Chronic progressive primary lumbosacral plexus neuritis: MRI findings and response to immunoglobulin therapy.
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Aged↗
Biomedical subjects
M Sturzenegger
Publications and source records attributed to M Sturzenegger.
Determination of language dominance: Wada test confirms functional transcranial Doppler sonography.
OBJECTIVE: To determine the efficacy of the invasive Wada test in determining language dominance, and to validate the functional transcranial Doppler sonography (fTCD) examination in patients. BACKGROUND: Previous work shows that simultaneous bilateral fTDC may identify cognitive hemispheric dominance in healthy individuals. METHOD: fTDC and the Wada test were performed prospectively in 14 patients with various diseases (tumors, cerebrovascular events, head injury, intractable epilepsy). fTDC hemispheric dominance was determined based on the hemispheric blood flow velocity shift for language and visuospatial tasks. RESULTS: fTDC was performed easily in patients. One patient could not be examined by fTDC because of absent temporal bone window for ultrasonic transmission. Two Wada tests were inconclusive due to patient somnolence. One of these patients suffered from right frontal tumor and had aphasia remitted under steroids when examined. fTDC indicated a bilateral language dominance. In the remaining 11 patients the correlation between fTDC and Wada language lateralization indices was 0.75 (p = 0.008). If a post hoc cutoff score was taken for the fTDC language lateralization index, in eight patients, both fTDC and Wada testing determined the left hemisphere to be dominant for language; in the other three patients, language function was bilateral in both examinations. CONCLUSION: Although the current results are preliminary and require replication in a larger sample, fTDC seems to be able to assess hemispheric language dominance not only in healthy individuals, but also in patients. It might become an alternative noninvasive or complementary tool to the Wada test, particularly in patients in whom the Wada test is impractical or gives inconclusive results.
Adolescent↗
Magnetic resonance imaging findings in isolated spinal psammomatous melanotic schwannoma.
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Humans↗
[Viral encephalitis].
Viral encephalitis presents with fever, headache, focal and generalized neurologic symptoms and signs, seizures, and CSF pleocytosis. Herpes simplex Virus (HSV) 1 and arboviruses (flaviruses) are the most common causes of encephalitis in Switzerland. The initial work-up in a suspected encephalitis includes CSF analysis, EEG, and brain CT or MRI. The identification of the responsible agent usually occurs with polymerase chain reaction or serology. The differential diagnosis to other infectious and non-infectious acute CNS-disorders may initially be arduous. A specific treatment is possible only in encephalitis caused by viruses of the herpes group. Active immunization should be considered in subjects at high risk for tick-borne encephalitis. With early treatment the prognosis may be satisfactory also in HSV encephalitis.
Antiviral Agents↗
[Human prion diseases].
The interest in prion diseases, particularly the Creutzfeldt-Jakob type (CJD), rose dramatically in the last years for two reasons. 1) The general public wants to know whether eating beef may cause CJD. Discovering the new variant Creutzfeldt-Jakob disease (nvCJD) and experimental evidence that nvCJD and bovine spongiforme encephalopathy (BSE) are caused by the same prion strain make this idea probable. 2) Infectiologists and Neuroscientists recognise a model disease for a new infectious principle in that the same disease may occur as being inherited as well as transmitted. Additionally, it might allow new insights into the possible aetiologies of neurodegenerative disease.
Animals↗
Adverse effects of intravenous immunoglobulin therapy.
A growing body of literature documents that intravenous immunoglobulin prophylaxis and therapy is becoming applied to a steadily growing list of new indications. Some of these new indications have led to the use of intravenous immunoglobulin therapy in doctors offices, far from the hospital environment. Being stable products purified from blood or plasma donations, intravenous immunoglobulins must be considered as biological products in addition to their status as pharmaceutical products. This makes the study of adverse reactions reach beyond a mere drug safety surveillance programme into the realms of good manufacturing procedures guaranteeing not only intravenous tolerance but also sterility with regard to transfusion transmitted agents. The initially perceived adverse effects, stemming from complement activating aggregated immunoglobulin G, had the effect of slowing down widespread introduction of intravenous immunoglobulin therapy in the late 1970s. These adverse effects have now been eliminated with amendment of the appropriate manufacturing steps. However, new adverse effects, such as hyperviscosity, aseptic meningitis or renal insufficiency, have been observed which can be assigned to certain comnpounds of intravenous immunoglobulin, to administration regimens or to special patient characteristics. Adverse effects can be divided into 3 types: immediate adverse effects (those that occur during the infusion, e.g. anaphylactoid reactions); delayed adverse effects (those that occur hours to days after initiation of the infusion, e.g. renal, pulmonary, dermatological adverse effects, hyperviscosity, aseptic meningitis, arthritis, cerebral infarction, haemolysis and leucopenia) and; late adverse effects (e.g. transmission of infectious agents). We conclude from our analysis, that in general, intravenous immunoglobulin may be considered a well tolerated medical agent provided the indication for use is chosen carefully and use is monitored by a physician familiar with contraindications, risks, adverse effects and their appropriate management.
Animals↗
[Fluctuating consciousness with diagnostic CT].
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Adult↗
[Otogenic meningitis].
We present three patients with otogenic meningitis, whose illness varied in extent and clinical course. Meningitis and otitis media are associated in 19-22% of all meningitis-patients. Streptococcus pneumoniae is the predominant microorganism with hemophilus influenzae being the second most important. We discuss the importance and problems of spinal puncture, early CT-scan as well as further, more extensive and sophisticated examinations to exclude late complications or predisposing factors. We emphasize an early start of antibiotic treatment, which should not be delayed for diagnostic reasons. With the appearance of highly penicillin-resistant pneumococci antibiotic therapy may become more difficult in the future, but for the moment pneumococcal infections in Switzerland can initially still be treated with cephalosporins or high dose penicillin. The use of steroids, although of unproven efficacy, may be considered in some cases. An otolaryngologist should initially be consulted for diagnostic reasons as well as for possibly indicated early surgery.
Adult↗
[X-chromosomal bulbospinal muscular atrophy (Kennedy syndrome)].
Two brothers with slowly progressive weakness and congenital nystagmus are presented. DNA analysis confirmed X-linked recessive bulbospinal muscular atrophy (XBSMA, Kennedy's disease) by demonstration of increased size of a CAG-triplet repeat on the androgen receptor gene on the X-chromosome. XBSMA is characterized by almost symmetrical muscular atrophy, weakness and fasciculations predominantly of bulbar, facial and proximal muscles of the extremities, with onset in the third to fifth decade. Tendon reflexes are depressed and pyramidal signs are absent. Sensory symptoms are clinically rare, but sensory nerve action potentials are frequently abnormal. Additional symptoms are important for differential diagnosis, and include postural tremor, gynecomastia, diabetes mellitus, testicular atrophy and impotence. Differentiation of this hereditary disorder from treatable conditions such as multifocal motor neuropathy or amyotrophic lateral sclerosis is essential. Though life expectancy is normal, patients become disabled in the course of the disease and need supportive care. Periodic testing for diabetes is recommended, and genetic counseling should be provided for patients and their relatives.
Adult↗
Pulsatile tinnitus--a review of 84 patients.
Pulsatile tinnitus can be annoying for a patient and can also be the only clue to a potentially devastating and life-threatening disease. In order to understand its clinical spectrum and management better we analysed the files of 84 patients seen at our institution over a 10-year period. Noninvasive techniques (ultrasound, computed tomography, magnetic resonance imaging) and angiography were employed as investigations tailored to the individual patient. A vascular disorder [i.e. arteriovenous fistula, dissection of the internal carotid artery (ICA), fibromuscular dysplasia, aneurysm of the ICA and sinus thrombosis] was found in 36 patients (42%), most commonly a dural arteriovenous fistula or a carotid-cavernous sinus fistula. In 26 patients with a vascular abnormality, pulsatile tinnitus was the presenting symptom. In 12 patients (14%), nonvascular disorders such as glomus tumour or intracranial hypertension with a variety of causes explained the tinnitus. We conclude that patients with pulsatile tinnitus should be investigated with noninvasive techniques. If these are negative or to clarify abnormal findings of noninvasive techniques selective angiography is needed for diagnosis and to guide treatment.
Adolescent↗
EEG findings during basilar migraine attacks in children.
We present clinical and EEG findings in 4 children with basilar migraine (BM) (three female and one male, age 11 to 13.5 years). All patients had an EEG during the acute attack and a follow-up EEG within 4 to 18 days. In two patients the EEG, done within 4 h of the onset of symptoms (initial stage), showed diffuse polymorphic subdelta-delta activity. In two other children the EEG, performed 16 h after the onset of symptoms, showed delta-theta activity predominant over the occipital regions. Resolution of these abnormalities during follow-up was observed in all patients. We wish to stress the danger of misinterpretation of the slow wave activity in the EEG of patients with BM attacks. Together with the clinical findings and their evolution, EEG results should not be interpreted as a sign of a structural brain-stem lesion, such as infarction or inflammation.
Acute Disease↗
Avascular necrosis of the trapezoid bone.
A case of idiopathic avascular necrosis of the right trapezoid is presented. The aetiology was not clear. Treatment consisted of bone curettage, autologous bone graft and revascularization with a dorsal metacarpal artery.
Adult↗
Anticipation in familial cavernous angioma: ascertainment bias or genetic cause.
OBJECTIVES: Anticipation has been linked to unstable trinucleotide repeats in many neurological disorders. We examined the hypothesis of genetic anticipation in familial cavernous angioma (FCA) of the central nervous system. MATERIAL AND METHODS: The mean ASO of affected individuals was compared between successive generations in 55 families. Intergenerational pair-wise comparisons were employed to avoid several ascertainment biases. Regarding severity of disease both type of manifestation and number of cavernous angiomas were compared between generations. RESULTS: The mean ASO decreased significantly both from the first to the second generation (31.6 vs 17.8 years; P = 0.000) and from the second to the third generation (17.8 vs 6.7 years; P = 0.002). The pair-wise comparisons also showed significantly earlier ASO. No clear evidence for anticipation with regard to severity of disease was found. CONCLUSIONS: Molecular genetic studies will determine whether trinucleotide repeats are the underlying mechanism for our observation of anticipation in FCA.
Adolescent↗
Antiganglioside GM1 antibodies and their complement activating capacity in central and peripheral nervous system disorders and in controls.
So far, the pathogenic significance and use for diagnosis of antiganglioside GM1 antibodies (anti-GM1) are unclear. We therefore compared serum IgM and IgG antimonosialo ganglioside GM1 levels of 33 patients with presumed immune-mediated neuropathies, 100 patients with various other central or peripheral neurological disorders, and 110 controls by ELISA. We also measured the complement-activating capacity of anti-GM1 by C5b-9-GM1-ELISA to evaluate its value to distinguish between pathogenic and nonpathogenic autoantibodies. Low levels of anti-GM1 were observed in all disease categories and in controls (healthy blood donors). Twenty-four of the controls including the 10 with the highest serum IgM or IgG anti-GM1 were examined for neurological disorders in a double-blind checkup study. In the patients, elevated IgM anti-GM1 levels were predominantly found in those with neuropathies (NP), but barely in patients with central nervous system disease (CNSD). We found elevated IgG anti-GM1 levels predominantly in patients with NP of inflammatory origin (multifocal motor neuropathy, chronic inflammatory demyelinating polyneuropathy or Guillain-Barré syndrome), rarely in patients with NP of noninflammatory origin or CNSD, but not in the control disease group myasthenia gravis (MG). Median levels of IgM-, IgG-, (IgM+IgG)-, and C5b-9-binding anti-GM1 were significantly higher in patients with inflammatory NP as compared to the controls (p < 0.025). In addition, median levels of IgG- and (IgM+IgG)-anti-GM1 were significantly higher in inflammatory NP versus CNSD. Elevated complement-binding activity was associated with low or elevated IgM and/or IgG anti-GM1. Nevertheless, there was a significant correlation between anti-GM1 level (IgM+IgG) and the respective complement-activating capacity (r = 0.758; n = 243). Estimation of anti-GM1 and their respective complement-activating capacity may be helpful in the diagnosis of inflammatory neuropathies. However, neither an elevated anti-GM1 level nor an increased C5b-9 binding seems specific for a given disease category (e.g. peripheral nerve disease) nor a disease process (e.g. demyelination or inflammation).
Adolescent↗
Selective intra-arterial fibrinolysis of acute central retinal artery occlusion.
BACKGROUND AND PURPOSE: Occlusion of the central retinal artery (CRAO) causes a sudden decrease of monocular vision. Because early restoration of blood flow may improve outcome, we attempted to treat CRAO with selective intra-arterial fibrinolysis. METHODS: Intra-arterial fibrinolysis was performed within 6 hours after symptom onset in 17 patients with thromboembolic CRAO. Symptoms were painless, acute and severe decrease of vision. Urokinase (100 000 to 900 000 IU) was given through a microcatheter into the ophthalmic artery over 10 to 90 minutes. For comparison, the history and visual outcome of 15 control patients who did not receive fibrinolytics were evaluated. In both groups some of the patients underwent paracentesis and/or received carboanhydrase inhibitors. RESULTS: Patients who underwent fibrinolysis fared better than control patients (P=0.01). Three patients (17.6%) recovered completely after fibrinolysis and regained visual acuity of 20/20 (n=2) to 25/20 (n=1). Two additional patients (11.8%) showed a marked improvement to a visual acuity of 20/30. In 6 patients (35. 3%) vision improved slightly. They were able to count fingers, detect hand movements, or perceive light. In 6 patients (35.3%), fibrinolytic treatment was without effect. Among control patients, 1 patient (6.7%) showed partial, 4 patients (26.7%) minimal, and 10 (66.7%) no improvement of vision. CONCLUSIONS: A complete or marked improvement of visual acuity was achieved in one third of intra-arterial fibrinolysis patients but in none of the control patients. Intra-arterial fibrinolysis seems to have the potential to "lighten" the spontaneously poor outcome of CRAO.
Acute Disease↗
Mechanisms of cerebrovascular events as assessed by procoagulant activity, cerebral microemboli, and platelet microparticles in patients with prosthetic heart valves.
BACKGROUND AND PURPOSE: Cerebrovascular events (CVE) in patients with prosthetic heart valves (PHV) have remained a severe and frequent complication despite oral anticoagulation with or without aspirin. We studied the possible pathophysiological involvement of platelet-derived microparticles (PMP) as a contributing factor for the increased incidence of CVE in patients with PHV. METHODS: We compared in a retrospective, case-control study the clinical outcome after the implantation of the PHV with several different independent morphological and functional methods, including simultaneous transcranial Doppler monitoring of both middle cerebral arteries, PMP detection by flow cytometry with use of platelet-specific antibodies, coagulation markers, and determination of the procoagulant activity by Russell's viper venom time, a phospholipid-dependent coagulation assay. RESULTS: Eight of 26 patients with PHV had 9 CVE during 136 person-years of observation. Transcranial Doppler monitoring revealed an increased frequency of microembolic signals recorded over a 30-minute period in patients with CVE (75+/-25; median, 55; range, 27 to 248) compared with those without CVE (23+/-12; median, 7; range, 0 to 153; P<0.05) or with control subjects (0; P<0.001). Flow cytometry analysis showed increased levels of PMP in patients with compared to those without CVE (4.1+/-0.6% versus 2.4+/-0.4% of all fluorescence-positive events gated; P<0.05). Increased procoagulant activity was documented by the shortened Russell's viper venom time expressed as an increased level of platelet equivalents per microliter of plasma in patients compared with control subjects (+24.7+/-14.9%; P<0.01). Subgroup analysis revealed that patients with CVE had a higher excess of platelet equivalents per microliter of plasma than patients without CVE in relation to the controls (+68.7+/-36.7%; P<0.05). Mildly elevated thrombin-antithrombin III complexes (2.9+/-0.7; median, 2.3; normal, <2.0 microg/L) suggested incompletely suppressed thrombin formation, and fibrin generation (fibrinopeptide A) was in the upper normal range (2.1+/-0.2; median, 1.8; normal, <2.0 ng/mL), despite adequate anticoagulation (INR=3.6+/-0.1). CONCLUSIONS: Our data show increased microembolic signals, platelet microparticles, and procoagulant activity in symptomatic patients with PHV and provide a potential pathophysiological explanation of CVE.
Antithrombin III↗
Local intra-arterial thrombolysis in acute ischemic stroke.
BACKGROUND AND PURPOSE: We performed a retrospective analysis of the prognostic factors in patients treated with local intra-arterial thrombolysis (LIT). The purpose of this study was to evaluate the safety and efficacy of LIT using urokinase in patients with acute ischemic stroke of the anterior or posterior circulation and to determine the influence of clinical and radiological parameters on outcome. METHODS: Forty-three patients were treated with LIT using urokinase (median dose, 0.75x10(6) IU). The median National Institutes of Health Stroke Scale (NIHSS) score at hospital admission was 18 (range, 9 to 36). Nine patients had occlusions of the internal carotid artery (ICA), 23 of the middle cerebral artery (MCA), 1 of the anterior cerebral artery, and 10 of the basilar artery (BA). Outcome was assessed after 3 months and classified as good for Rankin Scale (RS) scores of 0 to 3 and poor for RS scores of 4 or 5 and death. RESULTS: Nine patients (21%) recovered to RS scores 0 or 1, 17 (40%) to scores of 2 or 3, and 7 (16%) to scores of 4 or 5. Ten patients (23%) died. Outcome was good in 17 patients (80%) with MCA occlusions, in 3 patients (33%) with ICA, and in 5 patients (50%) with BA occlusions. Good outcome was associated with an initial NIHSS score of <20 (P<0.001), improvement by 4 or more points on NIHSS score within 24 hours (P=0.001), and vessel recanalization (P=0.02). Recanalization was more likely if LIT was started within 4 hours (P=0.01). Symptomatic cerebral hemorrhage occurred in 2 patients (4.7%). CONCLUSIONS: LIT was most efficacious in patients with MCA and BA occlusions when the initial NIHSS score was less than 20 and when treated within 4 hours. It is of limited value in patients with distal ICA occlusions.
Acute Disease↗
Neurosyphilis mimicking Miller-Fisher syndrome: a case report and MRI findings.
A 36-year-old man presented with rapidly progressive unsteadiness of gait and diplopia. He had horizontal nystagmus, left sixth nerve palsy, severe ataxia, and areflexia. CSF protein content was 2.4 g/L. Despite a pleocytosis, Miller-Fisher syndrome was diagnosed. Two years later, reevaluation revealed active neurosyphilis in the freshly drawn and stored serum and CSF samples. Clinical findings were consistent with tabes dorsalis. MRI showed lesions confined to the posterior columns of the spinal cord.
Adult↗