Magnetic resonance imaging of cerebral fat embolism: A case report

Fat embolism syndrome (FES) is one of the most important causes of morbidity and mortality following multiple fractures. Neurological involvement (cerebral fat embolism) has been reported frequently. A case of cerebral fat embolism is reported. While CT scan revealed no abnormalities, MRI, performed in this patient 8 days after trauma, showed relative lowintensity areas on T1-weighted images and high intensity areas on T2-weighted images involving cerebral white matter, corpus callosum and basal ganglia. MRI follow-up (1 and 3 months post-trauma) showed nearly complete resolution of the abnormal signal. MRI seems to be a useful diagnostic tool for detecting and quatifying lesions in fat embolism syndrome.     

Rapid detection of Flt3 mutations in acute myeloid leukemia patients by denaturing HPLC

BACKGROUND: fms-related tyrosine kinase 3 (Flt3) is the most commonly mutated gene in human acute myeloid leukemia (AML) and has been implicated in its pathogenesis. Because screening of Flt3 in AML patients by PCR followed by gel electrophoresis is time-consuming and fails to detect some very small internal tandem duplications (ITDs), we developed a method for screening of FLT3 receptor mutations with PCR plus denaturing HPLC (D-HPLC). METHODS: Total mRNAs extracted from 34 AML patients were first analyzed for the presence of juxtamembrane length mutations and tyrosine kinase domain point mutations by a conventional method involving PCR amplification, restriction enzyme digestion, and agarose gel electrophoresis (PCR-RED-AGE). Subsequently, the same patient panel was analyzed by D-HPLC, using specifically designed primers and optimized running temperatures for the length and point mutation analysis. RESULTS: Thirty-four patients were analyzed by PCR-RED-AGE; 9 were positive for known Flt3 mutations: 6 of 34 (18%) for ITDs in exon 14 and 3 of 34 (9%) for point mutations in exon 20. The same patient panel was analyzed by D-HPLC, and additional nucleotide changes were discovered; in total, 14 sequence variations were identified: 7 of 34 (21%) for ITDs in exon 14; 2 of 34 (6%) for point mutations in exon 20; 1 of 34 (3%) for a new point mutation in exon 16; and 4 of 34 (12%) for polymorphisms in exons 13 and 14. Direct sequencing analysis identified nucleotide alterations in each of the "D-HPLC positives" but in none of the "D-HPLC negatives", yielding a specificity and sensitivity of 100% for D-HPLC-based screening. CONCLUSIONS: This novel D-HPLC-based procedure, which is optimized for identification of new point mutations in the catalytic and regulatory domains of FLT3 receptor, could potentially be useful for studies involving precise genotype determination, which could be critical for selection of innovative AML therapies targeting the FLT3 protein.     

A phase IV open label study to assess the safety, tolerability and immunogenicity of a Haemophilus influenzae type B (Hib) CRM197 conjugated vaccine administered to healthy infants at 2, 4, and 6 months of age

The objective of this prospective clinical study was to evaluate the safety, tolerability and immunogenicity of Chiron Hib vaccine (Vaxem Hib) in Thai infants. This trial was conducted at Bhumibol Adulyadej Hospital, Bangkok, Thailand from June to November 1999. Three intramuscular injections of the vaccine were given to 119 infants at 2, 4 and 6 months of age. Reactions and adverse events after the vaccination were recorded. Blood samples for anti-PRP antibody were collected before the first immunization, and after the second and third immunizations. After the second dose, 91% and 58% of the subjects had anti-PRP antibody titers of > or =0.15 microg/ml and > or =1.0 microg/ml, respectively. After the third dose, 99% and 90% of the subjects had anti-PRP antibody titer > or =0.15 mcirog/ ml and > or =1.0 microg/ml, respectively. Local and systemic reactions were mild and transient. The study indicates that Vaxem Hib vaccine is safe and well tolerated. Three doses of the vaccine are necessary to achieve adequate protection in infants.     

Brain magnetic resonance imaging in Wolf-Hirschhorn syndrome

Wolf-Hirschhorn syndrome (WHS) is a rare genetic disorder, which is caused by partial deletion of the short arm of one chromosome 4. Brain magnetic resonance (MR) imaging findings are lacking. We report on brain findings in 10 children with WHS. We evaluated the MR imaging films of 10 subjects affected by WHS, which had been confirmed by genetic study. The age range at MR imaging was between 1 month and 9 years. In 9/10 cases enlargement of the third lateral ventricles was present. In 9/10 cases a global reduction of cerebral hemispheres white matter was present. In 10/10 cases diffuse thinning of the corpus callosum was visible; it was severe in 7/10 cases. In 5/10 cases small foci of T (2) hyper intense signal were visible within the subcortical white matter. In three of the six cases studied within the first year of life frontal periventricular cysts were present. In three of the four cases studied after the first year of life a squared shape of the frontal horns of the lateral ventricles was visible. The MR imaging findings reported in WHS cannot be considered pathognomonic of the syndrome, however, they may suggest WHS.     

Antioxidant responses in different body regions of the polychaeta Laeonereis acuta (Nereididae) exposed to copper

Antioxidant enzymes, total antioxidant capacity (TOSC) and concentration of reactive oxygen species (ROS) were measured in anterior (A), middle (M) and posterior (P) body regions of Laeonereis acuta after copper (Cu; 62.5 microg/l) exposure. A catalase (CAT) activity gradient observed in control group (lowest in A, highest in P) was not observed in Cu exposed group. Glutathione-S-transferase (GST) activity in A region of Cu group was higher than in A region of the control group. DNA damage (comet assay) was augmented in the A region of Cu group. Since copper accumulation was similar in the different body regions, sensitivity to copper in A regions seems to be related to lowest CAT activity. In sum, copper exposure lowered TOSC, a result that at least in part can be related to lowering of antioxidant enzymes like CAT. DNA damage was induced in the anterior region, where a lower CAT activity was observed.     

Pure representational neglect and navigational deficits in a case with preserved visuo-spatial working memory

We describe a patient who, after right hemisphere damage, showed severe, persistent, pure representational neglect but no evidence of perceptual neglect and no deficits in spatial working memory when evaluated with a traditional clinical test (Corsi Block Tapping test). This finding provides evidence against a full explanation of representational neglect within the context of visuo-spatial working memory. Indeed, this patient showed a peculiar deficit in navigational tasks requiring re-orientation in a novel environment by means of his mental representation of the environment. Since no representational neglect was observed in tests requiring mental representation of single or multiple objects (i.e., o'clock test) we suggest that in our patient representational neglect is caused by damage to the cognitive system involved in coding and storing environmental information to be used during navigation.     

Detecting malingering in traumatic brain injury and chronic pain: a comparison of three forced-choice symptom validity tests

Individual and joint malingering detection accuracy of the Portland Digit Recognition Test (PDRT), Test of Memory Malingering (TOMM), and Word Memory Test (WMT) was examined in traumatic brain injury (TBI; 43 non-malingering, 27 malingering) and chronic pain (CP; 42 non-malingering, 58 malingering) using a known-groups design. At published cutoffs, the PDRT and TOMM were very specific but failed to detect about 50% of malingerers; the WMT was sensitive but prone to false positive errors. ROC analyses demonstrated comparable accuracy across all three tests. Joint classification accuracy was superior to that of the individual tests. Clinical and research implications are discussed.     

Using the Wechsler Memory Scale-III to detect malingering in mild traumatic brain injury

This study examined the classification accuracy of the WMS-III primary indices in the detection of Malingered Neurocognitive Dysfunction (MND) in Traumatic Brain Injury (TBI) using a known-groups design. Sensitivity, specificity, and positive predictive power are presented for a range of index scores comparing mild TBI non-malingering (n = 34) and mild TBI malingering (n = 31) groups. A moderate/severe TBI non-malingering (n = 28) and general clinical group (n = 93) are presented to examine specificity in these samples. In mild TBI, sensitivities for the primary indices ranged from 26% to 68% at 97% specificity. Three systems used to combine all eight index scores were also examined and all achieved at least 58% sensitivity at 97% specificity in mild TBI. Specificity was generally lower in the moderate/severe TBI and clinical comparison groups. This study indicates that the WMS-III primary indices can accurately identify malingered neurocognitive dysfunction in mild TBI when used as part of a comprehensive classification system.     

Anti-brain antibodies in PANDAS versus uncomplicated streptococcal infection

The objective of this study was to assess brain involvement through the presence of antineuronal antibodies in Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcus (PANDAS) and in uncomplicated active Group A streptococcal infection. We compared serum antibrain antibody to human basal ganglia sections assessed by indirect tissue immunofluorescence in two groups: a PANDAS group, comprised of 22 patients (mean age 10.1 years; 20 male, 2 female) who met strict National Institutes of Mental Health diagnostic criteria for PANDAS and had clinically active tics or obsessive-compulsive disorder, or both; and a GABHS control group consisting of 22 patients (mean age 9.1 years; 15 mol/L, 7 female) with clinical evidence of active Group A beta-hemolytic streptococcal (GABHS) infection confirmed by throat culture and elevated antistreptolysin O titers but without history or clinical evidence of tics or obsessive-compulsive disorder. We observed positive anti-basal ganglia staining (defined as detectable staining at 1:10 serum dilution) in 14/22 patients in the PANDAS group (64%) but only 2/22 (9%) in the GABHS control group (P < 0.001, Fisher's exact test). These results suggest that antibrain antibodies are present in children with PANDAS that cannot be explained merely by a history of GABHS infection.