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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.

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.

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.

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.

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.

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.

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 s- and 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).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.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.The increase of ICAM-3 levels might indicate the anti-inflammatory 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 —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.

Myotonic dystrophy (DM) is an autosomal dominant multisystem disorder involving muscle, brain, heart, eyes and endocrine organs, among others. The molecular basis is an unstable trinucleotide repeat at the 3'-untranslated end of the myotonin protein kinase gene on chromosome 19 q 13.3, and the number of repeats correlates with the severity of muscle weakness. We performed a clinical, psychometric and MRI study on 43 patients with DM and correlated findings with the molecular analysis. Nineteen patients had mild distal muscle weakness, 17 moderate und 7 severe weakness. Thirteen had marked cognitive deficits with reduced speed of cognition, low IQ, and apathy. MRI showed pathological muscle signal in 35 cases with a characteristic mosaic involving distal muscle groups, often sparing the posterior tibial muscle. Cerebral MRI showed significant subcortical white matter lesions in 20 cases and brain atrophy in 15 cases. Clinical and MRI findings of CNS and muscle both correlated with CTG repeat length, but did not parallel each other. DM is a significant disease of the brain as well as muscle, and several aspects of the disease correlate with molecular findings, with a threshold effect for repeats exceeding 1000 trinucleotides. The individual predominance of specific organ involvement probably depends on variable somatic mosaicism of the molecular defect.

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).

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.

In a 46 year old patient a syringomyelia and syringobulbia were diagnosed clinically 18 years after a transverse lesion of the cord and removal of an epidural spinal tuberculoma. Post-myelographic CT and NMR scans showed a communicating syringomyelia and syringobulbia. A flavectomy was performed and a syringopleural shunt implanted. The operation was successful by neuroradiological criteria. Clinically there was a worsening of the spastic gait during one-year follow up. Neuroradiological findings and etiology of the syrinx are discussed, and a causal relationship to the tuberculoma is proposed.

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.

Neurological manifestations of xeroderma pigmentosum, a rare autosomal recessive neurocutaneous syndrome, are variable. The association with progressive mental retardation, usually with onset in childhood, is well known. We present a case of x.p. with progressive presenile dementia. This combination has, to our knowledge, not yet been reported in the literature. Although no hints on another aetiology have been found, the coincidental combination of x.p. with M. Alzheimer has to be taken into consideration. CT scan and MRI showed a marked cerebral atrophy.

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.

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.

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.

The severity of neurological deficits, size of hypodense zone on CT, concentration of cAMP and activity of adenylate kinase in cerebrospinal fluid (CSF) were evaluated at predefined intervals in the acute stage of supratentorial cerebral ischaemic infarction in 52 patients. Patients with cerebral infarction had raised activities of adenylate kinase CSF as compared with normal persons. Patients with marked neurological deficits, only slight improvement of neurological signs and large infarction zones on CT had higher average activities of adenylate kinase and lower concentration of cAMP in CSF. Alterations of CSF adenylate kinase and CSF cAMP values were most distinct on the 3rd day after the stroke. Reasons for the changes may be metabolic disorders following brain ischaemia.

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.

Clinical symptoms, course of disease, cerebrospinal fluid (CSF) and cranial CT of 53 patients suffering from acute or subacute encephalitis were evaluated retrospectively. The virus could be identified in 21 (39%) patients. Nine of them had herpes simplex virus-encephalitis. 16 patients, eight with herpes simplex virus-encephalitis, died due to the disease. Complete restitution could be observed in 73% of survivors. Disturbance of consciousness and severe focal neurological deficit worsened prognosis towards letality and functional recovery. Most patients had initially elevated number of cellular elements and/or pathological protein concentration in CSF. CSF protein profile showed disturbance of blood-CSF-barrier function in a great number of patients during the first week of the disease whereas autochthonous production of immunoglobulin G was observed predominantly during the second and third week. Elevated concentration of CSF-lactate was seen in herpes simplex virus-encephalitis and in letal cases. 28 (53%) patients had pathological CT-findings. Generalized brain edema, focal hypodensities, focal and cortical contrast enhancement and hemorrhagic imbibation were observed. With one exception patients with herpes simplex virus-encephalitis had hypodense lesions in the temporal lobe. Besides this CT-findings did not allow conclusions regarding the etiology of encephalitis. Prognosis of encephalitis caused by other than herpes simplex virus was worse in case of pathological CT.

A 48-year-old man, who took by mistake a sip of ointment containing dichloroethane, survived, and showed a course of two phases of toxic symptoms. After an initial narcosis and an interval with few pathological symptoms seizures, myoclonia and somnolence occurred. Irreversible final disturbances were lasting mental defects, cerebellar dysarthria, ataxia, and hydrocephalus. Concomitant diseases were acute liver dystrophy, nephropathy, and anemia. The clinical picture of dichoroethane posoning is outlines, the pathogenesis of this particular cerebral lesion described, and the therapy discussed.

In 65 cases of ischemic cerebral infarction, CT scans and quantitative assessments of the neurological disturbances were undertaken at specific intervals during the 4 week period after stroke. Forty-three patients underwent lumbar puncture to determine the serum/CSF albumin ratio. The etiology of the infarction was evaluated on the basis of angiographic, ultrasonic and cardiologic findings. A hemorrhagic transformation of the infarction occurred in 28 patients, eleven within the first week, and 15 within the second. Risks of hemorrhage were correlated with a severe neurological deficit, disturbance of consciousness, large infarction with a mass effect, enhancement of contrast medium in CT (especially if occurring early), involvement of the cortex, and distinct blood/CSF barrier disturbances. Cardiac embolism was a frequent etiology in patients with secondary hemorrhagic infarction, especially when transformation occurred within the first week after stroke. In addition to a heterogeneous pattern of hemorrhage, frank hematoma predominated in those infarcts which underwent early transformation, while those transforming late often showed less hyperdense cortical hemorrhagic changes. Deterioration evident on clinical evaluation was caused by the hemorrhagic transformation in three cases, in each instance within the first week after stroke.

Fourteen of 330 patients with the clinical and laboratory supported definite diagnosis of Multiple Sklerose (MS) had epileptic seizures. The epilepsy of six patients probably originates from the MS. Four patients respectively suffered from genuin epilepsy or symptomatic epilepsy caused by other diseases than the MS. Most patients had GM, two GM and focal motor fits and one uncinatus fits. An epileptic focus in the EEG was evident in two patients. MRI- and CCT-scans frequently showed extensive cortex-neighboured lesions and multiple subcortical demyelination, especially localized in the temporal lobe. Epileptic seizures as a symptom of MS are very seldom. The longer the interval from the first episode of the MS to the first epileptic seizure the more probable epilepsy is caused by other reasons than the MS.

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 Barré 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.

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.

In a 46 year old patient a syringomyelia and syringobulbia were diagnosed clinically 18 years after a transverse lesion of the cord and removal of an epidural spinal tuberculoma. Post-myelographic CT and NMR scans showed a communicating syringomyelia and syringobulbia. A flavectomy was performed and a syringopleural shunt implanted. The operation was successful by neuroradiological criteria. Clinically there was a worsening of the spastic gait during one-year follow up. Neuroradiological findings and etiology of the syrinx are discussed, and a causal relationship to the tuberculoma is proposed.