MRI findings in Wernicke encephalopathy

During the past 7 years from January 1991 to November 1997, we experienced 31 cases of aortic root reconstruction utilizing Carrel patch method. Concomitant procedure were performed in 9 cases of them due to another cardiovascular disease. Complicated cardiovascular disease included 3 cases of ischemic heart disease, 3 cases mitral regurgitation and one case of Aortic arch aneurysm. Several concomitant procedures were performed; 5 cases of CABG, 2 cases of mitral annuloplasty, one case of CABG with mitral valve replacement and one case of aortic arch replacement. The mean extra corporeal circulation time was 190.6 +/- 39.3 minutes and aortic clamp time was 147.8 +/- 34.2 minutes in these 9 cases. There were no significant differences of operative results between the simple root reconstruction group and the concomitant procedure group. We concluded that the aortic root reconstruction using the Carrel patch method has few complications. Although further long-term follow-up is required, our experiences suggest that the aortic root reconstruction with the concomitant procedure can be carry out safely with the aid of appropriate assistance method.     

Pediatric sarcoidosis presenting with hypertensive encephalopathy

A 3-year-old girl with simultaneous presentation of hypertensive encephalopathy, subcutaneous nodules, hepatosplenomegaly and proteinuria was presented. The sarcoidosis was confirmed by histologic demonstration of non-caseating granulomas in the liver, left kidney, a cervical lymph node and subcutaneous nodules. The proteinuria resolved spontaneously. During the six-month period of steroid therapy, the renal mass and hepatosplenomegaly were dissolved but the cervical lymphadenopathy still persisted. The hypertensive state was difficult to control which required a multiple antihypertensive drug regimen.     

Imaging findings in renal hydatid disease

The aim of this work is to describe the image findings of renal hydatid disease, especially on MR. Four cases of echinococcal involvement of the kidney were retrospectively reviewed. All patients had intravenous urography (IVU) and US performed. Computed tomography examination was available in three patients and MR in two cases. Intravenous urography demonstrated communication of the cyst to the collecting system in one case. Ultrasound revealed multicystic appearance in three cases and unilocular in one case. Computed tomography demonstrated unilocular thick-walled or multilocular cysts with well-defined walls, calcified in one case. In multilocular cysts the CT densities of the fluid of daughter cysts was significantly lower than the fluid of mother cysts. This typical appearance was present in three of our cases. The presence of a hypointense rim and a multicystic appearance were distinctive in MR imaging. The combined findings of these different imaging modalities aid greatly in establishing the correct diagnosis. Magnetic resonance imaging is of value in determining the presence of a characteristic rim and enables the evaluation of anatomical relationships.     

Diffusion-weighted MR imaging in hypertensive encephalopathy: clues to pathogenesis

We investigated the effects of organic buffers on the NO-like biological activities of ONOO-. In HEPES buffer (50 mM), ONOO- (1 mM) induced a 20-fold increase in endothelial cGMP accumulation and the effect was comparable to that elicited by a maximally active concentration of the NO donor DEA/NO. ONOO- produced a 12-fold increase of cGMP in MOPS buffer (50 mM), but was virtually inactive in phosphate buffer (50 mM). Electrochemical detection of NO showed that the biological effects of ONOO- in HEPES or MOPS were due to accumulation of compounds that released NO in the presence of copper ions. CuCl2-induced formation of NO was completely blocked by the Cu(I) chelator neocuproine but unaffected by the Cu(II) chelator cuprizone, pointing to a Cu(I)-catalyzed decomposition pathway. Formation of NO from ONOO- was not detectable in phosphate buffer, in agreement with the lack of effect of ONOO- on cGMP accumulation in this buffer. These data demonstrate that certain buffer components present in cell culture media may yield artificial results in experiments with authentic ONOO-.     

Solvent-induced toxic encephalopathy: electrophysiological data in relation to neuropsychological findings

Male subjects with type 2A (n = 12) and 2B (n = 12) solvent-induced toxic encephalopathy and a reference group of healthy men (n = 12) without previous solvent exposure were studied using quantitative EEG and event-related potentials from an odd-ball and a dual-task paradigm. Subjects with toxic encephalopathy of types 2A and 2B showed markedly lower P300 amplitudes than did controls in both paradigms. In the relatively complex dual-task setting, subjects with 2A and 2B showed lower signal detection than did controls.     

Reversible MR findings of hemolytic uremic syndrome with mild encephalopathy

We report the reversible MR findings in a 7-year-old girl with hemolytic uremic syndrome and mild encephalopathy. The splenium of the corpus callosum showed isointense to low signal intensity on T1-weighted images and high signal intensity on T2-weighted images, representing local edema. These findings returned to near normal on MR images obtained 1 week later. The patient recovered without CNS impairment.     

Cadmium encephalopathy: a report with elemental analysis and pathological findings

We report a boy of East Indian origin, aged 2 years and 10 months, who died suddenly and unexpectedly. Autopsy findings showed marked cerebral swelling with herniation and histological evidence of marked cerebral edema with perivascular protein leakage, indicating blood-brain barrier disruption. Energy dispersive X-ray microprobe analysis of the brain demonstrated the presence of cadmium and a marked increase in sulfur, predominantly intracellular, both within neuroglial, and to a lesser degree endothelial, cells. Localization was predominantly in the nucleus. Analysis of the kidney showed cadmium deposition in renal tubules and in the basal lamina of podocytes within the glomerulus. Although the environmental source of cadmium remains unknown, we speculate that acute cadmium toxicity led to brain intracellular accumulation with resultant cellular dysfunction, blood-brain barrier disruption, and lethal cerebral edema.     

Imaging findings in diffuse axonal injury after closed head trauma

OBJECTIVE: We compared the activity and tolerance profile of a 0.05% retinaldehyde cream with a 0.05% retinoic acid cream and the retinaldehyde vehicle in patients with photodamaged skin of the face. METHODS: A silicone replica of the left crow's feet area was taken at baseline and at weeks 18 and 44. Skin replicas were then analyzed by means of an optical profilometry technique. Standard wrinkle and roughness features were then calculated and statistically analyzed. The tolerance profile of the test products was also clinically evaluated during the entire study. RESULTS: A total of 125 patients (40 in the retinoic acid group, 40 in the retinaldehyde group, and 45 in the vehicle group) were studied. At week 18, a significant reduction of the wrinkle and roughness features was observed with both retinaldehyde and retinoic acid. At week 44, a less pronounced reduction was demonstrated in both active groups. No statistically significant changes were observed with the retinaldehyde vehicle at any assessment point. A total of 135 patients constituted the safety population. Retinaldehyde was well tolerated during the entire study. In contrast, retinoic acid caused more local irritation, and affected compliance of the patients. CONCLUSION: Retinaldehyde was efficacious and well tolerated in patients with photodamage.     

Hypoxic-ischaemic encephalopathy: early and late magnetic resonance imaging findings in relation to outcome

Sixteen infants with hypoxic-ischaemic encephalopathy (HIE) were studied using serial magnetic resonance imaging (MRI) up to the age of 2 years. The infants had regular neurological and developmental assessments. An nuclear magnetic resonance (NMR) score was devised to quantify the early and late MRI findings and a neurological optimality score was used to quantify abnormal neurological signs at the time of the final examination. The follow up MRI score was compared with the neonatal MRI score and the outcome of the child. There was a strong positive correlation between the neonatal and follow up MRI scores and between MRI scores and optimality score. All infants with a normal outcome had patchy white matter abnormalities. All infants with an abnormal outcome had extensive white matter abnormalities. The outcome was most severe in those infants with additional basal ganglia atrophy with or without cyst formation. Infants with mild HIE who are developmentally normal at the age of 2 years do not have normal MRI scans and may be at risk of minor neurological problems by school age. Bilateral basal ganglia abnormalities are associated with severe developmental delay, but infants with mainly white matter and cortical abnormalities have less severe problems despite extensive tissue loss.     

Neuropathological findings in cattle with clinically suspect but histologically unconfirmed bovine spongiform encephalopathy (BSE)

Neuropathological observations were made in 200 clinically suspected cases of bovine spongiform encephalopathy (BSE) in which pathognomonic vacuolar changes were absent. Routine histological and immunocytochemical techniques were applied to formalin-fixed, paraffin-embedded sections of the central nervous system. Significant neuropathological findings were detected in 85 (42.5 per cent) cases. The most frequent lesion, detected in 46 (23 per cent) cases, was a focal white matter vacuolation principally affecting the substantia nigra, but its clinical significance was unclear. Listeriosis was diagnosed in 17 (8.5 per cent) cases. In three of seven cases of non-suppurative encephalitis, lesions suggested sporadic bovine encephalomyelitis, a disease not previously reported in the UK. Suppurative thromboembolic or granulomatous lesions accounted for other inflammatory changes. Neuroectodermal tumours were present in five cases (2.5 per cent); three were identical in form and considered to be atypical ependymoma. Cerebrocortical necrosis, oedema or both were detected in four cases. The remaining cases (4.5 per cent), comprised those in which the changes were minor and of doubtful significance. Incidental pathological findings included occasional degenerating or vacuolated neurones, which occurred in the red nucleus in 105 brains, in the habenular nucleus in 71 brains, and singly at other sites in 17 brains. In sections of 37 brains immunostained with antiserum to prion protein (PrP), no evidence of PrP accumulation was found, providing some evidence that the series did not contain bovine prion disease cases which, based on the histological diagnosis, had given a false negative result. It is suggested that, of 115 cases (57.5 per cent) which lacked significant histological lesions, some were suffering from metabolic disorders. The study identified diseases and lesions which feature in the differential diagnosis of BSE. Their more accurate diagnosis may become particularly important if, as predicted, the BSE epidemic declines.     

Imaging findings in patients with-right lower quadrant pain: alternative diagnoses to appendicitis

Patients with right lower quadrant (RLQ) pain referred for imaging studies with a clinical diagnosis of appendicitis may have other pathologic conditions mimicking appendicitis. Appropriate diagnostic imaging may establish other specific diagnoses and thereby play a significant role in determining proper medical or surgical treatment. In this pictorial essay, we present a spectrum of imaging findings in patients whose clinical features were suggestive of appendicitis, but the diagnoses of a broad spectrum of other diseases were established with the imaging studies. The differential diagnoses of diseases mimicking appendicitis are reviewed.     

182 offspring of cows with bovine spongiform encephalopathy (BSE) in Switzerland. 1. Clinical findings

This study involved 182 calves, heifers and cows that were the first generation progeny of cows with bovine spongiform encephalopathy (BSE). All animals underwent clinical and neurological examinations. Blood, milk, urine, ruminal fluid and cerebrospinal fluid samples were examined. Each cow underwent a specific examination for BSE which included assessment of behaviour, sensitivity and locomotion. This examination was performed twice, and there was very good agreement between the results. The most common symptom was nervousness (14 animals). In the first examination, 68 animals were mildly to moderately sensitive to touching of the head, 44 animals were hypersensitive to a halogen light and 35 animals were hypersensitive to a camera flash. Twenty-four animals reacted by kicking when the hindlimbs were touched with a broom. None of the animals had disturbances in locomotion. Based on the interpretation of all findings and the comparison of the results of the first and second examinations, 173 animals were diagnosed as free of BSE. In 9 animals, the disease could not be ruled out; however, it could not be confirmed in any of them. Histological and immunohistochemical examinations of the brain of all animals were negative for BSE (see Part 2 of the study for details). The results of this study indicate that abnormal clinical findings may occur in clinically healthy cattle. However, none of the progeny of cows with BSE exhibited clinical signs typical of BSE, such as disturbances in behaviour; sensitivity and locomotion.     

Imaging of en bloc renal transplants: normal and abnormal postoperative findings

Escherichia coli possesses a hexameric citrate synthase that exhibits allosteric kinetics and regulatory sensitivity, and for which the gene (gltA) has previously been cloned and sequenced. A citrate-synthase-deficient strain of E. coli (K114) has been mutated to generate a revertant (K114r4) that produces a dimeric citrate synthase with altered kinetic and regulatory properties. On cloning and sequencing the gltA gene from both K114 and K114r4, a single mutation was found that caused the replacement of Asp362 with Asn. Asp362 has been previously shown to be a catalytically essential residue in E. coli citrate synthase, and we demonstrate that the hexameric enzyme produced on expression of the gltA gene from K114 and K114r4 is inactive. The dimeric citrate synthase from K114r4 has been purified and shown to be immunologically distinct from the wild-type hexameric enzyme. Determination of its N-terminal amino acid sequence demonstrates that the mutant citrate synthase is encoded by a gene distinct from the E. coli gltA gene. The N-terminal sequence is compared with those of other eukaryotic, eubacterial and archaebacterial citrate synthases.     

Early infantile epileptic encephalopathy and glycine encephalopathy

INTRODUCTION: Early infantile epileptic encephalopathy (EIEE) with suppression burst activity in EEG (Ohtahara syndrome) is a rare type of epileptic encephalopathy in infancy and represents the earliest type of age-related symptomatic generalized epilepsy. The main etiologic factors associated to EIEE are cerebral dysgenesia and metabolopathies, principally nonketotic hyperglycinemia. CLINICAL CASE: We report a neonate with EIEE secondary to glycine encephalopathy, diagnosed by increased of LCR/plasma glycine index.     

Central (interior) osteophytes of the distal femur. Imaging and pathologic findings

RATIONALE AND OBJECTIVES: To characterize central osteophytes (COs) of the distal femur, imaging features that enable their differentiation from intra-articular bodies were identified, and the frequency of overlying articular cartilage abnormalities was determined. METHODS: The authors inspected 133 distal femoral specimens and retrospectively reviewed knee radiographs of 48 symptomatic patients and magnetic resonance images of 60 symptomatic patients for presence of COs. The location and imaging features of COs, and their association with marginal osteophytes (MOs), were recorded. Articular cartilage abnormalities, if any, were documented in patients who underwent magnetic resonance imaging. RESULTS: Specimen analysis showed a 41% prevalence of COs, 95% association of COs with MOs, and predominance of large COs in the medial femoral condyle (81%). Radiographic analysis revealed a 9.8% prevalence of COs in the distal femur. Magnetic resonance images demonstrated a 14.3% prevalence of COs, with the majority (73%) accompanied by an overlying cartilage that had abnormal signal intensity or morphology, or both. CONCLUSIONS: Central osteophytes are a common finding in the distal femur. Based on their location and cortical continuity with the underlying bone, most COs can be differentiated from intra-articular bodies.     

Bovine spongiform encephalopathy (BSE): clinical aspects, duty of notification and consequences of positive findings

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