[MRI bone marrow findings in 63 patients with type I Gaucher disease]. / MRT der Knochenmarkverändungen bei 63 Patienten mit Morbus Gaucher Typ I.

PURPOSE: To determine whether MR bone marrow findings in Gaucher patients may help to identify patients at high risk of developing severe Gaucher bone complications exemplified by avascular necrosis (AVN) of the femoral head. MATERIALS AND METHODS: MR images were obtained in 63 Type I Gaucher patients through a standard protocol using coronal T 1 and T 2-weighted sequences of the lower extremities. The location and extent of infiltrated marrow was established using a semi-quantitative MRI scoring method (Düsseldorf Gaucher score, DGS) and the morphological pattern of bone marrow involvement determined (whether homogeneous type A or non-homogeneous type B). The active marrow process with bone edema and AVN of the femoral head were also analyzed. RESULTS: Bone marrow involvement was observed in femoral sites more than in tibial sites. A high DGS was significantly correlated with type B morphology and femoral AVN (both p < 0.0001). Splenectomized patients showed a significantly higher Düsseldorf Gaucher score and type B morphology than non-splenectomized patients (both p < 0.05). AVN was seen in 46 % of patients with type B morphology versus 3 % in type A morphology (p < 0.0001). DGS and morphology of bone marrow involvement were not significantly correlated with active marrow processes. CONCLUSION: Type B marrow morphology and extensive marrow packing were significantly associated with AVN of the femoral head (both p < 0.0001). These patterns are considered predictive and may be employed in a disease management context to alert physicians to the need for urgent therapeutic measures.

Necrotizing fasciitis: microbiological characteristics and predictors of postoperative outcome.

OBJECTIVE: Necrotizing fasciitis is a life threatening soft-tissue infection with a high morbidity and mortality. Prompt treatment based on extensive surgical debridement and antibiotic therapies are the therapeutic principles. METHODS: The medical records of patients with necrotizing fasciitis (n = 26) from 1996 to 2005 were retrospectively analyzed. RESULTS: The localization of necrotizing fasciitis was most commonly the trunk (42.3 %). Type I polymicrobial infection was the dominating infection. The involvement of anaerobic bacteria was associated with an increase in the number of surgical revisions (p = 0.005). Length of postoperative intensive care unit stay, duration of postoperative ventilation and mortality were significantly increased in the ASA IV-V group. Computed tomography displayed only a limited significance as diagnostic tool for initial diagnosis. CONCLUSIONS: In severe cases the combination of necrotic skin and soft tissue gas facilitates the correct diagnosis, which should than be followed by immediate - and most often - repeated debridement. If anaerobes are isolated an early and aggressive second look is necessary.

Different dose-dependent correction of MIP-1beta and chitotriosidase during initial enzyme replacement therapy.

In tissue lesions of type I Gaucher patients, characteristic lipid-laden macrophages, 'Gaucher cells', are surrounded by inflammatory phagocytes. Gaucher cells secrete the elevated plasma chitotriosidase. The elevated plasma MIP-1beta in Gaucher patients stems from the phagocytes surrounding the Gaucher cells. Plasma chitotriosidase and MIP-1beta decrease upon successful enzyme replacement therapy (ERT) with mannose-terminated recombinant glucocerebrosidase (alglucerase). Previous histochemical analysis of Gaucher spleens revealed that Gaucher cells express little mannose receptor, in contrast to surrounding phagocytes. We therefore investigated the corrective effects of ERT on plasma MIP-1beta and chitotriosidase in more detail. We also compared effects of one year of treatment with a relatively low dose and a relatively high dose of ERT. A more rapid correction in plasma MIP-1beta, compared to chitotriosidase, was observed in most patients on low-dose ERT. Correction of plasma MIP-1beta and chitotriosidase levels was more pronounced in the higher-dosed patient group. Upon prolonged treatment, differences in the effects of enzyme dose were no longer significant. Normalization of plasma MIP-1beta and chitotriosidase levels was attained in the majority of patients. In conclusion, ERT with mannose-terminated gluocerebrosidase results in prominent corrections of plasma chitotriosidase, a marker of Gaucher cells, and in particular of plasma MIP-1beta, a marker of inflammatory phagocytes. The sharper response in plasma MIP-1beta to ERT is in line with the observation that especially phagocytes surrounding Gaucher cells express mannose-receptors.

"Silent" bone stress injuries in the feet of diabetic patients with polyneuropathy: a report on 12 cases.

BACKGROUND: Bone stress injuries are rarely being diagnosed in patients with sensory neuropathy, most likely because they may be silent in terms of pain. Load-related pain is considered a key feature of any bone stress injury, a symptom, which may be partially or completely absent in subjects with sensory neuropathy (loss of protective sensation). We evaluated the clinical course of bone stress injuries in insensitive feet in diabetic patients with polyneuropathy. METHODS: We investigated 12 consecutive diabetic patients with bone stress injuries of the foot (bone marrow edema, bone bruise and microtrabecular fractures, on magnetic resonance imaging MRI), which were undetectable on plain X-ray. All patients suffered from diabetic polyneuropathy, none of them had an active foot ulcer. RESULTS: The patients presented with a swollen foot, which was only mildly painful and did not prevent them from walking. Complaints were related to the swelling, which increased during load-bearing. In seven cases, a traumatic event preceding the onset of symptoms could be ascertained. MRI disclosed stress injuries in 2.5 (1-8) [median (range)] bones per foot. In 11 patients, treatment was started immediately by off-loading with total contact cast for 17 (8-52) weeks, followed by gradual increase in weight bearing. One patient unfortunately received off-loading treatment only after deforming fractures had developed. All bone injuries healed uneventuelly in eight patients, and with residual mild osteoarthrosis in three patients without gross deformities. However, the untreated patient developed severe Charcot foot deformity. CONCLUSIONS: In diabetic patients with polyneuropathy, symptoms of bone stress injuries of the foot are atypical, in that there is load-related swelling rather than load-related pain. Immediate diagnosis, and treatment with off-loading, leads to a restitutio ad integrum like in non-neuropathic patients. Delayed cessation of overuse, however, may cause irreversible joint and bone damage (Charcot foot).

Evaluation of the diabetic charcot foot by MR imaging or plain radiography--an observational study.

Magnetic resonance (MR) images and concomitant plain radiographs of 26 Charcot feet of different stages (stage 0 to III) were reviewed in retrospect for discordant findings. Bone and joint abnormalities were evaluated qualitatively, and quantitatively guided by the AO integral classification of injuries of the foot. MR images revealed traumatic bone and joint injuries (bone oedema, occult fractures, and joint effusion) already in stage 0, when X-ray still showed normal bone and joint anatomy (p = 0.02). Moreover, MR images revealed bone oedema, joint effusion and soft tissue oedema in addition to fractures and calluses in stage I (bone dissolution), stage II (bone coalescence), and stage III (bone remodeling), i.e., in stages with overt radiographic pathology. According to this observational study, MR imaging, in addition to radiography, provides important information as to the extent and the natural course of bone injury in the diabetic Charcot foot. MR imaging, thus, may improve disease staging, as well as treatment monitoring.

Radiation exposure of patients in comprehensive computed tomography of the head in acute stroke.

BACKGROUND AND PURPOSE: To assess patient radiation exposure in comprehensive stroke imaging using multidetector row CT (MDCT) combining standard CT of the head, cerebral perfusion (CTP), and CT angiography (CTA) studies. METHODS: Examination protocols for CT and CTA of cerebral and cervical vessels, as well as CTP were simulated using a Somatom Sensation Cardiac 64. Effective doses were derived from measurements with the use of lithium-fluoride thermoluminescent dosimeters (LiF-TLD) at several organ sites using an Alderson-Rando phantom. RESULTS: LiF-TLD measurements resulted in effective doses of 1.7 mSv for CT, 1.9 mSv for CTA of intracranial vessels, and 2.8 mSv for CTA of cervical vessels, respectively. Depending on examination parameters, effective doses varied between 1.1 and 5.0 mSv for cerebral CTP. For CTP, local doses in the area of the primary beam ranged between 114 and 444 mGy. CONCLUSIONS: Comprehensive stroke imaging may result in up to 9.5 mSv with possible local doses of 490 mGy. Although critical doses for organ damage (eg, cataract formation or hair loss) are not reached, physicians need to be aware of possible radiation induced sequelae particularly in repetitive examinations.

Imaging characteristics of hepatocellular carcinoma using the hepatobiliary contrast agent Gd-EOB-DTPA.

PURPOSE: To evaluate the ability of contrast-enhanced magnetic resonance imaging (MRI) with Gd-EOB-DTPA in comparison with non-enhanced imaging and spiral computed tomography (CT) to provide additional information for classification and characterization of hepatocellular carcinoma. MATERIAL AND METHODS: Forty patients with histopathology-proven hepatocellular carcinoma were selected for this subgroup analysis from a phase-III multicenter study in 235 patients with known or suspected liver lesions. The primary analysis was comparison of the proportion of hepatocellular carcinoma correctly classified and characterized by combined pre-/post-contrast MRI compared with pre-contrast MRI alone or with spiral CT. All images were evaluated on site, and in a blinded reading by three independent readers off site. RESULTS: In the on-site evaluation, the lesions were correctly classified as a malignant tumor with combined MRI in 90.3%, with pre-contrast imaging alone in 82.9% and with spiral CT in 87.8% (n.s.). The proportion of correct characterization (lesion type diagnosis) with combined MRI was 85.4%, 75.6% for pre-contrast imaging, and 77.5% for spiral CT (n.s.), respectively. In the blinded reading, one reader showed a significant increase in the proportion of correctly characterized lesions by 27% (P<0.05). The other two readers showed a reduction in the proportion of correct characterization by 12% and 15%, respectively (n.s.). CONCLUSION: With regard to lesion classification, no difference was found between combined pre-/post-contrast MRI and spiral CT. A non-significant trend in favor of Gd-EOB-DTPA-enhanced MRI with regard to characterization of hepatocellular carcinoma was found, although the CT scans were not optimized as the MRI scans.

Dose reduction in multi-slice CT of the heart by use of ECG-controlled tube current modulation ("ECG pulsing"): phantom measurements.

PURPOSE: To evaluate the effect of ECG-controlled tube current modulation on radiation exposure in retrospectively-ECG-gated multislice CT (MSCT) of the heart. MATERIAL AND METHODS: Three different cardiac MSCT protocols with different slice collimation (4 x 1, and 4 x 2.5mm), and a pitch-factor of 1.5 and 1.8 were investigated at a multi-slice CT scanner Somatom Volume Zoom, Siemens. An anthropomorphic Alderson-Rando phantom was equipped with LiF-thermoluminescence dosimeters at several organ sites, and effective doses were calculated using ICRP-weighting factors. Scan protocols were performed with ECG-controlled tube current modulation ("ECG pulsing") at two different heart rates (60 and 80 bpm). These data were compared to previous data from MSCT of the heart without use of "ECG pulsing". RESULTS: Radiation exposure with (60 bpm) and without tube current modulation using a 2.5 mm collimation was 1.8 mSv and 2.9 mSv for females, and 1.5 mSv and 2.4 mSv for males, respectively. For protocols using a 1 mm collimation with a pitch-factor of 1.5 (1.8), radiation exposure with and without tube current modulation was 5.6 (6.3) mSv and 9.5 (11.2) mSv for females, and 4.6 (5.2) mSv and 7.7 (9.2) mSv for males, respectively. At higher heart rates (80 bpm) radiation exposure is increased from 1.5 - 1.8 mSv to 1.8 - 2.1 mSv, using the 2.5 mm collimation, and from 4.6 - 5.6 mSv to 5.9 - 7.2 mSv, for protocols using 1 mm collimation. CONCLUSIONS: The ECG-controlled tube current modulation allows a dose reduction of 37 % to 44 % when retrospectively ECG-gated MSCT of the heart is performed. The tube current - as a function over time - and therefore the radiation exposure is dependent on the heart rate.

CT and MRI findings of multifocal hepatic steatosis mimicking malignancy.

We present two rare cases of multifocal hepatic steatosis as a variant of fatty liver. Multifocal hepatic steatosis can cause misleading findings in the differential diagnosis when using ultrasound and computed tomography. This case report describes the atypical findings of focal fatty liver infiltrations, which were misdiagnosed as diffuse metastatic disease. The correct diagnosis was established with magnetic resonance imaging using T1-weighted gradient-echo and T2-weighted Turbo spin-echo sequences with spectral fat suppression. Multifocal hepatic steatosis was proven by biopsy.

[Does dynamic contrast-enhanced MRI enable recognition of development of vertebral fractures in multiple myeloma?]. / Ermöglicht die dynamische kontrastverstärkte MRT die Detektion frakturgefährdeter Wirbelkörper beim Multiplen Myelom?

PURPOSE: To compare the value of MRI sequences with dynamic MRI measurements (d-MRT) for the assessment of risk of lumbar vertebral fractures in patients with multiple myeloma. MATERIAL AND METHODS: In 10 patients with multiple myeloma a sagittal T 1 -, T 2 -weighted spin-echo and an inversion-recovery sequence were performed. For dynamic measurements a fast gradient-echo sequence (turbo fast low angle shot 2 D) with machine-controlled Gd-DTPA administration was used. The presence of bone marrow abnormalities was determined and additionally the value of the highest signal increase (amplitude Alpha) was calculated for each of the 50 vertebral bodies. The subsequent development of vertebral fractures was assessed by MRI at a mean time interval of 6.2 months after the initial d-MRI. The pattern of marrow involvement and the amplitudes of the vertebral bodies that collapsed in the observation period were statistically compared with those of the vertebral bodies that did not collapse. RESULTS: During the follow-up period newly or progressive fractures occurred in 6 of 10 patients (7 of 50 vertebral bodies). The degree of pathological signal changes that preceded fractures was not significantly different (p > 0.05) from that of the other contemporary lesions identified in vertebral bodies that did not collapse in follow-up. In contrast, the amplitude of vertebrae that collapsed (A: 33.1 +/- 8.2) was significantly higher (p < 0.0001) than the amplitude of vertebrae that did not collapse (A: 16.7 +/- 4.2). The amplitude was a reliable predictor of vertebrae that collapsed in all cases. CONCLUSION: In contrast to the analysis of marrow lesions detected with non-dynamic MRI, this study suggests that the perfusion-parameter amplitude in dynamic contrast-enhanced MRI is a potentially relevant value to predict the risk of vertebral fractures in patients with multiple myeloma.

Response of Gaucher bone disease to enzyme replacement therapy.

In Gaucher disease, enzyme replacement therapy usually reduces liver and spleen volumes and improves haematological abnormalities within 1 year. In contrast, skeletal manifestations of Gaucher disease are thought to respond more slowly. For example, decreased bone marrow glycolipid infiltration and increased bone mineral density have been reported to take up to 3-4 years of treatment. In this report, we present recent studies using T1- and T2-weighted MRI and quantitative chemical shift imaging that demonstrate decreases in abnormal glucocerebroside infiltration and increases in normal fat content of bone marrow within the first year of treatment. There was no obvious relationship between age, gender, splenectomy status or genotype and the response of bone marrow to therapy. Although the dose of enzyme replacement therapy may be related to bone marrow response, no significant relationship was demonstrated in this report. Long-term enzyme replacement therapy induces continued degradation of Gaucher cell deposits, reconversion of fat marrow and increased bone mineral density. This treatment is also associated with improved or non-progressive bone symptoms and functional status in most adult patients, and it prevents the new occurrence of bone pain and bone crisis in nearly all patients. The development of more sensitive, quantitative imaging methods will help to evaluate disease severity better and to assess the response to therapy.

Imaging and quantifying skeletal involvement in Gaucher disease.

Radiological imaging is used in patients with Gaucher disease to estimate the disease burden, to evaluate the presence of specific skeletal complications and to track response to therapy. MRI is currently the best technique for assessing bone marrow involvement in Gaucher disease. Gaucher cell infiltrated bone marrow is characterized by an abnormal low signal intensity on conventional T1- and T2-weighted spin echo sequences, owing to a reduction in fat marrow, which gives a high signal intensity. Enzyme replacement therapy results in a degradation of Gaucher cell deposits with a reconversion of marrow fat and consequently an increased signal on T(1)-weighted images. Conventional MRI also detects other skeletal complications in Gaucher disease, including oedema resulting from acute bone infarction, infection and trauma, avascular necrosis, pathological fractures and vertebral compression. The main drawback of conventional MRI is that it is not quantitative. Quantitative chemical shift imaging is the most sensitive quantitative method for evaluating bone marrow but is not widely available. Alternative MRI-based methods include calculation of the T1 relaxation constant and proton spectroscopy. Scoring of imaging changes detected on conventional MRI may be useful in estimating disease burden and risk of complications. Dual-energy X-ray absorptiometry (DXA) is sensitive to generalized osteopenia and changes in bone mineral density with extended enzyme replacement therapy. However, DXA is insensitive to local changes and cannot yet be used to predict fracture risk in these patients. Until the ideal quantitative technique is developed, conventional MRI will remain the best diagnostic modality for assessing skeletal complications in Gaucher disease and monitoring response to enzyme replacement therapy.

[Dynamic MRI of the lumbar spine for the evaluation of microcirculation during anti-angiogenetic therapy in patients with myelodysplastic syndromes]. / Dynamische MRT der Lendenwirbelsäule zur Beurteilung der Mikrozirkulation unter anti-angiogenetischer Therapie bei Patienten mit myelodysplastischen Syndromen.

PURPOSE: Evaluation of MRI perfusion parameters of the lumbar spine in patients with myelodysplastic syndromes (MDS) to determine the vascularisation and anti-angiogenetic effects of thalidomide therapy. MATERIAL AND METHODS: In 20 healthy normal persons and 28 MDS patients a dynamic contrast-enhanced MRI (d-MRI) of the lumbar spine was performed. After the initial d-MRI-investigation 24 of the 28 MDS patients received an anti-angiogenetic therapy with thalidomide. With an average of 4.2 months after the beginning of therapy a d-MRI-follow-up examination in 9 of these patients was performed. The amplitude and exchange-rate constant were calculated and a statistical comparison of these values between healthy persons and MDS patients as well as a correlation with the clinical course was executed. RESULTS: Compared with the normal controls the MDS patients showed a higher amplitude (normal persons: 14.4 +/- 5.2, MDS: 24.8 +/- 8.1) and exchange-rate constant (normal persons: 0.124 +/- 0.042, MDS: 0.136 +/- 0.036). In 7 of 9 MDS patients undergoing thalidomide therapy a reduction of the amplitude and exchange rate constant values was evident in the d-MRI follow-up examinations. Clinically these patients showed a therapy response with complete or partial disease remission. CONCLUSIONS: In MDS patients significantly higher d-MRI parameters can be demonstrated than in normal persons. Under anti-angiogenetic treatment these values decrease in case of a response to therapy. Thus, d-MRI seems suitable for the evaluation of anti-angiogenetic therapy effects.

Proton MR spectroscopy of the lumbar spine in patients with glycogen storage disease type Ib.

Glycogen storage disease type Ib is an autosomal recessive inherited metabolic disorder resulting from deficiency of the microsomal glucose-6-phosphatase enzyme system. Six patients (three of which were treated with granulocyte colony stimulating factor) suffering from this disease were examined using image guided localized proton magnetic resonance (MR) spectroscopy. The relative signal intensities of water and lipid protons of the lumbar spine were determined. Comparison was made with iliac crest biopsies in the glycogen storage disease type Ib patients and localized proton MR spectroscopic values of the lumbar spine obtained by thirteen healthy volunteers. The data demonstrate for the first time that hypercellularity and myeloid hyperplasia in subjects with glycogen storage disease type Ib due to functionally impaired leucocytes results in a strongly increased water proton signal with a very low or absent lipid signal in localized proton MR spectroscopy. Upon granulocyte colony stimulating factor treatment, the water proton signal in the lumbar spine is not further augmented.

HRCT findings of amiodarone pulmonary toxicity: clinical and radiologic regression.

Amiodarone is an antiarrhythmic drug that can cause interstitial pneumonitis leading to pulmonary fibrosis. A 62-year-old man suffering from atrial fibrillation and recurrent dyspnea was treated with amiodarone. After 15 months of treatment, HRCT revealed bilateral interstitial and alveolar opacifications with high-attenuating pleural-parenchymal consolidations, suggesting amiodarone induced pneumonitis. Three months after cessation of amiodarone treatment, HRCT shows complete regression of pneumonitis. Amiodarone pneumonitis can be misinterpreted and mistreated in patients with further underlying diseases. The present case report demonstrates the diagnostic value of HRCT in the diagnosis of interstitial pneumonitis.

In vitro determination of cardiac ventricular volumes using MRI at 1.0 T in a porcine heart model.

PURPOSE: To determine the accuracy of a 1.0 T MR system with a standard gradient system for quantification of left and right ventricular volumes. A porcine heart model in vitro was used. METHODS: In eight explanted porcine hearts the atria were removed and the aorta and the pulmonary truncus were cannulated. Defined volumes were injected into the ventricles. Magnetic resonance imaging (MRI): FFE-EPI (Multishot EPI) was used. Papillary muscles and trabeculae were excluded. True volumes and MR measurements were analysed separately for both ventricles and by both investigators. RESULTS: The correlation of the true volumes and MR measurements was >0.99. MRI was found to be investigator independent in assessing right and left ventricular volumes in vitro. CONCLUSIONS: MRI at 1.0 T using standard equipment can be used to quantitate cardiac ventricular volumes in vitro with high accuracy.

Magnetic resonance imaging of bone marrow changes in Gaucher disease during enzyme replacement therapy: first German long-term results.

OBJECTIVE: Since 1991, enzyme replacement therapy (ERT) has been available for patients with Gaucher disease in Germany. The aim of this study was to analyse the MR pattern of bone marrow involvement and response to ERT in Gaucher disease type I. PATIENTS AND DESIGN: Thirty patients with Gaucher disease type I had MRI examinations prior to initiation of ERT with alglucerase/imiglucerase and during follow-up. Median MR follow-up and duration of ERT were 36 months. Coronal T1- and T2-weighted spin-echo images of the lower extremities were obtained to evaluate changes in the appearance of yellow marrow. MR images were categorized as having either a homogeneous (type A) or non-homogeneous patchy (type B) appearance of bone involvement and response to ERT was assessed by two radiologists. RESULTS: Overall, 19 of 30 patients (63%) showed an increased signal intensity on T1- and T2-weighted images after 36 months of ERT, consistent with partial reconversion of fatty marrow during treatment. Focal bone lesions surrounded by a low signal intensity (SI) rim did not respond to ERT, suggesting bone infarcts. Of the 11 patients with bone infarcts (low SI rim lesion), 82% had the non-homogeneous type B pattern (P=0.0021). In 86% of patients with splenectomy, bone infarcts were seen (P<0.05). CONCLUSIONS: MRI using T1- and T2-weighted spin-echo sequences is a valuable, non-invasive method for monitoring bone marrow response in patients receiving ERT. A non-homogeneous patchy signal intensity of bone marrow involvement correlates with the presence of bone infarcts (P=0.0021).

[MR-morphological changes of the metacarpophalangeal joints in patients with rheumatoid arthritis: Comparison of early and chronical stages]. / MR-morphologische Veränderungen der Metacarpophalangealgelenke bei Rheumatoider Arthritis: Vergleich früher und später Stadien.

UNLABELLED: MR-morphological changes of the metacarpophalangeal joints in patients with rheumatoid arthritis: Comparison of early and chronical stages. PURPOSE: Evaluation of MRI findings in the metacarpophalangeal (MCP) joints in patients with early (eRA) and chronical rheumatoid arthritis (cRA). MATERIAL AND METHODS: In 22 RA patients (9 with disease duration

[Gaucher disease: MR evaluation of bone marrow features during treatment with enzyme replacement]. / Morbus Gaucher: Analyse der Knochenmarkveränderungen in der MRT während Enzymersatztherapie.

PURPOSE: Enzyme replacement therapy (ERT) arrests and reverses the hematological and visceral symptoms of adult Gaucher disease, the most frequent lysosomal storage disorder. There are only a few studies available evaluating bone disease during ERT. The aim of this study was to investigate the features of bone marrow (bm) by magnetic resonance imaging (MRI) in these patients during ERT. MATERIALS AND METHODS: MRI was performed prospectively in thirty adult type I Gaucher patients before and during ERT with a mean follow-up of 3 years. Spin-echo sequences (T(1)/T(2)) of the lower extremities were obtained and the reconversion (response) or lack of reconversion (non-response) to fatty marrow during treatment was analyzed. The morphological features of bm involvement, a homogeneous or non-homogeneous distribution of bm changes and focal bone lesions surrounded by a rim of reduced signal intensity (SI), were analyzed. RESULTS: Infiltration of bm by Gaucher cells is characterized by a reduction of SI on both T(1)- and T(2)-weighted sequences. Bone marrow responses were seen in 19 patients (63 %) during treatment. Focal bone lesions, surrounded by a rim of reduced SI, did not respond to ERT and correlated with a non-homogenous distribution of bone involvement and splenectomy. CONCLUSION: In adult patients with type I Gaucher disease receiving ERT, treatment effects on bone disease can be demonstrated by MRI using Spin-echo sequences due to the partial reconversion of fat marrow. A non-homogeneous type of signal appearance and a status post splenectomy correlate with the presence of bone infarcts.