Biomedical subjects

V Bril

Publications and source records attributed to V Bril.

8 recordsLinked to original sources

PubMed1991

Endoneurial microvessels in human diabetic neuropathy. Endothelial cell dysjunction and lack of treatment effect by aldose reductase inhibitor.

Endoneurial microvascular abnormalities have been invoked in the pathogenesis of diabetic distal symmetric polyneuropathy. Detailed morphometric analysis of the endoneurial microvasculature was correlated with previously published data on nerve fiber morphometry and teased fiber analysis obtained from the same sural nerve biopsies. Biopsy specimens from neuropathic diabetic patients were obtained before and after 12 mo of aldose reductase inhibitor (ARI) treatment and compared to 15 carefully age-matched control subjects. Diabetic microvessels showed basement membrane thickening and loss of endothelial cell tight junctions. Microvascular density and the frequency of microvessels closed by endothelial cells increased with age in diabetic and control nerves and were unaffected by diabetes. The density of microvessels showing patent lumina did not differ between control and diabetic subjects and was not related to age or diabetes. Closed microvessels were composed of postcapillary venules that were otherwise devoid of ultrastructural abnormalities. We suggest that microvascular closure by endothelial cells may be a physiological condition and is unlikely to have any pathogenetic significance in diabetic neuropathy. Based on the current limited biopsy material, we conclude that 12 mo of ARI treatment that induced significant fiber repair and regeneration had no detectable effect on endoneurial microvascular abnormalities. These data suggest that endoneurial vascular pathology is not a rate-limiting factor in fiber damage or repair at this stage of diabetic neuropathy.

Adult↗

PubMed1988

Regeneration and repair of myelinated fibers in sural-nerve biopsy specimens from patients with diabetic neuropathy treated with sorbinil.

There is reason to believe that diabetic neuropathy may be related to the accumulation of sorbitol in nerve tissue through an aldose reductase pathway from glucose. Short-term treatment with aldose reductase inhibitors improves nerve conduction in subjects with diabetes, but the effects of long-term treatment on the neuropathologic changes of diabetic neuropathy are unknown. To determine whether more prolonged aldose reductase inhibition reverses the underlying lesions that accompany symptomatic diabetic peripheral polyneuropathy, we performed a randomized, placebo-controlled, double-blind trial of the investigational aldose reductase inhibitor sorbinil (250 mg per day). Sural-nerve biopsy specimens obtained at base line and after one year from 16 diabetic patients with neuropathy were analyzed morphometrically in detail and compared with selected electrophysiologic and clinical indexes. In contrast to patients who received placebo, the 10 sorbinil-treated patients had a decrease of 41.8 +/- 8.0 percent in nerve sorbitol content (P less than 0.01) and a 3.8-fold increase in the percentage of regenerating myelinated nerve fibers (P less than 0.001), reflected by a 33 percent increase in the number of myelinated fibers per unit of cross-sectional area of nerve (P = 0.04). They also had quantitative improvement in terms of the degree of paranodal demyelination, segmental demyelination, and myelin wrinkling. The increase in the number of fibers was accompanied by electrophysiologic and clinical evidence of improved nerve function. We conclude that sorbinil, as a metabolic intervention targeted against a specific biochemical consequence of hyperglycemia, can improve the neuropathologic lesions of diabetic neuropathy.

Action Potentials↗

PubMed1988

Histopathological heterogeneity of neuropathy in insulin-dependent and non-insulin-dependent diabetes, and demonstration of axo-glial dysjunction in human diabetic neuropathy.

Altered sorbitol and myo-inositol metabolism, (Na,K)-ATPase function, electrochemical sodium gradients, axonal swelling, and distortion and disruption of the node of Ranvier ("axo-glial dysjunction") directly implicate hyperglycemia in the pathogenesis of neuropathy in diabetic rats, but the relevance of this sequence to clinical neuropathy in heterogeneous groups of diabetic patients remains to be established. Fascicular sural nerve morphometry in 11 patients with neuropathy complicating insulin-dependent diabetes revealed a pattern of interrelated structural changes strikingly similar to that of the diabetic rat when compared to age-matched controls. 17 older non-insulin-dependent diabetic patients with comparable duration and severity of hyperglycemia and severity of neuropathy, displayed similar nerve fiber loss, paranodal demyelination, paranodal remyelination and segmental demyelination compared to age-matched controls, but axo-glial dysjunction was replaced by Wallerian degeneration as the primary manifestation of fiber damage, and fiber loss occurred in a spatial pattern consistent with an ischemic component. The mechanistic model developed from the diabetic rat does indeed appear to apply to human diabetic neuropathy, but superimposed hormonal, metabolic, vascular, and/or age-related effects alter the morphologic expression of the neuropathy in non-insulin dependent diabetes.

Age Factors↗

PubMed1984

Oculoskeletal myopathy with abnormal mitochondria.

A clinical, electrophysiological and pathological review of 14 patients having oculoskeletal myopathy with abnormal mitochondria was undertaken. These patients present with ophthalmoplegia, and mild skeletal muscle weakness. The clinical course is slowly progressive. Electromyographic examination shows myopathic changes. Serum enzymes are normal. The diagnosis is confirmed by skeletal muscle biopsy which shows abnormal mitochondria, including crystalloid inclusions on electron microscopy. These patients form a distinct clinical group in which the risk of sudden cardiac death is much less than it is in the Kearns-Sayre syndrome.

Adult↗

PubMed1984

Number of potential reversals (turns) and amplitude of the pattern of electrical activity of the abductor pollicis brevis muscle in patients with neurogenic diseases.

We have quantified the pattern of electrical muscle activity at a force of 0.3 kg, 30% of maximum force, and maximum force in the abductor pollicis brevis muscle of 21 patients with neurogenic disorders (10 with carpal tunnel syndrome, 9 with polyneuropathy and 2 with motor neurone disease) and of 15 normal subjects. In normal subjects, the number of turns, the mean amplitude and the ratio between them were independent of maximum force, age and sex of the subjects. In 21 patients with neurogenic disorders, the cumulative diagnostic yield of the analysis of the pattern of electrical activity was 86% compared to the diagnostic yield of the mean duration and mean amplitude of individual motor unit potentials of 90%. These diagnostic yields in a small distal muscle were of the same order as those of the brachial biceps muscle in previous studies.

Adolescent↗

PubMed1983

Automatic analysis of the EMG interference pattern.

A method is described for the automatic analysis of EMG interference patterns obtained at optional non-controlled force levels. It is a modification of Willison's method of measuring turns and amplitude of the signal. In the method, the mean amplitude is plotted against turns obtained during 300 msec for each of 20 recording sites. Different voluntary strengths are used and a scatter of points is obtained. Confidence limits enclosing these points are constructed for healthy subjects for 4 different muscles with concentric and monopolar electrodes. The normal values differ with age, muscle, sex and electrode. The method is tested on patients with myopathic or neurogenic EMG and a high diagnostic yield is obtained. The method is objective, reproducible, fast and needs relatively small computer facilities.

Adolescent↗

PubMed1979

Midbrain asterixis.

Asterixis is usually a manifestation of metabolic encephalopathy. It was the only skeletal motor sign in a patient with ophthalmoplegia caused by midbrain infarction; no metabolic abnormality was present. The asterixis was accompanied by signs of damage to the mesencephalic reticular formation. We propose that episodic lapses of postural control by the reticular formation are responsible for midbrain asterixis and suggest that this asterixis is a segmental form of drop attack.

Aged↗

PubMedDate not supplied

Cerebral infarction associated with lupus anticoagulants--preliminary report.

Hypercoagulability may contribute to stroke in young adults. Lupus anticoagulants (LA) were identified in six patients (4%) of 145 young adults with cerebral infarction. The clinical features of the 6 patients in this survey plus an additional patient from another institution with LA-associated stroke are presented. Four had systemic lupus erythematosus and 3 had idiopathic LA; all had mild thrombocytopenia. In 2 patients, no other conditions associated with stroke were discovered after thorough evaluation. Recurrent arterial thrombosis occurred in 4 of 7 patients during an average of two years of follow-up. Evidence suggests that inhibition of prostacyclin formation may occur with LA, promoting a prothrombotic state.

Adolescent↗