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Background Clinical phenotypic heterogeneity represents a major barrier to trials in motor neuron disease (MND) and objective surrogate outcome measures are required, especially for slowly progressive patients. We assessed responsiveness of clinical, electrophysiological and radiological muscle-based assessments to detect MND-related progression. Materials and methods A prospective, longitudinal cohort study of 29 MND patients and 22 healthy controls was performed. Clinical measures, electrophysiological motor unit number index/size (MUNIX/MUSIX) and relative T2- and diffusion-weighted whole-body muscle magnetic resonance (MR) were assessed three times over 12 months. Multi-variable regression models assessed between-group differences, clinico-electrophysiological associations, and longitudinal changes. Standardized response means (SRMs) assessed sensitivity to change over 12 months. Results MND patients exhibited 18% higher whole-body mean muscle relative T2-signal than controls (95% CI 7–29%, p p p Conclusion MUNIX and relative T2-weighted MR represent objective surrogate markers of progressive denervation in MND. Radiological changes were maximal in leg muscles, irrespective of clinical onset-site.

Background Clinical phenotypic heterogeneity represents a major barrier to trials in motor neuron disease (MND) and objective surrogate outcome measures are required, especially for slowly progressive patients. We assessed responsiveness of clinical, electrophysiological and radiological muscle-based assessments to detect MND-related progression. Materials and methods A prospective, longitudinal cohort study of 29 MND patients and 22 healthy controls was performed. Clinical measures, electrophysiological motor unit number index/size (MUNIX/MUSIX) and relative T2- and diffusion-weighted whole-body muscle magnetic resonance (MR) were assessed three times over 12 months. Multi-variable regression models assessed between-group differences, clinico-electrophysiological associations, and longitudinal changes. Standardized response means (SRMs) assessed sensitivity to change over 12 months. Results MND patients exhibited 18% higher whole-body mean muscle relative T2-signal than controls (95% CI 7–29%, p

Diabetic neuropathy is a chronic and often disabling condition that affects a significant number of individuals with diabetes. Long considered a disease of the peripheral nervous system, there is now increasing evidence of central nervous system involvement. Recent advances in neuroimaging methods detailed in this review have led to a better understanding and refinement of how diabetic neuropathy affects the central nervous system. Recognition that diabetic neuropathy is, in part, a disease that affects the whole nervous system is resulting in a critical rethinking of this disorder, opening a new direction for further research.

MRI imaging has significantly improved the detection of brain lesions over the past few decades. It has high sensitivity to intracranial pathology but confident preoperative tissue diagnosis is relatively unusual. MR spectroscopy provides in-vivo biochemical information and has been used to improve the low specificity of tumour diagnosis. During the last decade there have been a number of reports making the case that proton spectroscopy can distinguish different grades of glial tumours and in some situations provide information on histological type. We report four children who presented with neurological symptoms and focal masses on MRI. MRS in each of them gave results consistent with textbook descriptions of malignancy, but in all four cases the abnormalities were subsequently shown to be due to demyelination. We reiterate that spectroscopic appearances are nonspecific and spectroscopic data should be evaluated in the light of concurrent imaging features and the clinical presentation.

The stereological method of cross-counting based on the Cavalieri principle is widely used in neuroimaging to estimate the volume of cerebral structures. Although superficially simple, the stereological technique is validated by arcane mathematical proofs, so the cross size is determined by most investigators on a pragmatic basis with the assumption that the volume calculated is independent of the cross size used. We used three cross sizes (8x8, 5x5 and 3x3 voxels) to estimate the brain volume of six healthy control subjects. The volume estimate using a cross size of 3x3 was 9% larger than with a cross size of 5x5 and the latter was 15% larger than with a cross size of 8x8. We conclude that cross size significantly affects whole brain volume estimates and this result is presumably applicable to other structures whose cross-sectional areas form complex shapes. Investigators should be aware of this fact, especially when trying to make direct comparisons between volume estimates derived from the stereological method using different cross sizes.

The first generation of digital subtraction MR angiography using thick-slab contrast-enhanced 2D projection techniques has confirmed the potential of MRI to produce noninvasive subsecond angiograms of the craniocervical circulation. As time-resolved techniques become more sophisticated and 3D acquisitions can be obtained with high isotropic spatial resolution we may start to see the demise of catheter angiography as a diagnostic procedure.

This study was carried out using MRI (proton density--and T2-weighted) on 16 HIV-negative controls, 9 symptom-free HIV-positive patients and 25 with CDC IV HIV disease. The studies from this last group had previously been allocated by a radiologist to the following categories: 8 with focal mass lesions and normal-appearing white matter; 9 with diffuse encephalopathy (high signal on T2-weighted images, affecting most or all of the white matter) and 8 with patchy encephalopathy (high signal affecting only one or two areas within the white matter). Moran's I, a statistic of spatial autocorrelation, was calculated for the grey-scale values of a sampled pixel array from a central white matter region of each of the images. All values of Moran's I calculated in this study showed a large positive excess over the expected value under randomisation, indicating highly significant positive auto-correlation in the spatial arrangement of the grey-scale values. On T2-weighted images a statistically significant increase in the mean value of Moran's I, compared with controls, was found in the diffuse encephalopathy group, indicating that quantifiable changes in the spatial autocorrelation of pixel data can be related to recognised qualitative changes in the appearance of white matter in subjects with HIV disease. A lesser, but significant, rise in the mean value of Moran's I was also found in the focal mass lesion group, suggesting that changes in spatial autocorrelation may indicate pathological change in advance of qualitative MRI changes.