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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
PubMed2000

Bilateral stenting of symptomatic and asymptomatic internal carotid artery stenosis due to fibromuscular dysplasia.

Stent grafting of internal carotid artery (ICA) stenoses due to fibromuscular dysplasia has been rarely and only unilaterally carried so far. Bilateral carotid stent grafting of ICA stenoses due to fibromuscular dysplasia has not been reported previously. In a 37 year old woman with recurrent right hemispheric transitory ischaemic attacks, a non-disabling minor stroke, and recurrent right amaurosis fugax despite antithrombotic therapy, cerebral angiography disclosed a long segment narrowing, distal, high grade (95%) stenosis of the right ICA and a long narrowing, distal high grade (70%) stenosis of the left ICA. Morphological features of both stenoses were indicative of fibromuscular dysplasia. The right sided stenosis was stented with a PTFE-HEMOBAHN endoprosthesis; this was followed by a brief, postprocedural left sided hemiparesis. The left sided ICA stenosis was successfully stented by the same procedure. Nine months later, both stents were still patent and the patient was symptom free. Bilateral carotid stenting may remain an alternative to endarterectomy in bilateral ICA stenosis due to fibromuscular dysplasia when ischaemic events persist despite full antithrombotic therapy.

Adult↗
PubMed2000

Cardiac involvement in primary myopathies.

Simultaneous or temporarily staggered affection of both the skeletal as well as the cardiac muscle (cardiac involvement, CI) is a frequent finding in primary myopathies (MPs). CI leads to impulse generation defects, impulse conduction defects, thickened myocardium, left ventriculalr hypertrabeculation, dilatation of the cardiac cavities, secondary valve insufficiency, reduction of coronary vasodilative reserve, intracardial thrombus formation, and heart failure with systolic and diastolic dysfunction. CI has been found in Duchenne muscular dystrophy (MD), Becker MD, Emery-Dreifuss MD, facioscapulohumeral MD, sarcoglycanopathies, myotubular congenital MD, myotonic dystrophies type 1 and 2, proximal myotonic myopathy, myoadenylate deaminase deficiency, glycogenosis type II, III, IV, VII and IX, carnitine deficiency, mitochondriopathy, desmin MP, nemaline MP, central core disease, multicore MP, congenital fiber-type disproportion MP, Barth syndrome, McLeod syndrome and Bethlem MP. Patients with any of the above-mentioned myopathies should be cardiologically investigated as soon as their diagnosis is established, since sufficient cardiac therapy improves CI in MPs and since management of these patients is influenced by the degree of CI.

Cardiomyopathies↗
PubMed2000

Isolated left ventricular abnormal trabeculation is a cardiac manifestation of neuromuscular disorders.

OBJECTIVES: Isolated left ventricular abnormal trabeculation (ILVAT) is defined as >3 coarse trabeculations of the left ventricular wall, apically to the papillary muscles, in hearts without congenital malformations. The aims of the study were to assess by echocardiography the prevalence of ILVAT, look for cardiac findings in ILVAT and find out if ILVAT is associated with neuromuscular disorders. DESIGN AND RESULTS: In the course of 3 years, ILVAT was found in 15/17, 648 patients (0.08%). Twelve patients had heart failure, all had ECG abnormalities. All patients had neuromuscular disorders. A definite diagnosis could be established in 10 patients (metabolic myopathy, n = 9; Becker's muscular dystrophy, n = 1). The remaining 5 patients refused further neurological investigations. CONCLUSIONS: ILVAT is a rare disorder associated with ECG abnormalities, heart failure and neuromuscular disorders, which is a reason why both cardiologists and neurologists should be involved.

Adult↗
PubMed2000

Complex mitochondriopathy associated with 4 mtDNA transitions.

In a 33-year-old man, mitochondriopathy was diagnosed upon short stature, auditory impairment, gynaecomastia, hypogonadism, vertical ophthalmoplegia, cerebral atrophy, leucencephalopathy, cataract, hypertrabeculated left ventricle, hypothyroidism, diabetes mellitus, glomerulonephritis necessitating kidney transplantation, general wasting, polyneuropathy, abnormally high lactate levels on exercise, partially reduced cytochrome-c oxidase staining and abnormally structured mitochondria on muscle biopsy. Mitochondrial DNA (mtDNA) analysis revealed 1 novel (A15662G) and 3 known mtDNA transition(s) (T3398C, T4216C, G15812A) affecting the cytb and ND1 gene, respectively. Three of the patient's transitions were also detected in blood leukocytes of the patient's maternal grandmother, mother and brother. Mutant mtDNA was heteroplasmic at >75% in the patient's skeletal muscle.

Adult↗
PubMed2000

Chronic granulomatous meningitis with multiple cranial nerve lesions hydrocephalus, stroke, sinus thrombosis, and epilepsy.

Most frequently, chronic granulomatous meningitis (CGM) is caused by infectious agents. However, in some cases the cause of CGM remains undetermined. It is unclear whether antimicrobial agents, including antituberculous drugs, are helpful in such cases. We describe a 61-year-old man who had multiple cranial nerve lesions, epilepsy, sinus thrombosis, stroke, and hydrocephalus attributable to CGM. Repeated extensive search for a causative agent in the cerebrospinal fluid (CSF) and the meninges remained negative. Only a single culture of the sputum revealed growth of Mycobacterium tuberculosis, which prompted antituberculous therapy with isoniazid, rifampicin, and ethambutol. After 6 months of therapy, neurologic abnormalities were slightly improved. We conclude that antimicrobial/ antituberculous agents have only a minor short-term effect in long-lasting CGM of undetermined cause.

Aged↗
PubMed2000

Spinal dural arteriovenous fistula associated with syringomyelia.

The previously undescribed association of a spinal dural arteriovenous fistula with syringomyelia was found in a 60-year-old male, who developed increasing paresis, numbness of both lower extremities and sphincteric dysfunction. Symptoms and signs were attributed to a syringomyelia at T5-L1 and an arteriovenous spinal dural fistula at L1. The fistula was successfully immobilised with N-butyl-cyano-acrylate. Six months after the procedure, all abnormalities had nearly disappeared. Whether the relation between the fistula and the syringomyelia was coincidental or causative could not be determined.

Angiography↗
PubMed1999

Giant visually-evoked potentials without myoclonus in the Heidenhain type of Creutzfeld-Jakob disease.

In a 64-year old woman with progressive visual impairment for 4 weeks, probable Creutzfeld-Jakob disease without myoclonus was diagnosed after rapidly progressive mental deterioration had also developed, and CSF and EEG showed characteristic findings. Pattern-reversal and flash visually-evoked potentials, recorded 5, 6, 7 and 8 weeks after onset, showed a maximum P100 latency of 210 ms, 8 weeks after onset, and a maximum N75/P100 amplitude of 33.1 microV, 5 weeks after onset. While the P100 latency progressively increased, the N75/P100 amplitude continuously decreased after reaching its maximum. In the Heidenhain type of Creutzfeld-Jakob disease giant visually-evoked potentials may be recorded during the early stages of the disease, even in the absence of myoclonus. Visually-evoked potentials may prove useful in diagnosing Creutzfeld-Jakob disease with atypical initial presentation.

Creutzfeldt-Jakob Syndrome↗
PubMed1999

Isolated left ventricular abnormal trabeculation in adults is associated with neuromuscular disorders.

BACKGROUND: Isolated left ventricular abnormal trabeculation (ILVAT) is defined as > 3 coarse trabeculations of the left ventricular wall, apically to the papillary muscles, in hearts without congenital malformations. HYPOTHESIS: The aims of the study were to assess by echocardiography the prevalence of ILVAT, to confirm the diagnosis by cardiac magnetic resonance imaging (CMRI), to look for cardiac findings in ILVAT, and to determine whether ILVAT is familial and associated with neurological disorders. METHODS: During a 1-year period (July 1995 to July 1996) all patients in whom transthoracic echocardiography visualized ILVAT, were included in the study. The examination and measurements were performed according to established guidelines. RESULTS: During 1 year, ILVAT was found in 6 of 3,397 patients (0.2%). When applied, CMRI confirmed ILVAT. Four patients had heart failure, all had electrocardiographic (ECG) abnormalities. None of the investigated relatives showed ILVAT. One patient had Becker's muscular dystrophy, three had mitochondrial myopathy, one had polyneuropathy, and one had muscle wasting of unknown origin. CONCLUSIONS: Isolated left ventricular abnormal trabeculation is rare, visible on echocardiography and CMRI, associated with ECG abnormalities, sometimes with heart failure, and always with neuromuscular disorders. Thus, when ILVAT is found, the cardiologist should consider a neurology referral.

Adult↗
PubMed1999

[Antithrombotic therapy in atrial fibrillation and additional stroke/embolism risk factors. CORSurvey Study].

OBJECTIVES: To evaluate 1) how many patients with atrial fibrillation (AF) and heart failure were discharged from Austrian hospitals with antithrombotic therapy, 2) if the presence of risk factors for stroke/embolism (age > 65 years, arterial hypertension, diabetes, and previous stroke) influence the choice of antithrombotic therapy and if the presence of contraindications for oral anticoagulation (dementia, alcohol abuse) influence the choice of antithrombotic therapy, and 3) if there are differences among the types of departments in the use of antithrombotic therapy. PATIENTS: Included were 1566 patients (841 female, 725 male, mean age 76 years) with AF and heart failure. METHODS: At discharge, a questionnaire was completed including risk factors, contraindications for antithrombotic therapy, and antithrombotic medication. RESULTS: Oral anticoagulants (OAC) had 26% of the cases, acetyl salicylic acid (ASA) 31%, a combination of OAC and ASA 2%, and no antithrombotic therapy 41%. The risk factors age > 65 years, arterial hypertension, diabetes, and previous stroke did not influence the choice of antithrombotic therapy. Dementia but not alcohol abuse influenced the choice against OAC. The rate of OAC was higher in cardiological or cardiovascular rehabilitation clinics than in other departments. CONCLUSION: The results of this survey show that in medical practice the recommendations regarding antithrombotic therapy in atrial fibrillation are rarely considered, especially when additional risk factors are present.

Aged↗
PubMed1999

Cardiac involvement in Becker's muscular dystrophy, necessitating heart transplantation, 6 years before apparent skeletal muscle involvement.

In Becker's muscular dystrophy cardiac abnormalities usually occur after onset of neuromuscular symptoms. We describe a Becker muscular dystrophy patient in whom chronic heart failure, necessitating cardiac transplantation, was the initial manifestation. Neuromuscular symptoms occurred not earlier than 6 years after the initial cardiac symptoms and 5 years after heart transplantation. In conclusion, severe heart failure due to dilated cardiomyopathy may be the initial manifestation of Becker's muscular dystrophy and may predate neuromuscular symptoms for years.

Adult↗
PubMed1999

Subclinical skeletal muscle involvement in long-QT syndrome.

Deafness is said to be the only extracardiac manifestation of long-QT syndrome. Whether long-QT syndrome manifests in the skeletal muscle as well, has not been investigated so far. Six affected members of two families with long-QT syndrome without deafness (Romano-Ward syndrome) underwent a clinical neurological examination, nerve conduction studies and needle electromyography. The clinical neurological examination and nerve conduction studies were normal but abundant spontaneous activity (fibrillations and bursts of fibrillations) could be recorded from the right biceps brachii muscle (one patient) and the right abductor pollicis brevis muscle (all patients). Since all other causes were excluded, spontaneous discharges were interpreted to be related to the long-QT syndrome. In conclusion, long-QT syndrome does not seem to be confined to the heart but may involve the skeletal muscle subclinically as well.

Action Potentials↗
PubMed1999

Macro-EMG in mitochondriopathy.

OBJECTIVES: In a previous macro-EMG study on patients with mitochondriopathy, macro-MUAP amplitude has been shown to be the electromyographic parameter most often abnormal. We wanted to investigate the role of the macro-MUAP area and if macro-EMG is more helpful than conventional needle-EMG in detecting involvement of the skeletal muscle in patients with mitochondriopathy. METHODS: From the right brachial biceps muscles of 38 healthy subjects and 20 patients with primary mitochondriopathy, aged 23-72 y, conventional needle EMGs and macro-EMGs were recorded one after the other. RESULTS: Macro-MUAP amplitude and macro-MUAP area were not significantly different between patients and controls. The sensitivity of the macro-EMG was 35%, that of conventional needle EMG 40%. Both methods supplemented each other. CONCLUSIONS: The sensitivity of the macro-EMG to detect involvement of the skeletal muscle in patients with mitochondriopathy is similar to that of conventional needle EMG. Macro-MUAP area was not more helpful than macro-MUAP amplitude in this respect.

Adult↗
PubMed1999

Liquorpheresis (CSF filtration) in familial amyotrophic lateral sclerosis.

OBJECTIVES: Liquorpheresis (CSF filtration) has been shown to be of benefit in various neurologic disorders, including sporadic ALS. Liquorpheresis in patients with familial ALS, has not been investigated so far. METHODS: A 52-year-old woman with familial ALS is reported who underwent liquorpheresis during 4 consecutive days. During this period, 875 ml CSF were filtered off via an intrathecal catheter and a combined mechanical and ionic filter by means of a bi-directional syringe pump. RESULTS: Immediately after treatment and 2 weeks later there was subjective, but no objective, improvement of her symptoms, assessed by the Norris score and measurements of the elbow extension, vital capacity, F-waves of both ulnar nerves and automatic EMG of the right brachial biceps and anterior tibial muscles. CONCLUSION: Liquorpheresis does not seem to be helpful in the treatment of familial ALS.

Amyotrophic Lateral Sclerosis↗
PubMed1999

Cerebrospinal fluid filtration in amyotrophic lateral sclerosis.

By means of a randomized, controlled and open study the authors wanted to find out if cerebrospinal (CSF)-filtration was of substantial benefit to patients with sporadic amyotrophic lateral sclerosis (SALS). Five SALS patients, aged 51-75 years, being treated with riluzole underwent CSF-filtration daily over five days (group A). Five other SALS patients, aged 52-70 years, were treated only with riluzole (group B). Although all five patients in the first group reported a subjective benefit following CSF- filtration, the Norris score, the Frenchay score, the vital capacity, the ulnar nerve F-wave persistence and the peak-ratio of the brachial biceps and anterior tibial muscles did not change significantly after five days of therapy, either in group A or in group B. In conclusion, filtration of 200-250 ml CSF daily, over five days, does not seem to have a substantial therapeutic effect in patients with SALS.

Aged↗
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↗