Adult presentation of PHACES syndrome.

A significant percentage of children with hemangiomas may have PHACES syndrome which refers to the association of posterior fossa malformations, facial hemangiomas, arterial cerebrovascular abnormalities, cardiovascular anomalies, eye abnormalities and ventral defects like sternal clefting or supraumbilical raphe. A variety of factors have led to under diagnosis of PHACES syndrome in the past including lack of awareness and limited imaging modalities. Also, patients with PHACES syndrome with arterial cerebrovascular abnormalities can present with acute ischemic stroke. However, these patients usually present before one year of age. We describe a 29-year-old woman with no history of cerebrovascular disease who initially presented with symptoms of a stroke and was subsequently diagnosed to have PHACES syndrome exhibiting an array of multiple unusual imaging findings. We also discuss the current literature and recommendations about PHACES syndrome.

Idiopathic lingual artery aneurysm: CT findings and endovascular therapy. A case report.

SUMMARY: We describe a 65-year-old woman with an asymptomatic idiopathic lingual artery aneurysm which is suspected to be congenital. We review the literature on external carotid artery branch aneurysms, diagnostic evaluation and discuss treatment options for the various types and the specific chosen in the case presented.

Glutamate as a spectroscopic marker of hippocampal structural plasticity is elevated in long-term euthymic bipolar patients on chronic lithium therapy and correlates inversely with diurnal cortisol.

While an excess of glucocorticoids is associated with hippocampal pathology in mood disorders, lithium exerts robust neuroprotective and neurotrophic effects. Here, 21 stably remitted bipolar I patients who had been on chronic lithium maintenance therapy, on average, for more than a decade, and 19 carefully matched healthy controls were studied using 3 T (1)H-magnetic resonance spectroscopy of left and right hippocampus. Salivary cortisol samples were obtained to assess activity of the hypothalamus-pituitary-adrenal system. Absolute concentrations of N-acetylaspartate (NAA), choline-containing compounds and total creatine were similar in euthymic bipolar patients and healthy controls. Hippocampal glutamate concentrations were significantly increased as an effect of patient status (patients>controls) and laterality (left hippocampus>right hippocampus). Hippocampal glutamate content (Glu) was strongly correlated with NAA. Across groups and within the patient group, diurnal saliva cortisol levels showed a significant inverse relationship with both Glu and NAA. Taken together, these results add to the concept of bipolar disorder as an illness involving disturbed hippocampal structural plasticity under the opposing influences of lithium and glucocorticoids.

Magnetic resonance imaging results can affect therapy decisions in hyperacute stroke care.

BACKGROUND: Despite some limitations, a perfusion/diffusion mismatch can provide a working estimate of the ischemic penumbra in hyperacute stroke and has successfully been used to triage patients. PURPOSE: To evaluate whether the addition of magnetic resonance imaging (MRI) to clinical and non-contrast computed tomography (CT) data alters diagnosis and choice of therapy. MATERIAL AND METHODS: We retrospectively analyzed clinical records, and CT and MRI data fully available in 97 of 117 patients. Upon clinical examination and CT, a diagnosis and treatment path was scored and compared to treatment path after addition of MRI data. The MRI protocol included T2-weighted images, diffusion-weighted images (DWI), and perfusion-weighted images (PWI), and MR angiography (MRA). RESULTS: MRI data were acquired in less than 15 min. In 20 of 97 patients (21%), the diagnosis changed after MRI. In 25 of 97 patients (26%), the presumptive treatment plan was changed after MRI evaluation. Thirteen patients had their treatment changed from thrombolytic to nonthrombolytic therapy. Three patients were changed from nonthrombolytic to intraarterial (IA) thrombolysis. In one patient, treatment was changed from intravenous (IV) to IA thrombolysis, and in five patients it was changed from IA to IV thrombolysis. In two patients, systemic heparin was added to antiplatelet therapy. CONCLUSION: The expansion of the acute stroke protocol to include MRI altered the therapy plan in 26% of our patients. The utility of MRI, shown here to improve patient stratification into best-treatment options, demonstrates the value of using MRI to optimize care in hyperacute stroke patients.

Assessment of 3D-TOF-MRA at 3.0 Tesla in the characterization of the angioarchitecture of cerebral arteriovenous malformations: a preliminary study.

BACKGROUND: The characterization of brain arteriovenous malformation (AVM) angioarchitecture remains rewarding in planning and predicting therapy. The increased signal-to-noise ratio at higher field strength has been found advantageous in vascular brain pathologies. PURPOSE: To evaluate whether 3.0T time-of-flight (TOF) magnetic resonance angiography (MRA) is superior to 1.5T TOF-MRA for the characterization of cerebral AVMs. MATERIAL AND METHODS: Fifteen patients with AVM underwent TOF-MRA at 3.0T and 1.5T and catheter angiography (DSA), which was used as the gold standard. Blinded readers scored image quality on a four-point scale, nidus size, and number of feeding arteries and draining veins. RESULTS: Image quality of TOF-MRA at 3.0T was superior to 1.5T but still inferior to DSA. Evaluation of nidus size was equally good at 3.0T and 1.5T for all AVMs. In small AVMs, however, there was a tendency of size overestimation at 3.0T. MRA at 3.0T had increased detection rates for feeding arteries (+21%) and superficial (+13%) and deep draining veins (+33%) over 1.5T MRA. CONCLUSION: 3.0T TOF-MRA offers superior characterization of AVM angioarchitecture compared with 1.5T TOF-MRA. The image quality of MRA at both 3.0 and 1.5T is still far from equal to DSA, which remains the gold standard for characterization of AVM.

Computed tomography of the human pineal gland for study of the sleep-wake rhythm: reproducibility of a semi-quantitative approach.

PURPOSE: To propose a semi-quantitative computed tomography (CT) protocol for determining uncalcified pineal tissue (UCPT), and to evaluate its reproducibility in modification of studies showing that the degree of calcification is a potential marker of deficient melatonin production and may prove an instability marker of circadian rhythm. MATERIAL AND METHODS: Twenty-two pineal gland autopsy specimens were scanned in a skull phantom with different slice thickness twice and the uncalcified tissue visually assessed using a four-point scale. The maximum gland density was measured and its inverse graded on a non-linear four-point scale. The sum of both scores was multiplied by the gland volume to yield the UCPT. The within-subject variance of UCPT was determined and compared between scans of different slice thickness. RESULTS: The UCPT of the first measurement, in arbitrary units, was 39+/-52.5 for 1 mm slice thickness, 44+/-51.1 for 2 mm, 45+/-34.8 for 4 mm, and 84+/-58.0 for 8 mm. Significant differences of within-subject variance of UCPT were found between 1 and 4 mm, 1 and 8 mm, and 2 and 8 mm slice thicknesses (P<0.05). CONCLUSION: A superior reproducibility of the semi-quantitative CT determination of UCPT was found using 1 and 2 mm slice thicknesses. These data support the use of thin slices of 1 and 2 mm. The benefit in reproducibility from thin slices has to be carefully weighted against their considerably higher radiation exposure.

Bleeding complications after endovascular therapy of cerebral arteriovenous malformations.

BACKGROUND AND PURPOSE: Intracerebral hemorrhages after embolization of arteriovenous malformations (AVMs) are the most dreaded complications of this well-established therapy. Apart from the known risk factors, our center noticed a high incidence of complications during postinterventional monitoring in medical intensive care units (ICUs) and stroke units. MATERIALS AND METHODS: We report 125 consecutive interventions performed on 66 patients by using flow-dependent microcatheters and n-butyl cyanoacrylate as the embolic agent. Postinterventional intensive care monitoring was performed in an interdisciplinary operative ICU, a stroke unit, or a medical ICU. Patients were compared with regard to bleeding complications, AVM morphology, embolization result, postinterventional monitoring, and demographic factors. RESULTS: Intracerebral hemorrhages occurred in 7 patients. Significant differences in outcome were found between 66 patients monitored in the interdisciplinary operative ICU from medical ICU or stroke unit. This was also true when adjusted for age and extent of AVM reduction by using exact logistic regression. A partial AVM reduction of >60% was a considerable risk factor for hemorrhage (odds ratio [OR] = 18.8; 95% confidence interval [CI] [1.341, not available]. Age was also an essential risk factor. An age difference of 10 years leads to an OR of 2.545 (95% CI [1.56, 7.35]). DISCUSSION: A considerable AVM reduction in one session appears to increase the risk of hemorrhage technically. This suggests a distribution of the interventions in many partial steps.

[Pitfalls and special features of modern imaging diagnostics of the upper cervical spine]. / Fallgruben und Besonderheiten der modernen bildgebenden Diagnostik der oberen Halswirbelsäule.

Recent technical developments led to a change in the significance of the different imaging modalities in the whole spectrum of neuroradiology and thus also in the field of imaging of the cervical spine. The main part in diagnostic imaging of the upper cervical spine is taken by high-resolution multislice CT and MRI. Conventional X-rays serve as an initial screening technique if required.

Transient tetraplegia and vegetative dysfunction depending on cervical positioning caused by a cervical spinal chordoma.

A 39-year-old man with acquired torticollis suffering from cervicobrachialgia and neurological deficits is presented. Due to a change in head position a transient reproducible tetraplegia and severe vegetative dysfunctions were caused. The origin of this uncommon serious combination of symptoms and signs was a chordoma of the upper cervical spine. After surgical decompression the patient was free of neurological deficit and pain. Review of the literature did not reveal any similar case.

Comparison of metric analysis of spinal structures, exemplarily of the ligamentum flavum, obtained with CT and MRI.

PURPOSE: Comparison of metric analysis of spinal structures, exemplarily of the ligamentum flavum, obtained with computed tomography (CT) (soft tissue window and bone window) and magnetic resonance imaging (MRI) (T1 and T2 weighted images). MATERIAL AND METHODS: Forty-six lumbar ligamenta flava of 46 patients (25 women and 21 men) were examined at a Somatom Plus 4 (Siemens, Erlangen, FRG) and at a 1.5 T clinical scanner (Magnetom Vision, Siemens, Erlangen, FRG). Two independent neuroradiologists measured the thickness of the ligamenta flava in mm. Statistics included Pearson's correlation coefficient and the intra-class correlation coefficient. RESULTS: Mean values did not differ significantly. The correlation coefficients varied between 0.69 and 0.98. The best correlation occurred comparing the same techniques in different windowing and weighting (CT: r = 0.98; MRI: r = 0.95). Correlating different techniques the combination of CT bone window and T1 weighted images presented the best result (r = 0.75). CONCLUSIONS: Because of the excellent correlation between the examined techniques CT as well as MRI can equally be used to measure distances of spinal structures.

[Changes of the fat/water ratio in the erector trunci muscle in patients with lumbar disc herniation: a comparative study with 1H-MRS]. / Veränderungen des Fett/Wasser-Quotienten im Musculus erector trunci bei Patienten mit lumbalem Bandscheibenvorfall: eine vergleichende.

PURPOSE: To evaluate whether metabolic changes of the erector trunci muscle in patients with lumbar disc herniation can be detected with proton magnetic resonance spectroscopy ( (1)H-MRS). MATERIALS AND METHODS: In 10 patients with lumbar disc herniation and 16 healthy volunteers, proton spectra were obtained from the erector trunci muscle and analyzed for fat-water ratio. The axial images were evaluated for the degree of atrophy of the erector trunci muscle. The muscular tissue was histopathologically examined in 2 patients. RESULTS: The examination was well tolerated by all patients and volunteers and all acquired spectra could be analyzed. Patients with lumbar disc herniation have a significantly increased fat-water ratio of 0.19 compared to 0.09 in the control group, with a p-value of 0.003. This result correlates well with the bioptical findings of increased intracellular lipid deposits and lipomatous transformation of the muscle parenchyma. CONCLUSION: (1)H-MRS is a useful method to detect metabolic changes in lumbar back musculature and, as a non-invasive technique, might play a role in monitoring exercise in lumbar disc herniation. It can be expected that muscle regeneration will be shown as precisely as muscle degeneration. This hypothesis, however, remains to be proven.

Intracerebral pH affects the T2 relaxation time of brain tissue.

Signal changes in activated brain areas are detectable by MRI and MR spectroscopy (MRS). Shifts in pH occur during brain activation. Our aim was to investigate the relationship between changes in pH and T2 relaxation times. T2 was determined in vitro at 24 MHz in various liquids at different pH using a Carr-Purcell-Meiboom-Gill (CPMG) spin-echo sequence. We also studied five Fisher rats were studied at 2.4 tesla with a double-tuneable surface coil. After baseline measurements, potassium cyanide was injected, producing intracerebral acidosis. Alternating series of 1H CPMG spin-echo sequences and 31P spectra were acquired. True T2 relaxation times were calculated from a CPMG multi-echo train. Changes in intracellular pH determined from 31P spectra. In vitro measurements demonstrated a correlation between T2 and pH that could be described by a quadratic fit curve. Depending on the initial pH, changes of 0.2 induced changes in T2 of up to 150 ms. In vivo measurements confirmed these findings. After intraperitoneal injection of a sublethal dose of cyanide, T2 decreased by about 5% in four cases, followed by recovery after 2 h. The in vitro measurements demonstrated that changes in pH can lead to significant signal change on T2- or T2*- weighted images. The dependence of T2 on pH in vitro was confirmed in vivo; it may contribute to signal change in activated brain areas.

Reduced NAA in the thalamus and altered membrane and glial metabolism in schizophrenic patients detected by 1H-MRS and tissue segmentation.

Functional and structural abnormalities in the thalamus as well as a generalized phospholipid membrane disorder have been implicated in the pathogenesis of schizophrenic psychosis. To determine whether thalamic neuronal abnormalities and altered membrane-associated metabolites can be detected in schizophrenic patients, we used in vivo proton magnetic resonance spectroscopy (1H-MRS) in 32 acutely-ill, medicated schizophrenic patients and 17 age-matched controls. Thalamic and white matter metabolite concentrations (myo-inositol (mI), choline-containing compounds (Cho), total creatine (Cr) and N-acetylaspartate (NAA)) were estimated and corrected for atrophy (CSF) and gray and white matter contributions (GM, WM) by use of image-based voxel segmentation. Thalamic NAA was significantly reduced in schizophrenic patients, whereas Cho and mI were significantly increased in the parietal white matter. White matter Cr was significantly elevated in patients and correlated positively with the brief psychiatric rating scores (BPRS). Regional metabolite levels were inversely associated with GM and WM content reaching significance for mI and Cr in the thalamus and Cho and NAA in the white matter. Reduced NAA in the left thalamus of schizophrenic patients confirms and extends previous spectroscopic data and agrees well with histologic and imaging findings of reduced neuronal density and volume. Elevated Cho in line with 31P-MRS studies suggests increased myelin degradation thus further supporting a generalized membrane disorder in schizophrenic patients. In addition, we demonstrate the need to correct metabolite concentrations for regional tissue composition in studies employing patients with altered brain morphology.

Altered white and gray matter metabolism in CADASIL: a proton MR spectroscopy and 1H-MRSI study.

OBJECTIVE: Subcortical white matter hyperintensities (WMH) and small cystic lesions are the radiologic hallmark of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), a hereditary angiopathy causing stroke in young adults. To further characterize the cerebral pathology in vivo we analyzed metabolite concentrations in normal and abnormal appearing brain tissue using single and multiple voxel proton MR spectroscopy (1H-MRS and 1H-MRSI). METHODS: Twenty patients with CADASIL and 21 age-matched controls were studied with 1H-MRSI at the level of the centrum semiovale; short echo time 1H-MRS was performed in six patients (WMH) and 10 controls. LCModel fits were used to estimate absolute and relative concentrations of N:-acetylaspartate (NAA), choline-containing compounds (Cho), total creatine (Cr) within WMH, normal appearing white matter (NAWM), and cortical gray matter (GM) as well as myo-inositol (mI) and lactate in WMH. RESULTS: 1H-MRSI-Patients with CADASIL showed significantly reduced NAA, Cho, Cr, and total metabolite content (Met(tot)) in WMH and NAWM. Normalization to Met(tot) revealed that NAA/Met(tot) was reduced in all regions, whereas Cho and Cr were relatively elevated in WMH. Short echo time 1H-MRS showed decreased NAA, Cr, Met(tot), and NAA/Met(tot) and elevated mI/Met(tot) and lactate in WMH. Metabolite changes were larger in severely affected subjects. Rankin scores correlated negatively with NAA/Met(tot) (all regions) and NAA/Cho (WMH), and positively with Cho/Met(tot) (WMH) and Cr/Met(tot) (NAWM). CONCLUSION: Marked metabolic abnormalities were observed in abnormal and normal appearing white matter in patients with CADASIL. The findings suggest axonal injury, enlarged extracellular spaces, myelin loss, and gliosis. The cortical abnormalities may reflect structural damage or functional neuronal impairment secondary to white matter pathology. NAA reductions were correlated with clinical disability emphasizing the clinicopathologic relevance of axonal injury in CADASIL.