Dirty-appearing white matter in multiple sclerosis Preliminary observations of myelin phospholipid and axonal loss

“Dirty-appearing white matter” (DAWM) in multiple sclerosis (MS) is defined as a region(s) with ill-defined borders of intermediate signal intensity between that of normal-appearing white matter (NAWM) and that of plaque on T₂-weighted and proton density imaging. To delineate the histopathology of DAWM, four formalin-fixed cerebral hemisphere slices of three MS patients with DAWM were scanned with T₂- weighted and proton density sequences. The myelin water fraction (MWF) was obtained by expressing the short T₂ component as a fraction of the total T₂ distribution. Hemispheric sections were then stained with Luxol fast blue (LFB) for myelin phospholipids, for myelin basic protein (MBP) and 2’,3’-cyclic nucleotide 3’-phosphohydrolase (CNP) for myelin; Bielschowsky silver impregnation for axons; and for glial fibrillary acidic protein (GFAP) for astrocytes. Compared to NAWM, DAWM showed reduction in MWF, corresponding to a reduction of LFB staining. DAWM also showed reduced Bielschowsky staining. Quantitatively, the change in MWF in DAWM most consistently correlated with the change in LFB staining. The findings of this preliminary study suggest that DAWM is characterized by loss of myelin phospholipids, detected by the short T₂ component, and axonal reduction. † Deceased     

Pediatric functional magnetic resonance imaging (fMRI): issues and applications

Functional magnetic resonance imaging (fMRI) represents a useful tool for studying brain functions and the neural basis of cognition in healthy children and in those in disease states. Functional magnetic resonance imaging is a relatively new use of existing magnetic resonance imaging technology that allows scientists and practitioners to observe the brain at work. It is based on the observation that local increases in blood flow are related to neural activity. This review considers principles of functional magnetic resonance imaging, issues relevant to imaging children, and research using functional magnetic resonance imaging to examine cognitive processing in pediatric populations. The focus is specifically on language studies to review strengths, limitations, and practical applications of this technology with children. Future directions for functional magnetic resonance imaging are presented.     

Ascorbate decreases Fabry cerebral hyperperfusion suggesting a reactive oxygen species abnormality: an arterial spin tagging study

PURPOSE: To test the hypothesis that reactive oxygen species contribute to the cerebral hyperperfusion in Fabry disease. MATERIAL AND METHODS: We examined the effect of intravenous injection of ascorbate on cerebral blood flow (CBF) using magnetic resonance arterial spin tagging. Nineteen patients with Fabry disease and 15 control subjects were studied as part of a randomized double-blind placebo-controlled trial of enzyme replacement therapy (ERT). RESULTS: Vertebro-basilar hyperperfusion was observed in patients with Fabry disease. A decrease in systemic ascorbate levels relative to healthy controls was found in the patients. CBF decreased after ascorbate infusion in both control subjects and patients treated with ERT. The placebo group had a significantly delayed decrease in the CBF response after ascorbate infusion. Myeloperoxidase levels were elevated in Fabry patients, consistent with ongoing inflammatory processes in these patients. CONCLUSION: Increased CBF in Fabry disease may be related to increased production of reactive oxygen species, while low plasma ascorbate levels suggests a global redox imbalance. These abnormalities were improved by ERT. These observations have implications regarding oxidative processes contributing to accelerated atherosclerosis in Fabry disease.     

Renal artery aneurysms: diagnosis and surveillance with 3D contrast-enhanced magnetic resonance angiography

The use of contrast-enhanced magnetic resonance angiography (CE-MRA) in screening for suspected renovascular disease may result in increased detection of renal artery aneurysms. We report the CE-MRA findings at diagnosis and follow-up in nine hypertensive patients with unsuspected renal artery aneurysms. A search of renal CE-MRAs of suspected renal artery stenosis at two tertiary referral institutions over 5 1/2 years was performed. All patients underwent CE-MRA using a fast spoiled gradient echo technique (TR/TE/flip 5.1–6 ms/1.6 ms/40°), scan matrix 512×196–224, 1 excitation, FOV 400–450 mm×266–360 mm, 32–50 mm×1.5–2 mm interpolated slices. Gadolinium-enhanced 3D images were obtained during breath holding. Images were evaluated and post-processed on a workstation by a single operator. Nine patients with renal artery aneurysms out of a total of 912 cases were found, all involving the main artery or divisions proximal to the renal hilum. Renal arteries distal to the hilum were not consistently visualized. The aneurysm was bilobed in one patient, multilocular in another and unilocular in all others. Severe stenosis of the renal artery proximal to the aneurysm was present in two. Four patients underwent follow-up showing no change in aneurysm size. CE-MRA reliably identifies aneurysms involving the main renal arteries and proximal branches. Once diagnosed, CE-MRA offers a safe, non-invasive modality for surveillance of aneurysm if active intervention is not planned.     

Development and psychometric evaluation of the patient knowledge of,and attitudes and behaviours towards pressure ulcer prevention instrument (KPUP)

The Patient Knowledge of, and Attitude and Behaviour towards Pressure Ulcer Prevention Instrument (KPUP) was developed and validated using a two‐stage prospective psychometric instrument validation study design. In Stage 1, the instrument was designed, and it is psychometrically evaluated in Stage 2. To establish content validity, two expert panels independently reviewed each item for appropriateness and relevance. Psychometric evaluation included construct validity and stability testing of the instrument. The questionnaire was administered to a convenience sample of 200 people aged more than 65 years, living independently in the community; reliability and stability were assessed by test/retest procedures, with a 1‐week interval. Mean knowledge scores at ‘test’ were 11.54/20 (95% CI = 11.10‐11.99, SD: 3.07), and ‘retest’ was 12.24 (95% CI = 11.81‐12.66, SD: 2.93). For knowledge, correlation between the test/retest score was positive (r=. 60), attitude section‐inter‐item correlations ranged from r = −.31 to r = .57 (mean intraclass correlation coefficient of r = .42), and internal consistency for the retest was the same as the test (α = .41 for the eight items). For health behaviours, individual inter‐item correlations for test items ranged from r = −.21 to r = .41 for the 13 standardised items. Psychometric testing of the KPUP in a sample of older persons in the community provided moderate internal consistency and general high test‐retest stability.     

Iliac artery rupture related to balloon insertion for placenta accreta causing maternal hemorrhage and neonatal compromise

Purpose

The use of internal iliac artery balloons for prevention of hemorrhage in cases of placenta accreta is increasing. Most described complications of this technique are maternal and thromboembolic in nature. Complications related to vascular rupture are rare, their presentation is not well described, and the resultant neonatal consequences are infrequently reported.

Clinical features

A 35-yr-old term parturient with suspected placenta accreta underwent prophylactic endovascular placement of iliac balloons prior to Cesarean delivery. The patient complained of contraction-like pain during balloon placement, and an arterial wall tear was discovered after abdominal incision. This produced significant maternal bleeding and the birth of a neonate with an umbilical venous pH of 6.95 and Apgar scores of 3 and 7.

Conclusion

In addition to the known maternal risks, fetal risks must be considered when planning the placement of endovascular iliac balloons during pregnancy. We recommend continuous monitoring of maternal and fetal status when performing the procedure. Contraction-like pain during placement should raise the suspicion of arterial disruption.     

Plexiform neurofibromatosis of the mediastinum: CT appearance

We studied the CT findings in four patients with pathologically proved thoracic plexiform neurofibromatosis. In all four patients, CT showed an infiltrative process and masses that involved the mediastinum along the distribution of the sympathetic chains, phrenic, and vagus nerves. The lesions had lower attenuation values than did chest-wall muscle. In one patient, CT revealed calcifications and peripheral enhancement of nodular components after IV infusion of contrast material. In the appropriate clinical setting, CT detection of lesions in the distribution of the mediastinal nerves strongly favors the diagnosis of plexiform neurofibromatosis.     

Tumor vascular signals in renal masses: detection with Doppler US

The vascularity of 49 renal masses (26 malignant and 23 benign lesions) was investigated with duplex Doppler ultrasound. Doppler signals obtained at the margins of renal masses were defined as "tumor signals" when the Doppler-shifted frequency of the lesion exceeded the frequency shift in the ipsilateral main renal artery. These exceeded 2.5 kHz with a 3-MHz insonating frequency. Among the 26 renal masses that subsequently proved to be malignant, tumor signals were obtained in 15 of 18 (83%) untreated renal cell carcinomas, in three of four Wilms tumors, and in two patients with metastases to the kidney, but not in the one patient with lymphoma. None of the 23 benign renal masses demonstrated tumor signals. Tumor vascularity in malignant lesions gives rise to abnormal, high-velocity, Doppler-shifted signals that can help in the differential diagnosis of renal masses.     

The relationship between pulmonary artery pressure and pulmonary artery diameter in pulmonary hypertension

The pulmonary arteries dilate in response to many factors, principally increased pressure and flow. In patients who have pulmonary arterial hypertension but no increase in flow, we have compared main pulmonary artery size at computed tomography with pulmonary haemodynamic data obtained during right heart catheterisation. In patients with primary pulmonary hypertension and chronic thromboembolic pulmonary hypertension, dilatation correlated with raised pulmonary vascular resistance and reduced cardiac output but not with mean arterial pressure. In patients with chronic lung disease no correlations were shown though a trend between raised pressure and size was observed. We speculate that pulmonary artery compliance is an important factor which determines the degree of dilatation in response to raised pressure. Estimations of pressure cannot be made from measurements of pulmonary artery size without knowledge of the underlying lung disease.