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Biomedical subjects

J Finsterer

Publications and source records attributed to J Finsterer.

At least 19 recordsLinked to original sources
PubMed2003

[Fibrate and statine myopathy].

Fibrates and statines are useful in preventing arteriosclerosis and thromboembolic events. However, they should be administered with caution. The risk of fibrate/statine myopathy, the most common side effect of fibrate/statine therapy, increases with dosage, combination of a fibrate with a statine,hypothyroidism, renal insufficiency, and intake of cytochrome P450 inhibitors. In case of clinical signs of a fibrate/statine myopathy such as proximal myalgias, stiffness,weakness, and dark urine, appropriate investigations should be initiated. If they establish the diagnosis of fibrate/statine myopathy, lipid lowering drugs should be immediately withdrawn. If rhabdomyolysis is present, prompt and adequate therapy is required. Though muscle abnormalities are rare side effects, fibrate/statine therapy should be regularly monitored with regard to clinical and laboratory alterations. The new "superstatines" rosuvastatine and pitavastatine reduce serum cholesterol more effectively than the established statines, but it is not known if they also have fewer muscular side effects than established statines.

Animals↗
PubMed2003

Quality of blood pressure control and risk of cerebral bleeding in patients with oral anticoagulation.

BACKGROUND: We assessed how many patients on long-term oral anticoagulation (OAC) (i) suffer from arterial hypertension (AH), (ii) are aware of AH, (iii) need improvement of their therapy and (iv) suffer from cerebral bleeding. METHODS: Outpatients on long-term OAC were asked to measure blood pressure at least 4 times. Blood pressure was classified as normotensive if at least 75 % of all measurements were < 139/89 mm Hg; as mild/moderate AH if > 25 % of all measurements were 140 - 179 mm Hg systolic or 90 - 109 mm Hg diastolic; and as severe AH if > 25 % of all measurements were > 180 mm Hg systolic or > 110 mm Hg diastolic. Bleeding complications were registered. RESULTS: Of the 235 patients (108 female, 67 +/- 12 years), 80 % suffered from AH. Severe AH was present in 5 %. Only 56 % were aware of suffering from AH. An improvement of antihypertensive therapy was needed in 64 %. Over 225 days, only one cerebral bleeding occurred. Blood pressure was normotensive in 30 % with known AH. CONCLUSIONS: Blood pressure control seems better in OAC patients than in normals, if the patient is aware of AH. Patients with AH on OAC are not aware of AH in > 50 %. Repeated blood pressure measurements in OAC are recommended, even if patients are not aware of AH.

Administration, Oral↗
PubMed2002

Is left atrial appendage occlusion useful for prevention of stroke or embolism in atrial fibrillation?

Since in atrial fibrillation more than 90% of the thrombi are located in the left atrial appendage, an "elimination" of the left atrial appendage, either by resection or occlusion, seems an attractive alternative to oral anticoagulation. Although frequently regarded as an useless appendage, data from animal and human investigations show that the left atrial appendage may play an important role in the maintenance and regulation of the cardiac function, especially in arterial hypertension, atrial fibrillation, coronary heart disease, valvular heart disease and heart failure. Elimination of the left atrial appendage may impede thirst in hypovolemia, deteriorate hemodynamic responses to volume or pressure overload, decrease cardiac output and promote heart failure. Instead of preventing stroke, the consequences of left atrial appendage elimination may create new risk factors for stroke and thus might induce more harm than benefit to patients with atrial fibrillation. As long as the physiologic and pathophysiologic role of the left atrial appendage is not fully understood, left atrial appendage elimination should not be an alternative to oral anticoagulation.

Animals↗
PubMed2002

Cranial nerve lesions and abnormal visually evoked potentials associated with the M694V mutation in familial Mediterranean fever.

A 52-year-old Turkish man with familial Mediterranean fever (FMF) due to the homozygous M694V mutation in the MEFV-gene on chromosome 16p13.3, newly developed hemicrania, blurred and double vision, ptosis, ophthalmoparesis and peripheral facial nerve palsy. Except for double vision, all the other abnormalities disappeared spontaneously within 10 days after onset. Markedly prolonged latencies of the visually evoked potentials were also found. At follow-up, 8 months after onset of the neurological abnormalities, right-sided bradydiadochokinesia, right-sided discrete weakness and right-sided hypaesthesia were found. After the exclusion of other hereditary fever syndromes, migraine, stroke, Molaret's meningitis, Behçet's syndrome and mitochondriopathy by clinical, serological, CSF investigations, funduscopy, electroencephalography, and cerebral MRI and MRI angiography, the described neurological abnormalities were regarded as CNS and PNS manifestation of vasculitis or amyloidosis in FMF.

Evoked Potentials, Visual↗
PubMed2002

Elevated 14-3-3 protein and axonal loss in immunoglobulin-responsive, idiopathic acute transverse myelitis.

OBJECTIVES: To report the elevation of the 14-3-3 protein and the complete denervation of hand muscles in idiopathic acute transverse myelitis (IATM) of the cervical cord. CASE DESCRIPTION: In a 29-year-old woman with a 2-week history of neck pain and repeated attenuated flus, subacute quadriplegia, hypaesthesia of both arms, a T3 sensory level, and urinary dysfunction occurred. Based upon the clinical findings, the cervical MRIs, and an elevated 14-3-3 protein in the CSF, IATM C4-C7 was diagnosed. Ten, 17, 28 and 61 days after onset, nerve conduction studies revealed complete denervation of the right abductor pollicis brevis and abductor digiti minimi muscles but gradual improvement of the compound muscle action potential of the left abductor pollicis brevis muscle. F-waves of the right median nerve were absent. Tibial somatosensory evoked potentials showed a prolonged central conduction time. Transcranial magnetic stimulation evoked a response in the left but not the right abductor digiti minimi muscle. CONCLUSION: IATM may cause elevation of the 14-3-3 protein and loss of motor axons originating from affected anterior horn cells.

14-3-3 Proteins↗
PubMed2002

Respiratory chain complex-I defect mimicking myasthenia.

In a 67-year-old woman with ptosis, double vision, dysphagia, ambiguous Tensilon tests, normal acetylcholine-receptor antibodies, normal thymus, and repeatedly abnormal responses to low-frequency repetitive stimulation, ocular myasthenia was suspected. Pyridostigmin was ineffective, but corticosteroids improved the abnormalities. Despite this therapy, lower-limb weakness developed. Reevaluation disclosed abnormal increase of serum lactate during slight exercise, myogenic electromyography, ragged-red fibers, reduced oxidative enzyme staining and abnormally shaped and structured mitochondria on muscle biopsy, and a respiratory chain complex-I defect on biochemical investigation of the muscle homogenate. Respiratory chain disorder due to complex-I defect with abnormal decremental response to low-frequency repetitive stimulation was diagnosed. It is concluded that respiratory chain disorders due to a complex-I defect may mimic ocular myasthenia clinically, electrophysiologically, and even therapeutically.

Action Potentials↗
PubMed2002

Parkinson syndrome as a manifestation of mitochondriopathy.

OBJECTIVES: Although there is growing evidence for a relation between Parkinson syndrome (PS) and mitochondriopathy (MCP), little is known about the frequency of PS in MCP. MATERIAL AND METHODS: This study assessed the frequency of PS in patients with MCP, the phenotype of these patients, and their response to anti-Parkinson medication, during a 1-year period. RESULTS: Between April 1999 and March 2000 PS was diagnosed in nine of 76 patients with MCP (12%). The frequency of MCP among 144 patients with PS attending the department during the investigational period was 6.3%. Systems most frequently affected by the MCP in the nine patients were the peripheral nervous system, central nervous system, endocrinium, heart, intestines, eyes, ears and kidneys. PS in MCPs responded well to amantadine, L-DOPA, dopamine agonists and catechole-o-methyl-transferase inhibitors. CONCLUSION: Twelve per cent of the patients with MCP have phenotypic features of PS and 6% of the patients with PS have features of MCP. MCP patients with PS frequently show multisystem involvement. PS in MCP responds well to anti-Parkinson medication.

Aged↗
PubMed2002

Transverse syndrome as the dominant feature of solitary cervical plasmocytoma with dissemination.

STUDY DESIGN: A case report of transverse syndrome secondary to compression from an intravertebral plasmocytoma. OBJECTIVES: To report a rare case of cervical cord compression. SETTING: Vienna, Austria. CASE REPORT: A 47-year-old man presented with pain over the left scapula and subsequent development of pain and sensory disturbance. Investigations showed that the entire C7 vertebra was affected by a plasmocytoma. Decompression was carried out but no stabilisation of the spinal column was performed. Seven days after surgery the patient became paraplegic. Chemotherapy produced no improvement. CONCLUSION: Transverse syndrome may be a dominant feature of plasmocytoma and may resolve after immediate decompression but may recur if the spinal column is not stabilised.

Cervical Vertebrae↗
PubMed2002

Myotonic dystrophy type 2.

Myotonic dystrophy type 2 (DM2) is a clinically but not genetically heterogeneous, multisystem disorder, that is clinically similar to, but distinct from myotonic dystrophy type 1 (DM1). Initially, different phenotypes of DM2 were described by Ricker (proximal myotonic myopathy, PROMM), Ranum (myotonic dystrophy 2, DM2) and Udd (proximal myotonic dystrophy, PDM). Clinical features these three phenotypes had in common were diffuse, proximal or distal weakness, wasting, myotonia, cataract, cerebral, endocrine and cardiac abnormalities. Initially, the clinical differences between DM1 and PROMM seemed unmistakable, but meanwhile it has become apparent that the clinical differences between these entities are blurring. In 1999, Day et al., Meola et al. and Ricker et al. mapped the mutated gene of all three phenotypes to chromosome 3q. In 2001, the three different phenotypes were found to rely on the same mutation in the ZNF9 gene on chromosome 3q21.3. Although DM2 may be clinically heterogeneous, it is by result of a mutation in a single gene. The mutation responsible for DM2 is a CCTG-repeat expansion of 75-11 000 repeats in intron 1 of the ZNF9 gene on chromosome 3q21.3. Because of the clinical heterogeneity, the diagnosis of DM2 should rely on DNA analysis alone.

Anti-Inflammatory Agents↗
PubMed2002

Lethal fulminate S. aureus sepsis in M. Behçet overnight cold exposure.

Whether patients with Behçet's disease (BD) and immunosuppressive therapy are generally prone to acquire severe infectious diseases is unknown. A 48-year-old man under corticosteroids and azathioprine for BD since 1995 was admitted because of a transitory ischemic attack. Between the third and fourth hospital day he was accidentally locked up, insufficiently dressed, in the hospital's chapel over night. On the following day, he developed fever and deteriorating consciousness until he became comatose. CT scans of the brain were normal and there was only a slight pleocytosis. Despite adequate therapy, the patient's condition further deteriorated such that he died. Responsible for his decline was a fulminate sepsis, diagnosed upon fever, increased C-reactive protein, thrombocyte decline, multi-organ failure, rhabdomyolysis, growth of S. aureus on blood culture, and autopsy. Patients with BD and immunosuppressive therapy, may be more vulnerable to infections and may develop lethal overwhelming sepsis already after overnight cold exposure.

Adult↗
PubMed2001

Diagnostic yield of the lactate stress test in respiratory chain disorders under absolute and relative workload.

Usually, the lactate stress test is carried under a constant, low workload maintained for 15 min, although there are indications that the lactate response to exercise is a function of the relative workload, and that a workload for only 5 min does not decrease its sensitivity. Thus, this study compared the diagnostic yield of the lactate stress test when carried out under a constant workload of 30 W (LSTA) and under a workload of 30% of maximum (LSTR), and when the workload was maintained for 5 and 15 min. In 16 patients with respiratory chain disorders, 12 women, four men, aged 27--88 years, the LSTA and LSTR were carried out on 2 different days, within 48 h. For both tests serum lactate was determined before, during and after a 15-min workload on a bicycle. Upper reference limits at rest, 5, 10, 15 min after starting, and 15 min after finishing the exercise were 1.9, 2.0, 2.0, 2.0, and 1.6 mmol/l for the LSTA, and 2.3, 3.0, 3.2, 3.4 and 2.7 mmol/l for the LSTR. The sensitivity was 88% for the LSTA and 63% for the LSTR. The specificity of the LSTA was 94%. The diagnostic yield of both tests was similar when the workload was maintained for 5 or 15 min. In conclusion, the LSTA should be preferred to the LSTR. Three lactate determinations during 15 min have no advantage over a single lactate determination after a 5 min workload of 30 W.

Adult↗
PubMed2001

[Echocardiography in storage and neuromuscular disorders].

Storage disorders and neuromuscular disorders may lead to cardiac involvement which can be visualized by echocardiography. In storage disorders like hypothyroidism, haemochromatosis, amyloidosis, mucopolysaccharidosis and Fabry's disease, myocardial thickening and systolic dysfunction can be found. In amyloidosis, atrial enlargement and abnormal texture of the myocardium are additional findings. In advanced haemochromatosis all cardiac chambers may be dilated. In hypothyroidism and amyloidosis, a pericardial effusion can be present. In haemochromatosis and amyloidosis, a restrictive filling pattern may be detected using Doppler-sonography. Mucopolysaccharidosis and Gaucher's disease may lead to aortic and mitral stenosis. In neuromuscular disorders like glycogenosis, mitochondriopathy and myotonic dystrophy, myocardial thickening and systolic dysfunction are found, in spinal muscular atrophy myocardial thickening and in muscular dystrophy Becker/Duchenne systolic dysfunction. An abnormal myocardial texture may be present in glycogenosis, isolated left ventricular abnormal trabeculation (ILVAT) in mitochondriopathy, myotonic dystrophy and muscular dystrophy Becker/Duchenne. Using Doppler-sonography an impaired relaxation of the left ventricle may be detected in mitochondriopathy, myotonic dystrophy and spinal muscular atrophy. Most of these echocardiographic findings are unspecific and may be overlooked, especially if the storage or neuromuscular disorder is yet unknown. Establishing a correct diagnosis is important, since healing or functional improvement is possible in many of these disorders.

Amyloidosis↗
PubMed2001

Possible causes of symptoms in suspected coronary heart disease but normal angiograms.

BACKGROUND: In patients with suspected coronary heart disease and normal angiography, the causes of cardiac symptoms frequently remain undetermined. A correct diagnosis is desirable, however, since some of the underlying disorders may be curable, treatable, influence prognosis, or induce screening of the relatives. HYPOTHESIS: In such patients, the prevalence of arterial hypertension, hemochromatosis, hypothyroidism, hypoparathyroidism, tachycardiomyopathy, amyloidosis, and neuromuscular disorders as a possible cause for their symptoms and the seroprevalence of micro-organisms, known to cause myocardial damage, were assessed. METHODS: Consecutive patients with normal coronary angiograms were invited for two visits comprising clinical history and investigation, electrocardiograms, blood tests, and echocardiography. Patients were investigated neurologically if unexplained anginal chest pain or creatine kinase elevation persisted or if echocardiography showed isolated left ventricular abnormal trabeculations. RESULTS: In 71 patients (31 women, 40 men, mean age 60 years), the most common cause for cardiac symptoms was hypertension (66%), followed by neuromuscular disorders (13%), tachycardiomyopathy (9%), hypothyroidism (4%), and hemochromatosis (3%). The seroprevalence for Chlamydia species was 90%, Helicobacter pylori 70%, Chlamydia pneumoniae 63%, Borrelia burgdorferi sensu lato 15%, and Rickettsia conorii 10%. No possible cause was found in 24% of the patients. CONCLUSIONS: In patients with suspected coronary heart disease and normal angiograms, hypertension, neuromuscular disorders, tachycardiomyopathy, hypothyroidism, and hemochromatosis should be considered as possible causes.

Adult↗
PubMed2001

Left ventricular hypertrabeculation in myotonic dystrophy type 1.

BACKGROUND: Left ventricular hypertrabeculation (LVHT) has not been described in myotonic dystrophy Type I (MD1) before. CASE REPORT: A 42-year-old man developed typical features of MD1 since 1992. Creatinekinase was slightly, but recurrently elevated. Needle electromyograms were myogenic and showed extensive spontaneous activity. Muscle biopsy was compatible with MDI. DNA analysis revealed a heterozygous 300 CTG-repeat expansion in the myotonic-dystrophy proteinkinase gene on chromosome 19q13.3. Cardiac history and clinical cardiologic examination were normal. On ECG, ST elevation and atrial flutter were found. The AECG was normal except for atrial flutter. Surprisingly, transthoracic echocardiography revealed LVHT, previously described only in Becker's muscular dystrophy, mitochondriopathies, and Barth syndrome. CONCLUSION: A rare cardiac manifestation of MD1 may be LVHT which alone has no therapeutic implication.

Adult↗
PubMed2001

Spontaneous left ventricular hypertrabeculation in dystrophin duplication based Becker's muscular dystrophy.

BACKGROUND: Left ventricular hypertrabeculation is frequently associated with neuromuscular disorders. Whether left ventricular hypertrabeculation in these patients is congenital or develops during lifetime, is unknown. CASE REPORT: In a 65-year-old man with Becker's muscular dystrophy, due to a duplication in the dystrophin gene on chromosome Xq21 (dystrophin molecular weight: 500 kD), left ventricular hypertrabeculation was detected on transthoracic echocardiography although being absent in repeated previous echocardiographic examinations. Additionally, there was thickening of the left ventricular myocardium. The spontaneous occurrence of left ventricular hypertrabeculation was interpreted as progression of cardiac involvement in Becker's muscular dystrophy. CONCLUSION: Left ventricular hypertrabeculation may not exclusively be congenital, but may occasionally develop spontaneously during lifetime, being interpreted as progression of cardiac involvement in Becker's muscular dystrophy.

Aged↗