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2.
HNO ; 68(6): 447-450, 2020 Jun.
Article in German | MEDLINE | ID: mdl-31578600

ABSTRACT

Ectopic thyroid gland tissue can occur either as the only detectable thyroid gland tissue or in addition to a normotopic thyroid gland. After a total thyroidectomy thyroid-stimulating hormone (TSH) can induce a compensatory volume increase of previously asymptomatic ectopic tissue. This hyperplastic ectopic tissue can occur as an unclear cervical space-occupying lesion. Prior to surgical exploration of an unclear cervical mass the possibility of ectopic thyroid tissue should be included in the differential diagnostic considerations.


Subject(s)
Choristoma , Thyroid Dysgenesis , Humans , Neck , Thyroid Dysgenesis/surgery , Thyroid Gland , Thyroidectomy
4.
Acta Psychiatr Scand ; 136(6): 583-593, 2017 Dec.
Article in English | MEDLINE | ID: mdl-28922451

ABSTRACT

BACKGROUND: To determine event rates for specific medical events and mortality among individuals receiving electroconvulsive therapy (ECT). METHOD: Population-based cohort study using health administrative data of acute ECT treatments delivered in Ontario, Canada, from 2003 to 2011. We measured the following medical event rates, per 10 000 ECT treatments, up to 7 and 30 days post-treatment: stroke, seizure, acute myocardial infarction, arrhythmia, pneumonia, pulmonary embolus, deep vein thrombosis, gastrointestinal bleeding, falls, hip fracture, and mortality. RESULTS: A total of 135 831 ECT treatments were delivered to 8810 unique patients. Overall medical event rates were 9.1 and 16.8 per 10 000 ECT treatments respectively. The most common medical events were falls (2.7 and 5.5 per 10 000 ECT treatments) and pneumonia (1.8 and 3.8 per 10 000 ECT treatments). Fewer than six deaths occurred on the day of an ECT treatment. This corresponded to a mortality rate of less than 0.4 per 10 000 treatments. Deaths within 7 and 30 days of an ECT treatment, excluding deaths due to external causes (e.g., accidental and intentional causes of death), were 1.0 and 2.4 per 10 000 ECT treatments respectively. CONCLUSION: Morbidity and mortality events after ECT treatments were relatively low, supporting ECT as a low-risk medical procedure.


Subject(s)
Accidental Falls/statistics & numerical data , Cardiovascular Diseases/epidemiology , Electroconvulsive Therapy/statistics & numerical data , Gastrointestinal Hemorrhage/epidemiology , Hip Fractures/epidemiology , Lung Diseases/epidemiology , Seizures/epidemiology , Adolescent , Adult , Aged , Aged, 80 and over , Causality , Cohort Studies , Electroconvulsive Therapy/adverse effects , Electroconvulsive Therapy/mortality , Female , Humans , Male , Middle Aged , Ontario/epidemiology , Young Adult
5.
Qual Life Res ; 22(10): 2877-88, 2013 Dec.
Article in English | MEDLINE | ID: mdl-23605935

ABSTRACT

PURPOSE: Heart diseases are often associated with residual injuries, persisting functional restrictions, and long-term sequelae for psychosocial development. Currently, there are no disease-specific instruments to assess the health-related quality of life (HrQoL) of pre-school children. The aims of this study were to develop a parent proxy instrument to measure the HrQoL of children aged 3-7 years with a heart disease and to confirm its validity and reliability. METHODS: Items from the Preschool Pediatric Cardiac Quality of Life Inventory (P-PCQLI) were generated through focus groups of caregivers. In a pilot study, comprehensibility and feasibility were tested. Five subdimensions were defined theoretically. Psychometric properties were analysed within a multicentre study with 167 parental caregivers. RESULTS: The final 52-item instrument contains a total score covering five moderately inter-correlated dimensions. The total score of the questionnaire showed a very high internal consistency (Cronbachs' α = 0.95). Test-retest correlation was at r tt = 0.96. External validity was indicated by higher correlations (r = 0.24-0.68) with a generic paediatric quality of life questionnaire (KINDL) compared to the Strengths and Difficulties Questionnaire (r = 0.17 to 0.59). Low P-PCQLI total scores were significantly associated with inpatient as opposed to outpatient treatment (t = 6.04, p < .001), with at least moderate disease severity ((t = 5.05, p < .001) NYHA classification) and with poorer prognosis (t = 5.53, p < .001) as estimated by the physician. CONCLUSIONS: The P-PCQLI is reliable and valid for pre-school children with a heart disease. It could be used as a screening instrument in routine care, and for evaluation of HrQoL outcomes in clinical trials and intervention research.


Subject(s)
Health Status Indicators , Heart Diseases/psychology , Parents/psychology , Psychometrics/methods , Quality of Life/psychology , Surveys and Questionnaires/standards , Adult , Caregivers/psychology , Child , Child, Preschool , Chronic Disease/psychology , Feasibility Studies , Female , Heart Diseases/physiopathology , Humans , Hungary , Male , Pediatrics , Proxy , Psychometrics/instrumentation , Reproducibility of Results , Schools , Sickness Impact Profile
6.
Klin Padiatr ; 223(3): 152-8, 2011 May.
Article in English | MEDLINE | ID: mdl-21462102

ABSTRACT

BACKGROUND: Survivors of pediatric cancer are at increased risk for medical and psychosocial late effects. This study retrospectively investigated the utilization of oncological and psychosocial care by former adolescent cancer patients (≥ 5 years since cancer diagnosis) in Germany. PATIENTS: Based on data of the German Childhood Cancer Registry (N=1 876 survivors of cancer with an age at diagnosis between 15 and 18 years), the study cohort comprised 820 survivors of adolescent cancer (time since diagnosis: M=13.7, SD=6.0, age at follow-up: M=30.4, SD=6.0 years). METHOD: Survivors of adolescent cancer completed standardized questionnaires measuring symptoms of posttraumatic stress, depression and anxiety as well as items on their utilization of medical and psychosocial care. RESULTS: More than a quarter (26.2%) of the survivors was no longer attending regular oncological follow-up assessments. Less than half of the survivors (44.4%) had received psychosocial care, mostly during their in-patient cancer treatment and their post-acute rehabilitation phase. Out of 184 survivors showing clinically relevant symptoms of posttraumatic stress, anxiety and/or depression at time of the study, 12.0% received psychosocial care and 13.6% took psychotropic medication. CONCLUSION: It should be studied further why only a small proportion of the survivors showing clinically relevant symptoms received psychosocial or psychopharmacological treatment. Systematic oncological follow-up assessments should take psychological late effects into greater account.


Subject(s)
Aftercare/statistics & numerical data , Anxiety Disorders/rehabilitation , Community Mental Health Services/statistics & numerical data , Depressive Disorder/epidemiology , Depressive Disorder/rehabilitation , Neoplasms/psychology , Neoplasms/rehabilitation , Stress Disorders, Post-Traumatic/rehabilitation , Survivors/psychology , Survivors/statistics & numerical data , Adolescent , Adult , Anxiety Disorders/diagnosis , Anxiety Disorders/epidemiology , Anxiety Disorders/psychology , Attitude to Death , Depressive Disorder/diagnosis , Depressive Disorder/psychology , Drug Utilization/statistics & numerical data , Female , Germany , Hospitalization/statistics & numerical data , Humans , Longitudinal Studies , Male , Personality Assessment , Psychotropic Drugs/therapeutic use , Registries/statistics & numerical data , Rehabilitation Centers/statistics & numerical data , Stress Disorders, Post-Traumatic/diagnosis , Stress Disorders, Post-Traumatic/epidemiology , Stress Disorders, Post-Traumatic/psychology , Utilization Review/statistics & numerical data , Young Adult
7.
Phys Rev Lett ; 103(14): 141802, 2009 Oct 02.
Article in English | MEDLINE | ID: mdl-19905561

ABSTRACT

We report on the first axion search results from the Cryogenic Dark Matter Search (CDMS) experiment at the Soudan Underground Laboratory. An energy threshold of 2 keV for electron-recoil events allows a search for possible solar axion conversion into photons or local galactic axion conversion into electrons in the germanium crystal detectors. The solar axion search sets an upper limit on the Primakov coupling g(agammagamma) of 2.4x10(-9) GeV-1 at the 95% confidence level for an axion mass less than 0.1 keV/c2. This limit benefits from the first precise measurement of the absolute crystal plane orientations in this type of experiment. The galactic axion search analysis sets a world-leading experimental upper limit on the axioelectric coupling g(aee) of 1.4x10(-12) at the 90% confidence level for an axion mass of 2.5 keV/c2.

8.
J Mater Sci Mater Med ; 20(7): 1495-503, 2009 Jul.
Article in English | MEDLINE | ID: mdl-19259790

ABSTRACT

Chitosan crosslinked with glutaraldehyde or oxidised dextran was studied as a potential scaffold material in tissue engineering for cartilage regeneration. By mixing two solutions of both components it became a gel, which was frozen. After lyophilization a scaffold was generated with interconnected pores with diameters ranging between 120-350 microm. The mechanical properties (yielding point, elastic and viscous moduli), absolute porosity, pore morphology were determined depending on the ratio of chitosan to crosslinker. ATDC5 (murine cell line) and bovine articular chondrocytes (primary cells) were cultured for 14 days on the scaffolds. Cultivation with ATDC5 cells and bovine chondrocytes showed no negative influence of glutaraldehyde on cell vitality and growth.


Subject(s)
Cartilage, Articular/growth & development , Chitosan/chemistry , Chondrocytes/physiology , Dextrans/chemistry , Glutaral/chemistry , Glutaral/pharmacology , Tissue Engineering/methods , Animals , Biocompatible Materials/chemistry , Cartilage, Articular/cytology , Cattle , Cell Culture Techniques/methods , Cells, Cultured , Chondrocytes/cytology , Cross-Linking Reagents/chemistry , Materials Testing , Mice , Oxidation-Reduction
9.
Phys Rev Lett ; 102(1): 011301, 2009 Jan 09.
Article in English | MEDLINE | ID: mdl-19257177

ABSTRACT

We report results from the Cryogenic Dark Matter Search at the Soudan Underground Laboratory (CDMS II) featuring the full complement of 30 detectors. A blind analysis of data taken between October 2006 and July 2007 sets an upper limit on the weakly interacting massive particle (WIMP) nucleon spin-independent cross section of 6.6x10;{-44} cm;{2} (4.6x10;{-44} cm;{2} when combined with previous CDMS II data) at the 90% confidence level for a WIMP mass of 60 GeV/c;{2}. This achieves the best sensitivity for dark matter WIMPs with masses above 44 GeV/c;{2}, and significantly restricts the parameter space for some favored supersymmetric models.

10.
Physiol Res ; 58(3): 363-372, 2009.
Article in English | MEDLINE | ID: mdl-18637707

ABSTRACT

Recent observations suggest the presence of 20S proteasomes (20S) in the lung epithelial lining fluid. However, the physiological relevance of 20S in the alveolar space and possible contribution to disease processes are unknown. Thus, we evaluated whether extracellular proteasomes could have a pathophysiological role in the injured lung using a rat model of lung contusion (LC). Bronchoalveolar lavage fluids (BALF) were obtained at various time points for up to 168 h after LC or sham procedure. Enzyme activities, ELISA and Western blots indicated enzymatically active 20S, the 19S subunit Rpt5 and ubiquitin in BALF. 20S and ubiquitin increased significantly after LC, peaked at 24 h and normalized within 168 h. Mg(2+)/ATP-dependent peptidase activities were detectable 6-24 h after LC. BALF after LC also contained ubiquitin-protein-ligase activity. Addition of Mg(2+)/ATP to BALF after LC led to significant proteolysis and could be prevented with epoxomicin and EDTA. These data suggest for the first time that the Mg(2+)/ATP-dependent 26S proteasome complex exists outside the cell, is released into the lung epithelial lining fluid after LC and contributes to the proteolysis of the bulk of protein in the alveolar space of the injured lung. We infer that proteasome complexes may have a pathophysiological role during lung edema clearance.


Subject(s)
Bronchi/enzymology , Contusions/enzymology , Lung Injury/enzymology , Proteasome Endopeptidase Complex/metabolism , Pulmonary Alveoli/enzymology , Adenosine Triphosphate/metabolism , Animals , Bronchi/pathology , Bronchoalveolar Lavage Fluid/chemistry , Contusions/pathology , Disease Models, Animal , Lung Injury/pathology , Magnesium/metabolism , Male , Proteins/metabolism , Pulmonary Alveoli/pathology , Rats , Rats, Sprague-Dawley , Time Factors , Ubiquitin/metabolism
11.
Phys Rev Lett ; 96(1): 011302, 2006 Jan 13.
Article in English | MEDLINE | ID: mdl-16486434

ABSTRACT

We report new results from the Cryogenic Dark Matter Search (CDMS II) at the Soudan Underground Laboratory. Two towers, each consisting of six detectors, were operated for 74.5 live days, giving spectrum-weighted exposures of 34 (12) kg d for the Ge (Si) targets after cuts, averaged over recoil energies 10-100 keV for a weakly interacting massive particle (WIMP) mass of 60 GeV/c2. A blind analysis was conducted, incorporating improved techniques for rejecting surface events. No WIMP signal exceeding expected backgrounds was observed. When combined with our previous results from Soudan, the 90% C.L. upper limit on the spin-independent WIMP-nucleon cross section is 1.6 x 10(-43) cm2 from Ge and 3 x 10(-42) cm2 from Si, for a WIMP mass of 60 GeV/c2. The combined limit from Ge (Si) is a factor of 2.5 (10) lower than our previous results and constrains predictions of supersymmetric models.

12.
Phys Rev Lett ; 84(25): 5699-703, 2000 Jun 19.
Article in English | MEDLINE | ID: mdl-10991035

ABSTRACT

The Cryogenic Dark Matter Search (CDMS) employs Ge and Si detectors to search for weakly interacting massive particles (WIMPs) via their elastic-scattering interactions with nuclei while discriminating against interactions of background particles. CDMS data, accounting for the neutron background, give limits on the spin-independent WIMP-nucleon elastic-scattering cross section that exclude unexplored parameter space above 10 GeV/c2 WIMP mass and, at >75% C.L., the entire 3sigma allowed region for the WIMP signal reported by the DAMA experiment.

13.
Radiology ; 216(3): 683-91, 2000 Sep.
Article in English | MEDLINE | ID: mdl-10966695

ABSTRACT

PURPOSE: To evaluate changes in hydrogen 1 magnetic resonance (MR) spectroscopic findings in overt or subclinical hepatic encephalopathy (HE) after liver transplantation and to compare these changes with clinical outcomes and basal ganglia high signal intensity (BGH). MATERIALS AND METHODS: Twenty-two patients scheduled for liver transplantation and 17 healthy control subjects were examined with (1)H MR spectroscopy and standard nonenhanced MR imaging. Eight patients underwent complete MR imaging and (1)H spectroscopic examinations before liver transplantation and at 3-4-week, 12-28-week, and 10-12-month follow-up after liver transplantation. RESULTS: Before liver transplantation, typical (1)H spectroscopic changes-decreased myo-inositol (mI)/creatine (Cr) and choline (Cho)/Cr ratios and an elevated glutamine and glutamate (Glx)/Cr ratio-were found in 21 patients. Eighteen patients had BGH at T1-weighted imaging. Three to 7 months after liver transplantation, the mI/Cr and Glx/Cr ratios were within the normal range in five of eight and eight of eight patients, respectively, without any residual signs of subclinical or overt HE; however, at MR imaging, seven patients still had BGH. CONCLUSION: After successful liver transplantation, renormalization of HE-specific brain metabolite changes is detected at (1)H spectroscopy and precedes the disappearance of BGH. The neuropsychologic signs of subclinical or overt HE follow the changes seen at (1)H spectroscopy rather than those seen at MR imaging.


Subject(s)
Basal Ganglia/physiopathology , Energy Metabolism/physiology , Hepatic Encephalopathy/physiopathology , Liver Transplantation/physiology , Magnetic Resonance Imaging , Magnetic Resonance Spectroscopy , Adult , Basal Ganglia/pathology , Choline/metabolism , Creatine/metabolism , Female , Glutamic Acid/metabolism , Glutamine/metabolism , Hepatic Encephalopathy/diagnosis , Hepatic Encephalopathy/surgery , Humans , Inositol/metabolism , Male , Middle Aged , Neuropsychological Tests , Reference Values
14.
Semin Oncol ; 26(2 Suppl 6): 48-54, 1999 Apr.
Article in English | MEDLINE | ID: mdl-10598555

ABSTRACT

The mechanism of action of an antifolate may be investigated using a variety of experimental methods. These include experiments in a cell culture setting to observe possible protection against drug effects afforded by the end products of metabolic pathways, assessing the activity of purified target enzymes in the presence of the antifolate, and, finally, the measurement of drug effects on intracellular folate and nucleoside triphosphate pools. The current discussion is focused on studies using CCRF-CEM leukemia cells that were designed to compare and contrast mechanisms of action of the antifolates methotrexate, which is primarily a dihydrofolate reductase inhibitor, raltitrexed, a thymidylate synthase inhibitor, LY309887, a glycinamide ribonucleotide formyltransferase inhibitor, and MTA (multitargeted antifolate), which is a novel antifolate antimetabolite. The results of these studies support the hypothesis that MTA affects multiple enzymatic targets and has a distinct mechanism of action from methotrexate, raltitrexed, and LY309887.


Subject(s)
Acid Anhydride Hydrolases/drug effects , Antimetabolites, Antineoplastic/pharmacology , Deoxyribonucleotides/metabolism , Enzyme Inhibitors/pharmacology , Folic Acid Antagonists/pharmacology , Glutamates/pharmacology , Guanine/analogs & derivatives , Thymidylate Synthase/antagonists & inhibitors , Thymine Nucleotides/metabolism , Acid Anhydride Hydrolases/metabolism , Folic Acid/drug effects , Folic Acid/metabolism , Guanine/pharmacology , Humans , Methotrexate/pharmacology , Nucleoside-Diphosphate Kinase/metabolism , Nucleoside-Triphosphatase , Pemetrexed , Quinazolines/pharmacology , Ribonucleotides/metabolism , Tetrahydrofolates/pharmacology , Thiophenes/pharmacology , Tumor Cells, Cultured
15.
Rofo ; 170(3): 298-303, 1999 Mar.
Article in German | MEDLINE | ID: mdl-10230440

ABSTRACT

BACKGROUND AND AIMS: In chronic liver dysfunction with portal hypertension the risk of variceal bleeding can be lowered by intrahepatic portosystemic shunting (TIPS). Although less pronounced than in surgical shunting, hepatic encephalopathy (HE) is a well-known undesired side effect. In cerebral proton MR spectroscopy (MRS), HE can be detected by a specific pattern of brain metabolite changes (increase of glutamine/glutamate (Glx) and decrease of myo-inositol (ml) and choline (Cho)). The aim of this study was to examine whether, after TIPS implantation, there is a correlation of the reduction of the portosystemic pressure gradient (PSPG) and the cerebral metabolite changes and their correspondence to the clinical status. METHODS: We examined 10 cirrhotic patients (Child B, C) before and 3-20 days after TIPS implantation. Clinical examination was performed by a senior hepatologist. Localized MR spectra were acquired in parieto-occipital gray/white matter using a short echo time (TE = 5 ms) STEAM sequence. RESULTS: After TIPS we found an increase of Glx/(Cr + PCr) of 13%-40% and a decrease of ml(Cr + PCr) of 6%-46% with a positive (Glx: r = 0.71) respectively negative (ml: r = -0.59) correlation to the reduction of the PSPG. 7/10 patients with a reduction of the PSPG of more than 9 mmHg (9-17 mmHg) showed a clinical impairment of their HE. CONCLUSIONS: Short echo time cerebral MRS allows detection of finest HE specific metabolite changes and can therefore contribute positively to an individually optimized reduction of the PSPG during TIPS implantation.


Subject(s)
Brain/metabolism , Portasystemic Shunt, Transjugular Intrahepatic , Adult , Aged , Female , Hepatic Encephalopathy/diagnosis , Hepatic Encephalopathy/metabolism , Humans , Liver Cirrhosis/metabolism , Liver Cirrhosis/surgery , Magnetic Resonance Imaging/instrumentation , Magnetic Resonance Imaging/methods , Magnetic Resonance Spectroscopy/methods , Male , Middle Aged , Postoperative Period , Time Factors
16.
N Engl J Med ; 340(7): 565; author reply 566, 1999 Feb 18.
Article in English | MEDLINE | ID: mdl-10026045
17.
Alcohol Clin Exp Res ; 23(1): 158-63, 1999 Jan.
Article in English | MEDLINE | ID: mdl-10029218

ABSTRACT

An increased daily alcohol consumption results in neurological symptoms and morphological central nervous system changes, e.g. shrinkage of the frontal lobes and the cerebellar vermis. Brain shrinkage can be due to neuronal loss, gliosis, or alterations of (cell) membrane constitutes/myelin. Neuronal, glial, and metabolic changes can be measured in vivo with proton magnetic resonance spectroscopy. A total of 11 alcoholics and 10 age-matched volunteers were examined by magnetic resonance imaging and localized magnetic resonance spectroscopy at an echo time of 135 and 5 msec. Peak integral values were calculated for N-acetylaspartate (NAA), choline (Cho), myo-inositol (ml), glutamate/glutamine (Glx), and normalized to phosphocreatine/creatine (Cr). Patients had a significant shrinkage of the cerebellar vermis. NAA/Cr and Cho/Cr ratios were reduced in both sequences, but the NAA/Cr reduction was only significant in long echo time, although the Cho/Cr reduction was significant in short echo time. The ml/Cr and Glx/Cr ratios did not show any significant difference between volunteers and patients. The decrease of NAA/Cr in alcohol dependent patients is consistent with neuronal loss. The Cho/Cr decrease and an unchanged ml/Cr may reflect cell membrane modification or myelin alterations in alcohol-dependent patients. These changes lead to brain shrinkage, although hydration effects and gliosis are less likely.


Subject(s)
Alcoholism/pathology , Cerebellum/pathology , Magnetic Resonance Imaging , Adult , Aged , Alcoholism/metabolism , Aspartic Acid/analogs & derivatives , Aspartic Acid/metabolism , Atrophy/pathology , Cerebellum/metabolism , Choline/metabolism , Female , Humans , Male , Middle Aged
18.
Klin Monbl Augenheilkd ; 213(3): 186-7, 1998 Sep.
Article in German | MEDLINE | ID: mdl-9793918

ABSTRACT

PURPOSE: To present an unusual acute tumour of the lid. PATIENT: A 37-year-old female physician presented with a swelling and a slight hematoma of the right lower lid. Palpation revealed two firm painless nodules under an intact epidermis. The patient confirmed that the lesion had developed within only one day without a preceding trauma. Both eyes were morphologically and functionally normal. Motility was free, and there was no exophthalmus. After three weeks the palpable nodules had not changed substantially. Magnetic resonance imaging disclosed a space-occupying lesion resembling an old hemorrhage or an inflammation. Excision was performed which revealed a thrombus-like structure. Histology showed multiple endothelium-lined vessels that were densely filled with erythrocytes, hemorrhages within the connective tissue, and old hemosiderin deposits. However, no thrombus could be found. CONCLUSION: Although thrombosis was the preferred diagnosis on clinical findings, histology led to the diagnosis of a spontaneous hemorrhage most likely caused by an occult vascular malformation.


Subject(s)
Arteriovenous Malformations/diagnosis , Eyelids/blood supply , Hemorrhage/diagnosis , Adult , Arteriovenous Malformations/pathology , Diagnosis, Differential , Endothelium, Vascular/pathology , Eyelids/pathology , Female , Hemorrhage/pathology , Humans , Thrombosis/diagnosis , Thrombosis/pathology
19.
Biomed Tech (Berl) ; 43(7-8): 202-9, 1998.
Article in German | MEDLINE | ID: mdl-9745805

ABSTRACT

AIMS: To assess trabecular density and structures at various cancellous bone sites in the foot using MR imaging. To compare the MRI findings with the bone mineral density (BMD) values obtained by quantitative computed tomography (QCT) in the lumbar vertebrae. MATERIALS AND METHODS: Bone mineral densities were measured by QCT in lumbar vertebrae 1 to 3 in ten patients with suspected osteoporosis. Sagittal images of the feet were obtained from these patients and also from seven healthy volunteers applying spin-echo and gradient-echo MR techniques. RESULTS: MR imaging revealed differences between patients with osteoporosis and individuals with healthy bones. Comparison of MR and BMD data revealed a significant dependence of MR data on the bone density measured by QCT (alpha < 0.05). In addition, a good correlation (r = 0.73) was found between the results of the different MRI techniques. CONCLUSIONS: The MR imaging techniques investigated offer an interesting tool for the non-invasive assessment of bony structures, at least at peripheral locations with yellow bone marrow. To enable reliable application in the clinical routine setting, additional studies are required to identify suitable standardized methods and establish normal ranges.


Subject(s)
Bone Density/physiology , Foot Bones/pathology , Magnetic Resonance Imaging , Osteoporosis/diagnosis , Adult , Aged , Female , Humans , Male , Middle Aged , Reference Values , Sensitivity and Specificity
20.
AJNR Am J Neuroradiol ; 19(7): 1373-7, 1998 Aug.
Article in English | MEDLINE | ID: mdl-9726485

ABSTRACT

PURPOSE: Late juvenile neuronal ceroid lipofuscinosis (NCL) is a lysosomal neurodegenerative disorder caused by the accumulation of lipopigment in neurons. Our purpose was to characterize the MR imaging and spectroscopic findings in three children with late infantile NCL. METHODS: Three children with late infantile NCL and three age-matched control subjects were examined by MR imaging and by localized MR spectroscopy using echo times of 135 and 5. Normalized peak integral values were calculated for N-acetylaspartate (NAA), choline, creatine, myo-inositol, and glutamate/glutamine. RESULTS: MR imaging revealed volume loss of the CNS, most prominently in the cerebellum. The T2-weighted images showed a hypointense thalamus and hyperintense periventricular white matter. Proton MR spectra revealed progressive changes, with a reduction of NAA and an increase of myo-inositol and glutamate/glutamine. In long-standing late infantile NCL, myo-inositol became the most prominent resonance. Lactate was not detectable. CONCLUSION: MR imaging in combination with proton MR spectroscopy can facilitate the diagnosis of late infantile NCL and help to differentiate NCL from other neurometabolic disorders, such as mitochondrial or peroxisomal encephalopathies.


Subject(s)
Magnetic Resonance Imaging , Magnetic Resonance Spectroscopy , Neuronal Ceroid-Lipofuscinoses/diagnosis , Aspartic Acid/analogs & derivatives , Aspartic Acid/analysis , Brain/metabolism , Brain/pathology , Brain Diseases/diagnosis , Case-Control Studies , Cerebellum/metabolism , Cerebellum/pathology , Cerebral Ventricles/metabolism , Cerebral Ventricles/pathology , Child , Child, Preschool , Choline/analysis , Creatine/analysis , Diagnosis, Differential , Female , Glutamic Acid/analysis , Glutamine/analysis , Humans , Inositol/analysis , Lactates/analysis , Male , Mitochondrial Encephalomyopathies/diagnosis , Neuronal Ceroid-Lipofuscinoses/metabolism , Neuronal Ceroid-Lipofuscinoses/pathology , Peroxisomal Disorders/diagnosis , Thalamus/metabolism , Thalamus/pathology
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