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Biomedical subjects

J P Azulay

Publications and source records attributed to J P Azulay.

At least 55 records · Page 3Linked to original sources
PubMed1996

Erythrocyte Na(+)-K(+)-ATPase activity, metabolic control, and neuropathy in IDDM patients.

OBJECTIVE: To study the relationship among red blood cell Na(+)-K(+)-ATPase activity, metabolic control, and diabetic neuropathy. RESEARCH DESIGN AND METHODS: Na(+)-K(+)-ATPase activity has been measured in the red cell membrane of 43 long-standing IDDM patients (duration of diabetes 17.5 +/- 2 years, mean +/- SE), with 20 of the patients presenting with peripheral neuropathy. There were 23 healthy subjects serving as the control group. RESULTS: Na(+)-K(+)-ATPase activity was significantly lower in diabetic patients than in healthy subjects (236.5 +/- 7.5 vs. 294 +/- 10 nmol P1 . mg protein-1 . h-1, P < 0.05). Among diabetic patients, Na+/K(+)-ATPase activity was not dependent on the degree of diabetic control, nor was it correlated with either fasting blood glucose (r = 0.16, NS) or HbA1 (r = 0.01, NS). Na(+)-K(+)-ATPase activity was lower in patients with neuropathy than in those without it (212 +/- 8.5 vs. 261 +/- 6.6, P < 0.05). Furthermore, in a subgroup of 20 patients, a positive correlation was observed between erythrocyte Na(+)-K(+)-ATPase activity and nerve conduction velocity in the peroneal (r = 0.558, P < 0.02) and tibial nerve (r = 0.528, P < 0.05). CONCLUSIONS: These results suggest that diabetes-induced Na(+)-K(+)-ATPase activity dysfunction could be implicated in the pathogenesis of human diabetic neuropathy and the electrophysiological abnormalities observed in these patients.

Adult↗
PubMed1996

[Hiramaya disease: disease or syndrome?].

Hirayama's disease, a benign juvenile amyotrophy of "oblique" topography of an upper limb, has been acknowledged in the international literature. 4 cases are reported in this paper. However there present some differences: presence of antigangliosides antibodies in 1 case, cryoglobulinemia in another case developing bilaterally, conduction blocks in a third case. Finally, in the patient illustrating the typical form, the neuroradiological abnormalities considered as characteristic of cervical spine compression by Hirayama are lacking.

Adolescent↗
PubMed1996

[Automatic motion analysis of gait in patients with Parkinson disease: effects of levodopa and visual stimulations].

Gait analysis of 13 patients affected by Parkinson's disease (PD) and 7 healthy elderly volunteers was performed with a fully automatic motion analyser. The recording included stride parameters for walking velocity, stride length, stride duration and swing time. Maximal amplitudes of rotation of the hip, knee and ankle were also recorded. The analysis was performed for the PD's patients before and after L-Dopa intake. All the patients walked with and without transversal stripes on the floor. The contrasting white lines were 45 cm apart. After medication, the stride length, the velocity and the swing phase duration were significantly increased. The movements around the hip, knee and ankle joints that were initially reduced, poorly increased after L-Dopa intake. When stripes were placed on the floor, no significant changes occurred for the overall group of patients. Seven parkinsonian patients did improve with visual guidance, increasing their stride length and their speed. Some rotations of hip and knee were also influenced by stripes. This sub-group of patients was characterized by older age, a slower walking velocity, a smaller stride length and a shorter swing time. We correlated the sensitivity to visual cues to the defect of the visual contrast sensitivity that has been established in PD by several recent works. Locomotion on the transverse lines might have produced a motion perception at a rate determined by the speed of the patient. For appropriate intervals between stripes, one can hypothesise that the resulting visual stimuli belonged to a range of spatio-temporal frequencies that are decreased in patients with PD.

Aged↗
PubMed1996

[Multifocal motor neuropathies with persistent conduction blocks: a form of inflammatory demyelinating neuropathy].

Since the first cases described in 1986, multifocal motor neuropathy with persistent conduction blocks (MMN) appears to be a well-defined nosological entity. Clinical features include male-predominant occurrence, weakness often accompanied by cramps and fasciculations; topography of the motor involvement is characterized by assymetry, upper limb predominance, relation to the distribution of individual nerves or roots. Atrophy is variable but can lack despite severe weakness. Multifocal, purely motor and persistent conduction blocks are the electrophysiological hallmark of the disease. Serum antibodies against ganglioside GM1 are frequently associated with MMN but their diagnostic sensitibity and specificity are discussed. However high level of these antibodies may be a useful immunological marker of MMN. Their pathogenic role is probable, perhaps by their interaction with ionic channels localized in node of Ranvier. Morphological findings, immunologic abnormalities and improvement with intravenous immunoglobulin therapy define MMN as a type of inflammatory demyelinating neuropathy. Their relation with CIDP are discussed.

Adult↗
PubMed1996

[Treatment of multifocal motor neuropathies].

Multifocal motor neuropathy is characterized by a progressive asymetrical weakness, predominantly affecting the upper limbs with persistent conduction blocks on motor but not sensory nerves. Treatment woth prednisone and plasma exchanges have failed to demonstrate any positive effects. Some improvements have been reported with cyclophosphamide. Mainly immunoglobulin therapy has been evaluated with a beneficial response in almost 70% of the cases. These benefits obtained over periods of less than six months have recently been confirmed by a long-term evaluation of 18 patients treated by repeated infusions.

Adrenal Cortex Hormones↗
PubMed1995

Palmaris brevis spasm syndrome.

Palmaris brevis spasm syndrome is a rare and benign condition of localised muscular hyperactivity. In five men, the hypothenar eminence underwent spontaneous, irregular, tonic contractions of the palmaris brevis muscle. An EMG showed spontaneous high frequency discharges of normal motor units, without evidence of neuropathy or of nerve compression. This syndrome resembles other restricted muscle hyperactivity syndromes although there are some differences. Curiously, the palmaris brevis muscle is not under voluntary control. The mechanism of the syndrome could be an ephaptic transmission possibly secondary to the transient and repeated stretching of the ulnar nerve superficial branch. In one patient a root compression was the probable origin.

Adult↗
PubMed1994

Contrast sensitivity improvement with sulfamethoxazole and trimethoprim in a patient with Machado-Joseph disease without spasticity.

A double-blind, placebo controlled, cross-over trial of sulfamethoxazole and trimethoprim was performed in a 62-year-old male patient who suffered from Machado-Joseph disease for 25 years. The patient, with cerebellar ataxia, akinetic-rigid syndrome and motor weakness but without any pyramidal features, had been chair-bound for 3 years before the trial. Bactrim therapy markedly improved performance on a physical examination which tested standing and gait, as compared to placebo session. Walker-assisted gait was possible again. For the first time, evaluation of spatio-temporal contrast sensitivity was performed and also revealed an improvement after Bactrim therapy as compared to placebo. These results suggest that Bactrim may be effective in degenerative neurological diseases and that Bactrim may have an overall effect on neurotransmission rather than solely possessing antispastic properties.

Analysis of Variance↗
PubMed1994

Mitochondrial respiratory failure in skeletal muscle from patients with Parkinson's disease and multiple system atrophy.

We studied mitochondrial respiratory chain function in skeletal muscle taken from 27 patients with idiopathic Parkinson's disease (PD; 21 Dopa-treated PD patients and 6 de novo patients), 5 patients with multiple system atrophy (MSA) and from 43 age-matched controls in order to determine the occurrence of mitochondrial respiratory chain abnormalities in parkinsonian syndromes. In our control subjects, we found a significant age-related decrease in the activity of respiratory chain complex I. As compared to carefully age-matched control subjects, activity of complex (NADH:ubiquinone reductase) was significantly lower in muscle mitochondria from patients with PD and MSA and a mean remaining activity < 30% of controls was observed. Mean activities of complexes III (ubiquinol:cytochrome c reductase) and IV (cytochrome c oxidase) were also lower in PD patients than controls, but a low activity (remaining activity < 30% of controls) was observed in only 5 PD patients for complex I and III or I and IV. No deficit in complex II activity (succinate:ubiquinone reductase) was observed. Our results support the hypothesis of a wide-spread mitochondrial complex I deficiency in PD and MSA as compared to age-matched controls, who showed age-related deficiency. This deficit can be found in de novo PD patients as well as in treated patients. The observed respiratory enzyme chain deficiency could not be explained by the dose and duration of L-Dopa or dopaminergic agonist treatment, the severity of the disease, anxiety or depression since no significant correlation was found between these parameters and enzyme complexes activities.

Adult↗
PubMed1994

A single report of hemiplegic arm stretching related to yawning: further investigation using apomorphine administration.

We observed a stroke patient with an infarct of the internal capsule interrupting the pyramidal tract who stretched his hemiplegic arm during spontaneous and apomorphine-induced yawning. The putative mechanism by which yawning can induce the paradoxical motor response of the plegic arm in the patient might be the functional efficiency of a pathway projecting directly from the stimulated basal ganglia to lower motor systems in the brainstem.

Apomorphine↗
PubMed1994

Intravenous immunoglobulin treatment in patients with motor neuron syndromes associated with anti-GM1 antibodies: a double-blind, placebo-controlled study.

We studied the effects of intravenous immunoglobulin (IVIg) in 12 patients with motor neuron syndromes associated with high titers of anti-GM1 antibodies. Five of the patients had conduction blocks. The study design was a double-blind, placebo-controlled, crossover trial with IVIg (0.4 g/kg body weight per day injected for 5 consecutive days). We evaluated the patients before and 5, 28, and 56 days after drug administration using a computerized analyzer for muscle strength, the Norris scale for disability, motor nerve conduction velocities for patients with conduction blocks, and measurements of immunologic markers. Compared with placebo, IVIg induced a significant increase in muscle strength only in the patients with conduction blocks.

Adult↗
PubMed1994

[Spinal dural fistula with peri-medullar venous drainage].

Clinical and neuroradiological findings of 8 patients with a spinal dural arteriovenous fistula are reviewed. Disturbance of micturition or defecation and weakness of the legs were always present and the most frequent initial symptom was a progressive spastic paraparesis. Duration of symptoms before diagnosis was 2 years. Lumbar puncture showed elevation of proteins and myelography demonstrated dilated perimedullar posterior veins. In every case, magnetic resonance imaging of the spinal cord (T2- weighted images) revealed intramedullary high signal intensity of the conus medullaris and selective angiography confirmed the site of the dural fistula. Each patient was treated with endovascular method consisting in liquid adhesive embolization (0.2 cc of N-butyl cyanoacrylate) with hyperselective catheterism of the dorsospinal artery. Embolization procedure was successful in 6 cases with large improvement of leg weakness and partial regression of disturbed micturition and defecation. The pathophysiological mechanisms explaining the clinical signs are discussed.

Aged↗
PubMed1994

[What is left of Morvan's fibrillary chorea?].

In 1890, Morvan described a syndrome of myokimia associated with muscle pain, excessive sweating and sleep disorders. The course was severe and the patient died five weeks after the onset. Several cases were published after this first report in the French literature. The last for 10 years the disease seems to have disappeared. In fact, it seems that the progress in electromyography introduced clear definitions of spontaneous muscle discharges (myokimia, neuromyotonic discharges, fibrillations, fasciculations) allowing recognition of a disease characterized by a syndrome of spontaneous and continuous muscle fiber activity associated with cramping, slow relaxation (pseudomyotonia), excessive sweating and stiffness. Different terms have been used to describe this entity: neuromyotonia (Mertens and Zschocke, 1965) or continuous muscle fibre activity (Isaacs, 1961). Classification of these syndromes has to distinguish inherited from acquired forms, idiopathic cases from cases where neuromyotonia is associated with a peripheral neuropathy, a cancer or an immunological disease. Moreover, the frequent occurrence of this later association and recent reports of improvement after plasma exchanges suggest a possible autoimmune aetiology for this group. Finally, we suggest that the term of "Maladie de Morvan" must be used instead of chorée fibrillaire but only concerning patients who exhibit central disorders including insomnia, hallucinations and altered behaviour.

Acrodynia↗
PubMed1994

[Isolated proximal muscular weakness disclosing myasthenic syndrome].

Three cases of chronic limb-girdle myasthenia gravis (one man and two women) are reported. The duration of the disease was three, five and 20 years respectively. None had oculobulbar weakness during this period nor fluctuating symptoms. The primary diagnosis was myopathy in two cases and myelopathy in the other one. In view of this diagnosis, several features were atypical: absence of marked wasting, preservation of tendon reflexes and normal serum creatine kinase activity. Finally, diagnosis of myasthenia gravis was established by the occurrence of a characteristic decremental muscular response to repetitive stimulation and by positive edrophonium test. It seems that the rate of positivity of acetylcholine receptor antibody is lower than in the common form of the disease. Two patients responded to acetylcholinesterase inhibitors and one to high-doses intravenous human immunoglobulins. These and previous cases described in the literature raise the problem of the definition of an unusual form of myasthenia gravis or of a new type of neuromuscular disease characterized by the presence of distinctive changes on muscular biopsies, specially tubular aggregates as in one of our cases. However, this unusual form of chronic limb-girdle weakness has to be recognized because treatments are effective in most cases.

Adult↗