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Purpose of this study was to define a subgroup of TIA/stroke patients who should be examined by transthoracal and transesophageal echocardiography or Holter-electrocardiography to identify those with cardiogenic brain embolism reliably; 300 consecutive patients with acute focal brain ischemia underwent a standardized diagnostic protocol for the evaluation of the etiology including, clinical examination by a cardiologist and routine electrocardiography, Holter-electrocardiography, transthoracal and transesophageal echocardiography. 188 patients had a potential cardiac source of embolism. In particular echocardiography was diagnostic in 163 patients, and Holter-electrocardiography 10; 159 of these 188 patients (84.6%) had competitive etiologies, predominantly large vessel atherosclerosis. In 136 patients cardiogenic brain embolism was assumed as quite definite or possible. To identify these patients reliably, transthoracal and transesophageal echocardiography would have been necessary in 89% of the entire group of patients (all with clinically cardiological abnormalities, pathological routine ECG, without vascular risk factors, or no atherosclerosis in duplex sonography), and Holter-electrocardiography in 54%.

Introduction - MELAS is most often due to an maternally transmitted A-G transition mutation of mitochondrial DNA (mt DNA) at position 3243. In this study we report on the clinical spectrum associated with the mutation in the largest family reported so far. Patients and methods - In a family with three MELAS cases we identified 47 persons at risk for the mutation ; sufficient data was available on 29. Mitochondrial disease was diagnosed in two of 9 deceased members (posthumous molecular analysis in one) ; 27 surviving family members underwent examination and 25 a molecular analysis of mt DNA from lymphoblasts. Then had a muscle biopsy and two were later autopsied. Results - All 26 cases investigated by molecular analysis showed the mutation at position 3243. The 18 symptomatic patients without stroke-like episodes had sensorineural hearing loss in 15 cases, diabetes in 6, nephropathy in 7, mild myopathy in 4, cardiomyopathy in 2, cerebellar disease in 4 and mental retardation in 2 cases. Eight carriers were asymptomatic. Autopsy showed > 80% mutant mt DNA in all tissues except blood (20%) examined in a MELAS patient, but

During a four-week trip to Nigeria a 54-year-old German developed a fever of 39 degrees C. Later on he had lymphadenopathy, pretibial oedema, dyspnoea and weight loss. After 16 weeks a wreath-like pale pink skin rash, increased pulse rate with pulse deficit and hepatosplenomegaly were noted. Abnormal laboratory findings were an increased blood sedimentation rate (95 mm), raised immunoglobulin M (483 mg/dl), haemoglobin of 12.0 g/dl, mean corpuscular volume of 76 fl and Borrelia IgM antibody titre of 1:512. The electrocardiogram was suggestive of myocarditis: the cardiac symptoms were controlled with digoxin and verapamil. The patient's general condition deteriorated while he was receiving antibiotic treatment with tetracycline and penicillin. Cerebrospinal fluid (CSF) showed an increased cell count (39/microliters) and albumin (0.98 g/dl). There was a mild, predominantly proximal, tetraplegia which--on the basis of electromyographic and biopsy findings--was thought to be due to polyneuritis and myositis. At this stage blood smear and CSF examination revealed Trypanosoma. He thereupon received suramin (1.0 g) and prednisolone (120 mg down to 40 mg) daily, to which melarsoprol was added after 6 days (0.5 ml up to 5.0 ml daily for 36 days). Almost all symptoms then regressed within 6 weeks.

Technical improvements accomplished over the last few years allow imaging of intracranial parenchymal structures as well as of basal cerebral arteries by ultrasound. Low frequency probes (2-2.5 MHz) and colour Doppler technology are required for these examinations. Recent experience in clinical practice proves that perfusion patterns in basal cerebral arteries can be depicted more accurately and with better reproducibility compared to conventional transcranial Doppler sonography. Simultaneous B-Mode imaging allows detection of cerebral haemorrhage, brain tumours and ventricular enlargement. Preliminary data on the sensitivity and specificity are promising and encourage further prospective studies.

Objective - The expression of intercellular adhesion molecule-3 (ICAM-3), a member of the Ig supergene family, is restricted to immune competent cells. Expression of soluble and cell surface ICAM-3 (s- and c-ICAM-3) is preferentially seen in the state of low activation of the immune system. We studied the relevance of the expression levels of sand c-ICAM-3 in cerebrospinal fluid (CSF) and blood as markers for disease activity as well as the influence of high-dose methylprednisolone (MP) treatment upon the expression of s- and c-ICAM-3 in blood of patients with multiple sclerosis (MS). Materials and methods - A total of 33 patients (relapses n = 25, remission n = 8) with relapsing-remitting MS were included into the study. CSF and blood were acquired from all of them. Of the patients 24 were treated with high-dose MP. In those, blood was additionally collected at the 10th day of the therapy and after 3 months. Expression of c-ICAM-3 was determined by two colour FACS analysis, whereas the concentration levels of s-ICAM-3 were measured by ELISA, Results - In CSF we detected a significant decrease of the expression levels of c-ICAM-3 on CD3 + T cells in 25 patients suffering from an acute relapse in contrast to 8 patients with remission (P=0.04). In comparison to the levels before treatment and after 3 months, at the 10th day of MP treatment we obtained highly significant changes of the expression values of c-ICAM-3 both on CD3 + T cells (P=0.0004; P=0.005) and CD14 + monocytes/macrophages (P=0.0006; P=0.008) on the 10th day of high-dose MP treatment from 24 MS patients. Conclusion - The increase of ICAM-3 levels might indicate the antiinflammatory effect of the MP treatment. It could be interesting to search for similar effects investigating the new immune modulatoring therapy forms of MS.

Background and Purpose —Transcranial color-coded duplex sonography (TCCS) of intracranial veins and sinuses in adults is a new, emerging application of ultrasonographic imaging. This study reports a standardized examination protocol for venous TCCS and provides reference data for clinical application. Methods —In 130 healthy volunteers (mean age, 45.9±16.9 years; range, 14 to 77 years) the intracranial venous system was examined using frequency-based transtemporal TCCS. Identification rate, blood flow velocity , resistance index, and systolic/diastolic ratio were recorded for each examined venous vessel. Results —Intracranial veins and sinuses show a low pulsatile forward flow with maximal systolic blood flow velocity up to 20 cm/s. Significant side differences of blood flow velocity in the paired venous structures could not be detected. Venous flow velocities decreased with age, whereas resistance indices and systolic/diastolic ratios increased. Women showed higher flow velocities than men. Mean identification rates for all age groups ranged from 70% to 90% for the deep middle cerebral vein, the basal cerebral vein, and the great cerebral vein of Galen. The straight sinus, the transverse sinus, and the rostral part of the superior sagittal sinus could be detected in 55% to 70% of cases. Detection rates were dependent on age and decreased as age increased. Conclusions —Venous TCCS can reliably image a significant part of the cerebral venous system. This method can provide information on venous hemodynamics in normal subjects and pathological cases.

Injection-induced palsy of the ischiatic nerve with concomitant damage of other peripheral nerves occurred in two patients following intramuscular injection in the upper outer quarter of the large gluteal muscle. In close chronological connection skin changes developed in both cases representing drug-induced cutaneous embolism, and in one case extensive muscular necrosis was observed. In both cases the cause may be assumed to be ischaemic neuropathy following embolic occlusion of the vasa nervorum as a result of accidental intraarterial injection.

To ascertain the varieties of neuroborreliosis, 330 patients were identified at the Departments of Neurology in Wurzburg and Giessen from 1979 to 1994. Patients who fullfilled at least one of three strict case definitions based on clinical and laboratory criteria were included in the study. Ninety-one per cent of the patients had second-stage neuroborreliosis (duration of symptoms ≤6 months). The most common syndrome was a painful spinal meningoradiculitis, alone (37%) or in combination with a cranial radiculitis (29%). Meningoradiculitis cranialis (9%), isolated meningitis (4%) and erythema chronica migrans-associated mono/ polyneuritis (3%) were further stage II features. Central nervous system involvement occurred either as an acute meningomyelitis or meningomyeloradiculitis (5%) and meningoencephalitis or meningenocephaloradiculitis (4%). Less than 9% of the patients ran a chronic course (stage III) with a disease duration between 6 months and 9 years, either as acrodermatitis chronica atrophicans associated mono- or polyneuritis (2%) or a chronic progressive encephalomyelitis (6%). Cerebrovascular neuroborreliosis (1%) occurred in both stages; however, the primary nature of the course was a chronic one. Involvement of other organs except the skin was rare (joints 3%, heart 1%) but elevated hepatic enzymes were frequent. Our study demonstrates that neuroborreliosis has to be considered in the differential diagnosis of a wide variety of neurological conditions. Cerebrospinal fluid analysis and the search for specific intrathecal antibody production are important diagnostic procedures.

Eight carriers of the A3243G mutation of mitochondrial DNA without stroke-like episodes were monitored for up to 7 years in clinical and metabolic studies, by magnetic resonance imaging (MRI) and positron emission tomography (PET). None developed mitochondrial encephalopathy (MELAS), but 2 developed diabetes mellitus, 1 terminal kidney failure and 2 cardiomyopathy. One patient improved markedly under ubiquinone. Electroencephalography showed progressive slowing in 2 cases, but electrophysiological tests and MRI were otherwise noncontributary. PET showed widespread cortical and basal ganglion metabolic deficits in 6 cases. We conclude that internal medical complications are more common than MELAS in adult carriers of the mutation. PET findings, firstly reported in such patients, suggest that chronic subclinical encephalopathy is very frequent, and PET may play a role in monitoring in the future. Dieser Beitrag ist mit Zustimmung des Rechteinhabers aufgrund einer (DFG-geförderten) Allianz- bzw. Nationallizenz frei zugänglich.

Abnormal amplification of a CTG repeat on chromosome 19 is the molecular basis of myotonic dystrophy (DM). Expansion of the repeat has been correlated with severity of several clinical features of the disease. We performed extensive cognitive testing, cerebral magnetic resonance imaging (MRI) and a molecular analysis in 28 cases of DM to determine the relationship between the molecular defect and brain disease. Performance in two or more cognitive tests was pathological in 10 cases. Fourteen patients had subcortical white matter lesions on MRI, 14 had cerebral atrophy. Amplification of the CTG repeat showed a strong correlation with cognitive test deficits when exceeding a length of over 1000 trinucleotides. MRI lesions were associated with impaired psychometric performance, but MRI and molecular findings were only weakly related. Disease duration influenced the appearance and amount of white matter lesions on MRI. Quantification of CTG repeat size may allow an early estimate on the probability of brain involvement in DM; cognitive dysfunction is associated with white matter lesions and cerebral atrophy later on in the course.

It is well established from pediatric experience and animal experiments that intracerebral blood can be demonstrated by B-mode real-time duplex scanning. This has recently become feasible in adults as well. The present study investigated the changes in the sonographic appearance of intracerebral hematomas over the course of time. Starting in May 1991, 23 consecutive patients with intracerebral hematoma confirmed by computed tomography (21 spontaneous and 2 traumatic hematomas) were investigated within 1 year. They were monitored by repeated ultrasound scanning via the transtemporal approach. The sonographic appearance of the hematomas on B-mode scans and the angle-corrected blood flow velocity in the basal cerebral arteries were assessed. There was unequivocal localization of the hematoma in 18 patients (78%). In 3 cases (13%), an adequate acoustic window could not be found. One small intracerebral hemorrhage was overlooked, and one extensive hemorrhage in the basal ganglia was misdiagnosed as a lobar hematoma. There was an alteration of the appearance of the hematoma with time. This was divided into three sonographic stages (initial stage, days 1 to 5; intermediate stage, days 6 to 10; and capsular stage, from day 10). In 14 of the 20 patients with an appropriate acoustic bony echo window, the blood flow velocity in the middle cerebral artery could be measured; in 1 of these patients, the signs of increasing intracranial pressure were apparent from Doppler frequency spectrum. In 5 patients, the intracerebral hematoma could be imaged but not the ipsilateral middle cerebral artery. One female patient showed cerebral circulatory arrest at the time of examination, which took place within 24 hours after the onset of clinical symptoms. Most intracerebral hematomas in adults can be imaged in B-mode. Their sonographic appearance changes over the course of the disease. The advantages of this noninvasive method are its easy bedside operation and its suitability for follow-up; it is also less stressful than other imaging procedures. It yields a combination of structural and functional diagnostic information. In approximately 13% of the cases, the investigation was not feasible because of inadequate ultrasonic penetration of the intact skull.

Because the frequency of hemorrhagic transformation of cerebral infarcts is still a matter of controversy, we performed a prospective magnetic resonance imaging study in a series of consecutive patients with cardioembolic stroke. Among 200 consecutive patients with transient ischemic attack or ischemic stroke, 41 were identified with a computed tomographically proven supratentorial infarct due to cardiogenic embolism. Magnetic resonance imaging (T1-weighted) was performed 3 weeks after the stroke in 35 of these 41 patients. Eight patients received anticoagulants. Magnetic resonance images showed hemorrhagic transformation in 68.6% (24) of the 35 infarcts, always without clinical deterioration. In a stepwise forward logistic regression analysis only the volume of infarction edema on the initial computed tomogram was linked significantly with the risk of hemorrhagic transformation (p = 0.037). Hemorrhages were a regular finding on magnetic resonance images of infarcts exceeding a volume of 10 cm3 (94.4%, 17 of 18). Hemorrhagic transformation is a regular finding in medium-sized and large cardioembolic infarcts. Thus, in therapeutic and preventive studies of acute stroke the severity, not the frequency, of hemorrhages into brain infarcts should be the matter of interest.

We examined whether the pattern of cerebral hemorrhage changed after the introduction of computed tomography. Using a prospective data base we analyzed the case-fatality ratio, early mortality, and the incidences of hypertension and anticoagulant medication in 488 consecutive cases with supratentorial hemorrhage between 1978 and 1989. Blood pressure at admission of all patients for the years 1978-1979 and 1988-1989 and the Mathew and activities of daily living scores for the years 1978-1979, 1982-1983, and 1988-1989 were assessed retrospectively. The case-fatality ratio decreased from 49% in the period 1978-1981 to 31% in 1986-1989 (p = 0.006); early mortality (day 1-4) decreased from 28% to 12% (p = 0.0017). The incidence of hypertension decreased from 78% in the period 1978-1981 to 64% in 1986-1989 (p = 0.01). Systolic and diastolic blood pressure at admission sank (p = 0.09). The decrease of the case-fatality ratio correlated best with a less severe initial disturbance of consciousness (p less than 0.01) and with a higher Mathew score (p = 0.038). The activities of daily living score remained unchanged. The case-fatality ratio and early mortality decreased after the introduction of computed tomography. This was basically due to a decreased incidence of comatose and stuporous patients with severe neurological deficit paralleled by a decrease of mean systolic and diastolic blood pressure values at admission.

Clinical, CT, Doppler, and angiographic findings of 404 consecutive patients with brainstem/cerebellar strokes were registered in a data bank. One hundred and sixty-nine of the patients (41.8%) had a

Results of clinical investigations and CCT of 23 patients with thalamic infarctions were evaluated retrospectively. Nineteen patients underwent a clinical and 15 a neuropsychological examination one to 5 years after their stroke. Regarding the affected vascular territory, the patients were divided into the following subgroups: paramedian infarctions (posterior thalamo-subthalamic arteries, 13 patients, among them 5 bilateral infarcts); anterolateral infarcts (tuberothalamic arteries, 5 patients); posterolateral infarcts (thalamo-geniculate arteries, 5 patients). The leading symptoms of paramedian thalamic infarcts were disturbance of consciousness, amnesia and vertical gaze palsy. The patients with anterolateral thalamic infarctions became acutely confused and disorientated, whereas those with posterolateral infarcts suffered from focal neurological deficits in the first place. Five patients with leftsided thalamic infarctions of varying localization were aphasic. Two patients died within the observation period. A slight hemiparesis was detected in two patients and a vertical gaze palsy in 4, respectively, by the neurological reexamination one to 5 years after the stroke, whereas the clinical findings in 11 patients were completely normal. However 5 patients had a chronic psychosyndrome. The neuropsychological examination showed in some cases disorders of visual retention and verbal function as well as deficits in concentration. Neither the character nor the degree of the neuropsychological deficits did depend on the affected vascular territory but on the existence of further cerebral infarcts on CT-scan.

Acute episodes of focal neurological dysfunction are a well-recognized complication of the mitochondrial encephalomyopathies. Because of rapid remission, biochemical tests and other diagnostic procedures are mostly performed after the acute phase. We report the case of a patient suffering from mitochondrial disease manifesting primarily with seizures, progressive deafness and dementia, who experienced multiple stroke-like episodes. Other members of the family with evidence of mitochondrial dysfunction are presented briefly. EEG and biochemical findings in the acute stage are correlated with clinical symptoms, showing characteristics distinct from the chronic illness. The possible involvement of dietary factors in the provocation of stroke-like episodes is discussed and regulation of glucose intake suggested as a strategy in the prevention of stroke-like episodes.

We investigated incidence, age distribution in relation to etiology, and localization of spontaneous intracerebral hematoma in 100 consecutive cases. Incidence in the total population of the Giessen area was estimated to be greater than 11/100,000 inhabitants/yr and increased with age. There was a trend toward higher incidence in males. Overall mortality was 27%, 22% of 58 patients aged less than 70 years and 33% of 42 patients aged greater than or equal to 70 years. Hypertensive putaminal hematoma showed the highest mortality rate (42%, 10 of 24 cases). Chronic alcoholism and anticoagulant medication influenced the mortality rate unfavourably. We found the following localizations and etiologies to have a specific relation with age: 1) lobar hematomas from vascular malformations, group aged less than 40 years; 2) hypertensive putaminal hematomas and hypertensive thalamic hematomas, group aged 40-69 years; and 3) lobar hematomas, group aged greater than or equal to 70 years. Alcoholism was an additional factor in 38% of the 13 middle-aged men with hypertensive putaminal hematomas. Fourteen cases of spontaneous intracerebral hematoma were possibly due to cerebral amyloid angiopathy. Six of these 14 patients had recurrent lobar hematomas, but only three of the six could be histologically investigated. In these three cases, cerebral amyloid angiopathy was proven.

In this study 11 patients aged between 34 and 57 years with clinical and angiographic findings typical of carotid dissection were thoroughly examined with transcranial Doppler ultrasound (TCD) repeatedly during a follow-up period of 1-6 months. Stagnating blood flow velocities in the downstream middle cerebral artery (MCA) were recorded initially as well as enhanced velocities due to postischaemic hyperperfusion syndrome. MCA embolism originating from the extracranial carotid artery with or without resolution could be detected by TCD in 5 cases. TCD findings in another 2 cases pointed to haemodynamic upset. In 3 cases, clinical as well as TCD data did not allow strict differentiation between embolic and haemodynamic complications, suggesting more complex pathophysiological mechanisms as the cause of infarction. Carotid recanalization was seen in 9 cases. The follow-up investigations moreover demonstrated that intracranial haemodynamics may change from day to day in patients suffering from internal carotid artery dissection. TCD data may thus improve the understanding of pathogenetics as well as rationales for individual therapeutic intervention in this particular disease.

Neurologic, cardiologic, and dopplersonographic findings of 303 patients undergoing coronary bypass surgery were evaluated in a prospective study. About 5% of the patients suffered from a prior stroke. Nearly 9% had a significant stenosis of an internal carotid artery detected by dopplersonography. Central nervous system complications occurred in 18.2% of the patients, predominantly temporary mental disturbances. Old patients and those with complicating heart failure were at higher risk for mental disorders. Approximately 3% of the patients suffered from a stroke with a permanent deficit. Half of them had a potential source of cardiogenic embolism. The risk of a complicating stroke was raised by the presence of a stenosis of a carotid artery, a heart failure, or an arrhythmia. The frequency of peripheral nervous system complications was 13.5% with temporary symptoms in about half of the cases. Brachial plexus paresis predominated with a particular risk in mammaria bypass grafting.

Background and Purpose —The use of the conventional temporal bone window for transcranial color-coded duplex sonography (TCCS) often results in difficulties in obtaining angle-corrected flow velocity measurements of the A2 segment of the anterior cerebral artery, the posterior communicating artery, and the midline venous vasculature because of the unfavorable insonation angle. The same applies to B-mode imaging of the frontal parenchyma. However, transorbital TCCS raises problems with the insonation of the orbital lens. To overcome these drawbacks, we studied the feasibility of frontal bone windows for TCCS examinations. Methods —In 75 healthy volunteers (mean age, 45.3±17.0 years; age range, 17 to 77 years), the circle of Willis and the venous midline vasculature were insonated through a lateral and paramedian frontal bone window. Insonation quality of parenchymal structures (B-mode) was graded on a 3-point scale depending on the visibility of typical parenchymal landmarks. In a similar manner, the quality of the color-/Doppler-mode imaging of the arteries of the circle of Willis and the internal cerebral veins was assessed. In 15 patients (mean age, 62.7±13.7 years; age range, 33 to 83 years), the color-/Doppler-mode imaging quality of the intracranial vessels before and after application of an ultrasound contrast-enhancing agent was compared. Results —B-mode insonation quality was optimal to fair in 73.3% of cases using the lateral and in 52.0% of cases using the paramedian frontal bone window, with defined parenchymal structures used as reference. Insonation quality decreased in those older than 60 years. In those younger than 60 years, angle-corrected flow velocity measurements of the A2 segment of the anterior cerebral artery and the internal cerebral vein were possible in 73.6% and 60.0%, respectively. Contrast enhancement resulted in a highly significant improvement in the imaging quality of the intracranial vessels. Conclusions —The transfrontal bone windows offer new possibilities for TCCS examinations, although the insonation quality is inferior to the conventional temporal bone window in terms of failure of an acoustic window. This can be compensated for by application of an ultrasound contrast-enhancing agent.

Objective - The intercellular adhesion molecule-1 (ICAM-1) is a member of the Ig supergene family. ICAM-1 is expressed on various cells like peripheral blood lymphocytes, endothelial cells or thymic cells and the cell surface form is supposed to be shed into a soluble form. The expression of ICAM-1 is induced by cytokines like Interleukin-1, TNF alpha or interferon gamma. The aim of the study was to investigate whether changes of cell surface and soluble ICAM-1 in the cerebrospinal fluid (CSF) and blood are indicative for disease activity in patients with multiple sclerosis (MS). Material and methods - In all patients with relapsing-remitting MS (relapse: n=31, remission: n= 11) and controls (n= 13) the expression of cell surface ICAM-1 (c-ICAM-1) was determined by two colour flow cytometry. Soluble ICAM-1 (s-ICAM-1) was measured by ELISA. Follow-up examinations were done 3 months later. Results - In 31 patients with a current relapse we found significantly decreased expression levels of c-ICAM-1 on leukocytes in CSF (P

One hundred untreated neuroborreliosis patients were investigated by IgG/IgM immunoblot to find out if different stages and syndromes are characterized by different patterns of their Borrelia burgdorferi specific immune responses in CSF and serum. Stage III (n = 10) was characterized by a broad, highly specific intrathecal immune response (the mean number of IgM bands in CSF was 3.1 and of IgG bands 6.3). All patients recognized one or more of the following proteins (p35, p21, p18) or the 5 kd glycolipid. In contrast, the immune response in stage II (n = 90) was less restricted (28%) and heterogeneous (mean number of IgM bands 1.4 and of IgG bands 3.4). It was mainly directed against the highly crossreactive p41 antigen (91%). The different clinical features of stage II were comparable regarding the intrathecal immune response. However anti-glycolipid and anti-p35, -p21, -p18 IgG antibodies were detected in a small subset of patients, mainly corresponding to more severe courses of the disease. Our data are compatible with a direct agent-related pathomechanism in neuroborreliosis. Antibodies against certain proteins and the glycolipid of B. burgdorferi seem to have a prognostic value as to the development of more severe disease or transition to stage III.

Magnetic resonance imaging (MRI) permits efficient visualization of white matter lesions (WML). A growing body of literature deals with the correlation of WML and cognitive dysfunction with conflicting results. We studied the influence of lesion pattern as well as size by analyzing MRI and psychometric test performance in 2 patient collectives with different WML patterns. 22 patients with myotonic dystrophy (MD) and mainly subcortical WML were compared with 39 patients with multiple sclerosis (MS) and mainly periventricular lesions. 73% of MD patients had WML, the extent of which correlated with cognitive deficits. Severely impaired patients had psychometric findings compatible with "subcortical" dementia. In MS the extent of WML alone did not correlate significantly with cognitive deficits. Significant cognitive dysfunction was observed with extension of WML to areas of white matter immediately underlying cortex, but not with exclusively periventricular lesions. Cerebral atrophy had less impact. Comparison of MD and MS indicates that WML immediately subjacent to cortex are likely to cause significant cognitive deficits, whereas extensive periventricular demyelination may cause no major dysfunction. This may relate to early disturbance of associative fibers by subcortical lesions. Our results emphasize the significance of pattern as well as total extent of WML. Myotonic dystrophy is a useful model to study the effect of subcortical lesions, due to a typical lesion pattern unusual in other conditions.

Patent foramen ovale as a possible stroke risk factor can be diagnosed with transcranial Doppler sonography (TCD) by detecting intravenous contrast medium crossing from the right to the left atrium. The present study evaluates the reliability of this method. We performed TCD and transesophageal echocardiography simultaneously in 50 patients using galactose microbubbles. We observed bubble signals passing the middle cerebral artery in 7 patients less than 20 seconds after injection; we found positive TCD tests in 14 patients using the Valsalva maneuver. With transesophageal echocardiography patent foramen ovale could be detected in 15 patients (sensitivity, 0.93; specificity, 1; P < .01). TCD with echo contrast is a reliable screening tool for patent foramen ovale. A standardized procedure including the Valsalva maneuver is essential to prevent false-negative results.

CSF cytology, immunocytochemistry and biochemical tumor markers were compared in 12 patients with a meningeal carcinomatosis regarding diagnostic significance and therapy control; 50 samples were investigated. Cytology was tumor-positive in 39 samples and immunocytochemistry in 45 samples. Intrathecally produced tumor markers were found in 47 samples. On initial examination only immunocytochemical testing correctly classified all cases. Cytology and biochemical tumor markers revealed positive results in 10 respectively 11 of 12 patients, combined use of these two methods would increase the sensitivity to 100%. On follow-up examination tumor markers correlated best with symptoms and were early indicators for clinical relapse. These results could be achieved only if several monoclonal antibodies and biochemical tumor marker tests were combined. It is concluded that immunocytochemistry and biochemical tumor markers are of major help regarding the problems of false-negative cytology and reliable therapy control in meningeal carcinomatosis.

Functional outcome and recurrence rate were evaluated retrospectively in a series of 566 consecutive patients with a cardioembolic TIA or stroke. Seventy-five patients had a TIA/RIND, 163 a minor stroke, 238 a major deficit, and 90 a fatal event. In a logistic regression analysis the probability of a disabling or fatal outcome was significantly influenced by age (p = 0.0023), a previous stroke (p = 0.0262), concomitant carotid artery disease in Doppler sonography (p = 0.0210), and the underlying heart disease (p = 0.0001). Fatal or major strokes predominated among patients with nonvalvular atrial fibrillation, prosthetic heart valves, acute or chronic myocardial infarction. Kaplan-Meier estimates revealed a cumulative risk of recurrent cerebral embolism of 2.9% within three weeks. In a proportional hazards model only the underlying heart disease was significantly linked with the risk of recurrent embolism (p = 0.021).

A rare case of aseptic thrombosis of the right vein of Labbe in a young woman is reported. Cerebral venous thrombosis was suggested by computed tomography and confirmed by angiography. Mild left-sided neurological deficits resolved almost completely. The combination of a nonspecific clinical picture with an atypical lesion on CT may favour the diagnosis of cortical venous thrombosis.

One hundred patients with spontaneous subarachnoid hemorrhage due to aneurysm or presumed aneurysm consecutively admitted to a neurological clinic and subjected to CCT during the first 72 hours were examined retrospectively. The outcome after two months as defined by the Glasgow Outcome Scale (GOS) was relatively good: 23% of the patients suffered management mortality (GOS I) (postoperative lethality 8%), 3% showed GOS-Grade II, 14% grade III, 17% grade IV, and 43% grade V. The extent of intracranial hemorrhage correlated well with the initial Hunt-Hess Grade which, in turn, had a strong influence on case fatality and the degree of disability. Lethal factors were: 1. massive subarachnoid hemorrhage together with a massive ventricular hemorrhage (p < 0.001), 2. massive subarachnoid hemorrhage together with an intracerebral hematoma > 20 ml (p < 0.05). Case fatality was lower when angiography was negative. In our study rebleeding (12%) and delayed cerebral ischemia (DCI) (18%) were less frequent and the lethality due to acute hydrocephalus (5%) and delayed cerebral ischemia (5%) was less pronounced than in comparable studies. The degree of disability (GOS) was directly related to the amount of intracranial blood, to the development of acute or chronic hydrocephalus, delayed cerebral ischemia and rebleeding. DCI occurred in 60% of patients with marked hydrocephalus. Rebleeding was more frequent in patients with acute hydrocephalus. Hydrocephalus, DCI, and rebleeding were associated with a poorer initial grade on the Hunt and Hess Scale.

Myotonic dystrophy (MD) is characterized by myotonia, weakness and extramuscular symptoms, including intellectual impairment. We performed magnetic resonance imaging (MRI) of brain and muscle in 25 MD patients: 81% had cerebral atrophy (severe in 36%); 68% had focal white matter lesions, which were large and multiple in 27%. Brain MRI findings correlated with mental impairment; the severity of both correlated with disease duration. Changes in brain and muscle MRI were progressive with time, but independent of each other. Muscle MRI findings were fatty degeneration and loss of bulk. In the calves, the medial gastrocnemius muscles were involved earliest and the posterior tibial muscles relatively spared. In the thighs the vastus muscles were damaged most often and the rectus femoris least. Focal muscle damage was efficiently visualized, sometimes preceding clinical detection. Muscle MRI was less sensitive than conventional methods for early diagnosis, but ideal for follow-up, owing to its non-invasiveness and examiner-independence.

This study was performed to gather information about long-term prognosis after infratentorial transient ischemic attacks and minor strokes and about the factors influencing it. We included 226 patients with transient ischemia and 169 patients with a minor stroke of the brain stem/cerebellum consecutively admitted to a neurological department. Medical records and the findings of computed tomography, Doppler ultrasonography, and angiography were evaluated retrospectively. Follow-up information was gathered from the patients and their physicians by questionnaires. Complete follow-up information was available for 381 patients. During a mean follow-up of 3.9 years, 15.7% of the 381 patients suffered a stroke and 6.8% a myocardial infarction; 15% died. Kaplan-Meier estimates revealed a cumulative stroke rate of 5.1% within the first year and a risk of stroke, myocardial infarction, or death of any cause of 9.8%. In a proportional hazards model, the time-dependent risk of stroke was significantly increased by increasing age (p = 0.018), minor stroke (p = 0.0005), hypertension (p = 0.022), previous stroke (p = 0.0006), and carotid artery occlusive disease (p = 0.0065). The probability of stroke, myocardial infarction, or death was influenced by age (p = 0.0001), minor stroke (p = 0.006), diabetes (p = 0.015), previous stroke (p = 0.002), infarct on a computed tomogram (p = 0.041), and carotid artery disease (p = 0.032). Long-term prognosis after brain stem/cerebellar transient ischemic attacks and minor strokes is significantly influenced by age, diabetes, hypertension, previous stroke, and concomitant carotid artery disease. Patients with transient ischemic attacks have a better prognosis than those with minor stroke.

We evaluated the efficacy of transcranial Doppler ultrasonography in 23 patients suffering from acute middle cerebral artery occlusion. The diagnosis of occlusion was most suggestive when all basal arteries except the affected middle cerebral artery were detectable. Enhanced blood flow velocity in the anterior cerebral artery due to leptomeningeal collateralization was used as a corroborating criterion. With frequent follow-up examinations, we monitored reperfusion of the M1 segment resulting from recanalization or embolus migration in 16 patients. Those patients undergoing recanalization within days after onset of the first symptoms revealed variable clinical courses and lesion patterns on computed tomography, indicating the crucial importance of early and efficient leptomeningeal collateral blood supply. Transcranial Doppler ultrasonography was able to exclude middle cerebral artery occlusion with accuracy, which provides important clinical information. However, distal branch occlusions could not be detected with sufficient exactness.

Clinical, CT and EEG findings of 100 consecutive patients suffering from epileptic seizures following hemispheric cerebral infarction were evaluated retrospectively. All patients were followed up for an average of 49 months. Twelve patients suffered from cardiogenic brain embolism, 20 had an occlusive disease of the internal cerebral artery. Forty-seven patients had a single middle cerebral artery infarct, 6 each a posterior cerebral artery or watershed infarct. CT showed lacunes in only 6 cases, 8 had multiple larger infarcts and 27 were normal. Seventy-six patients suffered from generalized seizures, 54 from partial fits, predominantly focal motor seizures. Seventy-one patients had their first seizure within the 1st year after stroke, 30 within 2 weeks after the infarct. The interval between stroke and the first epileptic fit exceeded 1 year in the remaining 29 cases. If the first fit occurred in the acute phase after stroke, the risk of further ones was significantly lower than when the first fit occurred in the chronic stage. The frequency of fits mainly depended on the occurrence of epileptic potentials in the EEG and the interval between stroke and the first seizure. During follow-up 27 patients suffered a recurrent stroke, and 52 patients died. Cardiac failure was the predominant cause of death; only 4 patients died as a consequence of an epileptic fit.

Using a computerized gamma camera regional cerebral blood flow and regional cerebral blood transit time are measured after injection of Xenon 133 and Technetium 99m respectively into the internal carotid artery. Equipment, technical procedures and methodology are described. Typical cases are presented to describe the usefullness of this procedure.

A 40-year-old man suffered from a right parietal oligodendroglioma. Two months after the onset of clinical symptoms complete removal of the tumor was attempted. At second craniotomy four years later the tumor was found to invade the falx cerebri. The patient died seven years after the first admission to the hospital. Necropsy revealed the right parietal oligodendroglioma invading the sagittal sinus and the left hemisphere as well as metastases in the lung, the lymph-nodes, the vertebra, and the femur.

During 1977 to 1985 2008 patients suffering from acute ischemic cerebral infarcts were admitted to the Department of Neurology, Giessen University. 213 (10.6%) died during their stay at hospital of 28 days in average. Time of survival, cause of death, localization and etiology of the infarcts were evaluated retrospectively on base of the medical reports, the angiographic, doppler-sonographic, computerized tomographic, and autopsy findings. The same number of patients died because of direct sequelae of stroke and secondary complications respectively. Those with supratentorial infarcts, who died in consequence of a vegetative dysregulation generally did not survive the first week after ictus, often had infarcts exceeding the supply area of one large cerebral artery and frequently had evidence of cardiac embolism. Pathogenetic factors for extension of the ischemic cerebral damage subsequently causing transtentorial herniation were spreading thrombosis, reinfarction, and fatal secondary hemorrhage. Patients dying in consequence of an infratentorial infarct often had a thrombosis of the basilar artery or a large cerebellar infarction. Secondary fatal complications mostly occurred after the first week after stroke. Pulmonary edema, pulmonary embolism and myocardial infarction predominated with different time pattern.

Total CSF protein and CSF/serum albumin and alpha 2-macroglobulin ratios as indicators of a disturbed blood-brain barrier were determined in 39 cases of ischaemic cerebral infarction proved by computed tomography (CT). About 50% of the patients had a barrier disturbance, whereat the CSF serum albumin ratio was shown to be the most sensitive parameter. A disturbed blood-brain barrier was more often found in cases of large infarction, as shown by CT, and occurred most frequently in the first 2 weeks of illness. No correlation was seen between enhancement in CT and disturbance of the blood-brain barrier.

The significance of the measurement of lactate, lysozyme and PHI in CSF for differential diagnosis of meningitis was examined in 58 cases of viral, 36 of bacterial and 5 of tuberculous etiology. In the early phase of the illness CSF lactate was found to be the most sensitive parameter for distinction of viral from bacterial or tuberculous meningitis respectively. Except for one case CSF lactate exceeded 3.8 mmol/l in all cases of bacterial etiology, whereas this value was never reached in any case of viral meningitis. While lactate concentration was maximal on the day of admission and declined continuously thereafter, PHI activity reached its maximum on the third day after beginning of the therapy. At this time all patients with a bacterial or tuberculous meningitis had PHI activities about 50 U/l. This value wasn't exceeded in any case of viral meningitis. In a few cases some days after onset of therapy a distinction of bacterial meningitis from viral forms was still possible by PHI determination but not by lactate measurement. Determination of lysozyme also could be helpful in the later phase of the disease.

Clinical data and Cerebrospinal fluid (CSF) findings of 20 patients suffering from a meningoradiculitis were analyzed. Most patients had a tick bite or an erythema migrans before neurological symptoms occurred. All suffered from severe radicular pain. Predominant neurological symptoms were peripheral palsies of the facial nerve followed by motor paresis of the trunk and the extremities of mostly asymmetric distribution. Predominant CSF alterations were a long lasting inflammatory cellular reaction and an impairment of the blood-CSF barrier. In contrast to the CSF of 17 patients suffering from a polyradiculitis Guillain Barre with a similar total protein content, an intrathecal immunoglobulin IgG synthesis could be confirmed for the majority of the patients. CSF finding would be compatible to a viral as well as a borrelia etiology.

CT contrast enhancement of the ischemic infarction, blood-CSF barrier function for albumin, and severity of neurological symptoms were evaluated at predefined intervals in 41 patients with supratentorial ischemic infarctions. Contrast enhancement was most frequently observed in the 2nd and 3rd week after the stroke. This late CT enhancement was not related to infarction size and severity of blood-CSF barrier disturbance. The rare appearance of CT enhancement in the 1st week was usually associated with extensive infarctions and accompanied by blood-CSF barrier disturbances. These barrier disturbances, which occurred with higher frequency and greater severity in extensive infarctions (peak 3rd day), generally persisted for several weeks. We suggest that contrast enhancement in the 1st week after an ischemic stroke is due to diapedesis from necrotic capillaries; the more frequently observed late enhancement might be the result of a blood-brain barrier disturbance which in turn is hypothetically attributed to increased pinocytotic activity of regenerated endothelial cells.

Bericht uber einen Patienten, der mit 36 Jahren an einer Papulosis atrophicans maligna erkrankte und 3 Jahre spater neurologische Symptome entwickelte, die dem Bild einer lumbosacral beginnenden aufsteigenden todlichen Polyradiculitis mit Hirnnervenbeteiligung entsprachen. 4 Monate nach den ersten neurologischen Symptomen kam es auch zu gastrointestinalen Erscheinungen. Ergebnisse der Muskel- und Nervenbiopsien werden mitgeteilt. Auf die wenigen im Schrifttum mitgeteilten Beobachtungen dieser seltenen Krankheit wird Bezug genommen. Dabei stellt sich heraus, das die Beteiligung des Nervensystems an der Symptomatologie dieser Krankheit nach den dermatologischen und intestinalen Symptomen an dritter Stelle liegt.

Die Meswerte des Ophthalmodynamogrammes von 57 Patienten mit einer Carotisobliteration wurden mit den Werten einer Kontrollgruppe verglichen und statistisch analysiert. 2 von 3 Patienten mit einem kompletten Carotisverschlus oder einer signifikanten Stenose hatten eine pathologische Seitendifferenz der Pulsationsvolumina der Aa. ophthalmicae, wahrend die Druckwerte entweder nicht auszuwerten oder nicht seitenverschieden waren.