Early infantile form of Krabbe disease with optic hypertrophy: serial MR examinations and autopsy correlation.

The development of white matter lesions in a case of autopsy-proved early infantile form of Krabbe disease was monitored by serial MR examinations. Hypertrophy of the optic nerves was present late in the course of the patient's disease and is a remarkable feature in this case.

MR assessment of brain maturation: comparison of sequences.

PURPOSE: To evaluate the role of short-inversion-time inversion-recovery (STIR) sequences in assessment of brain maturation. METHODS: Twenty-seven infants and young children with normal neurologic development were examined by 1.5-T MR using a circularly polarized head coil. Axial T1-weighted and T2-weighted and spin-echo and STIR images were obtained. Signal intensity of different anatomic structures at individual sequences was classified relatively to reference sites and temporal sequence of signal intensity was observed. RESULTS: Signal intensity changes on T1-weighted and T2-weighted spin-echo sequences occurred at ages described in various previous publications. On STIR images intensity changes became apparent at a time between T1-weighted and T2-weighted images. The advantages of the STIR sequence were improved assessment of myelination of subcortical cerebral white matter from 6 to 14 months and good contrast between white matter lesions and cerebrospinal fluid. CONCLUSION: Our results suggest that from 0 to 6 months myelination can be assessed best using a combination of T1-weighted and T2-weighted images; from 6 to 14 months a combination of T2-weighted and STIR images seems to be advantageous; after 14 months the use of only T2-weighted sequences is sufficient. After 14 months STIR images may be useful in detecting small periventricular white matter lesions or in cases with retarded myelination and isointensity between gray matter and white matter.

MRI in basal ganglia diseases.

76 patients suffering from different basal ganglia diseases (28 cases with M. Parkinson, secondary parkinsonism and Parkinson diseases; 5 cases with Chorea Huntington; 5 cases with Fahr disease and 38 cases with M. Wilson) MRI featured 2 characteristical patterns: 1. abnormal deposition of minerals, 2. focal atrophies of involved organs. Thus MRI provides with informations about: 1. differential diagnosis in clinically misleading courses, 2. stage and, as a consequence, prognosis of some diseases, 3. biochemical processes of diseases in vivo.

Superparamagnetic particles as oral contrast medium in magnetic resonance imaging of patients with treated ovarian cancer--comparison with plain MRI.

In 23 patients with treated ovarian cancer, 24 magnetic resonance (MR) examinations of the abdomen and pelvis were performed before and after administration of an oral superparamagnetic contrast medium. Depiction of bowel loops was judged sufficient in 47% with plain scans and in 85% with enhanced scans. Minor artefacts attributed to the agent were noted in 32% of examinations and were classified as disturbing in 8%. The diagnostic information obtained after contrast enhancement was estimated to be superior to that from plain MRI in 20% of patients. The use of oral contrast medium did not alter the MR diagnosis of "tumour recurrence" or "disease-free" in any patient. Our results suggest that superparamagnetic iron oxide is an effective and reliable approach to negative bowel contrast enhancement, increasing the confidence level when distinguishing intestines from solid structures.

[Gd-DTPA in the MR diagnosis of pituitary adenomas]. / Gd-DTPA in der MR-Diagnostik von Hypophysenadenomen.

25 patients with surgically (n = 14) and/or biochemically (n = 20) confirmed pituitary adenomas were examined on a 1.5 T superconducting MR system by means of multislice spin-echo technique, 3 mm thick sections and a 256 x 256 matrix. T1-weighted sagittal and coronal sections were obtained before and after administration of Gd-DTPA (0.1 mmol./kg. BW). 13 microadenomas and 12 macroadenomas were found. In unenhanced T1-weighted images 48% of the adenomas were isointense, 48% were hypointense and 4% were hyperintense. 58% of the adenomas enhanced to a lesser degree, 15% enhanced to a higher degree than the normal pituitary gland. 12% showed equal enhancement and 15% showed hypointense and hyperintense areas simultaneously. In 3 patients (12%) there was evidence of adenoma only in the enhanced images. The delineation of the adenoma from the cavernous sinus was possible in 95% of the enhanced, but only in 25% of the native scans.

[The importance of magnetic field strength in the MR diagnosis of multiple sclerosis: a comparison of 0.5 and 1.5 T]. / Bedeutung der Magnetfeldstärke in der MR-Diagnostik der multiplen Sklerose: Ein Vergleich von 0.5 und 1.5T.

The value of magnetic resonance (MR) to establish the diagnosis of multiple sclerosis (MS) is well known. This study was undertaken to compare MR imaging of the brain of MS patients at high (1.5T) and mid (0.5T) field strength. 25 patients with MS underwent two consecutive MR studies within one hour, each consisting of axial proton density and T2-weighted spin-echo images. Lesions in the supratentorial white matter and corpus callosum and those in the brain stem and cerebellum were separately counted. At 1.5T significantly more lesions were seen than at 0.5T (p < 0.05). Although T2-weighted images at 1.5T added significant information compared to images obtained at 0.5T, in none of our 25 patients the diagnosis was missed at 0.5T. However, at 1.5T dissemination in space was better demonstrated, suggesting MR scanning with high field-units to be favourable in patients with clinically suspected MS.

[Linear versus circular polarization of head coils: comparison on phantoms and in the clinic]. / Linear versus zirkular polarisierte Kopfspule--Vergleich am Phantom und in der Klinik.

Two different head coils--one with linear polarization and the other with circular polarization--were compared under the same measurement conditions. Comparison was done on a phantom with water-filled and gadolinium-filled pin-holes, as well as on anatomical MR images of 23 volunteers. In three volunteers the whole brain was examined while, in the remaining 20 volunteers the sella region or cerebellopontine angle region was examined. Criteria for comparison were signal-to-noise ratio, background noise, and detail resolution (phantom), as well as subjective criteria--image sharpness, anatomical, contrast, and recognition of anatomical details--, evaluated on anatomical MR images by four radiologists independently of each other. The results show a significant improvement of signal-to-noise ratio, lower background noise and therefore marked improvement of images sharpness, and moderate improvement in the recognition of anatomical details using the circular polarized head coil; as for as detail resolution and anatomical contrast were concerned, however, no significant difference was seen between the two coils. Major advantages of the circular, polarized head coil in clinical application are shorter measurement times (reduced number of acquisitions), as well as thinner slices without loss of signal-to-noise ratio.

[Cardiovascular reflexes, vibration thresholds and electroneurography parameters of the peroneal and sural nerves in type I diabetics]. / Untersuchung der kardiovaskulären Reflexe, der Vibrationsschwelle und der elektroneurographischen Parameter des N. peronaeus und suralis bei Typ-I-Diabetikern.

In 26 type I diabetics ranging in age from 18 to 65 years with a duration of illness of between 1 and 34 years the following investigations were performed: 1. case history and questionnaire; 2. neurological examination; 3. determination of the vibration threshold; 4. electroneurography of the peroneal nerve and the sural nerve; 5. determination of the cardiovascular reflexes; 6. medical examination and additional findings; 7. ophthalmological investigation. 21 patients showed evidence of sensorimotor polyneuropathy (SM-PNP), the average age of this group (41 years) being 10 years higher than in the group without SM-PNP (31 years). The values of HbA1c were pathological in 17 of 21 cases with SM-PNP, and 2 of 5 cases without SM-PNP. Retinopathy was found rarely in both groups. 13 patients showed evidence of autonomic neuropathy (ANP). The mean duration of illness (15.4 years) and the average age of patients (36.5 years) in this group was distinctly higher than in the group without ANP (mean duration of illness: 8.9 years, mean age: 31.9 years). 12 patients with ANP and 7 patients without ANP had abnormally high HbA1c levels. Diabetic SM-PNP was most frequently (in 19 of 21 cases) diagnosed by electroneurographical investigation of the peroneal nerve. In the diagnosis of diabetic ANP the anamnesis (8 positive findings) and the determination of the heart rate variation during deep breathing (7 positive findings) are complementary. Among the 13 patients with ANP, 12 also had SM-PNP, whereas among the 21 patients with SM-PNP only 12 showed evidence of concomitant ANP.

[AIDS of the central nervous system]. / AIDS im ZNS.

The central nervous system of nearly every HIV-positive patient becomes affected by the AIDS virus itself or by one of the associated diseases during the course of the illness. Early diagnosis of lesions which demand therapeutic consequences is of the most importance concerning prolongation of life and improvement in its quality. In spite of the frequent underestimation of cerebral involvement by imaging methods and their unspecific findings they are often the only diagnostic means which permit-timely diagnosis and, at least in some diseases, therapeutic monitoring. Indications for cranial computed tomography (CCT) or magnetic resonance tomography (MRT) are already present with mild or transient neurological or psychiatric symptoms or the extracerebral manifestation of neurotropic organisms or tumours which metastasize to the brain, even in patients without subjective complaints.

[Stenosis of the extracranial carotid artery]. / Stenose der extrakraniellen Arteria carotis.

Magnetic resonance angiography (MRA) as well as intraarterial digital subtraction angiography (DSA) were performed in 45 patients suffering from cerebrovascular disease, both procedures within one week of each other. MRA und DSA were evaluated separately for every patient retrospectively by two experienced radiologists on consensus basis. The results were then compared using catheter angiography as gold standard. Evaluation was confined to the common and internal carotid arteries (CCA, ICA) in the neck subdivided into 5 grades of stenosis. A comparative assessment was impossible in 9% cases due to motion artifacts and patient malposition. Out of 18 verified CCA stenoses 13 were demonstrated by MRA and 39 out of 44 ICA stenoses were assessed by MRA. There was a highly significant correlation between degree of stenosis detected by the two methods. Spearman Rank correlation coefficients of 0.824 and 0.913 were found for CCA and ICA, respectively.

[Angiologic, neurologic and orthopedic findings in vibration exposed chain saw operators]. / Angiologische, neurologische und orthopädische Befunde bei vibrationsexponierten Kettensägenarbeitern.

The present study was designed to evaluate and correlate angiological, neurological and orthopaedic findings in lumberjacks, professionally exposed to vibration by chain-saws with a frequency of 40 to 120 Herz. Raynaud's phenomenon was diagnosed in 16 of 33 subjects. The nerve conduction velocity was delayed in 13 of 32 lumberjacks, the sulcus nervus ulnaris syndrome was diagnosed in 41%, the vibration threshold was abnormal in 10/32 cases. Orthopaedic examination and X-ray studies revealed spondylotic (59%) and spondyloarthritic (65%) alterations of the cervical spine; cysts in bones of the distal upper extremities were observed in 75% of cases. Frequency and severity of angiological, neurological, and orthopaedic pathological findings correlated with the total chain-saw operating time. However, there was no correlation between the presence of digital vascular hyperreactivity and the sulcus nervus ulnaris syndrome or changes in nerve conduction velocity.

Case report: lipoma of the liver--ultrasound, CT and MR imaging.

A case of lipoma of the liver is presented; ultrasound, computed tomography (CT) and magnetic resonance imaging (MRI) findings are reviewed and compared with previous reports. The ultrasound pattern and appearance of the tumour in CT as well as MRI are discussed.

MRI of spondylitis.

MRI of proven spondylitis was performed in 39 patients. Acute spondylitis appeared hypointense on T1-weighted and hyperintense on T2-weighted images. Following intravenous administration of Gd-DTPA, enhancement of the marrow and disc was seen. With progressive healing an increasing signal was seen on T1-weighted images. This was caused by the presence of focal fat marrow representing the first sign of therapy response. As healing progressed, the T1-signal increased due to fatty marrow, while the T2-signal decreased as well as the enhancement following Gd-DTPA administration. Therefore, MR imaging can be recommended as the method of choice for early diagnosis of spondylitis and assessment of therapy response.

[Sensorimotor and autonomous polyneuropathies in diabetic children and adolescents]. / Sensomotorische und autonome Polyneuropathie bei kindlichen und jugendlichen Diabetikern.

In 28 juvenile and adolescent diabetics (14 males, 14 females) ranging in age between 9 and 20 years (mean: 14.9) we investigated several electrophysiological parameters, the vibration threshold and the cardiovascular reflexes in order to determine the incidence of sensomotor and autonomic polyneuropathy. 6 patients showed evidence of sensomotor polyneuropathy, 2 more had signs of autonomic polyneuropathy. Both groups with polyneuropathy did not differ significantly from the whole group of diabetics concerning the mean onset of illness and several metabolic parameters. The mean duration of illness and the average age was clearly higher in the patients with sensomotor polyneuropathy than in the whole group. Out of 5 patients with diabetic retinopathy 3 had signs of sensomotor polyneuropathy. No patient was clinically affected by polyneuropathy. In 1 patient the distal latency and the amplitude of the compound muscle action potential of the peroneal nerve showed pathologic values, in a second patient the maximal conduction velocity of the median nerve was slowed. 4 patients had abnormally high vibration threshold. The heart rate variation during rest was reduced in 2 patients. Significant differences between the diabetics and the controls could only be found concerning the maximum conduction velocities of the peroneal and the median nerve. Our results will be compared and discussed.

Jarisch-Herxheimer reaction in a patient with neurosyphilis.

A 40 year old man presented with progressive personality changes in the previous six months. Specific serological tests for syphilis in blood and CSF were highly positive and CSF sedimentation showed signs of an inflammatory process. Ten hours after the start of penicillin treatment a severe symptomatic Jarisch-Herxheimer reaction with alteration of level of consciousness, pupillary changes, and focal neurological signs developed. Jarisch-Herxheimer reaction may occur in various settings, particularly in the treatment of syphilis. Investigation of CSF before the treatment may predict a potential risk. Corticosteroid treatment has been suggested for prevention.

[Nuclear magnetic resonance tomography findings in obsessive-compulsive disorder]. / Kernspintomographische Befunde bei der Zwangserkrankung.

Eighteen patients with a DSM-III-R diagnosis of obsessive-compulsive disorder (OCD) were imaged by MR and compared to a control group of 18 patients with clinically diagnosed unspecific headaches. Weighted spin-echo sequences were carried out with a 1.5T unit in both axial and coronal planes T1 (TR 700/TE 15 ms) and T2 (TR 2500/TE 15.70). The ferritin distributions in the basal ganglia and in the midbrain were evaluated; in addition, the width of the pars compacta in the midbrain on the basis of the criteria of Braffmann et al. 1988, the widths of the inner subarachnoid spaces on the basis of the ventricular index quotient VIQ (TerBrugge, 1986), and the widths of the outer subarachnoid spaces were conducted. The latter was undertaken by three independent investigators whose results were in agreement with one another. In 12 of the OCD patients (n = 18) there was a total of 25 pathological MRI findings; in the control group there were 6 pathological MRI findings in only 6 of the patients (n = 18). In conclusion pathological changes were found at different locations, but a connection between a specific neuroanatomic system and obsessive-compulsive disorder could not be demonstrated.

Atypical vertebral artery in a patient with an intra-and extraspinal cervical neurenteric cyst.

A 15 year old girl experienced Horner's syndrome on the right side, paraesthesia of the right arm and meningitis. CT and MRI showed a cystic lesion on the right side of the cervical spine at C3/C4 which descended with an extraspinal portion down to C6. Histology revealed a complex neurenteric cyst. The ipsilateral vertebral artery showed an atypical course. This abnormal artery besides a partial fusion of the vertebrae C2/C3/C4 suggest a complex malformation at the site of the cyst. Disturbed developmental relationships in this case indicate that pathological blood vessels may represent a risk factor in the surgical treatment of neurenteric cysts.

[Diagnosis of cerebrovascular disorders using nuclear magnetic resonance tomography]. / Diagnostik zerebrovaskulärer Störungen mittels Kernspintomographie.

Regarding cerebrovascular diseases magnetic resonance imaging (MRI) provides informations which could not be obtained before by any other imaging method. MRI permits an early detection of ischemic infarctions, exact delineation of lesions near to skull base, and microangiopathy. Further diagnosis of arteriovenous malformations and venous thromboses can be done without using contrast media. Exclusion of acute hemorrhage or subarachnoidal hemorrhage is still a domain of computed tomography (CT). Other MR methods, like magnetic resonance angiography (MRA) and magnetic resonance spectroscopy (MRS) are expected to replace angiography and positron emission tomography (PET) in the near future, at least for some purposes.

Cranial MR imaging and cerebral 99mTc HM-PAO-SPECT in patients with subacute or chronic severe closed head injury and normal CT examinations.

Eighteen patients in the subacute or chronic state following severe closed head injury with normal cranial CT scans were examined by MR and 99mTc HM-PAO SPECT. Correlations were sought between these 2 imaging modalities and the clinical outcome, as defined by the Glasgow Outcome Scale (GOS) score. Both MR and SPECT revealed cerebral damage in all patients examined but structural and functional alterations did not coincide topographically in 64.9% of lesions. Nevertheless, complementary injury patterns suggesting poor recovery were found; cortical contusions and diffuse axonal injury (MR) in conjunction with cortical and thalamic hypoperfusion (SPECT) were noticed in 8 out of 12 patients with unfavorable outcome (GOS = III and IV). The synthesis of MR and SPECT information clearly enhanced the ability both to accurately assess posttraumatic brain damage and to improve patients' outcome prediction.

Septo-optic dysplasia and growth hormone deficiency: accelerated pubertal maturation during GH therapy.

We report four patients (three male, one female) with septo-optic dysplasia and growth hormone deficiency. All had GH therapy for a period of four to eight years until reaching final height. In all four cases bone maturation during puberty was accelerated (1.4 to 1.9 "years"/year), resulting in a final height which was clearly below the predicted height. The progress of pubertal stages was very short in all patients. In three patients TSH and prolactin release after TRH stimulation were increased. These data support a hypothalamic original of the endocrine disorder. Insufficient GH release, even after repeated GHRH stimulation, is in contrast to this assumption. In one case there was a late manifestation of neurohormonal diabetes insipidus, which indicates the possibility of later disease progression. MR imaging of the brain demonstrated variable malformation of the septum pellucidum, chiasma and nervus opticus or the pituitary gland, respectively.