[Hemiballism-hemichorea syndrome].
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Anti-Dyskinesia Agents↗
Biomedical subjects
J Simbrunner
Publications and source records attributed to J Simbrunner.
Imaging of the neonatal CNS.
Imaging of the central nervous system is one of the major tasks of Paediatric Radiology, particularly in newborns, who present with a variety of conditions that need more or less urgent imaging. Imaging is usually performed primarily by bedside US, in rare cases supplemented by a skull or spine radiograph. For more detailed information and preoperatively, MRI has become the neuroimaging tool. Thus, CT today is only used for acute trauma assessment, for assessment of potential cerebral calcifications or when MRI is not available. In cases with vascular anomalies or unsuccessful punctures, image guided interventions (embolisation) or image guidance for access (lumbar puncture, puncture of skull collections ...) may become necessary. This article tries to give a brief overview on the common disease entities, their typical imaging features in the major modalities applied and the implications of imaging potential for indication and choice of imaging method. In general, acute assessment may become everywhere and major features of important diseases should be recognised not to miss conditions which need urgent treatment or referral to a dedicated paediatric unit. Many other conditions will only be seen at centres with a dedicated neonatal care unit and dedicated paediatric radiologist who then also will be able to provide proper imaging with adapted protocols and methods for these partially severely sick babies. As these specific features and adapted capabilities as well as dedicated training and clinical experience are necessary for providing best results and proper handling in neonates, many neonatal conditions will not be imaged at a peripheral site, but primarily should be referred to a paediatric (radiology) centre.
Algorithms↗
Degree of hypomyelination and magnetic resonance spectroscopy findings in patients with Pelizaeus Merzbacher phenotype.
As only 10 - 30 % of patients with a Pelizaeus Merzbacher disease (PMD) phenotype carry mutations of the proteolipid protein (PLP) gene, we were interested if the degree and time-dependent progression of abnormal MRI and MRS findings would discriminate patients with mutations of the PLP gene (Pelizaeus Merzbacher disease, PMD) from patients without a defect of the PLP gene (Pelizaeus Merzbacher-like disease, PMLD). For a standardised intraindividual follow-up and for comparison of the degree of hypomyelination, we have applied a newly developed semiquantitative myelination score on a total of 18 MRI series of 4 PMD and 4 PMLD patients. We found severe hypomyelination (< 50 % of normal) in 2 PMD and in 2 PMLD patients, moderate hypomyelination (< 75 % of normal) in 2 PMD and mild hypomyelination (> 75 % of normal) in 2 PMLD patients. Our score revealed a clear correlation between the degree of hypomyelination and the severity of clinical handicap in PMD but not in PMLD patients. MRS showed heterogeneous cerebral metabolite patterns in both patient groups and seems to reflect a mixture of unspecific changes due to primary hypomyelination and secondary gliosis and demyelination. Neither by MRI nor by MRS were patterns found that would allow differentiation between PMD and PMLD patients.
Adolescent↗
Feasibility of MR urography in neonates and infants with anomalies of the upper urinary tract.
The aim of this study was to evaluate the feasibility and diagnostic potential of dynamic MR urography (MRU) in neonates and infants with sonographically detected abnormalities of the upper urinary tract. Thirty infants (age range 5 days to 3 years, mean age 7.9 months; male:female: 22:8) underwent MRU using T2 and contrast-enhanced dynamic T1-weighted sequences. The results were compared with the findings of ultrasound ( n=30), intravenous urography (IVU, n=19) and/or scintigraphy ( n=25) based on the criteria suggestive of obstructive uropathy. Oral sedation was sufficient to perform MRU with diagnostic quality in 20 of 21 patients younger than 1 year; 9 older patients needed intravenous sedation. Diagnosis of the 66 renal units (58 kidneys, 29 successful examinations) included normal systems (contralateral units), duplex systems, vesico-ureteral reflux, obstructive megaureter, ureteropelvic junction obstruction and accompanying renal parenchymal disease, with complex pathology in 10 patients. Magnetic resonance urography demonstrated anatomy better than IVU, particularly the renal parenchyma, (ectopic) ureters, and poorly functioning dilated systems. Magnetic resonance urography was superior to US in showing ureteral pathology. Tiny cysts in dysplastic kidneys were better seen by US. Gadolinium-enhanced dynamic MRU allowed accurate assessment of obstruction applying IVU criteria. Here MRU matched IVU results, and most of the scintigraphic findings. Magnetic resonance urography can be performed in young infants with diagnostic quality using oral sedation. Magnetic resonance urography correctly depicts anatomy and allows assessment of the urinary tract better than US and IVU, with additional functional information. Magnetic resonance urography thus has the potential to replace IVU for many indications.
Feasibility Studies↗
Idiopathic sclerotic inflammation of the orbit with left optic nerve compression in a patient with multifocal fibrosclerosis.
We present the MR imaging findings in a 43-year-old male patient with bilateral idiopathic sclerosing inflammation of the orbit. Bilateral enhancing retrobulbar masses, with concentric compression of the retrobulbar segment of the left optic nerve, were seen. MR imaging proved to be the only means to distinguish between the different intraorbital structures and to determine the exact site of optic nerve compression. To our knowledge, this is the first documented case of MR imaging findings of this entity.
Adult↗
Diffusion-weighted MR imaging of the spinal cord.
Diffusion-weighted MR imaging may increase the sensitivity and specificity of MR imaging for certain pathologic conditions of the spinal cord but is rarely performed because of several technical issues. We therefore tested a novel phase-navigated spin-echo diffusion-weighted interleaved echo-planar imaging sequence in seven healthy volunteers and six patients with intramedullary lesions. We performed diffusion-weighted MR imaging of the spinal cord with high spatial resolution. Different patterns of diffusion abnormalities observed in patient studies support the possible diagnostic impact of diffusion-weighted MR imaging for diseases of the spinal cord.
Adult↗
Diffusion-weighted imaging of patients with subacute cerebral ischemia: comparison with conventional and contrast-enhanced MR imaging.
BACKGROUND AND PURPOSE: The importance of diffusion-weighted imaging (DWI) for delineating acute ischemic lesions has been investigated extensively; however, few studies have investigated the role of DWI in the subacute stage of stroke. Because these lesions tend to appear bright throughout the first days of ischemia, owing to restricted diffusion, we speculated that DWI could also improve the detection of subacute infarcts as compared with conventional and contrast-enhanced MR imaging. METHODS: Interleaved echo-planar DWI with phase navigation was performed on a 1.5-T MR unit in a consecutive series of 53 patients (mean age, 66 +/- 14 years) with suspected recent cerebral ischemia. The interval between onset of clinical symptoms and MR imaging ranged from 1 to 14 days (mean, 6 +/- 4 days). Contrast material was given to 28 patients in a dose of 0.1 mmol/kg. RESULTS: DWI clearly delineated recent ischemic damage in 39 patients (74%) as compared with 33 (62%) in whom lesions were identified or suspected on conventional T2-weighted images. DWI provided information not accessible with T2-weighted imaging in 17 patients when evidence of lesion multiplicity or detection of clinically unrelated recent lesions was included for comparison. Subacute ischemic lesions were also seen more frequently on DWI sequences than on contrast-enhanced images (20 versus 13 patients). DWI was more likely to make a diagnostic contribution in the first week of stroke and in patients with small lesions or preexisting ischemic cerebral damage than was conventional MR imaging. CONCLUSION: Recent ischemic damage is better shown on DWI sequences than on conventional and contrast-enhanced MR images throughout the first days after stroke and may provide further information about the origin of clinical symptoms. Adding DWI to imaging protocols for patients with subacute cerebral ischemia is recommended.
Adult↗
Clinical and magnetic resonance findings in the temporomandibular joints of patients before and after orthognathic surgery.
It is difficult to achieve the correct position of the condyle in the temporal fossa during orthognathic surgery in angle class II patients with disorders of the temporomandibular joint. This led us to examine the TMJ of 25 of our own patients before and shortly after orthognathic operations. We recorded the clinical and magnetic resonance imaging findings of the temporomandibular joint preoperatively and three months postoperatively. The patients had skeletal class II dysgnathia and had been treated with fixed orthodontic appliances for a mean of two years and three months before operation. Operation resulted in a mean reduction of maximal incisor distance of 12 mm. In five of the 25 patients, the pattern of mouth-opening changed. Nine patients had less pain than before surgery, and nine had fewer abnormal joint sounds. The magnetic resonance imaging showed displacement of the articular disc in 38 of the 50 joints preoperatively and in 28 postoperatively. Degenerative joint changes were not improved by operation. Improvement of the disc position was achieved by repositioning of the condylar-disc complex during orthognathic surgery in angle class II patients. Clinical and magnetic resonance imaging findings regarding the temporomandibular joint in class II patients correlated significantly both preoperatively and postoperatively.
Adult↗
Diffusion-weighted imaging with navigated interleaved echo-planar imaging and a conventional gradient system.
PURPOSE: To demonstrate the technical feasibility and precision of a navigated diffusion-weighted (DW) MR imaging method with interleaved echo-planar imaging and test its diagnostic sensitivity for detection of ischemic stroke. MATERIALS AND METHODS: Apparent diffusion coefficient (ADC) measurements were performed in phantoms, and six healthy adult volunteers were examined to determine intrasubject (precision) and intersubject (reference range) variations in absolute ADC and relative ADC (rADC) measurements. DW imaging maps and lesion rADC values were also obtained in 34 consecutive stroke patients to evaluate the sensitivity and reliability of DW-interleaved echo-planar imaging for detection of ischemic brain damage. RESULTS: Phantom and volunteer ADC values were in excellent agreement with published data. The intrasubject variation of rADC was 6.2%. The ADC precision ranged from 6.5% in the subcortical white matter in the frontal lobe to 12.9% in the head of the caudate nucleus. Interleaved echo-planar imaging enabled rapid acquisition of high-quality images of the entire brain without substantial artifacts. Within the 1st week, the sensitivity of DW-interleaved echo-planar imaging for detection of acute infarction was 90% (18 of 20 true-positive studies) and independent of lesion location. CONCLUSION: DW-interleaved echo-planar imaging with phase navigation and cardiac triggering is robust, reliable, and fast. With high sensitivity for detection of early ischemic infarction, it is useful for examining stroke patients by using MR systems with conventional gradient hardware.
Adult↗
A six year experience with the postoperative radiosurgical management of pituitary adenomas.
Since April 1992, 73 consecutive patients with pituitary adenomas were treated with radiosurgery. There were 31 hormonally inactive adenomas and 42 hormonally active adenomas. All but three patients had been subjected to one or more surgical procedures prior to radiosurgery. Three patients had received fractionated radiotherapy. In the inactive adenoma group, the mean target volume was 4.4 ¿ 3 cm3 and the mean prescription dose was 13.8¿1.5 Gy. In the prolactinoma patients, the mean target volume was 6.7 ¿ 9 cm3 and the mean prescription dose was 14.2 ¿4 Gy. In the acromegalic patients, the mean target volume was 2.9¿2.5 cm3 and the mean prescription dose was 16¿4 Gy. ACTH secreting adenomas had a mean target volume of 3.6 ¿ 5.5 cm3 with a mean prescription dose of 17 ¿4.8 Gy. The mean follow-up time was 28.9 ¿ 21.5 months. Follow-up data was available in 83.6% of the patients. Tumor control was achieved in 98.3% and the endocrinological cure rate was 57%. Pituitary function deteriorated in 19.2%. No patient suffered from radiation induced visual damage. It would seem that postoperative radiosurgery for residual or recurrent pituitary adenomas may be a safe technique that can increase the frequency of therapeutic success.
Adenoma↗
Cerebral arteriovenous malformations: diagnostic value of echo-enhanced transcranial Doppler sonography compared with angiography.
BACKGROUND AND PURPOSE: The purpose of our study was to examine the clinical value of echo-enhanced transcranial power Doppler sonography (EE-TCD), including it ability to assess hemodynamic parameters of the intracranial vasculature, in patients with suspected cerebral arteriovenous malformations (AVMs) and to compare this method with angiography. METHODS: Sixteen patients with suspected cerebral AVMs were examined with EE-TCD and angiography. As an echo-enhancing agent, SHU 508A (Levovist) was administered intravenously by bolus injection in nine patients and by continuous infusion in seven. Sonograms were reviewed without knowledge of other imaging results and were correlated with angiographic findings. RESULTS: Angiography showed AVMs in 12 of 16 patients. Eleven lesions were located in the anterior or middle fossa and one was in the posterior fossa. EE-TCD was slightly less sensitive in the detection of AVMs (92%, 11/12 lesions), since in one patient the lacking acoustic window did not allow a transcranial examination. EE-TCD slightly underestimated AVM size compared with angiographic findings but showed feeding arteries with sufficient acoustic properties. In seven patients (58%), angiography revealed a coincidental blood supply from another intracranial or extracranial vessel, which was missed by EE-TCD in all cases. Assessment of peak systolic velocities and resistive indexes resulted in a higher (mean, 191.1 cm/s) and a lower (mean, 45.7%) value, respectively, in the feeding arteries as compared with the contralateral arteries (mean, 101.8 cm/s and 55.6%, respectively). Side-to-side differences were significantly higher in patients with AVMs than in those without a malformation. Signal enhancement was markedly longer with continuous infusion (mean, 520 seconds +/- 28.2) than with bolus injection (mean, 145 seconds +/- 10.5) of the contrast agent. CONCLUSION: In our limited study group, EE-TCD was a sensitive method for the detection of AVMs, and Levovist proved to be a safe and effective echo-enhancing substance.
Adolescent↗
An in vivo 13C magnetic resonance spectroscopic study of the relationship between diet and adipose tissue composition.
13C magnetic resonance spectroscopy (MRS) is a noninvasive technique used in the study of lipids. We applied 13C MRS to assess the effects of long-term dietary variation on adipose tissue composition in humans. In vivo 13C MRS was used to analyze the fatty acid composition of adipose tissue in 88 healthy volunteers with significantly different diets (38 vegans, 11 vegetarians, and 39 omnivores) assessed by analysis of dietary records. Results were compared with the serum lipid profile. 13C MRS revealed clear differences in the adipose tissue composition of vegans, which contained more unsaturated (P < 0.01) and fewer saturated fatty acids (P < 0.01) compared with omnivores and vegetarians. The vegan subjects had a significantly lower intake of saturated fatty acids and higher intake of polyunsaturated fatty acids than either the omnivore or the vegetarian groups (P < 0.01). These findings were associated with significantly lower levels of serum total cholesterol and low density lipoprotein-cholesterol in the vegan group compared with the omnivores. Our results demonstrate the use of 13C MRS for the noninvasive study of adipose tissue composition and its application to the study of the interaction between long-term dietary and metabolic risk factors in humans.
Adipose Tissue↗
Development of supratentorial astrocytomas.
Six cases of supratentorial astrocytomas are presented in this study in order to demonstrate the typical development of this kind of tumors. All patients were controlled regularly by CT scan. Five of them were operated two times, one patient three times. The tumor growth was quite similar in all cases. Time interval between the first intervention and tumor recurrence was larger than between second intervention and tumor recurrence. In all cases the tumors tended to "explosive" growth after the second operation. There are discussed pros and cons of an early reintervention after tumor recurrence.
Adult↗
MR imaging of the TMJ. MR diagnosis and intraoperative findings.
Magnetic resonance imaging provides the possibility of a non-invasive method for the investigation of the soft tissues of the temporomandibular joint (TMJ). Between March 1991 and August 1992 we performed static and dynamic magnetic resonance images (MRI) on 47 TMJs of 37 symptomatic patients using a MR-tomograph with a 6.5 cm surface coil. MR diagnosis could be verified in 15 joints at operation and in 3 cases by arthrotomography. Disc position was confirmed in 80%, perforations were missed in 2 out of 3 cases and two more perforations became obvious during operation. One patient refused MR examination, in 2 cases MRI had to be abandoned because of the patient's claustrophobia. In our work we wanted to show that MRI is a reliable tool to determine disc position, disc shape and disc behaviour during motion. Detection of perforations is still the domain of arthrography.
Adolescent↗
[An unusual case of a blow-out fracture. Entrance of a bone fragment into the frontal lobe after a ski accident].
Due to a ski accident a 16-year old girl suffered from headache, periorbital hematoma and ptosis of the right eyelid. No penetrating injury could be seen, but in the CT scan a blow-out fracture of the orbital roof and floor was verified. Since there was no evident lesion of the eye muscles, an operative indication could only be a neurosurgical one. Because of the traumatic connection between frontal cerebrum and sinus maxillaris we expected a CSF rhinorrhoea, which could not be confirmed.
Adolescent↗
[Congenital hypopituitarism and giant cell hepatitis in a three month old girl].
CASE REPORT: A three month old girl, with recurrent hypoglycemia and neonatal cholestasis, is reported. A metabolic disease could be excluded. The liver biopsy revealed giant cell hepatitis and intrahepatic bile duct hypoplasia. ACTH, Cortisol and hGH measured during hypoglycemia were low. Magnetic tomography (MR) of the brain showed an "empty sella". After beginning a replacement therapy with hydrocortisone, growth hormone and thyroxine there was no further episode of hypoglycemia. Transaminases and bilirubin levels normalized. The girl is in good condition, growth and development are normal. DISCUSSION: Hypoglycemia is often the first sign in childrens with neonatal hypopituitarism. The association of liver disease and hypopituitarism has been documented in a few reports. The pathophysiological mechanism leading to the liver dysfunction is not well understood. The prognosis of neonatal hypopituitarism as well as the concomitant liver disease is good under sufficient replacement therapy.
Bile Ducts, Intrahepatic↗