(99m)Tc-DPD scintigraphy is a highly sensitive technique for imaging cardiac ATTR amyloidosis and is an important investigation in the diagnostic pathway of patients with cardiac amyloidosis. It is not specific for ATTR in isolation but must be interpreted in a broad clinical context to avoid dangerous diagnostic errors. Diffuse skeletal muscle uptake identifies muscle as a hitherto unrecognized site that merits investigation as a target organ in ATTR amyloidosis.

High-grade (Perugini grade 2 or 3) cardiac uptake on bone scintigraphy with 99m Technetium labelled 3,3-diphosphono-1,2-propanodicarboxylic acid (99m Tc-DPD) has lately been confirmed to have high diagnostic sensitivity and specificity for cardiac transthyretin (ATTR) amyloidosis. We sought to determine whether patient stratification by Perugini grade on 99m Tc-DPD scintigraphy has prognostic significance in ATTR amyloidosis. Methods and Results Patient survival from time of 99m Tc-DPD scintigraphy was determined in 602 patients with ATTR amyloidosis, including 377 with wild-type ATTR and 225 with mutant ATTR amyloidosis (ATTRm). Patients were stratified according to Perugini grade (0-3) on 99m Tc-DPD scan. The prognostic significance of additional patient and disease-related factors at baseline were determined. In the whole cohort, the finding of a Perugini grade 0 99m Tc-DPD scan (n=28) was invariably associated with absence of cardiac amyloid according to consensus criteria as well as significantly better patient survival compared to a Perugini grade 1 (n=28), 2 (n=436) or 3 (n=110) 99m Tc-DPD scan (p<0.005). There were no differences in survival between patients with a grade 1, grade 2 or grade 3 99m Tc-DPD scan in wild-type ATTR (n=369), V122I-associated ATTRm (n=92) or T60A-associated ATTRm (n=59) amyloidosis. Cardiac amyloid burden, determined by equilibrium contrast cardiac magnetic resonance imaging, was similar between patients with Perugini grade 2 and Perugini grade 3 99m Tc-DPD scans but skeletal muscle/soft tissue to femur ratio was substantially higher in the latter group (p<0.001). Conclusion 3 99m Tc-DPD scintigraphy is exquisitely sensitive for identification of cardiac ATTR amyloid, but stratification by Perugini grade of positivity at diagnosis has no prognostic significance.

Definitions of treatment failure and the labelling of patients as non-responsive typically require treatments to have been offered and failed. For pharmacological treatments, treatment quality is relatively easy to define; this is much more difficult with psychological treatments. This study examined patient recollections of previous therapy for obsessive compulsive disorder (OCD). A Treatment History Questionnaire was administered to a sample of 57 apparently treatment refractory OCD patients from a specialist national OCD treatment unit and a national charity for OCD sufferers. On average, respondents reported an 8 1 2 year wait between the obsessional symptoms interfering significantly with their lives and being diagnosed. Forty-three percent recalled having received either cognitive behaviour therapy (CBT) or behaviour therapy as the first treatment; 31% of the group did not know what type of therapy they had received. The components of therapy that respondents recalled were analysed and contrasted with minimal therapy criteria. These criteria appear not to have been met in most patients who understood that they had received "CBT". The implications of this study for assessment of treatment integrity and the classification of patients as "treatment resistant" are discussed.

PRRT treatment offers increased OS, PFS, EFS, and response to treatment compared to (131)I-MIBG therapy in patients with progressive/malignant PGLs.

SUMMARY:As we defeat infectious diseases and cancer, one of the greatest medical challenges facing us in the mid-21st century will be the increasing prevalence of degenerative disease. Those diseases, which affect movement and cognition, can be the most debilitating. Dysfunction of the extrapyramidal system results in increasing motor disability often manifest as tremor, bradykinesia, and rigidity. The common pathologic pathway of these diseases, collectively described as parkinsonian syndromes, such as Parkinson disease, multiple system atrophy, progressive supranuclear palsy, corticobasal degeneration, and dementia with Lewy bodies, is degeneration of the presynaptic dopaminergic pathways in the basal ganglia. Conventional MR imaging is insensitive, especially in early disease, so functional imaging has become the primary method used to differentiate a true parkinsonian syndrome from vascular parkinsonism, drug-induced changes, or essential tremor. Unusually for a modern functional imaging technique, the method most widely used in European clinics depends on SPECT and not PET. This SPECT technique (described in the first of 2 parts) commonly reports dopamine-transporter function, with decreasing striatal uptake demonstrating increasingly severe disease. ABBREVIATIONS:DaT ϭ dopamine transporters;