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Biomedical subjects

J Finsterer

Publications and source records attributed to J Finsterer.

At least 37 records · Page 2Linked to original sources
PubMed1999

Immunoadsorption in multifocal motor neuropathy.

To our knowledge, this is the first reported application of immunoadsorption in a patient with multifocal motor neuropathy (MMN). The diagnosis relied on the typical clinical features, markedly increased IgM-GM1 antibodies, multiple motor conduction blocks, and motor nerve biopsy. Immunoadsorption was carried out in seven cycles of two sessions each within 97 days. From the first therapy, muscle force as well as nerve conduction velocity and conduction blocks deteriorated continuously. As a consequence, immunoadsorption cannot be recommended as a treatment for MMN.

Antibodies, Anti-Idiotypic↗
PubMed1999

High-altitude illness induced by tooth root infection.

High-altitude illness may occur after recent pulmonary infection, but high-altitude illness after root canal therapy has not been described previously. A 44-year-old man is presented who skied to a 3333 m high peak in the Eastern Alps one day after he had undergone root canal therapy because of a tooth root infection. After 4 hours above 3000 m severe symptoms of high-altitude illness, including pulmonary oedema, developed. His condition improved after immediate descent. The next day he presented with local and general signs of infection which were successfully treated with gingival incisions and antibiotics. In conclusion, acute tooth root infection and root canal therapy may induce high-altitude illness at an altitude just above 3000 m.

Adult↗
PubMed1999

Diagnostic work-up in peripheral neuropathy: an analysis of 171 cases.

This study was set up to evaluate retrospectively the efficacy of a standard diagnostic procedure, including non-invasive and invasive (spinal tap, nerve/muscle biopsy) investigations, in the diagnosis of peripheral neuropathy. The medical records of 171 in-patients with the final diagnosis of peripheral neuropathy of determined or undetermined cause were reviewed and each individual diagnostic work-up was analysed. Basic investigations included the patient's history, a clinical examination and basic laboratory tests. Depending on the individual presentation, course, and severity, further non-invasive and invasive examinations were added according to the department's standard diagnostic procedure. The aetiology could be clarified in 124 patients (73%) and remained unclear in 47 cases. Excluding cases with acute and chronic inflammatory polyneuropathy (n=14), the number of idiopathic peripheral neuropathies dropped to 33. Non-invasive investigations were sufficient to reveal the underlying aetiology in 114 cases (83 %). It is concluded that, with the application of a standard procedure for the diagnosis of peripheral neuropathy, the aetiology can be clarified in 81% of patients. In the other 19% of patients the aetiology remains idiopathic. In the majority of cases, non-invasive investigations were sufficient for diagnosis.

Adult↗
PubMed1999

Cardiac involvement in Werdnig-Hoffmann's spinal muscular atrophy.

Despite the increasing interest in cardiac involvement (CI) of neuromuscular disorders, only few data are available on CI in spinal muscular atrophy (SMA). We tried to determine the cardiac manifestations of SMA, their incidence rates and the necessity of cardiac therapy in patients with SMA and CI. Eight patients with SMA, aged 10-79 years, underwent clinical cardiologic examination, ECG, echocardiography and ambulatory ECG. The most frequent findings were angina pectoris, palpitations, exertional dyspnea, ST abnormalities, couplets, thickened myocardium and diastolic dysfunction. In conclusion, cardiac manifestations of SMA mainly comprise ECG abnormalities and thickened myocardium. CI in SMA frequently necessitates cardiac therapy.

Adolescent↗
PubMed1999

Hypothyroid myopathy with unusually high serum creatine kinase values.

Depending on the degree of hormone deficiency, skeletal muscle involvement may occur in hypothyroidism. Usually, hypothyroid myopathy is associated with creatine kinase values <5,000 U/l. We report a 54-year-old man suffering from increasing fatigability, hoarseness, gait disturbances and a creatine kinase of 9,000 (normal: <80 U/l). He presented with bradyphrenia, macroglossia, dysarthria, myxedema, monoparesis, reduced deep tendon reflexes and stocking-type sensory disturbances. Free triiodthyronine was 0.25 pg/ml (normal: 0.6-1.9 pg/ml), free thyroxine <0.1 ng/dl (normal: 0. 6-1.8 ng/dl) and the thyroid-stimulating hormone >48.0 (normal: 0. 1-4.0 IU). Clinical neurologic examination and electromyography were compatible with myopathy and polyneuropathy. Other causes of myopathy, except hypothyroidism, were excluded. After L-thyroxine therapy (1.7 microg/kg BW/day) during 3 months, the patient's symptoms and signs vanished, except for sensory disturbances, and creatine kinase values and electromyography became normal. Severe hypothyroidism may be associated with highly elevated creatine kinase and myopathy. Adequate therapy leads to complete recovery, including myopathy.

Creatine Kinase↗
PubMed1998

Lactate stress test in the diagnosis of mitochondrial myopathy.

The aim of the study was to determine the sensitivity and specificity of the lactate stress test in the detection of mitochondrial myopathies. Thirty one healthy subjects, 10 patients with non-mitochondrial myopathy and 26 patients with mitochondrial myopathy underwent lactate stress testing at a standardized workload of 30 W during 15 min on a bicycle ergometer. Lactate was determined before the exercise (R1), 5, 10, 15 min after starting the exercise (S5, S10, S15) and 15 min after finishing the exercise (R2). A result was interpreted as pathologic if more than two of the five lactate values were above the corresponding upper reference limits. The upper reference limits for the venous lactate at R1, S5, S10, S15 and R2 were 1.9, 2.0, 2.1, 2.0 and 1.7 mmol/l respectively. The lactate stress test was pathologic in 1/10 of the non-mitochondrial myopathies and in 18/26 of the mitochondrial myopathies. The sensitivity of the lactate stress test was 69%. The specificity of the test was 90%. In conclusion, the lactate stress test proved to be helpful for evaluating the integrity of the oxidative metabolism in the majority of patients with mitochondrial myopathy.

Adolescent↗
PubMed1998

Transesophageal echocardiography to assess embolic risk in patients with atrial fibrillation. ELAT Study Group. Embolism in Left Atrial Thrombi.

BACKGROUND: Transesophageal echocardiography visualizes the left atrium and its appendage, thrombi, and spontaneous echocardiographic contrast. OBJECTIVE: To assess the association of transesophageal echocardiographic characteristics with stroke or embolism in atrial fibrillation. DESIGN: Multicenter observational follow-up study. SETTING: Hospitals in Austria and Slovakia. PATIENTS: 409 outpatients with nonrheumatic atrial fibrillation and without recent stroke. INTERVENTION: Patients with thrombi received anticoagulation, and patients without thrombi received aspirin. MEASUREMENTS: Primary events were stroke or embolism. Secondary events were death not caused by stroke or embolism and need for anticoagulation. RESULTS: In the left atrium or left atrial appendage, 10 patients (2.5%) had thrombi and 47 (12%) had spontaneous echocardiographic contrast. The appendage had a mean (+/- SD) length of 44+/-10 mm, a mean width of 23+/-6 mm, and a mean area of 5.8+/-2.5 cm2. Follow-up ranged from 1 to 74 months (mean, 58 months). Fifty patients had stroke or embolism, 53 died of a cause other than stroke or embolism, and 38 required anticoagulation. On univariate analysis, thrombi (risk ratio, 3.9 [95% CI, 1.4 to 10.1]; P = 0.009), length of the left atrial appendage (risk ratio, 1.6 [CI, 1.05 to 2.5]; P = 0.03), and width of the left atrial appendage (risk ratio, 2.4 [CI, 1.2 to 4.81; P = 0.01) were associated with stroke or embolism. Multivariate analysis identified hypertension (risk ratio, 3.6 [CI, 1.8 to 8.4]; P = 0.001), previous stroke (risk ratio, 3.7 [CI, 1.5 to 7.5]; P = 0.002), and age (risk ratio, 1.1 [CI, 1.0 to 1.11; P < 0.001) as risk factors for stroke or embolism and provided evidence of an association between thrombi and stroke or embolism (risk ratio, 2.4 [CI, 0.9 to 6.9]; P = 0.09). CONCLUSIONS: In outpatients with atrial fibrillation and without recent stroke, thrombi of the left atrium or left atrial appendage and length and width of the left atrial appendage were associated with stroke or embolism in univariate analysis. In a multivariate analysis, age, hypertension, and previous stroke were risk factors for stroke or embolism, and thrombi of the left atrium or left atrial appendage were possible risk factors. In these patients, history may be more useful than transesophageal echocardiography for the assessment of embolic risk.

Atrial Fibrillation↗
PubMed1998

Myotonic dystrophy: molecular genetics and diagnosis.

Myotonic dystrophy (DM) is the most common adult muscular dystrophy and follows an autosomal dominant pattern of inheritance. Up to now, the clinical diagnosis of DM was based on symptoms presented such as encephalopathy, facies myopathica, paresthesia, atrophy, myotonia, mental retardation, cataract, diabetes, cardiac conduction defects and electromyography. Since 1991 the specific molecular defect in DM is known and a respective diagnosis is possible. The mutation responsible for DM is the expansion of an unstable trinucleotide repeat, (CTG)n, in the 3'-untranslated region of the myotonin protein kinase gene. It is now generally accepted that the CTG repeat length correlates with the clinical category and the age at onset of the disease; therefore genetic tests are essential in monitoring and management of DM-patients and their family members. Based on the average incidence in Europe about 1000 affected individuals can be expected in Austria, a high percentage of whom is, however, not recognized as carries of the DM-mutation. After having established a genetic diagnosis in Austria allowing the detection of this mutation in DM-patients and their relatives, improvement of the diagnostic procedure should be possible.

Adult↗
PubMed1998

Reversal of dilated to hypertrophic cardiomyopathy after alcohol abstinence.

Left ventricular dilation and systolic dysfunction develop in 14-16% of patients with hypertrophic cardiomyopathy. Such findings may easily be misdiagnosed as dilated cardiomyopathy. It is unknown whether left ventricular dilatation and systolic dysfunction in patients with hypertrophic cardiomyopathy are reversible. A 35-year-old man had been a heavy drinker for 13 years and was abstinent for 1 year. Five years previously he suffered cardiac arrest and, based on echocardiographic, radionuclide, and cardiac catheterization findings, the diagnosis of alcohol-induced dilated cardiomyopathy was established. At presentation the heart was of normal size, with concentric left ventricular hypertrophy and only slightly reduced systolic function. Hypertrophic cardiomyopathy was diagnosed since no other cause for left ventricular hypertrophy could be detected. In hypertrophic cardiomyopathy, alcohol may induce reversible systolic dysfunction and left ventricular dilatation.

Adult↗
PubMed1998

Increased sensitivity to rocuronium and atracurium in mitochondrial myopathy.

PURPOSE: To describe the prolonged effect of the intermediate-acting, non-depolarising neuromuscular blocking agents rocuronium and atracurium in a 29-yr-old apparently healthy woman. CLINICAL FEATURES: Because of abdominal pain the patient was scheduled for explorative laparoscopic pelvic examination. General anaesthesia was induced with fentanyl, midazolam and propofol. Muscle relaxation was achieved with 0.6 mg.kg-1 rocuronium. Anaesthesia was maintained with nitrous oxide and propofol. Two Hz train-of-four stimulation every 15 sec evoked no twitch responses until 60 min after rocuronium. Further relaxation was achieved with 0.075 mg.kg-1 atracurium after which twitch responses recurred after 45 min. Fifteen minutes later neuromuscular blockade was successfully reversed with atropine and neostigmine. The postanaesthetic course was uneventful. Because of the increased sensitivity to rocuronium and atracurium the patient was re-evaluated postoperatively. History revealed occasional double vision, fatigue, muscle cramps, stiffness and myoglobinuria. Clinical neurological examination showed ptosis, tremor, ataxia and bradydiadochokinesia. A standardised lactate stress testing on a bicycle was pathological and, after muscle biopsy, the diagnosis of mitochondrial myopathy was established. CONCLUSION: An increased sensitivity to rocuronium and atracurium may occur in patients with mitochondrial myopathy. In these patients appropriate dosing of muscle relaxants and adequate monitoring of the neuromuscular blockade are required. If an increased sensitivity to rocuronium and atracurium occurs in an apparently healthy subject, further neurological investigations should follow.

Adult↗
PubMed1998

Chest pain in cardiac syndrome X--caused by neuromuscular disorders?

We wanted to find out if chest pain in cardiac syndrome X can be a manifestation of neuromuscular disorders. Five patients with cardiac syndrome X (3 women, 2 men), aged 34 to 70 years, consented with a clinical neurological examination, muscle enzyme testing, electroneurography of the right median and peroneal nerves and electromyography of the right brachial biceps and anterior tibial muscles. A neuromuscular disorder was found in 1 of the 5 investigated patients. The 60-year-old man presented with a monoparesis of the left leg and sensory dysfunction of the left upper and lower limb. He was diagnosed as having either posttraumatic myelopathy or radiculopathy. Since chest pain in cardiac syndrome X can be caused by neuromuscular disorders, a comprehensive neurological examination is recommended in patients with this disorder.

Adult↗
PubMed1998

Influence of spontaneous activity on peak-ratio analysis.

OBJECTIVES: To prove or disprove this assumption that in neuropathy patients with abundant spontaneous activity, peak-ratio interference pattern analysis may lead to false negative results. METHODS: Spontaneous activity >100 microV, automatically analysed by turn/amplitude analysis and expressed as (turns/second)/2 ((T/S)/2), and interference patterns, analysed by the peak-ratio technique, were recorded, one after the other, from the right anterior tibial muscle of 21 patients with neuropathy, aged 36-87 years. RESULTS: The mean number of spontaneous discharges ((T/S)/2) was 12.3 (range 5.5-26) and its mean amplitude 261 microV (range 146-478 microV). Despite this abundant spontaneous activity, peak-ratio analysis was neurogenic in 81% of the patients. All peak-ratio parameters were independent on the amount and amplitude of spontaneous discharges. CONCLUSIONS: Spontaneous discharges >100 microV could be adequately assessed by means of the turn/amplitude analysis and did not influence peak-ratio analysis in neuropathies.

Adult↗