MRI ankle and subtalar characteristics in haemochromatosis arthropathy: a case-control study.

AIM: To examine the magnetic resonance imaging (MRI) features of the ankle and subtalar joints that might distinguish genetic haemochromatosis (GH). MATERIALS AND METHODS: The present study was a retrospective case-control study comparing 30 MRI studies of GH patients with ankle or subtalar arthropathy with 30 matched controls with ankle pain. Anonymised images were scored using a semi-quantative tool adapted from the MRI osteoarthritis knee score. Scores were generated for bone marrow lesions size, number, and distinguishing the proportion of each lesion consisting of subchondral cyst versus oedema. Articular cartilage loss and osteophytes were documented. The primary comparator was bone marrow lesion size. Paired Student's t-test and the chi-squared test were utilised to compare outcomes. RESULTS: Bone marrow lesion/cyst size and number, presence and extent of full-thickness cartilage loss, and osteophyte scores were significantly higher in ankle joints of GH cases (p<0.01). In the middle subtalar articulation, there were significantly higher scores for full-thickness cartilage loss and extent and osteophytes in GH cases (p<0.05). There were no significant differences in the posterior subtalar articulation. CONCLUSION: The finding of both numerous and large cysts on ankle MRI should raise suspicion of GH. Other MRI features of potential diagnostic value include large osteophytes and the presence of extensive full-thickness cartilage loss in the ankle joint and middle subtalar articulation.

The anatomy of apathy: A neurocognitive framework for amotivated behaviour.

Apathy is a debilitating syndrome associated with many neurological disorders, including several common neurodegenerative diseases such as Parkinson's disease and Alzheimer's disease, and focal lesion syndromes such as stroke. Here, we review neuroimaging studies to identify anatomical correlates of apathy, across brain disorders. Our analysis reveals that apathy is strongly associated with disruption particularly of dorsal anterior cingulate cortex (dACC), ventral striatum (VS) and connected brain regions. Remarkably, these changes are consistent across clinical disorders and imaging modalities. Review of the neuroimaging findings allows us to develop a neurocognitive framework to consider potential mechanisms underlying apathy. According to this perspective, an interconnected group of brain regions - with dACC and VS at its core - plays a crucial role in normal motivated behaviour. Specifically we argue that motivated behaviour requires a willingness to work, to keep working, and to learn what is worth working for. We propose that deficits in any one or more of these processes can lead to the clinical syndrome of apathy, and outline specific approaches to test this hypothesis. A richer neurobiological understanding of the mechanisms underlying apathy should ultimately facilitate development of effective therapies for this disabling condition.

Earliest evidence for the use of pottery.

Pottery was a hunter-gatherer innovation that first emerged in East Asia between 20,000 and 12,000 calibrated years before present (cal bp), towards the end of the Late Pleistocene epoch, a period of time when humans were adjusting to changing climates and new environments. Ceramic container technologies were one of a range of late glacial adaptations that were pivotal to structuring subsequent cultural trajectories in different regions of the world, but the reasons for their emergence and widespread uptake are poorly understood. The first ceramic containers must have provided prehistoric hunter-gatherers with attractive new strategies for processing and consuming foodstuffs, but virtually nothing is known of how early pots were used. Here we report the chemical analysis of food residues associated with Late Pleistocene pottery, focusing on one of the best-studied prehistoric ceramic sequences in the world, the Japanese Jomon. We demonstrate that lipids can be recovered reliably from charred surface deposits adhering to pottery dating from about 15,000 to 11,800 cal bp (the Incipient Jomon period), the oldest pottery so far investigated, and that in most cases these organic compounds are unequivocally derived from processing freshwater and marine organisms. Stable isotope data support the lipid evidence and suggest that most of the 101 charred deposits analysed, from across the major islands of Japan, were derived from high-trophic-level aquatic food. Productive aquatic ecotones were heavily exploited by late glacial foragers, perhaps providing an initial impetus for investment in ceramic container technology, and paving the way for further intensification of pottery use by hunter-gatherers in the early Holocene epoch. Now that we have shown that it is possible to analyse organic residues from some of the world's earliest ceramic vessels, the subsequent development of this critical technology can be clarified through further widespread testing of hunter-gatherer pottery from later periods.

Prediction of erosion progression using ultrasound in established rheumatoid arthritis: a 2-year follow-up study.

OBJECTIVES: This study aimed to determine whether a range of single-time-point ultrasound (US) measures of synovial disease and serologic characteristics were able to predict progression of US-defined erosive disease in patients with established rheumatoid arthritis (RA). MATERIALS AND METHODS: Forty patients were studied prospectively. At baseline, subjective US measures of bone damage and synovial disease, including grayscale and power Doppler (PD) scores pre- and post-Sonovue contrast, were obtained from one proximal inter-phalangeal or metacarpo-phalangeal joint per patient. After a minimum of 2 years, the same joints were scanned to obtain a new US erosion score. RESULTS: Follow-up US erosion scores were obtained in 25 joints. Progressive US determined that bone damage occurred in 12/25 joints, including four of eight treated with anti-tumor necrosis factor therapy. Baseline erosion scores were significantly higher in joints that did not show progressive bone damage in the entire cohort (p = 0.05, n = 25) and a subgroup treated with disease-modifying anti-rheumatic drugs (p = 0.015, n = 17). There were no other significant differences in baseline US or serologic scores between joints that developed progressive damage and those that did not. CONCLUSIONS: The majority of single-time-point US measures of synovial disease were not able to identify metacarpo-phalangeal or inter-phalangeal joint destined to develop progressive US-determined bone damage in patients with established RA. This may reflect the use of single-time-point measures, insensitivity of the US erosion score, and the long duration of RA disease in this study.

The relation between composite ultrasound measures and the DAS28 score, its components and acute phase markers in adult RA.

OBJECTIVES: Ultrasound (US) provides measurements of synovial morphology and vascularity. However, on an individual joint basis in RA, US measures do not relate well to clinical signs. This study investigates the relationship between composite US measures and the 28-joint disease activity score (DAS28), its components and acute phase markers in adult RA. METHODS: RA synovial disease activity was recorded in 50 patients by: (i) the DAS28 score; (ii) ESR and CRP; and (iii) US using Grey scale (GS) and power Doppler (PD) measures of PIP and MCP joints to derive composite US scores based on abnormal counts and severity. A total of 25 control subjects were studied to define normal US appearances. The relation between each measure of synovial disease was determined by Spearman correlation analysis. RESULTS: There was a significant relation between the DAS28 and the GS joint count (GSJC, Spearman's r = 0.4; P = 0.004) and severity score (GSJS, r = 0.34; P = 0.016) and the PD joint count (PDJC, r = 0.32; P = 0.028). There was a significant relation between the ESR and PDJC (r = 0.37; P = 0.007) and PD joint severity score (PDJS, r = 0.38; P = 0.006) and between the CRP and PDJS (r = 0.29; P = 0.04). The remaining components of the DAS28 related poorly to all US measures, except the tender joint count, which related significantly to the GS but not the PD measures. CONCLUSIONS: Composite US markers of synovial disease relate significantly to the DAS28 score and ESR/CRP in adult RA, but not as well with individual clinical joint counts and the patient's global assessment.

A comparison of clinical vs ultrasound determined synovitis in rheumatoid arthritis utilizing gray-scale, power Doppler and the intravenous microbubble contrast agent 'Sono-Vue'.

OBJECTIVES: Synovitis in rheumatoid arthritis (RA) is assessed clinically by the presence of joint tenderness and swelling. Synovial thickening and increased vascularity may also be detected by high-resolution ultrasonography (US) and power Doppler (PD). This study investigated the relationship between clinical and sonographic features of synovial disease utilizing US, PD and the contrast agent Sono-Vue. METHODS: Forty RA patients were recruited. One proximal inter-phalangeal or metacarpophalangeal joint was selected per patient, as being unambiguously either: swollen and tender, just swollen, just tender or neither swollen nor tender (Nil). Ten joints were selected per clinical group. On US, the mean synovial thickness was measured and synovial hypertrophy and erosions were graded subjectively. Synovial vascularity demonstrated by PD was scored subjectively pre- and post-contrast. RESULTS: All grades of synovial vascularity were found in each clinical group including the Nil group. There were significant differences between the four clinical groups for both synovial hypertrophy (P = 0.024) and PD scores pre- (P = 0.022) and post- (P = 0.039) contrast. Tender-only joints showed significantly less vascularity than other groups. Post-contrast, the median PD scores increased in all but the Nil group, in some cases from the normal to abnormal range. CONCLUSION: Synovitis demonstrated by US and PD is not predicted by patterns of disease as described by joint swelling and tenderness despite unambiguous selection of joints. Synovial vascularity was the least in tender-only joints and was heterogeneous in all other groups, including Nil joints. These findings question the reliability of traditional clinical signs in RA synovitis assessment.

Catel-Manzke syndrome: a case report of a female with severely malformed hands and feet. An extension of the phenotype or a new syndrome?

An unusual case of a female infant with Catel-Manzke syndrome is presented. Additional features not previously reported include three accessory ossicles at the bases or associated with the proximal phalanx of the index, middle, ring and little fingers bilaterally. There are also numerous bony abnormalities in both feet. Previous cases have shown no more than 2 accessory ossicles in the hand and these usually involve the index alone. The foot abnormalities are more extensive than any previously seen in this syndrome. This is only the 8th female case out of a total of 27 reported cases.

The diagnostic contribution of the frontal lumbar spine radiograph in community referred low back pain--a prospective study of 1030 patients.

AIM: The diagnostic contribution of the anteroposterior (AP) view was studied to assess whether this view could be omitted safely, thus reducing the radiation burden received by patients undergoing lumbar spine radiography. MATERIALS AND METHODS: Prospective analysis of 1030 consecutive referrals for lumbar spine radiography from general practice. RESULTS: In the majority of cases (90.5%) the AP view was non-contributory. In 4.2% the diagnosis was strengthened and it was altered in 4.6%. However, in the latter group only 1.3% of the total were considered significant alterations. These were cases of possible, but not definite, pars defects and sacroiliitis. Specific important conditions such as infection, malignancy and benign tumours were not missed on the lateral view alone, in our study population. The radiation burden is reduced by 75% by omitting the AP view. CONCLUSION: A single lateral view is an adequate examination, with the proviso that sacroiliac joint disease is not assessed on this view and some pars defects and facet joint degenerative changes may be overlooked. The radiation protection gains are considerable. A single lateral lumbar view is now our routine practice unless sacroiliitis is a specific clinical concern.

Effect of MRI noise on cochlear function.

A disadvantage of magnetic resonance imaging (MRI) is the high level of noise produced (peaking between 122 dB and 131 dB). We used otoacoustic emissions (OAEs) as a method to quantify the effect of MRI-generated noise on the cochlea. OAEs were measured in 16 patients before and after MRI and in 16 controls. OAEs decreased in patients after MRI, but the controls, who were not exposed to MRI noise, showed no decrease over the same period. The change in OAEs shows a clear effect of MRI noise on cochlear function, despite use of earplugs. The importance of correctly fitted earplugs cannot be underestimated.

Contrast-enhanced magnetic resonance angiography of the iliac arteries: optimization by injection simulation.

Protocols for contrast-enhanced magnetic resonance angiography (CE-MRA) of the iliac arteries were optimized by computer simulations based on an impulse response function (IRF) of contrast agent (CA) concentration as a function of time obtained for 20 patients. Protocols with sequential, centric, and elliptical k-space coverage, different repetition rates (5 and 10 ms), and CA doses (0.1, 0.2, and 0.3 mmol/kg b.w.) were compared in terms of signal-to-noise ratio (SNR), distortion of vessel profiles, and sensitivity to timing errors. IRF-based simulations successfully characterized CA recirculation. Slow-rate CA infusions were found to achieve relatively high enhancement. In terms of SNR, there is no advantage in increasing the repetition rate. Distortion of vessel profiles is more likely in elliptic and centric k-space coverage. Protocols based on sequential k-space coverage and relatively long CA infusions proved to be particularly suited to large-FOV iliac examinations as they are relatively insensitive to timing errors.

Speech and language therapy for adults in Hackney: an assessment of need.

With the development of separate community and the hospital trusts in the London borough of Hackney, at a time of increasing numbers and range of referrals, a number of gaps in service became apparent. The need for community adult speech and language therapy (SLT) in Hackney was identified, based on previous studies and other relevant literature. Comparison with services locally and nationally shows clearly that Hackney, in having virtually no community SLT for adults, is significantly disadvantaged. It was intended that this work would result in the widest consultation and discussion by the relevant agencies to explore ways of meeting this need.

Sensitivity of quantitative (1)H magnetic resonance spectroscopy of the brain in detecting early neuronal damage in systemic lupus erythematosus.

OBJECTIVE: To quantify N-acetylaspartate (NAA), total creatines (tCr), total cholines (tCho), and myo-inositol (mI) levels in normal and abnormal appearing white matter of patients with neuropsychiatric systemic lupus erythematosus (NPSLE) in order to determine the specific changes in metabolite concentrations. METHODS: Axial proton density and T(2) weighted magnetic resonance images, and short echo time (TE 30 ms) (1)H spectra were acquired with a GE SIGNA 1.5 T magnetic resonance system. Concentrations of NAA, tCr, tCho, and mI were determined, using brain tissue water as a reference, from nine patients (seven female, mean age 40.3 years, range 16-65) with NPSLE and eight healthy women (mean age 43 years, range 31-65). RESULTS: A significant rise of tCho (12.4%, p<0.05) and mI (31.4%, p<0.005) and a significant reduction in NAA (-12%, p<0.05) was found in normal appearing white matter compared with controls. Analysis according to severity of the clinical NPSLE features (subgrouped as major or minor) showed that SLE major had reduced NAA compared with SLE minor (-18.4%, p<0.05) and controls (-20%, p<0.005). The SLE major group showed a significant rise of mI (32%, p<0.01) and the SLE minor group a significant increase in tCho (18.6%, p<0.05) compared with controls. Longitudinal analysis of brain metabolites in normal appearing white matter showed consistent abnormalities in NAA, tCho, and mI in a patient with stable clinical features and a constant rise of tCho, but transient rise of mI was seen during a flare of disease in another patient. CONCLUSION: Quantitative (1)H magnetic resonance spectroscopy (MRS) suggests a particular course of neurometabolite changes that precedes irreversible reductions in NAA and permanent neuronal loss. Initially, in patients with SLE minor, there is a significant rise in tCho and a trend (reversible) for mI also to be raised. In patients with SLE major the NAA is significantly and permanently reduced and mI is significantly raised, whereas the tCho levels are near normal. Further investigations are needed to determine how specific MRS is as a clinical marker for brain disturbance in SLE.

The high proliferative potential-quiescent (HPP-Q) cell assay allows an optimized evaluation of gene transfer efficiency into primitive hematopoietic stem/progenitor cells.

Various protocols have been described to optimize gene transfer into hematopoietic cells. However, most of these methods do not specify whether they are associated with an improved transduction of the more primitive stem/progenitor cells, the best candidates for long-term engraftment. The majority of these primitive cells remains in quiescence because of the negative control of TGF-beta1, effective on these cells at low concentrations (10 pg/ml). In this study, CD34- cells were activated by a 10 h pretreatment with anti-TGF-beta1 followed by four successive retroviral supernatant incubations of 6 h each. After 12 h (two incubations), a significant increase in TGF-beta1 mRNA in CD34+ cells was observed. We wondered whether neo-synthesized autocrine TGF-beta1 could induce reversion to quiescence of the more primitive CD34+ cells transduced after one cell cycle. This would prevent their subsequent detection in a classic clonal assay. Using the HPP-Q assay comparing a rapid mixed colony assay with or without anti-TGF-beta1, we indeed observed, that in clonal growth conditions the more primitive transduced cells were activated and detectable only with anti-TGF-beta1. Therefore, this assay represents not only a rapid means to detect quiescent multipotent stem/progenitor cells but also a necessary step for the detection of the more primitive transduced cells which have returned to quiescence after retroviral induction of TGF-beta1 secretion.

[Iodine 131 uptake by a bronchogenic cyst in a patient with differentiated carcinoma of the thyroid gland]. / Fixation à l'iode 131 d'un kyste bronchogénique chez une patiente ayant un carcinome différencié thyroïdien.

BACKGROUND: After thyroidectomy for differentiated thyroid carcinoma, extracervical uptake of iodine 131 is suggestive of metastasic dissemination. False positives can however occur. CASE REPORT: Differentiated thyroid carcinoma was found in a female patient with a non-functional nodule. Two years after subtotal thyroidectomy and an ablative dose of iodine 131, the whole body scan showed abdominal mediastinal uptake with low serum thyroglobulin level. Considering the possibility of tumor recurrence or lymph node metastasis, the mass was excised. The histology diagnosis was mediastinal bronchogenic cyst. DISCUSSION: To date, iodine uptake in a bronchogenic cyst has not been reported among other false-positives previously described. The pathophysiology of this iodine 131 uptake in a bronchogenic cyst is still unknown: the presence of NIS symporter or a protein which can organify iodine in the mucus cells of the cyst remains to be proven.

[Cranial pachymeningitis]. / Les pachyméningites crâniennes.

INTRODUCTION: Pachymeningitis is a fibrous inflammatory process with non-specific symptoms, involving the dura mater. Due to MRI development, diagnosis is both easier and earlier. CURRENT KNOWLEDGE AND KEY POINTS: We report seven cases and review current literature. Clinical features are headaches and cranial nerve palsies. CSF shows inflammatory changes, while MRI evidences thickening of the dura mater. Disease etiologies in the present study were tuberculosis in two cases, sarcoidosis, Lyme disease, lymphoma and dural puncture; in one case only the disease was of unknown origin. The condition of six patients improved with specific treatment. FUTURE PROSPECTS AND PROJECTS: Further MRI development should allow detection of new forms of pachymeningitis and standardization of patients' management through the study of more important series.

Case weighting in a speech and language therapy service.

This study present an approach designed to calculate input provided by a speech and language therapy service. A case weighting system was developed in order to evaluate the cost of services, predict the need for intervention, balance and prioritise the caseload among therapists. It is suggested that this approach can be use widely in clinical practice.

A case of verbal auditory agnosia: missing the word ... missing the sound....

The neuropsychological nature of verbal auditory agnosia is not fully understood. This study aims to describe the language deficits and the remediation strategies used in a person with verbal auditory agnosia. In addition, it will address the theoretical issues concerning the nature of the phenomenon and the clinical implications in the management of people with this disorder.