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Biomedical subjects

Josef Finsterer

Publications and source records attributed to Josef Finsterer.

At least 19 recordsLinked to original sources
PubMed2006

Noncompaction in Duchenne muscular dystrophy: frustrated attempt to create a compensatory left ventricle?

The histological workup of the myocardium of a patient with Duchenne muscular dystrophy and left ventricular hypertrabeculation/noncompaction (LVHT) revealed an extremely thin left ventricular wall and a noncompacted layer double in size compared to the compacted layer. Within the compacted layer islets of fibrous tissue predominated, surrounded by areas of myxoid appearance hardly producing collagen, and occasionally normal or dystrophic cardiomyocytes. The noncompacted layer consisted largely of intact cardiomyocytes rarely intermingled with collagen-producing, fibrous tissue. This variant appearance of the compacted and noncompacted layer was found in all areas of noncompaction. These histopathological findings suggest that LVHT represents a compensatory attempt to overcome the failing compacted but dystrophic myocardium.

Adult↗
PubMed2006

Successful heart failure therapy in mitochondrial disorder with noncompaction cardiomyopathy.

OBJECTIVE: Effective heart failure therapy with angiotensin-converting enzyme inhibitors and beta-blockers in a patient with mitochondrial disorder and asymptomatic previously stable hypertrophic cardiomyopathy and left ventricular noncompaction (NCCMP) has not been reported. CASE REPORT: In a 58-year-old male with juvenile seizures, impaired hearing, recurrent pancreatitis, diabetes, recurrent emesis and diarrhea, discrete weakness for hip flexion, general wasting, and reduced tendon reflexes, elevated muscle-enzymes, abnormal lactate-stress-test, and mitochondrial dysfunction on muscle biopsy, mitochondrial disorder was diagnosed at age 51 year. Echocardiography revealed myocardial thickening and NCCMP. Cardiac abnormalities did not progress upon repeated follow-ups. At age 57 year he developed acute heart failure during respiratory infection. Echocardiography additionally revealed reduced left ventricular systolic function, and a restrictive filling pattern. Within seven weeks of therapy with ramipril (2.5 mg/day) and bisoprolol (1.25 mg/day) the restrictive filling pattern disappeared and fractional shortening normalized. CONCLUSION: This case shows that heart failure in a patient with mitochondrial disorder and previously stable hypertrophic cardiomyopathy and NCCMP promptly resolves under therapy with angiotensin-converting enzyme inhibitors and beta-blockers.

Adrenergic beta-Antagonists↗
PubMed2006

Left ventricular hypertrabeculation/noncompaction with PMP22 duplication-based Charcot-Marie-Tooth disease type 1A.

A 50-year-old women with Charcot-Marie-Tooth hereditary neuropathy type 1A due to the PMP22 duplication on chromosome 17p11.2-12 developed a left bundle branch block and progressive dilatation of the left ventricle since age 40 years and recurrent heart failure since age 44 years. At age 50 years left ventricular hypertrabeculation/noncompaction was first recognized on transthoracic echocardiography. A possible causal relation between the cardiac abnormalities and the PMP22 duplication is discussed.

Bundle-Branch Block↗
PubMed2006

Acquired noncompaction in Duchenne muscular dystrophy.

Acquired left ventricular hypertrabeculation/noncompaction (LVHT) is rare and has been described in patients with mitochondriopathy, Barth syndrome, and Becker muscular dystrophy. Here we report acquired LVHT in a 28-year-old man with Duchenne muscular dystrophy who required non-invasive, positive-pressure ventilation for muscular respiratory failure since age 16 years. Transthoracic echocardiography at age 22 years revealed enlarged left atrium and ventricle and reduced fractional shortening, but no LVHT. Transthoracic echocardiography at age 27 years revealed enlarged left atrium and ventricle, systolic dysfunction, mitral insufficiency, and, surprisingly, LVHT. The cause and pathomechanism of acquired LVHT in this patient remained speculative.

Adult↗
PubMed2006

Severe, persisting, steroid-responsive Dengue myositis.

OBJECTIVES: Short-term, general muscle affection is frequent in Dengue infection, but severe, persisting, myositis has not been reported. STUDY DESIGN: Case report. RESULTS: The patient is a 38 years old, HIV-negative male who developed sudden-onset fever up to 40.0 degrees C, headache, and sore eyes upon looking into light when on holidays in Thailand. One day after onset severe myalgias occurred in the shoulder girdle and hip girdle muscles. Clinical examination was normal, but blood work revealed elevated creatine-phosphokinase, glutamate-oxalate transaminase, and glutamate pyruvate transaminase, leucopenia and thrombocytopenia. Antibodies against Dengue viruses type 2 and 4 were positive and classical Dengue fever was diagnosed. The infection resolved upon symptomatic therapy, but myalgias, responsive only to opiates, resolved persistently not before the administration of corticosteroids, 2 months after onset. CONCLUSIONS: The case shows that Dengue fever may also cause persisting, severe, myositis for weeks, which do not respond to non-steroidal analgesics, but promptly to corticosteroids.

Adrenal Cortex Hormones↗
PubMed2006

Chronic long-standing headache due to neurocysticercosis.

A 35-year-old Chinese woman presented with a 26-year history of persistent headache, relieved only by diuretics. Characteristic CT findings, peripheral eosinophilia, lymphocytic CSF pleocytosis, elevated CSF IgG, positive oligoclonal bands, antibody-positive ELISA, and Western blot results with Taenia solium antigen, and a favorable response to albendazole led to the diagnosis of neurocysticercosis.

Adult↗