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Biomedical subjects

C Brechenmacher

Publications and source records attributed to C Brechenmacher.

At least 55 records · Page 3Linked to original sources

Variability of morphological features in early infantile polyneuropathy with defective myelination.

Four cases of early infantile polyneuropathy with defective myelination are reported. The peripheral nerve was studied by light and electron microscopy; different morphological characteristics have been noticed in these patients. Case 1 presented aspects of defective myelination with atypical "onion bulb" formation composed of multiple layers of basement membrane. In case 2, defective myelination and atypical "onion bulb" formation were associated with aberrant hypermyelination. Cases 3 and 4 were brothers, who presented axonal damage and atypical "onion bulb" formation.

Cell Count↗

[Myocardiopathies of Friedreich's disease].

Cardiac involvement in Friedreich's disease is classically a hypertrophic myocardiopathy, concentric or assymmetrical with or without dilatation. It has nothing specific in comparison to other myocardiopathies. Nevertheless, forms with a dilated myocardiopathy are also possible, but much more unfrequent. A propos of three cases, we have studied the different aspects of myocardiopathies in Friedreich's ataxia. The problem raised by the case of an hypertrophic myocardiopathy evolving toward a dilated form, unusual element of this pathology, is presented. The therapeutic potential of hypertrophic myocardiopathies is classically represented either by beta-blockers or by the more recent slow calcium inhibitors.

Adult↗

[Flecainide by the intravenous route: a new method for decreasing auricular fibrillation].

The action of intravenous flecainide was studied in patients presenting a tachyarrhythmia secondary to a permanent atrial fibrillation of recent onset. We obtained 9 improvements in 15 patients included in the protocol which consisted in the intravenous injection of 2 mg/kg of flecainide in 10 minutes. The side effects were minimal: no sign of cardia insufficiency nor alteration of the arterial blood pressure was noted. Nevertheless, although interesting, this method to decrease atrial fibrillation is not, in our opinion, destined to replace external electrical shock, except in some special cases.

Adult↗

[Embolic intracardiac thrombosis on a catheter for permanent stimulation. An echocardiographic finding].

If endovenous thromboses on permanent cardiac stimulation catheter are rather frequent, it is usual to note the absence of complications, especially embolic complications. On the contrary, intracardiac thromboses, occurring always on a permanent stimulation catheter, are rare but may generate embolisms: the authors report a new case discovered on bidimensional sonocardiography recorded following pulmonary embolisms.

Aged↗

[Agranulocytosis caused by captopril].

Captopril, an inhibitor of the conversion enzyme, is a medication with a known efficacy in the treatment of arterial hypertension and congestive cardiac insufficiency. Its side-effects are few. Among them, agranulocytosis is a severe complication, all the more severe and frequent as it occurs in patients with chronic renal insufficiency, collagen disease, or patient treated with medication having a leucopenic potential. Our case reports an agranulocytosis secondary to captopril in a patient with cardiac insufficiency presenting none of these aggravating factors. The mechanism of agranulocytosis secondary to captopril remains currently debatable, but does not seem, in the present case, to be dose related. Thus, a captopril prescription must comply with certain rules; decrease of the dosage in case of renal insufficiency, usual precautions in patients with collagene diseases. The association to a medication with leucopenic potential is to be avoided. Hematologic monitoring will be adapted to each particular case.

Aged↗

[Continuous electrocardiogram recording (Holter). Normality criteria].

The interpretation of a continuous electrocardiographic recording according to Holter's method is valid only in terms of well defined standard criteria. These criteria are beginning to become more specific, in spite of uncertainties linked especially to study protocols. Finally, if these criteria were established among healthy populations, their application to pathological situations comes up against the notion of physical condition which may modify all norms.

Arrhythmias, Cardiac↗

[Residual arterial hypertension after excision of an extra-adrenal pheochromocytoma].

Residual arterial hypertension after excision of a pheochromocytoma of the Zuckerkandl organ, has brought the problem of its etiology. The short term approach consisted in performing, after specific biochemical dosages, a scintigram with IMBG, a scan and a magnetic resonance imaging, to look for a second pheochromocytoma. A negative workup enabled to conclude to an essential arterial hypertension. Nevertheless, this does not exclude the possibility of a long term malignancy, requiring clinical monitoring and IMBG scintigraphy.

Chromaffin System↗

Relapsing inflammatory demyelinating polyneuropathy in a diabetic patient.

Inflammatory demyelinating polyradiculoneuropathies exhibit well-known ultrastructural lesions of the peripheral nerve, both in acute cases, i.e., Guillain-Barré syndrome, and in relapsing, sub-acute and chronic cases. We present a case of relapsing inflammatory demyelinating polyradiculoneuropathy in a diabetic patient with a biopsy exhibiting these lesions, as well as a widening of the outermost myelin lamellae in some fibers. Such associated lesions are classic in experimental inflammatory demyelinating polyradiculoneuropathies, but have not been reported in human pathology.

Animals↗

[Pheochromocytoma of the organ of Zuckerkandl].

The authors report the case of a pheochromocytoma of the organ of Zuckerkandl in a 46 year old man. The clinical presentation was severe paroxysmal hypertension; the tumour was located by arteriography, CT scanning, and by scintigraphy with iodine labelled benzylguanidine. The blood pressure returned to normal after surgery. The embryological, anatomical and physiological features of the organ of Zuckerkandl are reviewed: the authors discuss briefly the modern methods of diagnosis and localisation of the tumour.

Adrenal Gland Neoplasms↗

[The contribution of magnetic resonance imaging in congenital heart diseases].

The diagnostic value of magnetic resonance imaging (MRI) was assessed in 30 patients with congenital heart disease, including 7 patients with postoperative sequellae. The images obtained by synchronizing the MRI spectrometer with the electrocardiogramme were recorded in 2 or 3 different planes (sagittal, frontal and transverse) and compared to clinical, angiographic and/or echocardiographic data. The MRI provided high resolution tomographic images enabling spatial reconstitution of the heart by the use of different planes. These images were particularly useful for showing the position and dimensions of the ventricles (both chamber size and wall thickness) and their relationship to the atria and great vessels. These results confirm the value of this new non-invasive imaging technique in the diagnosis of congenital heart disease, not counting the additional information on blood flow and tissue characterisation that will soon become available.

Heart Defects, Congenital↗

Acute inflammatory demyelinating polyneuropathy in a diabetic patient: predominance of vesicular disruption in myelin sheaths.

A diabetic woman underwent an incision of the right big toe for an abscess and developed a typical Guillain-Barré syndrome 48 h later. A biopsy of a peripheral nerve, performed 10 days later, showed modifications usually seen in diabetic patients, as well as the characteristic ultrastructural modifications of the Guillain-Barré syndrome (GBS). Moreover, 22% of myelinated fibers exhibited vesicular disruption of the myelin sheaths. This lesion is rarely encountered on the biopsies of peripheral nerve in GBS and concerns only a few myelinated fibers. Such a prominence of myelinic vesicular disruption and its occurrence in a diabetic patient are discussed.

Acute Disease↗

[Tocolysis using salbutamol in cases of cardiac rhythm disorders in the mother. Apropos of 4 cases].

Tocolysis was carried out in a case of paroxysmal junctional tachycardia (Bouveret) and there was an attack of paroxysmal tachycardia which resolved spontaneously. In a case of Wolff-Parkinson-White syndrome and in two cases of ventricular double extra-systoles, tocolysis was very well tolerated. A short discussion based on these four case histories is presented. The indication for the use of tocolytic drugs was discussed with the cardiologist. The cardiac state of these pregnant women under treatment was very closely monitored.

Adult↗

[Efficacy of oral bepridil in ventricular arrhythmias in stable coronary insufficiency].

The electrophysiological properties of bepridil suggest a possible ventricular antiarrhythmic effect. This prospective multicentre trial was undertaken to study the antiarrhythmic efficacy of oral bepridil on ventricular arrhythmias in patients with stable coronary insufficiency and more than 1 000 ventricular extrasystoles per 24 hours or ventricular tachycardia. The patients were divided into 3 groups according to the arrhythmias observed and the dosage used. In group I, the dosage was 300 mg/day and 12 out of 44 patients (27%) responded favourably to two criteria of efficacy (decrease of 84% of the number of ventricular extrasystoles; suppression of complex ventricular extrasystoles). In group II, the dosage was 300 mg/day, and then increased to 500 mg in non responders. Using the same criteria of efficacy, 13 out of 19 positive results were observed (68.4%). Group III comprised all cases of ventricular tachycardia; bepridil was effective in 3 out of 6 cases, associated with amiodarone in 1 case. The results demonstrate an antiarrhythmic effect of bepridil which is modest at 300 mg/day but which becomes more marked at the dosage of 500 mg/day.

Administration, Oral↗

[Accessory heart conduction pathways].

For several decades, the existence in some hearts of accessory conduction pathways other than the principal pathway (AV node and His bundle) has been postulated. With the development of clinical electrophysiology it seemed important to try and correlate anatomical findings with electrophysiological anomalies. The results of 10 years of research in this direction are summarized here. Ten patients, most of whom died of cardiac arrhythmia, previously had intracavitary electrophysiological studies. The anatomical study required approximately 15.000 histological sections for each of the 10 hearts. During these 10 years we gradually became convinced of the existence of 3 accessory pathways, viz. Kent bundles, atrio-His bundle tracts and Mahaim fibres. In addition, correlations between the anatomical hypotheses inferred from electrophysiology and the results of this anatomical study proved excellent in most cases.

Arrhythmias, Cardiac↗

Uncompacted myelin lamellae in two cases of peripheral neuropathy.

Peripheral nerve biopsies from two patients with chronic sensorimotor neuropathy were studied. The first case was a non-Hodgkin malignant lymphoma and did not show any dysglobulinemia. The second case had a benign monoclonal gammopathy IgG, Lambda type. Direct immunofluorescence showed no deposits in the first case and slight deposits of anti IgG sera on a few myelinated fibers in the second case. There were numerous fibers showing uncompacted myelin lamellae, 7% in the first case and 4% in the second case. Some of these fibers had axons containing more tubules than filaments. The very few cases reported on neuropathies showing that uncompacted myelin lamellae were frequently associated with dysglobulinemic neuropathy. However, this ultrastructural abnormality of the myelin sheath can be observed without any dysglobulinemia.

Aged↗

Ultrastructural study of peripheral nerve in arteritic diabetic patients.

The peripheral nerve was taken from 25 diabetic patients with arteritis just before or after amputation of a leg. The ultrastructural study of myelinated fibers showed several alterations. Loss of fibers varied greatly from case to case. This was accompanied in some cases by hypertrophy of the Schwann cells around demyelinated fibers as has been observed in some ordinary diabetic neuropathies. Unusual abnormalities consisted of particular axonal degeneration and evidence of aberrant remyelination. This axonal injury was peculiar because of the accumulation of organelles which tended to transform some of the fibers into enlarged axons. Such a lesion might correspond to a hypoxic axonopathy.

Aged↗