Cerebral metastases of an allogenic renal cell carcinoma in a heart recipient without renal cell carcinoma.
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Brain Neoplasms↗
Biomedical subjects
D Krieger
Publications and source records attributed to D Krieger.
Neuropsychiatric profile and hyperintense globus pallidus on T1-weighted magnetic resonance images in liver cirrhosis.
BACKGROUND & AIMS: Hyperintense globus pallidus on T1-weighted magnetic resonance images (MRIs) in cirrhotic patients are reported to reflect severity of liver disease; however, their consequence for hepatic encephalopathy is unknown. The aim of this study was to outline a pattern of neuropsychiatric abnormalities in chronic liver failure and its correlation with MRI findings. METHODS: Fifty-one consecutive patients were enrolled in the study. Neuropsychiatric assessment used a standardized protocol, including dichotomized neurological parameters, brief psychiatric rating and psychometric tests, as well as electroencephalography. The severity of liver failure was graded using standard laboratory parameters and the Child-Pugh's classification. Signal intensity of the globus pallidus was determined on sagittal T1-weighted MRIs. RESULTS: Two aspects of neurological dysfunction could be distinguished by principal components analysis: impairment of complex cerebral function and subcortical motor performance. Both neurological categories correlated with severity of liver failure, grade of electroencephalographic abnormalities, and psychometric test results. Additionally, prior bouts of overt encephalopathy indicate progressive dementia. T1-weighted globus pallidus signal intensity did not correlate with any clinical or laboratory test result. CONCLUSIONS: This study shows a characteristic pattern of neurological findings in patients with liver failure and hyperintense globus pallidus on T1-weighted MRIs. Although neurological dysfunction parallels hepatic failure, MRI abnormalities of basal ganglia do not indicate severity of actual hepatic encephalopathy.
Adolescent↗
A novel two-nucleotide deletion in the ornithine transcarbamylase gene causing fatal hyperammonia in early pregnancy.
Ornithine transcarbamylase (OTC) deficiency shows X-linked inheritance. Typically, symptomatic females (who constitute 15%-20% of all carriers) have markedly reduced enzyme activity and show first symptoms in late infancy or early childhood. Here we present the case of a previously asymptomatic 24-year-old woman who died of severe hyperammonemia associated with orotic aciduria but normal OTC activity in the fourth month of pregnancy. DNA analysis revealed a novel mutation in form of the deletion of two nucleotides (T892, G893) in exon 9 of the OTC gene, leading to a frame shift and an aberrant gene product. We suggest that OTC deficiency should be suspected in any patient who presents with hyperammonia in the presence of otherwise normal liver function.
Adult↗
Critical illness polyneuropathy: clinical findings and outcomes of a frequent cause of neuromuscular weaning failure.
OBJECTIVE: To describe clinical and electrophysiologic features and outcomes of critically ill patients with neuromuscular causes of failure to wean from mechanical ventilator support. DESIGN: A prospective, consecutive, case series. SETTING: Neurological, neurosurgical, and medical intensive care units in a university hospital. PATIENTS: Seven patients during a 3-yr period with failure to wean from mechanical ventilation not explained by pulmonary complications. INTERVENTIONS: Muscle and nerve biopsy in three patients. MEASUREMENTS AND MAIN RESULTS: Detailed electrodiagnostic studies were done in all patients 3 to 6 wks (median 4.5) after the onset of the acute illness and were repeated 3 months to 3.5 yrs later in those patients who survived. Primary illnesses included various intracranial and medical conditions. All patients had moderate-to-severe limb weakness with marked muscle atrophy. Tendon reflexes were decreased in three patients, exaggerated in two patients with intracranial lesions, and absent in two patients. Electromyography demonstrated severe acute denervation, with striking involvement of proximal muscles. Muscle and nerve biopsies showed severe neurogenic atrophy and axonal degeneration without inflammation. There was no evidence of primary myopathy. Two patients died of complications of sepsis. Of the survivors, three patients had no further weakness at the time of reexamination, except for peroneal nerve palsy in one patient. Two patients, still in the recovery period, showed markedly improved conditions but still showed slight weakness of the proximal muscles. By electrophysiology, signs of chronic neurogenic damage were demonstrable in all survivors at follow-up. CONCLUSIONS: Critical illness polyneuropathy is a frequent cause of neuromuscular weaning failure in critically ill patients, regardless of the type of primary illness. Involvement of proximal (including facial and paraspinal) muscles is striking. Tendon reflexes are often preserved. Patients with central nervous system injury may likewise develop critical illness polyneuropathy. In these latter patients, tendon reflexes may even be exaggerated. Recovery from critical illness polyneuropathy is usually rapid and clinically complete, although incomplete on electrodiagnostic study. Residual peripheral nerve lesion, generally of the peroneal nerve, is the most frequent feature of incomplete recovery. The need for careful electrophysiologic testing is emphasized to clarify the nature and extent of neuromuscular disturbances in critically ill patients. Failure to recognize the development of neuropathy in these patients may lead to erroneous conclusions about the ability to wean them from the ventilator.
Adult↗
Cortical hyperintensity on proton density-weighted images: An MR sign of cyclosporine-related encephalopathy.
PURPOSE: To describe cortical hyperintensities in proton density-weighted images in six patients with presumed cyclosporine-induced neurotoxicity. METHODS: In six patients with clinical evidence of cyclosporine-related encephalopathy, MR imaging was performed after the onset of symptoms and signs (mean, 24 days after liver transplantation). Five of these patients had serial MR imaging for a period that varied from 2 to 20 months. Along with the imaging studies, the patients' clinical status was evaluated and various laboratory parameters, including blood pressure and levels of cyclosporine, cholesterol, and magnesium, were monitored. RESULTS: In all six patients, initial MR studies showed hyperintensity of several cerebral gyri that was unequivocal only on proton density-weighted images. Although in five patients these signal abnormalities were limited to the cortex, one patient had increased signal in the subjacent white matter as well. In one patient, the images were also remarkable for areas of cortical hyperintensities on T1-weighted images. In another patient, cortical enhancement occurred after administration of gadopentetate dimeglumine, with a normal cortical signal on the precontrast images. The abnormal cortical signal began to fade after cyclosporine reduction, but in two patients it remained visible for at least 20 months. The neurologic symptomatology associated with cyclosporine-induced neurotoxicity included seizures (three patients), speech disorder (three patients), and disturbance of consciousness (three patients). CONCLUSION: Cyclosporine-induced neurotoxicity occurring in patients after liver transplantation appears to affect the cerebral cortex preferentially. Because its MR equivalent resembles changes resulting from hypoxic injury or cortically centered vasculitis, we suspect the underlying mechanism may be a vascular injury that results in cortical hypoperfusion.
Adolescent↗
Manganese and chronic hepatic encephalopathy.
Clinical observations and animal studies have raised the hypothesis that increased concentrations of manganese (Mn) in whole blood might lead to accumulation of this metal within the basal ganglia in patients with end-stage liver disease. We studied ten patients with liver failure (and ten controls) by magnetic resonance imaging (MRI) and measurement of Mn in brain tissue of three patients who died of progressive liver failure (and three controls) was also done. Whole blood Mn concentrations in patients with liver cirrhosis were significantly increased (median 34.4 micrograms/L vs 10.3 micrograms/L in controls; p = 0.0004) and pallidal signal intensity indices correlated with blood Mn (Rs = 0.8, p = 0.0058). Brain tissue samples reveal highest Mn concentrations in the caudate nucleus, followed by the quadrigeminal plate and globus pallidus. Mn accumulates within the basal ganglia in liver cirrhosis. Similarities between Mn neurotoxicity and chronic hepatic encephalopathy suggest that this metal may have a role in the pathogenesis of chronic hepatic encephalopathy. Further studies are warranted because the use of chelating agents could prove to be a new therapeutic option to prevent or reverse this neuropsychiatric syndrome.
Adult↗
Rare diseases mimicking acute vertebrobasilar artery thrombosis.
Acute ischaemia of the vertebrobasilar circulation leads to a variety of clinical manifestation and is mostly due to cardiogenic or artery-to-artery embolism. We describe four neurological emergency situations involving vertebrobasilar artery aclusion of other origins; basilar migraine, extrinsic compression by rheumatoid inflammatory tissue, generalized vasculitis in subacute rheumatic fever and basilar artery dissection. The differential diagnosis of acute vertebrobasilar artery occlusion may have an important impact on patient management.
Acute Disease↗
Serial somatosensory and brainstem auditory evoked potentials in monitoring of acute supratentorial mass lesions.
OBJECTIVE: To determine the relevance of serial evoked potentials (brainstem auditory evoked potentials and somatosensory evoked potentials) and clinical parameters (pupillary response and intracranial pressure) in patients with acute supratentorial mass lesions. DESIGN: Prospective case series of comatose patients with acute supratentorial mass lesions. SETTING: Neurocritical care unit of a tertiary care center. PATIENTS: Thirty consecutive patients with the following study inclusion criteria: a) clinical and computed tomography evidence of an acute supratentorial mass lesion; b) implantation of an intracranial pressure monitoring device; and c) a persistent comatose state during the observation period. INTERVENTIONS: Brainstem auditory evoked potentials, somatosensory evoked potentials, intracranial pressure, and pupillary responses were recorded at the time of three particular events: a) immediately after implantation of an epidural intracranial pressure monitoring device; b) during intracranial pressure therapy; and c) at termination of intracranial pressure therapy. Evoked potential results were ranked into three categories: a) normal on both sides; b) abnormal or absent on one side; and c) evoked potentials on both sides abnormal or absent. Spearman's rank correlation was performed to analyze serial recordings. Cross tables were generated to determine the prognostic value of evoked potentials and clinical parameters. Fisher's exact test was applied to calculate statistical significance. MEASUREMENTS AND MAIN RESULTS: Intracranial pressure values correlated with pupillary responses and brainstem auditory evoked potentials during and at the termination of intracranial pressure therapy. Pupillary findings correlated with brainstem auditory evoked potentials only at the time of termination of intracranial pressure therapy. There was no correlation between somatosensory evoked potentials and clinical parameters. Pupillary responses indicated a good or poor recovery during and at the termination of intracranial pressure therapy. Brainstem auditory evoked potentials and intracranial pressure values distinguished between good and poor outcome only at termination of intracranial pressure therapy. Somatosensory evoked potential results did not predict outcome. CONCLUSIONS: Shortly after manifestation of supratentorial mass lesions, the results of evoked potentials and clinical parameters indicate increased intracranial pressure and incipient transtentorial herniation but do not predict sequelae. Our results indicate that after institution of effective therapy, pupillary abnormalities and brainstem auditory evoked potentials serve as valuable prognostic predictors. In contrast, somatosensory evoked potentials reflect neither therapeutic efficacy nor outcome in our patient population.
Acute Disease↗
Decompressive surgery in space-occupying hemispheric infarction: results of an open, prospective trial.
OBJECTIVE: Space-occupying hemispheric infarctions, requiring neurocritical care treatment, demonstrate high mortality and morbidity rates. This study was performed to determine the beneficial effects of decompressive craniotomy on mortality and morbidity rates. DESIGN: Open, nonrandomized, control trial. Outcome was rated at discharge from the hospital (Glasgow Outcome Scale) and at follow-up (Barthel Index, Oxford Handicap Scale). SETTING: Patient recruitment from the Department of Neurology, University of Heidelberg (primary care center) over 65 months. PATIENTS: Thirty-two patients were prospectively selected for surgical treatment; 21 patients were treated conservatively. INTERVENTIONS: Extended craniotomy and dura patch enlargement were performed in all surgically treated patients. MEASUREMENTS AND MAIN RESULTS: At discharge, the outcome of six (18.8%) of 32 surgically treated patients was good compared with 0 (0%) of 21 conservatively treated patients. Fifteen (46.9%) of 32 surgically treated patients were moderately to severely disabled compared with five (23.8%) of 21 conservatively treated patients, and 11 (34.4%) of 32 surgically treated patients died compared with 16 (76.2%) of 21 conservatively treated patients. At follow-up in surgically treated patients, the Barthel Index (mean 62.6) showed an excellent level of daily activity in one patient, minimal assistance (Barthel Index of > or = 60) in 15 patients, and dependency in five patients. The Oxford Handicap Scale indicated no handicap in one patient, moderate handicaps in 15 patients, and moderately severe handicaps in five patients. In the control group, all five surviving patients needed assistance and all but one patient demonstrated a moderately severe handicap. CONCLUSIONS: Hemicraniotomy may improve survival in massive hemispheric stroke victims, decreasing mortality rates to < 35%. The disability rate remains high (24%), although some patients seem to benefit significantly.
Adolescent↗
A testable model of global cortical organization.
This report advances a testable model of cortical organization which is tied to gamma band (20-150 Hz) neuroelectric oscillations. The elements of the model function as pattern recognizers and are assumed to be composed of a few cm2 of cortical tissue. Presumed modes of instability in the activity of this network of large units are proposed as the underlying neurophysiological basis of a variety of psychopathologies. The model is used to predict macroscopic neuroelectric measureables which will be present in patients who display these psychopathologies. Two techniques are described for identifying gamma band oscillations in neurophysiological recordings.
Animals↗
[Craniectomy in space-occupying middle cerebral artery infarcts].
Space occupying supratentorial ischemic stroke has a high mortality. The benefit of decompressive surgery in these patients is still matter of debate. In a prospective study we performed craniectomy in 37 patients with acute middle cerebral artery infarction and progressive deterioration under conservative antiedematous therapy. Twenty-one patients treated conservatively during the same period served as control group. All survivors were reexamined between one to two years after surgical decompression. In addition, neuropsychological tests were performed, including an Aachener Aphasie Test (AAT) in those patients with infarction of speech-dominant hemisphere. Clinical evaluation was graded using the Barthel index (BI). Mortality rate in the operated group was 37%. Twenty-three patients survived acute stroke and were reexamined. Despite complete hemispheric infarction, no patient suffered from complete hemiplegia or was permanently wheel chair bound. In speech dominant hemispheric infarction (n = 8) only mild to moderate aphasia could be detected. Mean BI was 64. Mortality rate in the conservatively treated group was 76%. The clinical outcome following craniectomy for the treatment of severe ischemic hemispheric infarction is unexpectedly good. Therefore, decompressive surgery should be considered in cases of space-occupying hemispheric infarctions and conservatively uncontrollable intracranial pressure.
Adolescent↗
Neurophysiologic assessment in the management of spinal dysraphism.
Neurophysiologic techniques provide a valuable addition to the armamentarium of tools for the evaluation of sensory and motor function in the pediatric spinal cord. These techniques include median, radial, and ulnar nerve evoked potentials from the upper extremity; common peroneal and tibial nerve evoked potentials from the lower extremity; dermatomal potentials; and compound muscle action potentials and compound nerve action potentials. The techniques that evaluate the sensory system have been used extensively and effectively as research tools, as adjuncts to diagnostic evaluation, and for intraoperative monitoring. There is a considerable literature that describes the properties of SEPs in the infant and young child. Techniques for assessing the descending pathways have been developed in the last 10 years. These techniques hold great promise as both diagnostic and intraoperative monitoring tools. Many questions, however, still exist concerning their value and use. The rapidly increasing capability available in computer systems is also providing enhanced capability in the acquisition, display, and analysis of neurophysiologic data. It is now common to acquire multiple responses simultaneously, e.g. tibial SEPs, pudendal SEPs, and motor potentials. It is also possible to apply computationally intensive numerical algorithms in real time to enhance signal quality and reduce the time required to produce an interpretable display. Finally, it is possible to monitor multiple cases simultaneously from remote locations. These enhanced computational capabilities are helping to optimize the contribution of neurophysiologic monitoring to patient care.
Child↗
Current issues of science-based practice.
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Nursing Care↗
Clipping of proximal paraclinoid aneurysms with support of the balloon-catheter "trapping-evacuation" technique. Technical note.
A method is described in which we use a combined endovascular balloon-catheter technique and open microneurosurgical approach for clipping aneurysms of the proximal paraclinoidal intracranial segment of the internal carotid artery. By temporary occlusion of the cervical carotid artery and continuously retrograde sucking of blood from the distal vessel via a double-lumen balloon-catheter, clip application to large and critically located aneurysms is facilitated applying evacuation-decompression to the trapped arterial segment under intra-operative SEP-monitoring.
Adult↗
Prospective evaluation of the prognostic significance of evoked potentials in acute basilar occlusion.
OBJECTIVE: To establish valid prognostic parameters in patients with acute basilar artery occlusive disease. DESIGN: A prospective study. SETTING: Neurocritical care unit at the University of Heidelberg. PATIENTS: Twenty-three patients (12 male, 11 female; 32 to 69 yrs of age, median 54) with acute basilar occlusions. INTERVENTIONS: Angiography, brainstem auditory and somatosensory evoked potentials. MEASUREMENTS AND MAIN RESULTS: Clinical and electrophysiologic data were obtained before angiography and thrombolytic therapy. Outcome was classified according to a slightly modified Glasgow Outcome Scale at discharge from the intensive care unit (ICU). Level of consciousness was determined in four classes: awake (n = 4); somnolence (n = 7); stupor (n = 4); and coma (n = 8). Bilateral recordings of brainstem auditory and somatosensory evoked potentials were ranked in three categories: normal; one side normal; and both sides abnormal. Of 23 sets of evoked potential recordings, brainstem auditory evoked potentials were normal in seven patients, one side abnormal in four patients, and both sides abnormal in 12 patients. Somatosensory evoked potentials were normal in eight patients, one side abnormal in eight patients, and both sides abnormal in seven patients. A combination of both evoked potential modalities demonstrated normal results in three patients, one side abnormal recordings in six patients, and both sides abnormal findings in 14 patients. Outcome was ranked in three groups: five individuals had a good recovery or moderate disability; two patients remained severely disabled; and 16 patients persisted either in a locked-in state or died. Statistical analysis using Fisher's exact test demonstrated a significant correlation between the initial brainstem auditory evoked potential findings and outcome (p < .005), while for the initial somatosensory evoked potentials a significant correlation with outcome was not identified (p = .089). All patients with normal brainstem auditory and somatosensory evoked potential findings did well, whereas all patients with bilateral (both sides) abnormal brainstem auditory evoked potential and bilateral abnormal somatosensory evoked potential remained locked-in or died. CONCLUSION: Initial brainstem auditory evoked potential and somatosensory evoked potential testing are valid prognostic parameters on which to base therapeutic decisions in patients with acute basilar occlusion.
Acute Disease↗
Prognostic and clinical relevance of pupillary responses, intracranial pressure monitoring, and brainstem auditory evoked potentials in comatose patients with acute supratentorial mass lesions.
OBJECTIVE: To test the hypothesis that the clinical condition and outcome in patients with acute supratentorial mass lesions can be assessed by determination of pupillary abnormalities, measurement of intracranial pressure, and results of brainstem auditory evoked potentials. DESIGN: Prospective case series of 55 patients presenting with supratentorial mass lesions. SETTING: Neurologic and neurosurgical intensive care unit of a tertiary care center. PATIENTS: Fifty-five comatose patients (26 female/29 male; 9 to 70 yrs of age [mean 44]). INTERVENTIONS: Pupillary abnormalities were rated "normal," "unilaterally enlarged," "unilaterally fixed," and "bilaterally abnormal." The outcome was rated using the Glasgow Outcome Scale. Intracranial pressure values were graded into five categories. Brainstem auditory evoked potentials were rated "bilaterally normal," "unilaterally abnormal," or "bilaterally abnormal," according to normative data. Statistical evaluation was performed by frequency analysis (Fisher's exact test, two-tailed) and calculation of contingency coefficients. MEASUREMENTS AND MAIN RESULTS: Outcome was poor in 24 patients, good in eight patients, and 23 patients were severely disabled. Statistical analysis showed prognostic significance of both pupillary abnormalities (p = .0000542; contingency coefficient = .589) and increased intracranial pressure (p = .0084; contingency coefficient = .352). Brainstem auditory evoked potential categories correlated significantly with pupillary abnormalities (p = .000276; contingency coefficient = .505) and increased intracranial pressure (p = .0301; contingency coefficient = .502) but did not predict outcome (p = .645; contingency coefficient = .321). CONCLUSIONS: Pupillary abnormalities may serve as a reliable parameter, which may even be superior to brainstem auditory evoked potential testing and intracranial pressure monitoring for prediction of outcome in comatose individuals with supratentorial mass lesions. Brainstem auditory evoked potentials can be used to support the clinical relevance of abnormal pupillary status and increased intracranial pressure but are of no prognostic value. Increased intracranial pressure is associated with abnormalities in pupillary status and brainstem auditory evoked potentials. Examination for pupillary abnormalities in combination with intracranial pressure monitoring and brainstem auditory evoked potential testing seems to be a useful strategy in managing patients with supratentorial mass lesions in critical care units.
Acute Disease↗
[Lemierre's syndrome with splenic abscesses].
A week after onset of a pharyngo-tonsillitis a previously healthy 23-year-old man developed high fever (41.4 degrees C), leukocytosis (12,200/microliters) with marked shift to the left, thrombocytopenia (86,000/microliters) and increased transaminases (GOT 83 U/l, GPT 113 U/l). Chest x-ray film suggested intrapulmonary abscesses with left-sided pleural effusion. The suspected diagnosis of "post-tonsillitis" septicaemia (Lemierre's syndrome) was confirmed by demonstrating anaerobic, fusiform, gram-negative bacteria (Fusobacterium nucleatum and necrophorum) in several blood cultures. Despite antibacterial treatment (amoxicillin/clavulanic acid, imipenem/cilastatin, clindamycin) he had recurrent pain referred to the kidney region and persisting fever. Repeated ultrasound and radiological examinations revealed new foci in the spleen, which were enlarging. Laparotomy with splenectomy performed on day 17 after the begin of treatment confirmed multiple splenic abscesses, but abscess pus and splenic tissue were sterile. After altogether 6 weeks of antibiotic treatment, finally with chloramphenicol, the patient was discharged in a good general state.
Abscess↗
German-Austrian Space Occupying Cerebellar Infarction Study (GASCIS): study design, methods, patient characteristics. The Steering and Protocol Commission.
An open prospective multicentric therapeutic trial to determine the timing and type of therapeutic intervention in patients presenting with secondary deterioration following cerebellar stroke is described. According to the results of retrospective studies a controlled approach comparing different therapies is ethically not feasible. Participants use the same scores and protocol for patient data collection but many choose different therapeutic procedures.
Austria↗