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Biomedical subjects

J Finsterer

Publications and source records attributed to J Finsterer.

At least 73 records · Page 4Linked to original sources

EMG-interference pattern analysis.

The EMG interference pattern, built up of single motor unit action potentials, may be analyzed subjectively, or objectively by computer aided, quantitative methods, like counting of zero-crossings, counting of spikes, amplitude measurements, integration of the area under the curve, decomposition techniques, power spectrum analysis and turn/amplitude analysis. Since the shape of the interference pattern of healthy muscles is dependent on age, sex, force, muscle, temperature, fatigue, fitness level, recording site and surrounding tissue, electrode type, sensitivity, filters, sampling frequency and threshold level, all methods of analyzing the IP have to be standardized. Quantitative methods of analyzing the EMG interference pattern may be used for monitoring botulinum toxin therapy of dystonia and spasticity, quantifying spontaneous activity, assessment of chronic muscle pain, neuro-urological and proctological function, and diagnosing neuromuscular disorders. For diagnostic purposes, the methods favored are those that use needle electrodes and do not require measurement or monitoring of muscle force. The most well-evaluated methods are those using turn/amplitude analysis, like the cloud methods and the peak-ratio analysis. Peak-ratio analysis has the advantage that reference limits are easy to obtain and that its utility is well established and confirmed by several investigations. Overall, automatic methods of EMG interference pattern analysis are powerful tools for diagnostic and non-diagnostic purposes.

Adult↗

Concentric-needle versus macro EMG. II. Detection of neuromuscular disorders.

OBJECTIVE: Little is known about the relation and sensitivity of macro-EMG (MA-EMG) compared with concentric-needle EMG (CN-EMG) in the detection of neuromuscular disorders. METHODS: CN-EMGs and MA-EMGs were recorded from the right brachial biceps muscle of 40 healthy subjects, aged 17-83 years, 20 patients with neurogenic disorders, aged 25-75 years, and 20 patients with myopathy, aged 18-76 years. Motor unit action potentials (MUAPs) were examined. RESULTS: In patients with neurogenic disorders CN-MUAP duration, CN-MUAP amplitude, percent polyphasia, MA-MUAP amplitude, MA-MUAP area and fibre density were significantly increased. In patients with myopathy, only fibre density was significantly increased. In patients with neurogenic disorders, the sensitivity of CN-EMG was 80%, and that of MA-EMG 85%. In myopathies, the sensitivity was 50% for each technique. Pooling the results of both EMG techniques, the sensitivity increased to 90% in patients with neurogenic disorders, and to 65% in myogenic disease. CONCLUSIONS: MA-EMG has a similar sensitivity in the detection of neuromuscular disorders as CN-EMG. Particularly when myopathy is suspected, both techniques should be applied if one is unrevealing.

Action Potentials↗

Phenotype variability in 130 adult patients with respiratory chain disorders.

Despite continuously improving diagnostic facilities, respiratory chain disorders (RCDs) are easily overlooked or misdiagnosed. We thus studied phenotype variability and the diagnostic potential of clinical and laboratory investigations in patients with RCD. We retrospectively evaluated clinical and laboratory investigations in 130 patients with RCD: 63 women and 67 men, aged 17-87 years, diagnosed between January 1992 and December 1999. mtDNA mutations were found in 20 patients; a respiratory chain defect but no mutation in 4; an abnormal lactate stress test but no mutation or biochemical defect in 66; and ragged-red fibres or reduced oxidative enzyme staining but no mutation, biochemical defect or abnormal lactate stress test in 40 patients. The most frequent initial manifestation of RCD were limb weakness, muscle pain and sensory disturbances. The most frequent clinical findings at diagnosis were muscle pain, fatiguability, limb weakness, reduced tendon reflexes and muscle wasting, irrespective of the diagnostic evidence. Mean age at onset, disease duration and time until diagnosis were 39, 14 and 13 years, respectively, without sex differences. The family history was positive in 29% of the patients. Hyperlipidaemia was found in 45%, hyper-CK-aemia in 42%, short stature in 33%, thyroid dysfunction in 17%, diabetes in 12%, and epilepsy in 8% of the patients. Laboratory investigations that prove useful to support the diagnosis of RCD are muscle biopsy, electromyography, lactate stress testing, echocardiography and mtDNA analysis. Systems most often involved in RCDs were the PNS, CNS, endocrine system and heart. The diagnosis of RCD requires awareness of the great phenotypic heterogeneity and an individualized, integral, multidisciplinary approach.

Adolescent↗

Relation of cardiac abnormalities and CTG-repeat size in myotonic dystrophy.

It is unclear if the severity of cardiac involvement in patients with myotonic dystrophy (MD) is related to the size of the CTG-repeat expansion. This open, uncontrolled, observational, prospective study aimed to find out if there is a relation between the severity of cardiac involvement in MD and the CTG-repeat size. In 21 patients with MD, (8 women, 13 men, aged 11-88 years) a detailed cardiologic examination, including history, clinical examination, electrocardiography (ECG), transthoracic echocardiography and ambulatory 24-h ECG, was carried out and cardiac involvement was assessed according to a previously described scoring system. Additionally, the CTG-repeat size was determined from nuclear DNA of blood leukocytes. The correlation between the CTG-repeat size and the mean heart rate, PQ-interval, QTc-interval, fractional shortening, left ventricular enddiastolic diameter, septal thickness, posterior wall thickness, mean heart rate on 24-h ECG and cardiac involvement score was r=0.47, r=0.086, r=0.11, r=-0.27, r=-0.34, r=-0.06, r=-0.12, r=0.16 and r=0.09 (all p>0.05), respectively. In patients 21-30, 31-40 and 41-50 years of age, cardiac involvement increased with increasing CTG-repeat size. In younger patients, the number of CTG-repeats needed to develop a reasonable cardiac involvement was higher than in older patients. Depending on age, cardiac involvement increases with increasing CTG-repeat size obtained from blood leukocytes in patients with MD.

Adolescent↗

Visually evoked potentials in respiratory chain disorders.

OBJECTIVES: Little is known about the frequency of abnormal visually evoked potentials (VEPs) in patients with respiratory chain disorders (RCDs). We thus wanted to investigate the frequency of abnormal VEPs in RCDs, how often VEPs are abnormal despite normal visual acuity, and which of the VEP variables are most often abnormal. MATERIAL AND METHODS: Reversal checkerboard VEPs of 26 patients with RCDs, aged 32-74 years, were evaluated. RESULTS: VEPs were abnormal in 17 of the 26 cases (65%). The P100 latency was prolonged at least unilaterally in 16 patients. The P100/N145 amplitude was decreased in a single patient. VEPs were abnormal without visual impairment in 9 cases (53%). CONCLUSION: VEPs prove useful to detect clinical or subclinical impairment of the optical tract in patients with RCDs. In the majority of the cases, the P100 latencies are prolonged while the P100/N145 amplitude remains normal.

Adult↗

Generalised spinal cord atrophy, Chiari-I malformation, and syringomyelia.

OBJECTIVES: To report the simultaneous occurrence of generalised spinal cord atrophy, Chiari-I malformation, and syringomyelia. SETTING: Vienna, Austria. CASE DESCRIPTION: An 83-year old woman presented with predominantly lower limb and distal tetraparesis, diffuse wasting, spasticity of both lower limbs, contractures, and severe kyphoscoliosis. Radiographic examinations revealed generalised spinal cord atrophy, Chiari-I malformation, and syringomyelia between C5 and C7. The generalised spinal cord atrophy was assumed to be due to either secondary atrophy or spontaneous collapse of a formerly more extensive syringomyelia. All other causes for generalised spinal cord atrophy were excluded. CONCLUSIONS: Generalised spinal cord atrophy in a patient with Chiari-I malformation and syringomyelia may be due to either secondary atrophy or spontaneous collapse of a formerly holocord syringomyelia. Myelography and MRI imaging of the spine are helpful in diagnosing this condition.

Aged↗

Severe, isolated thrombocytopenia under polytherapy with carbamazepine and valproate.

It is not known whether polytherapy with carbamazepine (CBZ) and valproate (VPA) increases the risk of thrombocytopenia. A 67-year-old woman with symptomatic epilepsy since age 6 years, classified as Lennox-Gasteau syndrome, developed severe, isolated thrombocytopenia of 5 GIGA/L (normal: 150-360 GIGA/L) after being on CBZ (1200 mg/day) for 15 days and additional VPA (300 mg/day) for 5 days in combination. After discontinuation of CBZ and VPA and two thrombocyte transfusions, the thrombocyte count normalized within 3 days. Because she had been taking VPA for only 5 days in addition to CBZ, it could not be confirmed whether it was CBZ alone or the combination of CBZ and VPA that was responsible for the severe thrombocytopenia.

Aged↗

Serum creatine kinase elevation in a medical department.

Serum creatine kinase (CK) levels are diagnostic markers for acute myocardial infarction. Many other causes however, including neuromuscular disorders, may induce serum CK elevation as well. Aim of the study was to investigate the prevalence of potential causes for serum CK elevation in a medical department. In particular we were interested in the recognition of patients in whom serum CK elevation was due to a neuromuscular disorder. Included in this prospective study were 100 consecutive patients in whom the CK level, determined at admission, was increased (> 70 IU/l in female, > 80 IU/l in male patients). After admission we looked for the presence of causes known to induce CK elevation. Patients with no potential cause for CK elevation were invited for follow-up investigations three months later. If no potential cause could be found and if CK was elevated again on this occasion, the patient was referred for a comprehensive neurological investigation. The prevalence of patients with CK elevation was 11.2%. The 100 patients (44 female, 56 male) were aged from 23 to 94 (mean 67) years. In 95% CK elevation was only up to 500 IU/l. The most frequent cause for serum CK elevation was acute myocardial infarction in 32%. Further frequent causes were drug intake (32%), fall (24%), haematoma (17%), intramuscular injection (16%) and malignancy (11%). In 61% of the cases at least two potential causes for serum CK elevation could be detected. Neuromuscular disorders were found in only 2%. This study shows that serum CK elevation occurs in 11% of patients admitted to a medical department and can be explained by acute myocardial infarction in only 32%. In almost two thirds of the patients, more than one potential cause for serum CK elevation can be found thus making CK elevation a rather unspecific finding. Neuromuscular disorders are rarely found as a cause of serum CK elevation in a medical department.

Accidental Falls↗

Cerebrospinal-fluid lactate in adult mitochondriopathy with and without encephalopathy.

Despite improved diagnostic facilities, the diagnosis of mitochondriopathy (MCP) is sometimes difficult to establish. This study aimed to investigate whether CSF lactate can be of diagnostic help in this respect. Cerebrospinal-fluid (CSF) lactate and its relation to resting lactate and lactate stress testing were investigated in 26 MCP patients, aged 30-84 years. The upper reference limit of CSF lactate, obtained from 28 healthy subjects, was 1.6 mmol/l. Seven MCP patients (27%) had elevated CSF lactate. Five of these patients had central nervous system (CNS) abnormalities other than elevated CSF lactate. In two patients with increased cerebrospinal-fluid lactate, lactate stress testing was normal or could not be carried out. In conclusion, CSF lactate is elevated in one quarter of adult MCP patients. CSF lactate is preferentially elevated in patients with additional CNS abnormalities other than elevated CSF lactate. If the results for resting lactate or lactate stress testing are normal or unavailable, determination of CSF lactate may be of diagnostic support in single cases.

Adult↗

Intramyocardial hematoma mimicking abnormal left ventricular trabeculation.

Intramyocardial hematoma may present as a tumor or pseudoaneurysm on echocardiography. A 68-year-old man was admitted with a subacute posterior wall infarction complicated by ventricular fibrillation. Echocardiography showed isolated left ventricular abnormal trabeculations, a finding suggesting an associated skeletal muscle disorder, in the lateral wall. At cardiac surgery, performed 6 weeks later because of severe 3-vessel disease, an intramyocardial hematoma of the lateral wall was excised, and myocardial and skeletal muscle biopsies were taken, which showed neither isolated left ventricular abnormal trabeculations nor skeletal muscle disorder. Postoperatively, echocardiography revealed no abnormal trabeculations.

Aged↗

Seroprevalence of antibodies to microorganisms known to cause arterial and myocardial damage in patients with or without coronary stenosis.

Infections are assumed to play a role in coronary artery disease (CAD) and cardiomyopathies. It is unknown whether the seroprevalence of antibodies to these microorganisms is higher in patients with than without CAD. The seroprevalence of antibodies to Bartonella henselae, Borrelia burgdorferi, Chlamydia pneumoniae, Coxiella burnetii, Helicobacter pylori, human granulocytic Ehrlichia, Leptospira, Rickettsia conorii, and Treponema pallidum was assessed prospectively in patients with exertional dyspnea or anginal chest pain who underwent coronary angiography because of suspected CAD. Patients with normal angiograms (NA) were those in whom no more than 50% stenosis of any coronary artery was found. Patients with CAD were patients who underwent percutaneous transluminal coronary angioplasty. There were 50 patients with CAD (9 female) and 62 with NA (25 female), with a mean age of 62 years. All patients had antibodies to at least one microorganism: to B. henselae, 8% of CAD patients and 5% of NA patients; to B. burgdorferi IgG, 14% CAD and 6% NA; to B. burgdorferi IgM, 6% CAD and 3% NA; to C. pneumoniae lipopolysaccharide (LPS) IgA, 76% CAD and 77% NA; to C. pneumoniae LPS IgG, 80% CAD and 90% NA; to C. burnetii, 0% CAD and 5% NA; to H. pylori, 92% CAD and 68% NA; to human granulocytic Ehrlichia, 8% CAD and 3% NA; to Leptospira IgG, 4% CAD and 2% NA; to R. conorii, 10% in both groups; and to T. pallidum, 2% CAD and 0% NA. The seroprevalence of antibodies to micro-organisms known to induce arterial and myocardial damage does not differ between patients with CAD and NA.

Adult↗

Nail-patella syndrome associated with respiratory chain disorder.

Nail-patella syndrome (NPS) has not been described to be associated with a respiratory chain disorder (RCD) before. In a 42-year-old man with the typical phenotype of an NPS, weakness and wasting of the shoulder girdle muscles, muscle cramps, fatigability, hyperhidrosis, chest pain and creatine kinase elevation were observed. Echocardiography revealed left ventricular hypertrabeculation. Needle electromyography was myopathic, lactate stress testing was abnormal, muscle biopsy showed typical features of an RCD and mtDNA analysis revealed the A3243G MELAS mutation. In conclusion, this case demonstrates that NPS may be randomly associated with RCD. NPS patients should undergo detailed cardiological and neurological investigations, in order not to overlook a double trouble partially mimicking NPS.

Adult↗

Evaluation of risk factors for stroke/embolism and of complications due to anticoagulant therapy in atrial fibrillation.

BACKGROUND AND PURPOSE: We sought to assess in outpatients with atrial fibrillation and oral anticoagulation (1) whether the complication rate is influenced by the presence of the risk factors age >65 years, arterial hypertension, diabetes, or previous stroke; (2) whether the complication rate is influenced by the number of additional drugs taken by patients; and (3) whether problems and interventions differ between patients with or without complications. METHODS: - Clinical characteristics, drugs, problems, interventions, and complications were registered during 2 years. RESULTS: - Three hundred sixty patients (mean age, 68 years; 43% female) were observed for 383 patient-years. Patients aged >65 years had more serious, life-threatening, or fatal complications (11% versus 5.3%/100 patient-years; P=0.0428) than younger patients. Patients with diabetes had more life-threatening and fatal complications (2.8% versus 0.6%/100 patient-years; P=0.0354) than patients without. The complication rate did not differ regarding the presence of previous stroke or hypertension. Patients who took </=3 drugs had fewer complications than patients who took more (4.3% versus 24.4%/100 patient-years; P=0.0041). Patients with complications complained more of chest (48% versus 28%/100 patient-years; P=0.0113) and abdominal pain (30% versus 13%/100 patient-years; P=0.0057), more frequently failed to keep appointments (134% versus 107%/100 patient-years; P=0.0321), had a higher tracking rate (134% versus 105%/100 patient-years; P=0.0272), and took more additional drugs (4.6 versus 3.5 drugs per day; P=0.0063) than patients with no complications. CONCLUSIONS: - Patients with increased age or diabetes mellitus or those who take >3 drugs per day have an increased complication rate and thus need especially careful monitoring of oral anticoagulation, including adequate pain control.

Administration, Oral↗

Video-EEG monitoring in respiratory chain disorders.

Subclinical epileptiform activity in patients with respiratory chain disorders (RCDs) has not been previously investigated by video-EEG monitoring. The purpose of this study was to look for the type and frequency of epileptiform activity during a 24 h-video-EEG recording in RCD patients. Eleven patients with RCD, 7 women and 4 men, aged 24-72 years, underwent a clinical neurologic examination, blood tests, CT/MRI scans of the brain, routine scalp EEG and continuous video-EEG monitoring over 24 consecutive hours. Ten patients had normal 24 h-video-EEG recordings. One of these patients had no CNS involvement; her resting EEGs showed diffuse background slowing. In one patient, automatic spike detection revealed 12 spikes during a 6 hour period. Resting scalp EEG in this patient showed extensive, bilaterally synchronous, pseudoperiodic polyspike-waves. In conclusion, subclinical epileptiform activity could be recorded by 24 h-video-EEG monitoring in only 1 of 11 RCD patients. The single patient with subclinical epileptiform activity presented with intermittent myoclonic jerks.

Adult↗

Isolated left ventricular abnormal trabeculation: follow-up and association with neuromuscular disorders.

OBJECTIVES: To assess in patients with isolated left ventricular abnormal trabeculation (ILVAT), by a prospective follow-up study, whether the echocardiographic appearance of ILVAT changes, whether cardiac symptoms change, how many patients are hospitalized because of cardiac problems or die, and whether ILVAT is associated with neuromuscular disorders in all patients. PATIENTS AND METHODS: ILVAT was diagnosed in 33 patients--nine women and 24 men--aged 30 to 73 years. At baseline, all patients underwent a cardiological and neurological investigation. Between October and December 1999 they were invited for a clinical and echocardiographic follow-up. results: During a mean follow-up duration of 19 months, ILVAT remained unchanged. Cardiac symptoms worsened in one patient and improved in 10. Five patients were hospitalized and four patients died, two of them suddenly. ILVAT was associated with neuromuscular disorders in 26 patients (81%). conclusions: Although ILVAT does not change over time, cardiac function may deteriorate. Because the rate of arrhythmias is high and even causes death in some patients, effective antiarrhythmic therapy should be considered. ILVAT is assumed to be a cardiac manifestation of a neuromuscular disorder or a special type of a cardiomyopathy.

Adult↗