SPECTRUM OF CLINICAL AND FDG-PET PATTERNS OF PATIENTS WITH FRONTOTEMPORAL DEMENTIA (FTD) IN A TERTIARY CARE HOSPITAL

Introduction. Frontotemporal dementia (FTD) is a common cause of cognitive impairment, behavioral changes and language deficits. in this study we assessed the clinical and FDG-PET characteristics of our patients and compared them with available international and indian data on FTD. Methods. All patients were evaluated with a neuropsychological battery followed by 18F-FDG-PET scan, in addition to all necessary dementia work-up. Results. 15 FTD patients (m:f = 11:4) had a mean age of presentation of 67.4 (8.6) years, with a mean interval of 1.7 (0.7) years from the symptom onset to diagnosis. those with 10 or less years of education had an earlier presentation. a positive family history was present in 20%. The mean MMSE score was 18.8 (4.6). Disinhibition was the most common symptom seen in 60%. Apathy was less frequently noted. FDG-PET scan showed predominantly anterior cingulate and anterior temporal hypometabolism, with asymmetry in 67%. 2 cases diagnosed as fvAD had a revised diagnosis of bvFTD after FDG-PET scan. 1 patient of nfvPPA was mute at presentation with a history of agrammatism with hypometabolism in left inferior frontal and superior temporal regions. Conclusions. There were significant variations compared to international/Western literature, with later age of presentation, shorter interval from onset to presentation, lower MMSE scores, with disinhibition rather than apathy as the commonest symptom. FDG-PET showed similar areas of involvement but with less extensive hypometabolism compared to other studies with a lesser frequency of asymmetry. FDG-PET scan is a useful adjunct for evaluation of FTD patients.

Clinical, Imaging, and Pathologic Characteristics of Patients With Right Versus Left Hemisphere-Predominant Logopenic Progressive Aphasia

Objective:To assess and compare demographic, clinical, neuroimaging and pathologic characteristics of a cohort of patients with right versus left hemisphere-predominant logopenic progressive aphasia (LPA).Methods:This is a case-control study of patients with LPA who were prospectively followed at Mayo Clinic and underwent an [18F]-fluorodeoxyglucose (FDG)-PET scan. Patients were classified as rLPA if right temporal lobe metabolism was ≥1 standard deviation lower than left temporal lobe metabolism. Patients with rLPA were frequency-matched 3:1 to typical left-predominant LPA based on degree of asymmetry and severity of temporal lobe metabolism. Patients were compared on clinical, imaging (MRI, FDG-PET, amyloid-beta- and tau-PET) and pathologic characteristics.Results:Of 103 prospectively recruited LPA patients, 8 (4 females) were classified as rLPA (7.8%); all rLPA cases were right-handed. rLPA patients had milder aphasia based on the Western Aphasia Battery-Aphasia Quotient (p=0.04) and less frequent phonologic errors (p=0.015). rLPA had shorter survival compared to typical LPA: hazard ratio 4.0(1.2- 12.9), p=0.02. There were no other differences in demographics, handedness, genetics, neurological or neuropsychological tests. Compared to the 24 frequency-matched typical LPA patients, rLPA showed greater frontotemporal hypometabolism of the non-dominant hemisphere on FDG-PET and less atrophy in amygdala and hippocampus of the dominant hemisphere. Autopsy evaluation revealed a similar distribution of pathologic findings in both groups, with Alzheimer’s disease pathologic changes being the most frequent pathology.Conclusions:Right LPA is associated with less severe aphasia but has shorter survival from reported symptom onset than typical LPA, possibly related to greater involvement of the non-dominant hemisphere.

Obsessive compulsive disorder: a case of extreme obsessional slowness in an 18-year-old presenting to the national OCD unit

ObjectiveObsessional slowness in OCD is a rare phenomenon on which there is minimal published literature. This is a particularly severe and atypical case of early onset OCD with extreme obsessional slowness and mutism. To the best of our knowledge, there have been no reports of similar severity published in this age group. This report seeks to provide discussion of important organic causes that may need to be considered as well as information on treatment approach.Case reportAn 18-year-old male was admitted to the National OCD Unit, Springfield Hospital with a history of autism and normal development until the age of 14, after which symptoms of OCD with fear of contamination emerged, followed by progressive motor slowness and mutism.Due to the severity of OCD and self-neglect he had two previous admissions to CAMHS wards and required a course of ECT to treat catatonic symptoms age 17.Pharmacological treatment has included Aripiprazole 5 mg and Fluoxetine 60 mg, which the patient was taking at admission. The latter was subsequently switched to Sertraline 250 mg and Aripiprazole increased. As it was hypothesized that his obsessional slowness stemmed from severe levels of anxiety, Buspirone was also added.Therapy has been intensive, although communication difficulties have made targeting specific fears challenging as the exact nature of the intrusive thoughts remains unclear.DiscussionFollowing combined neurology and neuropsychiatry review, the patient spent four weeks in a general hospital for further investigation as it was initially felt an organic cause was likely. Initial differentials included Juvenile Onset Parkinson's or Wilson's disease. Both were subsequently ruled out and despite multiple investigations, no obvious organic cause was found. A markedly abnormal FDG PET scan showed findings usually seen in advanced dementia, but not necessarily clinically correlating to his current presentation.The OCD unit have continued to provide intensive input and tailored treatment programme, encouraging actions against any rules he has in place. Prompting and pacing, verbal exercises and regular stretching exercises due to stooped posture which he attributed to needing to obey certain rules have been used.ConclusionIt is important for clinicians to be aware of obsessional slowness in OCD and this report highlights a particularly rare and severe example in a young adult who has been difficult to treat. Organic causes may need to be considered and MDT approach to treatment is essential.

Eccrine Porocarcinoma: A Challenging Diagnostic and Therapeutic Tumoral Entity

Eccrine porocarcinoma is a rare malignant cutaneous tumor with high rates of extracutaneous spread, and its diagnosis and management can be quite challenging. This is a case of an 82-year-old woman presenting with an asymptomatic and chronic pubic skin lesion for whom the work-up required many investigations and procedures to confirm the diagnosis of metastatic eccrine porocarcinoma. Indeed, the patient underwent a wide local excision of the skin lesion, imaging with an FDG-PET scan, a colonoscopy, and two inguinal node dissections. As illustrated in this case, surgery should always be considered to achieve disease remission. Other treatments such as chemotherapy and radiotherapy have also been reported in the literature without clear standard guidelines.

Amahrelis : Adcetris Maintenance after Autologous Stem Cell Transplantation in Hodgkin Lymphoma : A Real Life Study from Sfgmtc and Lysa Groups

Background : AETHERA randomized controlled study (Moskowitz, Lancet 2015) showed that the administration of Brentuximab Vedotin (BV) maintenance after autologous stem cell transplantation (ASCT) improved progression free survival (PFS) in BV-naive refractory/relapsed (R/R) Hodgkin lymphoma (HL) patients. However, since BV approval for R/R HL in 2012, many patients are receiving salvage BV before ASCT, alone or combined with chemotherapy. In the AMAHRELIS retrospective nationwide French study, we investigated the real-life outcome of patients with R/R HL mostly treated with BV-based salvage therapies and who received post-ASCT BV maintenance. Objectives : As primary 0objective, we assessed 2 years-PFS of patients treated with post-ASCT BV maintenance from 2012 to 2017 in France. As secondary objectives, we correlated variables (such as use of salvage BV before ASCT, centrally reviewed TEP-assessed response) with survival, and evaluated reported tolerance of BV using the CTCAE v4.0 criteria. Methods : We conducted this observational retrospective study in 58 national centers. Inclusion criteria were R/R HL patients who received at least two infusions of BV after ASCT, at the exclusion of patients in progression after transplant. Among 1134 patients who underwent ASCT for R/R HL between 2012 and 2017 in France based on the French society of bone marrow transplantation database, 835 (74%) patients were screened and data were available for 794 (70%) patients. FDG-PET scan at relapse and before transplantation were recorded and centrally reviewed (still ongoing). FDG-PET scan were reported using the Deauville score (DS), and complete remission was defined by a DS < 4. Post-transplant status was evaluated based on CT-scan or on FDG-PET scan results. This study was approved by the SFGMTC and the LYSA. Results : Fifteen percent (115/794) of patients met eligibility criteria, and were enrolled in this study. Among them, 95% met inclusion criteria for BV maintenance according to the AETHERA study as primary refractory disease (43%), early relapse (27%) or extranodal disease (49%). The mean number of BV injections after ASCT was 11 (3-18). Patients characteristics were : mean age was 34 y (range between 16-70y), 54% were male, and 57% of patients were stage III or IV at relapse. Notably, 70% of patients received BV as salvage therapy and 81% achieved a complete remission before ASCT. The median follow-up period was 35 months. The 2 years survival for the whole cohort was 75,3% for PFS (95% CI : 68,4-84,3) and 96,4% for overall survival (95% CI : 94,2-100) (Figure 1). The use of BV as part of salvage therapy before transplant had no impact on PFS. We observed a trend to an increased occurrence of neuropathy in patients receiving BV before transplant without impact on early treatment discontinuation. We tested several variables for correlation with survival using a univariate Cox model including high risk patients defined as primary refractory disease or early relapse and disseminated disease, extranodal relapse, use of BV before transplant, number of salvage lines, remission status before and after transplant (complete response versus partial response or stable disease), time between ASCT and BV onset and number of BV cycles after transplant. Among these variables, high risk status, less than 10 post transplant BV cycles and absence of post transplant complete remission significantly correlated with a reduced survival probability, and remained significant after analysis using a multivariate Cox model (Table 1). Conclusion : From the real-life AMAHRELIS study, we confirmed the very good outcome of R/R HL patients in the era of post-transplant BV maintenance, with a 2y-PFS of 75% similar to the results of the AETHERA landmark study (2y-PFS of 63%). The results of current strategies with pre- and post-transplant BV outperformed historical series based on high dose therapies, including those of tandem transplant for high risk patients. Future studies incorporating BV strategies with immune checkpoint inhibitors might improve outcome in R/R HL patients. Disclosures Meignan: ROCHE: Consultancy, Honoraria, Other: TRAVEL, ACCOMMODATIONS, EXPENSES (paid by any for-profit health care company). Sibon:takeda france: Consultancy. Stamatoulas Bastard:Pfizer: Other: TRAVEL, ACCOMMODATIONS, EXPENSES; Celgene: Honoraria; Takeda: Consultancy. Fornecker:Takeda: Consultancy; Roche: Consultancy. Casasnovas:Takeda: Consultancy, Honoraria, Other: TRAVEL, ACCOMMODATIONS, EXPENSES (paid by any for-profit health care company), Research Funding; Roche: Consultancy, Honoraria, Other: travel, accomodations, expenses, Research Funding; Gilead: Consultancy, Honoraria, Other: TRAVEL, ACCOMMODATIONS, EXPENSES (paid by any for-profit health care company), Research Funding; Abbvie: Consultancy, Honoraria; MSD: Consultancy, Honoraria; Amgen: Consultancy, Honoraria. Cartron:Gilead: Honoraria; Jansen: Honoraria; Abbvie: Honoraria; Celgene: Consultancy, Honoraria; Sanofi: Honoraria; F. Hoffmann-La Roche: Consultancy, Honoraria. Ghesquieres:Gilead: Consultancy, Honoraria, Other: TRAVEL, ACCOMMODATIONS, EXPENSES; CELGENE: Consultancy, Other: TRAVEL, ACCOMMODATIONS, EXPENSES; Roche: Consultancy, Other: TRAVEL, ACCOMMODATIONS, EXPENSES; Janssen: Honoraria. Brice:Takeda: Consultancy; Roche: Consultancy. Hermine:AB Science: Consultancy, Current equity holder in publicly-traded company, Honoraria, Patents & Royalties, Research Funding; Celgene BMS: Consultancy, Research Funding; Roche: Consultancy; Novartis: Research Funding; Alexion: Research Funding. Rubio:Medac: Consultancy; Gilead: Honoraria; MSD: Honoraria; Novartis: Honoraria; Neovii: Research Funding. Deau Fischer:Takeda: Consultancy; Roche: Consultancy.

Splenic lesion mimicking breast metastasis: The first description of splenic parenchymal endometriosis

Introduction: Splenic parenchymal endometriosis has never been described to date. We report here the case of real parenchymal endometriosis of the spleen. Case description: In this case, a 54-year-old female patient presented a histologically proven metastatic recurrence of breast cancer in the internal breast chain. The CT-scan also detected a large cystic structure developed from the spleen, but non-suspected to be metastasis. The patient was treated with chemotherapy (paclitaxel) and a combination of targeted therapies (everolimus and trastuzumab). While a complete radiological and biological response was noted at 2 months, the splenic cyst gradually decreased over the years. When targeted therapies were stopped, a reincrease of the splenic lesion and de novo significant hypermetabolism of the splenic parenchyma on 18F-FDG PET scan were observed. A splenectomy was finally performed and revealed splenic parenchymal endometriosis. Conclusion: This case once again highlights the complexity of endometriosis disease, from a pathophysiological point of view, but also the difficulties of radiological characterisation, and diagnostic management.