatology he underwent an endoscopy 20 days postinjury, where a stent was placed in the proximal pancreatic duct remnant and a bulging fluid collection of the lesser sac was drained transgastrically. He made an uneventful recovery and remains well 7 months postinjury, but a stricture with upstream dilatation of his main pancreatic duct has developed. CONCLUSIONS: The clinical status of the patient rather than the grade of pancreatic injury should be the principal determinant to guide treatment. Endoscopic stenting and drainage is an attractive minimally invasive procedure and it may obviate the need for surgery. However, further investigation is required regarding the safety and outcome.

The Role of Virtual Cystoscopy, after Multidetector Computed Tomography Imaging Reconstruction without the Use of Contrast Medium, in the Diagnosis and Evaluations of Bladder Tumors: Preliminary Study.

Introduction. Although conventional cystoscopy is considered to be the gold standard for diagnosis and follow-up of bladder tumors, it remains an invasive and costly procedure. With the advent of the multidetector CT (MDCT) scanners supported by specialized software virtual cystoscopy (VC) is possible. We assess the role of VC in diagnosing and evaluating bladder lesions. Materials and Methods. Between September 2010 and October 2011, 25 consecutive patients with cystoscopically confirmed bladder tumor underwent VC. The radiologists involved in this prospective study were blinded to the exact findings. After draining any residual urine with a catheter, the bladder was retrogradely insufflated with 200-600 cc of air. No intravenous or intravesical contrast was used. MDCT scan was performed in supine and prone positions and three-dimensional reconstruction of the urinary bladder was performed. Results. The examination was well tolerated by all patients with no complications. In total, 43 lesions were detected both with conventional cystoscopy and VC. Tumor size measured by CT ranged from 3 to 80 mm in diameter. The pathological report revealed noninvasive transitional cell carcinomas in all cases. Conclusion. VC has promising results in detecting exophytic bladder lesions. In the future it could be part of the diagnostic algorithm for bladder tumors.

A multimodal approach to acute biliary pancreatitis during pregnancy: a case series.

The treatment of acute biliary pancreatitis during pregnancy remains controversial. We present our experience of treating 7 pregnant women with acute biliary pancreatitis and verified or suspected choledocholithiasis, by using magnetic resonance cholangiopancreatography (MRCP), endoscopic retrograde cholangiopancreatography (ERCP), and sphincterotomy followed by laparoscopic cholecystectomy. MRCP was performed in all patients to confirm the presence of common bile duct stones, their size and number. ERCP and sphincterotomy were performed without the use of radiation. The procedure was terminated only when all stones (the number clarified at MRCP), were retrieved into the duodenum. All patients underwent laparoscopic cholecystectomy the following day. Neither post-ERCP nor postoperative major complications were noted. All but one patient reached a healthy natural-term labor. One patient had a planned cesarean section on 35th week. The combination of MRCP, nonradiation ERCP, and immediate laparoscopic cholecystectomy provides definite treatment and seems to put both mother and fetus at lower risk than presumed.

Diffuse pattern of bone marrow involvement on magnetic resonance imaging is associated with high risk cytogenetics and poor outcome in newly diagnosed, symptomatic patients with multiple myeloma: a single center experience on 228 patients.

Magnetic Resonance Imaging (MRI) and specific cytogenetic abnormalities offer important prognostic information for myeloma patients. However, limited data are available about the association between cytogenetic abnormalities and MRI patterns of marrow infiltration. To address this issue, we analyzed 228 consecutive newly diagnosed, symptomatic patients who were diagnosed and treated in a single center. On bone marrow MR images, 95 (41%) patients had diffuse, 94 (41%) had focal, 35 (15%) were normal, and 4 (1.7%) patients had variegated pattern of marrow infiltration. High risk cytogenetics were more commonly observed with diffuse MRI pattern (50% vs. 31% in focal and normal patterns). Patients with diffuse MRI pattern had poorer survival compared to others and responded better to novel agent-based therapies than to conventional chemotherapy (objective response: 88% vs. 46%, P < 0.001). There was a significant improvement of patients' survival with a diffuse MRI pattern when treated upfront with novel agents compared to conventional chemotherapy (47 vs. 24 months; P < 0.001). Diffuse MRI pattern along with ISS-3 and high risk cytogenetics could identify a very high risk group of patients with extremely poor median survival (21 months) and an only 35% probability of 3-year OS. Our study shows that symptomatic myeloma patients with a diffuse MRI pattern at diagnosis very often show high risk cytogenetic abnormalities and are benefiting from upfront novel agent-based therapies. Diffuse MRI pattern in combination with high risk cytogenetics and ISS-3 can identify a subset of myeloma patients with very poor prognosis who may need innovative treatment strategies and possibly more aggressive therapies.

Plain X-ray, computed tomography and magnetic resonance imaging findings of telangiectatic osteosarcoma: a case report.

An 18-year-old male patient presented with chronic nonspecific pain of three months located at his left proximal tibia. The patient was admitted to our department for plain X-ray, computed tomography and magnetic resonance imaging examination. Plain X-ray and computed tomography revealed a geographic lytic lesion at the medial aspect of the proximal tibia. Biopsy of the lesion showed telangiectatic osteosarcoma. Image findings of all modalities are presented.

An atypical case of noninfected iliopsoas bursitis - MRI findings.

The authors present an unusual case of atypical iliopsoas bursitis in an 81 years-old female patient. The patient asked for medical advice due to a chronic left hip and lower abdominal pain with no specific characteristics. Physical examination revealed a palpable left-sided pelvic mass. Ultrasound examination demonstrated a purely cystic mass that was presumed to be an ovarian cystic malignancy. The patient was referred for an MRI examination, which showed a cystic lesion extending from the left iliac crest to the anterior aspect of the ipsilateral hip joint (maximum diameter 18 cm), coursing below the iliopsoas tendon indicative of an extremely distended iliopsoas bursa.

Endovascular treatment of a vertebral artery pseudoaneurysm in a drug user.

A 26-year-old drug abuser who presented with sepsis was found to have a pseudoaneurysm in the left vertebral artery. This aneurysm was presumed to be post-traumatic, since the patient reported multiple attempts to inject drugs in the left jugular vein 15 days prior to admission. The pseudoaneurysm was treated effectively with stent-graft placement.