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PURPOSE: Moyamoya syndrome (MMS) is a rare intracranial arterial vasculopathy which can occur in neurofibromatosis type 1 (NF1) disease, representing a cause of cerebrovascular reserve (CVR) impairment, possibly leading to ischemic stroke. Here, we evaluated noninvasive imaging techniques used to assess CVR in MMS patients, describing clinical and imaging findings in patients affected by MMS-NF1. METHODS: Following strict inclusion and exclusion criteria, in this retrospective observational study, we evaluated imaging data of nine consecutive MMS-NF1 patients (M/F = 5/4, mean age: 12.6 ± 4.0). Subjects underwent a multimodal evaluation of cerebral vascular status, including intracranial arterial MR Angiography (MRA), MRI perfusion with dynamic susceptibility contrast (DSC) technique, and 99mTc-hexamethylpropyleneamine oxime (HMPAO) SPECT. RESULTS: In 8 out 9 patients (88.8%, 6/8 symptomatic), time-to-peak maps were correlated with the involved cerebral hemisphere, while in 6 out 9 patients (66.6%, 5/6 symptomatic), mean transit time (MTT) maps showed correspondence with the affected cerebrovascular territories. Cerebral blood flow (CBF) calculated using DSC perfusion failed to detect the hypoperfused regions instead identified by SPECT-CBF in all patients, while MTT maps overlapped with SPECT-CBF data in all cases and time-to-peak maps in 60.0%. CONCLUSIONS: Although SPECT imaging still represents the gold standard for CBF assessment, our results suggest that data obtained using DSC perfusion technique, and in particular MTT maps, might be a very useful and noninvasive tool for evaluating hemodynamic status in MMS-NF1 patients.
Asunto(s)
Enfermedad de Moyamoya , Neurofibromatosis 1 , Adolescente , Circulación Cerebrovascular , Niño , Humanos , Imagen por Resonancia Magnética , Enfermedad de Moyamoya/complicaciones , Enfermedad de Moyamoya/diagnóstico por imagen , Neurofibromatosis 1/complicaciones , Neurofibromatosis 1/diagnóstico por imagen , Tomografía Computarizada de Emisión de Fotón ÚnicoRESUMEN
We evaluated whether perfusion brain abnormalities by single-photon emission computed tomography (SPECT) imaging improves diagnostic and prognostic assessment in Sydenham chorea. Twenty-three children with acute autoimmune chorea underwent technetium-99m hexamethylpropyleneamine oxime brain SPECT imaging. In 16 children, SPECT was repeated during the follow-up. A pattern of basal ganglia hyperperfusion was observed in 20 (87%) patients. In 4 of 10 patients with generalized chorea, perfusion was comparable in right and left striatum and right and left thalamus. In 13 patients with hemi-chorea and in 3 with generalized chorea, unilateral hyperperfusion was detected. Three patients with generalized chorea had normal perfusion. Tracer uptake of basal ganglia of the patients at the acute phase was higher than at the follow-up ( P < .001). SPECT seems a useful noninvasive tool in pediatric patients with Sydenham chorea to support the clinicians during the acute phase of disease and to monitor the course of autoimmune chorea.
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Encéfalo/diagnóstico por imagen , Corea/diagnóstico por imagen , Adolescente , Ganglios Basales/diagnóstico por imagen , Niño , Femenino , Humanos , Masculino , Tálamo/diagnóstico por imagen , Tomografía Computarizada de Emisión de Fotón ÚnicoRESUMEN
PURPOSE: We aimed to investigate the integrity of the Resting State Networks in spinocerebellar ataxia type 2 (SCA2) and the correlations between the modification of these networks and clinical variables. METHODS: Resting-state functional magnetic resonance imaging (RS-fMRI) data from 19 SCA2 patients and 29 healthy controls were analyzed using an independent component analysis and dual regression, controlling at voxel level for the effect of atrophy by co-varying for gray matter volume. Correlations between the resting state networks alterations and disease duration, age at onset, number of triplets, and clinical score were assessed by Spearman's coefficient, for each cluster which was significantly different in SCA2 patients compared with healthy controls. RESULTS: In SCA2 patients, disruption of the cerebellar components of all major resting state networks was present, with supratentorial involvement only for the default mode network. When controlling at voxel level for gray matter volume, the reduction in functional connectivity in supratentorial regions of the default mode network, and in cerebellar regions within the default mode, executive and right fronto-parietal networks, was still significant. No correlations with clinical variables were found for any of the investigated resting state networks. CONCLUSIONS: The SCA2 patients show significant alterations of the resting state networks, only partly explained by the atrophy. The default mode network is the only resting state network that shows also supratentorial changes, which appear unrelated to the cortical gray matter volume. Further studies are needed to assess the clinical significance of these changes.
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Encéfalo/fisiopatología , Imagen por Resonancia Magnética/métodos , Red Nerviosa/fisiopatología , Ataxias Espinocerebelosas/fisiopatología , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Atrofia/patología , Encéfalo/patología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Red Nerviosa/patología , Descanso , Ataxias Espinocerebelosas/patología , Adulto JovenRESUMEN
BACKGROUND: This study was designed to assess the impact of obesity and acquisition protocol on (123)I-metaiodobenzylguanidine (MIBG) indexes of cardiac sympathetic innervation. METHODS: Forty-five patients with heart failure (HF) (38 men, age 58±15 years) underwent (123)I-MIBG cardiac imaging. Of these patients, 10 were obese [body mass index (BMI) ≥30 kg/m(2)]. Ten-minute planar images of the thorax in anterior view were performed 15 minutes ("early" image) and 3 hours and 50 minutes ("late" image) after tracer administration in both supine- and prone-position. Early and late (123)I-MIBG heart-to-mediastinum (H/M) ratios and washout rate were computed. RESULTS: In overall study population, early and late (123)I-MIBG H/M ratios and washout rate were comparable between supine- and prone-position acquisitions. Obese patients had a lower early and late (123)I-MIBG H/M ratios both in supine (P<0.01) and prone (P<0.05) positions compared to non-obese subjects. CONCLUSIONS: Our results indicate that in HF patients, obesity has a significant impact on (123)I-MIBG indexes of cardiac sympathetic innervation. Prone-position did not change early and late (123)I-MIBG H/M ratios and washout rate compared to supine position both in obese and non-obese HF patients.
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Previous MRI studies of functional connectivity in pre-symptomatic mutation carriers of Huntington's disease (HD) have shown dysfunction of the Default-Mode Network (DMN). No data however are currently available on the DMN alterations in the symptomatic stages of the disease, which are characterized by cortical atrophy involving several DMN nodes. We assessed DMN integrity and its possible correlations with motor and cognitive symptoms in 26 symptomatic HD patients as compared to 22 normal volunteers, by analyzing resting state functional MRI data, using the Precuneal Cortex/Posterior Cingulate Cortices (PC/PCC) as seed, controlling at voxel level for the effect of atrophy by co-varying for gray matter volume. Direct correlation with PC/PCC was decreased, without correlation with atrophy, in the ventral medial prefrontal cortex (including anterior cingulate and subgenual cortex), right dorso-medial prefrontal cortex, and in the right inferior parietal cortex (mainly involving the angular gyrus). Negative correlations with PC/PCC were decreased bilaterally in the inferior parietal cortices, while a cluster in the right middle occipital gyrus presented increased correlation with PC/PCC. DMN changes in the ventral medial prefrontal cortex significantly correlated with the performance at the Stroop test (p = .0002). Widespread DMN changes, not correlating with the atrophy of the involved nodes, are present in symptomatic HD patients, and correlate with cognitive disturbances.