Study of clinical features of amyloid angiopathy hemorrhage and hypertensive intracerebral hemorrhage.

OBJECTIVE: The purpose of this study was to differentiate between cerebral amyloid angiopathy (CAA) and hypertension (HTN) based on hemorrhage pattern interpretation. METHODS: From June 1994 to Oct., 2000, 83 patients admitted to our service with acute intracerebral hemorrhage (ICH) were investigated retrospectively; 41 patients with histologically proven diagnosis of cerebral amyloid angiography and 42 patients with clear history of hypertension were investigated. RESULTS: Patients with a CAA-related ICH were significantly older than patients with a HTN-related ICH (74.0 years vs 66.5 years, P < 0.05). There was a significantly higher number of hematomas > or = 30 ml in CAA (85.3%) when compared with HTN (59.5%). No basal ganglional hemorrhage was seen in CAA, but in 40.5% in HTN. In CAA-related ICH, subarachnoid hemorrhage (SAH) was seen in 26 patients (63.4%) compared to only 11 patients (26.2%) in HTN-related ICH. Intraventricular hemorrhage was seen in 24.4% in CAA, and in 26.2% in HTN. Typical features of CAA-related ICH included lobar distribution affecting mainly the lobar superficial areas, lobulated appearance, rupture into the subarachnoid space, and secondary IVH from the lobar hemorrhage. More specifically, multiplicity of hemorrhage, bilaterality, and repeated episodes also strongly suggest the diagnosis of CAA. Multiple hemorrhages, defined as 2 or more separate hematomas in multiple lobes, accounted for 17.1% in CAA-related ICH. CONCLUSION: There are certain features in CAA on CT and MRI and in clinical settings. To some extent, these features may contribute to distinguishing CAA from HTN related ICH.

Stroke pattern interpretation: the variability of hypertensive versus amyloid angiopathy hemorrhage.

INTRODUCTION: It is commonly felt that cerebral amyloid angiopathy (CAA) related intracerebral hemorrhage (ICH) can be distinguished from hypertension (HTN)-related ICH by certain typical features on computerized tomography (CT) and magnetic resonance imaging (MRI). The purpose of this study was to investigate the performance of clinicians who were asked to differentiate between CAA and HTN based on hemorrhage pattern interpretation and to assess the feasibility of such classification. METHODS: The admission scans from 83 patients who were admitted to our service with an acute ICH were presented to 5 clinicians in a randomized and blinded fashion (1 junior, and 1 senior neurosurgical resident, 1 attending neurosurgeon, and 2 neurosurgeon-neuroradiologists). There were no patients who received oral anticoagulants other than low-dose aspirin, or who suffered from vascular malformations or tumors. Scans from 41 patients with a histologically proven diagnosis of CAA and from 42 patients with a clear history of HTN were investigated. Hematoma evacuation was done in all CAA patients and in 59% of HTN patients (n = 25). RESULTS: The overall average classification accuracy was 66.8% (range: 62.7-69.9). For correct HTN classification it was 69.5% (range: 64.3-81), and 63.9% for CAA, respectively (range: 48.9-75.6). There were negligible differences in classification accuracy among all observers. Patients with a CAA-related ICH were significantly older than patients with a HTN-related ICH (74 vs. 66.5 years, p < 0.05). There was a significantly higher number of hematomas >30 ml in CAA (85.3%) when compared with HTN (59.5%). No basal ganglionic hemorrhage was seen in CAA, but in 40.5% in HTN. Intraventricular hemorrhage was seen in 24.4% in CAA, and in 26.2% in HTN. Two patients (4.9%) with CAA, and 7 patients with HTN (16.7%) presented with cerebellar hematomas. CONCLUSIONS: Three of 10 scans were not correctly diagnosed regardless of the examiner's level of training. This calls into question the reliability of classifying the underlying pathological condition based on hemorrhage pattern interpretation on CT or MRI. The definite diagnosis of CAA- versus HTN-related hemorrhage requires a histopathological confirmation and should not be based solely on hemorrhage pattern interpretation.

Differences that matter: major cytotoxic T cell-stimulating minor histocompatibility antigens.

Despite thousands of genetic polymorphisms among MHC matched mouse strains, a few unknown histocompatibility antigens are targeted by the cytotoxic T cells specific for tissue grafts. We isolated the cDNA of a novel BALB.B antigen gene that defines the polymorphic H28 locus on chromosome 3 and yields the naturally processed ILENFPRL (IFL8) peptide for presentation by Kb MHC to C57BI/6 CTL. The CTL specific for the IFL8/Kb and our previously identified H60/Kb complexes represent a major fraction of the B6 anti-BALB.B immune response. The immunodominance of these antigens can be explained by their differential transcription in the donor versus the host strains and their expression in professional donor antigen-presenting cells.

The same genetic regions encode minor histocompatibility antigens detected in the context of different MHC haplotypes.

The mammalian genome encodes numerous autosomal minor histocompatibilty (H) antigens that act as a barrier to allogeneic tissue transplantation when donor and recipient are matched for the major histocompatibilty complex (MHC). However, most of our understanding of the genetic basis of such minor H antigens is limited to analysis of mouse strains of the H2b haplotype. To broaden our understanding, we investigated minor H antigens detected in the context of other MHC alleles. As measured by graft survival and precursor cytotoxic T lymphocytes from blood, our results suggest that chromosomal segments known to encode minor H antigens detected in the context of the H2b haplotype are antigenically complex in that they also frequently encode minor H antigens that can be detected in the context of at least the H2d and H2k haplotypes. Genetic models to account for this complexity are discussed.

[Neuronavigation. Computer-assisted surgery in neurosurgery]. / Neuronavigation. Computergestütztes Operieren in der Neurochirurgie.

Computers in Neurosurgery were limited to diagnostics, planning stereotactic procedures, Radiosurgery and Radiation therapy. The possibility of intra-operative localisation only evolved since the mid 80's with the advent of more powerful computers. The computer adds more precision to microneurosurgical procedures, allowing neuronavigation in interaction with the computer. Diverse neuronavigationsystems are described in the literature [7-9, 11, 13]. We are working with the "ISG Viewing Wand" since april 1994. 113 operations were planed and executed aided by this system. We report on our experiences, the advantages and limitations of this system.

"Ultrahigh" dexamethasone in acute brain injury. Results from a prospective randomized double-blind multicenter trial (GUDHIS). German Ultrahigh Dexamethasone Head Injury Study Group.

In a prospective randomized double-blind multicenter trial, the efficacy and safety of a 51-hour ultra-high intravenous dexamethasone dosing regimen was investigated in patients with moderate and severe head injury. 300 patients between 15 and 55 years of age were randomized to receive either placebo or dexamethasone: 500 mg intravenous infusion within 3 h after trauma initially, followed by 200 mg after 3 h, thereafter 8 times 200 mg at 6 hourly intervals, resulting in a total administered dose of 2,3 g in 51 hours. Primary end points for assessment of efficacy were: Modified Glasgow Coma Scale (grading 3-16) on Day 5, modified Glasgow Outcome Scale (grading 1-6) 10-14 months after injury, and the time interval until consciousness improved above a level of modified GCS > or = 8. Secondary endpoints were CT results and neurological and laboratory data. The two groups were well matched with respect to important prognostic variables, such as age, severity of trauma, and interval between trauma and application of the drug. 269 patients (89.7%) were available for final examination after 10-14 months. Results were surprisingly favourable in both groups: Lethality in the dexamethasone and placebo group was 14.3 and 15.4%, respectively, and 61.7 and 57.4%, respectively, achieved social and professional rehabilitation after 10-14 months (outcome scale 6). No statistical difference was seen between the dexamethasone and the placebo group in any of the primary end points of efficacy and safety (incidence of upper gastrointestinal bleeding, infection, and thrombosis).(ABSTRACT TRUNCATED AT 250 WORDS)

Clinical signs associated with megadolichobasilar artery anomaly.

The elongation and ectatic course of the basilar artery (BA), called megadolichobasilar artery anomaly (MDBAA) is a macroscopic description of a neuroradiologic finding. Clinically ischemic brain stem syndromes and peripheral cranial nerve disturbances especially of the trigeminal and facial nerves, cerebellar dysfunction and CSF circulation disturbances are observed. Seldom a subarachnoid hemorrhage is proven. In CT and MRI often a tumorlike mass with a ringlike contrast enhancement combined with a nonhomogeneous lesion due to partial thrombosis, is detected. Angiography in most cases shows fusiform dilatation of the BA, elongation of the top of the basilar trunk, asymmetric tortuosity or dislocation of the irregular shaped wall of the BA. Fifteen patients within the last 12 years are demonstrated. The role of reticular fiber deficiency in the media and defects of the elastic lamina as the basis of these malformations is reported. We discuss coincidental findings of MDBAA with atherosclerosis, congenital factors causing generalized vasculopathy, metabolic disturbances in form of so called inborn errors of metabolism and endocrine deficiencies. In cases with clinical signs the morbidity is remarkably high.

Intradural manifestation of rheumatoid arthritis causing spinal cord compression.

Rheumatoid arthritis as a cause of medullary compression due to subluxation of rheumatically diseased joints is very common. However, spinal cord compression by rheumatoid nodules is seen rarely, usually by extradural lesions. We describe two cases of intradural rheumatoid nodules causing spinal cord compression.

[Malignant schwannoma of the trigeminal nerve]. / Malignes Schwannom des Nervus trigeminus.

We report on diagnosis, treatment and clinical course of a solitary schwannoma of the trigeminal nerve with consecutive metastatic spread in a 45-year-old woman. The patient presented a temporal lobe syndrome with psychomotor seizures. Trigeminal nerve function was intact apart from a transient hypaesthesia in the mandibular branch. Assuming a meningioma the tumor was removed via a temporal approach. Secondary to the definitive histological diagnosis radiation therapy was performed. After that the patient was symptom-free. 4 months later a large recurrent tumor was found involving the cavernous sinus and the pterygopalatine fossa. In a second operation the tumor was resected intra- and extradurally through an infratemporal preauricular approach in cooperation with an oral and maxillo-facial surgeon. At this time multiple pulmonary metastases developed showing no response to polychemotherapy (EVI). The patient died 13 months after onset of the disease. Hitherto, only 5 cases of a primary malignant schwannoma of the trigeminal nerve have been published in the world literature.

[Space-occupying cyst formation following surgery of malignant gliomas]. / Raumfordernde Zystenbildung nach der Operation maligner Gliome.

Incidence and follow-up of patients with space-occupying cystic lesions after surgery of malignant gliomas are demonstrated. Aetiological aspects and therapeutic possibilities are mentioned.

Cerebellar atrophy following diphenylhydantoin intoxication.

Seven epileptic patients with permanent ataxic dysfunction following DPH treatment are described. The ataxia correlates with cerebellar atrophy, though the extents of clinical and structural lesions are not necessarily proportional. Cerebellar atrophy is demonstrated by CT scans, the vermal region seems to be predominantly affected.--The tentatively increased susceptibility of female patients and of patients with pre-existing brain lesions, as well as the possible consequences pertaining to the course of the epilepsy are discussed.

[Fat embolism as a complication in shock. Clinical and therapeutic aspects (author's transl)]. / Fettembolie als Komplikation des Schockgeschehens. Klinische und therapeutische Aspekte.

In 36 patients the changes of the haemostasis in relation to hypovolaemic shock period were studied after polytrauma and or surgical operations. It was shown that a correlation between the hypovolaemic shock period and the degree of haemostatic disorder exists. The significance of this interaction for the manifestation of the fat embolism syndrome was demonstrated in two casuistics. It is concluded that the haemostatic disorder in shock enhances the development of the fat embolism syndrome. As a therapeutic consequence adequate volume substitution beside specific anticoagulation seems to be of utmost importance in the posttraumatic treatment.

[Large cerebral ateriovenous malformation (author's transl)]. / Ausgedehnte zerebrale arteriovenöse Gefässmissbildung.

A large cerebral arteriovenous malformation, which showed first no typical symptoms of an angioma as seizures or bleedings, but signs of a transitoric ischemic attack, is described. In computertomography there was not the angioma corresponding figure, but in angiography.

Computerized tomography in vascular malformations of the brain.

Computerized tomography scans of 166 patients - 99 cases with 117 aneurysms, and 67 cases with angiomatous malformations - were reviewed and correlated with angiographic and clinical findings. An intracranial hemorrhage was identified by CT scan in 103 patients, in 60 cases with aneurysms (17 only subarachnoid, 43 intracerebral), and in 43 cases with angiomas (6 only subarachnoid, 37 intracerebral). The source of hemorrhage was diagnosed with varying degrees of certainty with CT scan in 12 (12%) cases with aneurysms and in 27 (40%) cases with angiomas. Further CT findings were low density lesions in 18 cases indicating vasogenic edma, and calcifications in 10 angiomas. Intravenous infusion of contrast material was necessary to distinguish smaller aneurysms and angiomas. CT scan demonstrated the presence and location of thrombosed portions of giant aneurysms; these findings were less apparent with angiography. In 29 angiomas of medium or large size (macroangiomas) the malformation was demonstrated with CT scan in 25 (86%) cases and with angiography in 26 cases. 3 angiographically occult angiomas were diagnosed with CT as low density lesions and misinterpreted as gliomas. In all 36 microangiomas only the hemorrhage was precisely diagnosed with CT. The microangioma was identified angiographically in 24 cases and histologically in 21. In Sturge-Weber's disease the calcifications may be seen earlier with CT than with skull x-ray.

Cranial computertomography in children with tuberous sclerosis.

20 children with tuberous sclerosis were examined by computertomography (CCT). The test is useful to detect cerebral involvement with great certainty. The method is especially helpful in the early diagnosis of the disease. This offers the possibility of giving an early genetic advice. Characteristic CCT scan abnormalities in tuberous sclerosis are multiple densities in the walls and the roof of lateral ventricles. This specific finding can already be seen prior to the occurrence of calcifications in standard roentgenograms.