Changes within the "normal" cerebral white matter of multiple sclerosis patients during acute attacks and during high-dose cortisone therapy assessed by means of quantitative MRI.

Changes in the apparently unaffected cerebral white matter of multiple sclerosis (MS) patients were studied during acute attacks as well as during high-dose prednisolone therapy. Serial MR scans of patients with a clinically definite diagnosis were performed on four defined occasions: before an episode, within three days after its onset, after 10 days of therapy as well as four weeks later. Thirteen patients agreed to cooperate in forming a MRI data base and to be rescanned immediately after the onset of an acute relapse. Within one year, six patients had such episodes, one of them had a second bout. Both T1 and T2 relaxation times within the apparently normal white matter were significantly prolonged in all cerebral lobes compared to a control group of healthy volunteers. During the acute attacks as well as during therapy the T1 values remained as before. The T2 values were elevated only in two out of six cases during the episode. After therapy a considerable clinical improvement was seen in all cases, but a significant T2 decrease as a possible effect of cortisone was noted in only one case. We conclude that the prolonged relaxation times T1 and T2 within the apparently normal cerebral white matter of MS patients are the result of a number of molecular events differing considerably among individual patients and that serial measurements of these relaxation times do not consistently change during an acute relapse and do not reflect clinical improvement after high dose prednisolone therapy.

[Involvement of apparently normal white brain substance in the disease process of multiple sclerosis]. / Mitbeteiligung der scheinbar normalen weissen Hirnsubstanz am Krankheitsprozess der multiplen Sklerose.

In a group of patients with a clinically definite diagnosis of multiple sclerosis (MS) the cerebral white matter was investigated by means of quantitative nuclear magnetic resonance imaging (MRI). Measurements were taken from plaque-free regions and compared to a control group of healthy volunteers. A significant prolongation of T1 and T2 values was seen for all regions investigated (frontobasal, temporal, temporoparietal, and upper parietal white matter). In a comparison of MS patients with a short duration of the disease (mean: 1.7 years) with MS patients with a long-standing course of the disease (mean: 15.2 years) a significant prolongation of T2 relaxation times was found in the upper frontal and upper parietal white matter only for the latter group. In keeping with neuropathological findings the noted prolongation of T2 values might most probably be due to an increase of extracellular water secondary to astroglial proliferation. The increase of relaxation times could either be due to degeneration of commissural fibers or be the result of an exhausted capacity for remyelination. Both events are known to occur predominantly in the later stage of the disease. It is concluded that MS represents a disease which is not restricted to limited areas of demyelination but rather involves the entire cerebral white matter. These findings imply possibilities for the assessment of disease progression as well as for therapy.

[Incidence and clinical importance of chronic reactive periosteal new bone formation in the cervical region in patients with differing neurologic symptomatology]. / Inzidenz und klinische Bedeutung chronisch-reaktiver periostaler Knochenneubildungen im Zervikalbereich bei Patienten mit unterschiedlicher neurologischer Symptomatologie.

81 patients classed into three groups with clinical evidence of neurological symptoms and posttraumatic pain of the cervical spine and the incidence of degenerative disorders were studied noninvasively via CT scanning. Imaging of osseous structures in the axial plane by CT was excellent in all cases, even in the lower part of the cervical spine where soft-tissue discrimination is often impossible because of shoulder artifacts. In about half of the patients with nerve-root symptomatology as well as with signs of involvement of long tracts, narrowing of the foramen intervertebral, respectively of the spinal tract, was seen, attributable to degenerative osseous apposition with excellent clinical segmental and (according to radicular symptoms) side correlation. In contrast to these results the group of patients with posttraumatic clinical symptoms showed almost 50 per cent less preexisting degenerative disorders of the cervical spine. In conclusion, we assume that high-grade osseous appositions of the dorsal part of the vertebral body play an important role in the development of radicular symptomatology and cervical myelopathy, respectively. Hypertrophic changes of the processus articularis with narrowing of the spinal canal occurred in 14 per cent and were therefore of minor clinical significance.

Structural brain correlates of anterograde memory deficits in multiple sclerosis.

Progressive decline of anterograde memory functions has been increasingly recognized as a frequent symptom in chronic multiple sclerosis. In order to investigate the brain structures involved, magnetic resonance imaging was performed in 20 patients. Neuropsychological assessment included the WAIS and WMS subtests information, picture completion, similarities, digit span, logical memory, and paired associate learning. All patients with severely impaired memory functions (n = 5) showed bilateral lesions in the medial temporal lobe, whereas in those patients with moderate (n = 10) or no measurable impairment of memory testing (n = 5) either no lesions were seen in the medial temporal lobes or these lesions were restricted to one side. A post hoc cluster analysis strikingly confirmed these results. The differences could not be related to the age of the patients, the disease duration, or the level of education. Extensive lesions in the white matter of the frontal lobes, thinning and lining of the corpus callosum, and bilateral involvement of the anterior cingulate gyrus had no bearing on the neuropsychological results. These findings indicate that bilateral demyelination in the hippocampal regions is the most likely explanation for the impairment of anterograde memory in such patients.

[Reversible opening of the blood-brain barrier in the chemotherapy of malignant gliomas]. / Reversible Blut-Hirn-Schrankenöffnung in der Chemotherapie maligner Gliome.

The concept of a blood brain or blood tumour barrier blocking the passage of lipid insoluble cytoreductive drugs from blood into brain-tumor, leads to a protocol of intraarterial injection of high dose methotrexate (MTX) during reversible, osmotic blood brain barrier disruption (BBBD). Malignant Gliomas of grades III and IV in 9 patients and one primary CNS-lymphoma in one patient have been treated in 2 to 5 sessions per patient with BBBD plus polychemotherapy (MTX, cyclophosphamide, procarbazine). Median progression free intervals (PFI) which are still in continuation are 12.2 months. Median PFI which had been terminated by tumour recurrence are 7.75 months.

Topological characteristics of brainstem lesions in clinically definite and clinically probable cases of multiple sclerosis: an MRI-study.

Disseminated lesions in the white matter of the cerebral hemispheres and confluent lesions at the borders of the lateral ventricles as seen on MRI are both considered acceptable paraclinical evidence for the diagnosis of multiple sclerosis. Similar changes are, however, also found in vascular diseases of the brain. We therefore aimed at identifying those additional traits in the infratentorial region, which in our experience are not frequently found in cerebrovascular pathology. We evaluated MR brain scans of 68 patients and found pontine lesions in 71% of cases with a clinically definite diagnosis (17 out of 24) and in 33% of cases with a probable diagnosis (14 out of 43). Lesions in the medulla oblongata were present in 50% and 16%, respectively, and in the midbrain in 25% and 7%, respectively. With rare exceptions all brainstem lesions were contiguous with the cisternal or ventricular cerebrospinal fluid spaces. In keeping with post-mortem reports the morphological spectrum ranged from large confluent patches to solitary, well delineated paramedian lesions or discrete linings of the cerebrospinal fluid border zones and were most clearly depicted form horizontal and sagittal T2 weighted SE-sequences. If there is a predilection for the outer or inner surfaces of the brainstem, such lesions can be considered an additional typical feature of multiple sclerosis and can be more reliably weighted as paraclinical evidence for a definite diagnosis.

Blood brain barrier modification and chemotherapy. Interventional neuroradiology in the treatment of malignant gliomas.

Postoperative interventional neuroradiology was performed in patients with malignant gliomas to increase target efficacy of chemotherapy. In 8 glioma patients the blood brain or blood tumor barrier was reversibly opened by intraarterial injection of hyperosmolar fluid (Mannitol 25%). One additional patient had primary lymphoma of the central nervous system. During barrier modification chemotherapeutic agents were applied intraarterially and intravenously. A total of 22 blood brain barrier modification procedures have been carried out until now, ranging from one to five per patient. A presently continuing tumor regression or tumor progression free intervals have been noted in 5 patients. Therapeutic effects are being evaluated from repeated computed tomography and single photon emission computed tomography examinations.

[Real-time sonography of the skull in the newborn]. / Real-time-Sonographie des Schädels beim Neugeborenen.

The results of 293 examinations of the skull by sonography in 170 newborn are presented. In 73 patients the results could be compared with the findings on CT or at post mortem. In 48%, abnormal sonograms of the skull were obtained, due to congenital abnormalities, hydrocephalus or intracerebral bleeds. High accuracy and wide application make real time sonography the diagnostic procedure of first choice for the examination of the brain of the newborn. An attempt has been made to determine the optimum time for the examination and suitable intervals for follow-up.

BAEP abnormalities in tardive dyskinesia.

To evaluate brainstem dysfunction, brainstem auditory-evoked potentials (BAEPs) were recorded in 13 patients with tardive dyskinesia. Only patients under 60 years of age were included because of the spontaneous dyskinetic syndromes that frequently occur in elderly persons. Clinical assessment was performed with the Abnormal Involuntary Movement Scale (AIMS). BAEP measurements were taken in order to detect lesions in the brainstem. In 14 of 26 pathways (13 patients), abnormalities in the BAEPs were found which corresponded well to lesions found in former neuropathological reports of patients with tardive dyskinesia. The functional disturbance causing abnormal BAEPs may be an expression of structural brain changes in tardive dyskinesia patients.

Tardive dyskinesia: clinical correlation with computed tomography in patients aged less than 60 years.

In a prevalence study of 335 psychiatric in-patients 49 patients (14.6%) with tardive dyskinesia were found. In view of the high prevalence of spontaneous dyskinetic syndromes in elderly patients only patients under 60 years were included (n = 21; mean age: 44.9). Clinical rating was performed with the AIMS-scale. CT measurements of ventricular enlargement and cortical atrophy were obtained. Tardive dyskinesia cases did not differ significantly from healthy controls, though some patients with severe dyskinesia showed signs of brain atrophy. These findings did not provide evidence to support neuropathological reports describing neuronal cell loss and midbrain gliosis in such patients. It is concluded that such structural brain changes in tardive dyskinesia patients are not detectable with present CT technology: they may either be due to concurrent aging processes or, in the case of younger patients, can be confirmed only by more functional methods of testing.

[Acute cortical blindness: a reversible complication of acute kidney failure in a child with burns]. / Akute Rindenblindheit: Eine reversible Komplikation des akuten Nierenversagens bei einem Kind mit Verbrennung.

An 11 year old boy was admitted to the Department of Pediatrics Medical School of Vienna with 2nd and 3rd degree burns covering 30% of his body. He presented with complications--high fever, vomiting, diarrhea and dehydration--which had led to acute renal failure. After 6 hemodialyses renal function recovered after two weeks and the patient entered a polyuric phase. In connection with a transient dehydration the patient showed a sudden bilateral cortical blindness. The computerized tomogram (CT) showed vague evidence of an occipital cortical ischemia. We assume that several factors have played a role in this sudden occurrence. As a result of hypovolemia and coincident anemia and electrolyte inbalance, cerebral edema and cortical tissue hypoxia with emphasis in the occipital cortical region developed in the brain possibly already damaged by burn injury. A complete reversal of the clinical state was achieved. The patient was discharged with normal vision and normalized renal function.

Angiographic findings in relation to clinical course and results of computed tomography in cerebrovascular disease.

Clinical course, results of angiography and computerized tomography (CT) were compared in 204 patients with focal cerebral ischemias. The extent and the importance of the radiological results depend on the localization of the affected area, whereby essential differences exist between extra- and intracranial arteries as well as between the vertebrobasilar and the carotid arterial flow region. As to the carotid arterial system there appear to be significant correlations between the size of the infarction on the CT and the extent of the vessel stenosis on angiography. Furthermore the likelihood of deficit recovery is inversely proportional to the degree of pathology seen in both radiologic examinations. It is discussed whether these correlations, obtained from the examination of a great number of patients, can help to evaluate the significance of equivocal angiographic findings in individual cases.

[Primary diffuse leptomeningeal melanoblastosis and its CT appearances (author's transl)]. / Primäre diffuse Melanoblastose der Leptomeningen und CT-Veränderungen.

We report the case of a twelve-year-old boy who died, after a six months history, from primary diffuse leptomeningeal melanoblastosis, CT investigations showed a saw-tooth contrast enhancement at the brain surface. CT investigations may therefore be very helpful in meningeal lesions - apart from the importance of CSF investigations.

[Unilateral congenital anophthalmos with Trabecular agenesia and hydrophthalmos of the other eye (author's transl)]. / Einseitiger angeborener Anophthalmus mit Balken-agenesie und Hydrophthalmus am anderen Auge.

In a one-year-old female patient, unilateral anophthalmos was ascertained by clinical, echographic and X-ray examination. In addition, the cranial computer tomogram showed trabecular agenesia. The globe of the other eye was existent and was enlarged by hydrophthalmos. Pediatrically this case represents a syndrome with multiple anomalies of etiologically unknown origin; apart from the ocular malformation there are a severe cerebral developmental disturbance, an apallic syndrome, hypsarrhythmia, peculiar disproportional bodily structure with skeletal anomalies as well as an abnormal susceptibility to infections of the respiratory system and a marked, isolated IgA-deficiency. Neither chromosomal anomaly nor familiarity was discovered.

[The diagnostic value of cranial computerized tomography in exogenous psychoses and mental disorders associated with organic disease (author's transl)]. / Diagnostischer Wert der kranialen Computertomographie bei exogenen Psychosen und psychischen Störungen im Rahmen von körperlichen Erkrankungen.

205 patients hospitalized in a psychiatric department were subjected to cranial computerized tomography. The diagnosis on discharge according to the ICD key in each case was "psychoses or mental disorders associated with physical conditions". Pathological findings were obtained in 67.8% of the patients. In the case of space-occupying lesions the connection between psychiatric symptoms and syndromes and the underlying organic disease is obvious. Moreover, with inflammatory, vascular or traumatic diseases, a relationship to focal lesions found on the computer tomography appears likely if backed by appropriate neurological indications in the history or on clinical examinations. However, when alterations signifying diffuse brain atrophy are obtained on computer tomography any connections with psychiatric features should be very critically evaluated and, in individual cases, such connections had better not be looked for at all. Especially in cases of psychoses or mental disorders associated with physical conditions cranial computerized tomography is of high diagnostic value and the indications for its implementation in these cases should be judged very leniently.