A. Pratesi

Whole-Brain Histogram and Voxel-Based Analyses of Diffusion Tensor Imaging in Patients with Leukoaraiosis: Correlation with Motor and Cognitive Impairment

BACKGROUND AND PURPOSE: Cerebral white matter changes, termed leukoaraiosis (LA), appearing as areas of increased signal intensity in T2-weighted MR images, are common in elderly subjects, but the possible correlation of LA with cognitive or motor deficit has not been established. We hypothesized that histogram and voxel-based analyses of whole-brain mean diffusivity (MD) and fractional anisotropy (FA) maps calculated from diffusion tensor imaging (DTI) could be more sensitive tools than visual scales to investigate the clinical correlates of LA. MATERIALS AND METHODS: Thirty-six patients of the Leukoaraiosis and Disability Study were evaluated with fluid-attenuated inversion recovery for LA extension, T1-weighted images for volume, and DTI for MD and FA. The extent of LA was rated visually. The normalized total, gray, and white matter brain volumes were computed, as well as the 25th percentile, 50th percentile, kurtosis, and skewness of the MD and FA maps of the whole brain. Finally, voxel-based analysis on the maps of gray and white matter volume, MD, and FA was performed with SPM2 software. Correlation analyses between visual or computerized data and motor or neuropsychologic scale scores were performed using the Spearman rank test and the SPM2 software. RESULTS: The visual score correlated with some MD and FA histogram metrics (P < .01). However, only the 25th and 50th percentiles, kurtosis, and skewness of the MD and FA histograms correlated with motor or neuropsychologic deficits. Voxel-based analysis revealed a correlation (P < .05 corrected for multiple comparisons) between a large cluster of increased MD in the corpus callosum and pericallosal white matter and motor deficit. CONCLUSIONS: These results are consistent with the hypothesis that histogram and voxel-based analyses of the whole-brain MD and FA maps are more sensitive tools than the visual evaluation for clinical correlation in patients with LA.

Human herpesvirus 6-associated limbic encephalitis in adult recipients of unrelated umbilical cord blood transplantation.

Human herpesvirus 6-associated limbic encephalitis in adult recipients of unrelated umbilical cord blood transplantation

Early MR imaging and spectroscopy appearance of eIF2B-related leukoencephalopathy

Leukoencephalopathy with vanishing white matter (VWM) (OMIM 603896) is a progressive disease with autosomal recessive inheritance, linked to mutations in either of the five genes encoding the subunits of the eukaryotic translation initiation factor eIF2B.1,2 Before molecular genetic testing was available, the diagnosis of VWM could be suspected on the basis of MRI and proton MR spectroscopy (1H-MRS) findings, which characteristically show diffuse signal abnormality of the cerebral white matter with evidence of rarefaction and cystic degeneration.3,4 Here we report a patient with a childhood onset of ataxia and on MRI a diffuse leukoencephalopathy without rarefaction. Appearance of cysts within the affected white matter 15 years after onset prompted molecular genetic testing, which confirmed the diagnosis of VWM. The patients developmental milestones were normal. At age 9 years his gait was unstable and wide-based with frequent falls and he showed an intention tremor. MRI showed symmetric diffuse hyperintensity of the cerebral white matter on proton density images (figure). Laboratory workup for X-linked adrenoleukodystrophy, metachromatic leukodystrophy, mitochondrial dysfunction, and spinocerebellar ataxia types 1, 2, 3, and 6 was negative. At age 11 …

Bronchial artery embolization with detachable coils for the treatment of haemoptysis. Preliminary experience

Purpose . This paper reports our early experience in the treatment of haemoptysis with embolization of the bronchial arteries using detachable coilsMaterials and methods . Ten patients (mean age 45 years, range 23-83) with haemoptysis due to bronchoscopic biopsy of indeterminate lung nodules, lung cancer, tubercular bronchiectasis, cystic bronchiectasis or sarcoidosis underwent embolization of the bronchial arteries responsible for the bleeding using detachable coils. Patients were followed-up for a median of 14 monthsResults . In all patients the procedure halted the bronchial bleeding within 24 hours. Eight patients had no recurrence of haemoptysis. In one patient with lung cancer and another with sarcoidosis haemoptysis recurred within 1–3 months due to recruitment of additional feeding arteries and to a missed large feeding artery originating from the subclavian artery, which required a new procedureConclusions . Use of detachable coils for embolization of bronchial arteries in patients with haemoptysis is advantageous since it eliminates the risk of migration typical of other embolic materials and enables rapid and permanent vessel occlusionRiassuntoObiettivo . descrivere l’esperienza preliminare nel trattamento dell’emottisi mediante embolizzazione delle arterie bronchiali con spirali a distacco controllatoMateriali e metodi . Dieci pazienti (età media 45 anni, range 23-84) con emottisi causata da biopsia broncoscopia di noduli polmonari indeterminati, neoplasia polmonare, bronchiectasie tubercolari, bronchiectasie cistiche e sarcoidosi sono stati trattati con embolizzazione delle arterie responsabili del sanguinamento bronchiale utilizzando spirali a distacco controllato e seguiti con un follow-up medio di 14 mesiRisultati . In tutti i pazienti la procedura ha arrestato il sanguinamento bronchiale entro 24 ore. Otto pazienti non hanno avuto recidiva di emottisi. Un paziente con neoplasia polmonare ed un altro con sarcoidosi hanno presentato nuovi episodi di sanguinamento bronchiale che hanno richiesto una seconda procedura in un intervallo di tempo compreso tra 1 e 3 mesi, dovuti al reclutamento di nuovi apporti arteriosi e alla mancata embolizzazione di un apporto arterioso originante dall’arteria succlaviaConclusioni . L’impiego di spirali a distacco controllato per l’embolizzazione delle arterie bronchiali in pazienti con emottisi è vantaggioso in quanto elimina il rischio di migrazione proprio di altri materiali embolizzanti e permette una occlusione vascolare rapida e permanente

Computation of brain metabolite ratios in single-voxel proton MR spectroscopy: comparison between semiautomatic and automatic software

PurposeMetabolite ratios are the measurements most commonly utilised for clinical applications of brain proton magnetic resonance spectroscopy (1H-MRS) [1]. We evaluated the agreement between the metabolite ratios calculated with semiautomatic and automatic software.Materials and methodsTwo single-voxel spectra (3.375 ml) localised in the frontal grey matter (GM) and peritrigonal white matter (WM) were obtained in 20 healthy subjects by using a point-resolved proton spectroscopy sequence (PRESS, TE=144 ms). The spectra were processed using the semiautomatic software J-Magnetic Resonance User Interface (JMRUI) and the automatic software SpectroView. Agreement of the N-acetyl-aspartate (NAA)/creatine (Cr), NAA/choline (Cho) and Cho/Cr ratios calculated with the two methods was assessed by estimating the 95% limits of agreement (LAs) of the differences of the values obtained with the two software packages.ResultsMean values and standard deviations of NAA/Cr, Cho/Cr and NAA/Cho (semiautomatic//automatic software) were 1.99±0.53//1.73±0.36, 1.13±0.40//1.04±0.33, 1.85±0.62//1.89±0.69 for the GM and 2.24±0.41//2.37±0.27, 0.96±0.17//1.13±0.15, 2.37±0.43//2.11±0.23 for the WM. The 95% LAs were wider for GM spectra and ranged between −0.51, 0.17 for Cho/Cr in the WM and −1.54, 1.47 for NAA/Cho in the GM.ConclusionsThe difference between brain metabolite ratios calculated with the two software packages is not negligible and reflects spectral quality.RiassuntoObiettivoI rapporti fra metaboliti sono le misure più comunemente utilizzate nelle applicazioni cliniche della spettroscopia di risonanza magnetica di protoni (1H-MRS) dell’encefalo [1]. Abbiamo valutato l’accordo fra i rapporti dei metaboliti calcolati con un software semiautomatico e con un software automatico.Materiali e metodiIn 20 soggetti sani abbiamo ottenuto due spettri a voxel singolo (3,375 ml) localizzati nella sostanza grigia (SG) frontale e nella sostanza bianca (SB) peritrigonale utilizzando una sequenza PRESS (TE=144 ms). Gli spettri sono stati elaborati utilizzando il software semiautomatico JMRUI e il software automatico Spectro View. L’accordo fra i valori di N-acetil-aspartato(NAA)/creatina (Cr), NAA/colina (Cho) e Cho/Cr calcolati con i due metodi è stato stimato utilizzando i limiti di accordo (LA) al 95% delle differenze dei rapporti tra metaboliti calcolati con i due metodi.RisultatiI valori medi con deviazioni standard di NAA/Cr, Cho/Cr e NAA/Cho (software semiautomatico//automatico) erano 1,99±0,53//1,73±0,36, 1,13±0,40//1,04±0,33, 1,85±0,62//1,89±0,69 nella SG, 2,24±0,41//2,37±0,27, 0,96±0,17//1,13±0,15, 2,37±0,43//2,11±0,23 nella SB. I LA al 95% erano più ampi negli spettri ottenuti nella SG e si estendevano tra −0,51, 0,17 per Cho/Cr nella SB e −1,54, 1,47 per NAA/Cho nella SB.ConclusioniLa differenza tra i rapporti dei metaboliti calcolati con i due metodi non è trascurabile e dipende dalla qualità spettrale.

An unusual cause of cord compression: synovial cyst of the thoracic spine

A 71-year-old man with chronic back pain had acute pain exacerbation, followed in the next month by progressive weakness of the right thigh. Neurological examination revealed myelopathy with a sensory level at T11. Spinal MRI (fig 1) revealed a well demarcated oval shaped lesion contiguous with the right T11–T12 facet joint causing dislocation and compression of the spinal cord that showed a subtle hyperintensity on T2 weighted images (TR/TE 3500/120). The lesion …

Parkinsonism in polycythaemia vera probably due to manganism

Herrero Hernandez et al. reported postural tremor in one patient with polycythaemia vera which was tentatively attributed to manganese deposits in the basal ganglia and improved after therapy with a chelating agent. Herein we report a patient with polycythaemia vera who developed persistent parkinsonism and in whom MRI showed features consistent with manganism. In 1998 a 47-year-old man employee began to complain of headache and arterial hypertension that led to the diagnosis of polycythaemia vera, which were treated with repeated blood-lettings. The next year he began to complain of dysesthesia to the feet, nocturnal leg cramps, and unsteady gait. His wife reported that he was slightly confused. The neurological examination showed masked face, monotonous voice, rigidity of the neck and of the right upper arm, slightly ataxic gait with forward bent of the trunk and diffusely brisk tendon reflexes. The blood examination revealed an increase of the hematocrit (50.2%), and transferrin (504 mg/dL; NV 290-390 mg/dL) and a decrease of the sideremia (33 lg/dL; NV 50-150 lg/dL). The serum magnesium, copper and ammonium were within normal range. Serum or urine manganese was not assessed. Hepatic biopsy was negative for increased hepatic copper content. Cranial CT was negative. T1 weighted MR imaging revealed a symmetric hyperintensity of the basal ganglia, cerebral peduncles, red nucleus, quadrigeminal bodies, superior cerebellar peduncles, and dentate nuclei. He started levodopa therapy (100 mg/die) which was unsuccessful and interrupted few months later. Seven years later the clinical condition was substantially stable and the score of the Unified Parkinson’s Disease Rating Scale subscale 3 was 16. A control MRI showed progression of the T1 symmetric hyperintensity in the cerebral peduncels and evidence of the same signal change also in occipital white matter (Fig. 1). There was only a mild diffuse cerebellar atrophy. SPECT examination, obtained by injection of 111-185 MBq of [I]FP-CIT, showed only a mild decrease of dopamine transporter (DAT) density in the right putamen. Manganese level was finally assessed in blood and urine and it was within normal limits. The diagnosis of manganism in our patient was based on the clinical features, exclusion of Parkinson’s disease and Wilson’s disease, and on presence of the typical MRI appearance of symmetric hyperintensity in T1-weighted images of the basal ganglia and other brain structures caused by the paramagnetic properties of manganese. Although a direct demonstration of increased manganese in the serum of our patient was not available, the normal manganese levels observed at follow-up do not exclude the diagnosis since it is well known that, serum or urine levels correlate with actual, but not previous, levels of exposure. Manganese intoxication is usually caused by professional exposure as it typically occurs in miners, welders, and workers in steel industry, but it can also be observed in acute or chronic hepatic diseases and anemia. In particular, increased level of serum manganese is observed in anemia since sideropenia increases the intestinal absorption of manganese. We speculate that in our patient, the increased hematocrit and the frequent blood-lettings associated with polycythaemia vera could have determined manganese intoxication though sideropenia. Persistent parkinsonism is observed in chronic manganese exposure. Although polycythaemia secondary to central hypoventilation can be observed in patients with Parkinson’s disease, in 1977 Herishanu and Rosenberg described occurrence of L-dopa responsive parkinsonisms in three patients with polycythaemia vera, but did not suspect a role for manganism. Ours appears to be the first case of persistent parkinsonism in a patient with polycythaemia vera in whom manganism was documented by MRI and L-dopa was ineffective. Inefficacy of the L-dopa therapy is common in patients with manganese intoxication and is in agreement with the DATscan results in our patient showing only a mildly decreased uptake in the right putamen, despite having the disease for 7 years. This indirectly supports a presumable predominant post-synaptic site of damage of the nigro-striatal pathway in manganism as supported by the normal 6[F]fluorodopa uptake observed with position emission tomography in patients with manganese intoxication. However, our DATscan findings appear to be substantially at variance with the marked symmetric decrease of the 6[F]fluorodopa uptake recently reported in a further patient with parkinsonism due to manganism. However, the latter developed manganism on a background of chronic alcoholic hepatic disease, and it has been pointed out that different exposure mechanisms and factors related to the host metabolism might explain the heterogeneity of the nigro-striatal damage and its clinical expression observed in patients with manganism. Chelating agents have been recommended in patients with chronic manganese exposure, but they were not provided to our patient. In conclusion our observation indicates that permanent extrapyramidal signs with striatal and extra-striatal manganese deposition can complicate polycythaemia vera, probably through increase of the intestinal manganese absorption secondary to sideropenia. Published online 29 September 2008 in Wiley InterScience (www.interscience.wiley.com). DOI: 10.1002/mds.22319