Evaluation of the basal ganglia in neurofibromatosis type 1.

PURPOSE: Alterations of the brain microstructure and metabolism have been identified in patients with neurofibromatosis type 1 (NF1). In this study, we analyzed the basal ganglia of NF1 subjects without cognitive delay throughout a combined approach with magnetic resonance spectroscopy (MRS) and diffusion tensor imaging (DTI) in order to better define the metabolic and microstructural characteristics of these regions and, furthermore, to verify if metabolic and microstructural abnormalities may be present in normally developed NF1 patients. METHODS: A 3-T MRI with multivoxel MRS and DTI was performed in 14 NF1 patients and eight controls. N-acetyl-aspartate (NAA), choline (Cho), creatine (Cr) values and ratios, fractional anisotropy, and apparent diffusion coefficient (ADC) were calculated, for a total of four regions of interest (ROI) for each hemisphere. RESULTS: NF1 patients, compared to healthy controls, showed (a) decreased NAA in all the four ROI, (b) increased Cho and decreased Cr in three of the four ROI, (c) decreased NAA/Cho ratio in three ROI, and (d) increased ADC in all the four ROI. A trend of increased ADC was present in three of the four ROI of NF1 patients with unidentified bright objects (UBOs) and younger than 18 years. CONCLUSION: These data confirm the presence of neuroaxonal damage with myelin disturbances in NF1 patients. We showed that metabolic and microstructural anomalies can be present in the same time in NF1 patients without developmental delay or cognitive deficits. Relations between brain anomalies, UBOs, and cognitive functions need further studies.

Celiac disease: evaluation with dynamic contrast-enhanced MR imaging.

PURPOSE: To prospectively determine mural perfusion dynamics in patients with untreated celiac disease by using dynamic contrast material-enhanced magnetic resonance (MR) imaging and to compare these dynamics with those in a control population and in patients with celiac disease treated with a gluten-free diet. MATERIALS AND METHODS: Institutional review board approval and informed consent from all participants were obtained. Sixty consecutive patients with untreated celiac disease, 45 patients with celiac disease treated with a gluten-free diet for at least 1 year, and 30 control subjects were enrolled in this study. Dynamic contrast-enhanced MR imaging was performed by using a 1.5-T MR unit. For each MR imaging examination, maximum enhancement, slope of enhancement, and time-signal intensity curves were calculated at the level of the descending duodenal wall. Duodenal wall thickness was also evaluated. Statistical evaluation was performed by using one-way analysis of variance, and the results were confirmed by using the Bartlett test for equal variances and complemented by using Bonferroni multiple comparison, linear correlation, and the Student t test for paired data. RESULTS: Mean maximum enhancement of the duodenal wall was significantly higher in patients with untreated celiac disease (229.1 +/- 46.4 [standard deviation]) than in patients with treated celiac disease (109.8 +/- 27.8) and control subjects (94.7 +/- 17.9) (P < .001 for each comparison). All 60 untreated patients showed a curve characterized by fast enhancement and washout (type 4), while all 45 treated patients and the 30 control subjects showed a curve characterized by slow constant enhancement (type 2). Mean duodenal wall thickness was not significantly different between untreated patients (2.2 mm +/- 0.4), treated patients (2.0 mm +/- 0.3), and control subjects (2.0 mm +/- 0.4) (one-way analysis of variance, P = .4177; Bartlett test, P = .6951). CONCLUSION: The results of this study suggest that dynamic evaluation of the bowel wall by using contrast-enhanced MR imaging can be an effective and reproducible way to show the inflammation state in celiac disease.

Olfactory Dysfunction, Olfactory Bulb Volume and Alzheimer's Disease: Is There a Correlation? A Pilot Study1.

BACKGROUND: Olfactory dysfunction is present since the earliest stage of Alzheimer's disease (AD). In AD patients, the olfactory impairment has been correlated with atrophy of some structures of the olfactory system, but the role of the olfactory bulb remains unclear. OBJECTIVE: The aim of our work is to test if patients suffering from AD exhibit a statistically significant reduction of the average volume of the olfactory bulb (OBV) compared to healthy subjects. METHODS: 78 subjects were enrolled in the study and divided into three groups: 28 healthy elderly (22 females, 6 males, mean age 69.4 ± 9.2), 25 patients with mild cognitive impairment (MCI) amnestic type (14 females, 11 males, mean age 74.5 ± 7.5), and 25 mild AD patients (14 females, 11 males, mean age 73.7 ± 6.8). Every subject underwent an MRI study of the olfactory bulb and an olfactory assessment with the Sniffin' Stick Extended Test. RESULTS: The statistical analysis showed no correlation between the OBV and MCI or AD. Moreover, olfactory function and OBV were not correlated in any of the three groups. CONCLUSION: The reduction of OBV does not seem to represent an index of neuronal damage in the earliest stages of AD.