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G Serratrice

Publications and source records attributed to G Serratrice.

At least 19 recordsLinked to original sources
PubMed1992

[Polyneuritis, polyradiculoneuritis, polyneuropathies: development of a concept].

The concept of polyneuritis, polyradiculoneuritis and polyneuropathy has changed considerably since the last century. Charcot-Marie-Tooth disease has been dismembered into hypertrophic, neuronal and spinal forms. Within the group of hereditary sensory neuropathies, to Thévenard's ulcero-multilating acropathy have been added recessive forms of early onset and various types of congenital analgesia. Other hereditary polyneuropathies result from inborn errors of metabolism; in adults, these are chiefly Refsum disease, Fabry's disease, porphyria, amyloidosis and adrenoleucodystrophy. Guillain-Barré acute primary polyradiculoneuritis has come to be associated with chronic and recurrent forms and, more recently, with multifocal demyelinating neuropathy with persistent conduction blocks and antibodies to GM1. Young and Adams acute pandysautonomia is close to polyradiculoneuritis. Finally, many polyneuropathies, formerly labelled polyneuritis, are subsequent to an ever increasing number of known causes, notably infections (e.g. borreliosis or HTLV viruses) and drug-induced or industrial toxicity. Advances in explatory techniques have generated new concepts, including small and large fibre neuropathy, distal and central degenerations, myelin diseases and neuronal diseases.

Adult↗
PubMed1992

A double-blind placebo-controlled trial of L-threonine in amyotrophic lateral sclerosis.

Fifteen patients with the unequivocal diagnosis of amyotrophic lateral sclerosis (ALS) completed a 1-year randomized double-blind placebo-controlled trial of L-threonine (2 g daily). During the study, patients in the placebo group showed a decline in functional status consistent with the natural history of ALS, which was not statistically different from outcome in the patients in the L-threonine group.

Aged↗
PubMed1992

Nociceptive threshold in patients with epilepsy.

Clinical practitioners have often observed in the course of their daily work that the pain thresholds of epileptic patients seem to differ from those of healthy subjects. These patients can suffer from quite severe traumatic lesions without apparently experiencing any pain. Since they are usually under treatment for epilepsy, it is difficult to determine whether the absence of pain is due to these patients' epileptic condition or to its treatment, since most antiepileptic drugs also have analgesic effects. In the present study, it was proposed to assess the pain thresholds of 15 epileptic patients (10 with tonic-clonic seizures generalized at outset and 5 with temporal lobe epilepsy), by measuring the leg flexion nociceptive reflex (or RIII reflex) threshold: the stimulation threshold at which this reflex is triggered is known to be correlated with the pain threshold. The nociceptive threshold of the patients with generalized epilepsy was not found to differ from that of the control population, whereas that of the patients with temporal lobe epilepsy was spontaneously high and was not reversed upon injecting naloxone. These data are discussed from the point of view of the pain pathways and mechanisms possibly involved.

Adolescent↗
PubMed1992

Identifying the afferents involved in movement-induced pain alleviation in man.

It has been clearly established that the perception of nociceptive stimulus decreases in intensity when movement is initiated in the part of the body to which the stimulus is applied. The pain alleviation is probably at least partly due to the activation of afferents which occurs during the movement. The present experiments were carried out with a view to investigating which groups of afferent fibres is mainly responsible for the gating of the nociceptive messages which occurs during movements. In eight volunteers we investigated the changes in the amplitude of the nociceptive leg flexion reflex (RIII) when the subjects were at rest, when they were performing active or passive ankle movements and when the spindle proprioceptive pathway was mobilized by applying vibratory stimulation to the Achilles tendon. Similar experiments were also carried out with eight other volunteers after anaesthetizing the ankle skin mechanoreceptors. This method was chosen because a clear-cut correlation is known to exist between the amplitude of the nociceptive motor reflex and the intensity of the pain perceived by the subject. The data obtained clearly show that anaesthesia of cutaneous mechanoreceptors connected to the large diameter afferent fibres prevented the decrease in the motor response which otherwise accompanied both active and passive movements. Activation of the Ia fibre group by tendon vibration resulted on the contrary in an increase in the amplitude of the motor response. Movement-induced pain alleviation therefore does not mainly involve the activation of the Ia group of fibres.

Adult↗
PubMed1992

Analgesic effect of indomethacin shown using the nociceptive flexion reflex in humans.

This study investigated whether indomethacin has an analgesic effect on the central nervous system. As analgesics which affect the central nervous system produce a correlated decrease in the subjective sensation of pain and in the nociceptive reflex in humans, the amplitude of the nociceptive flexion of the biceps femoris was studied. Eight patients (six men, two women) aged 35-70 years (mean 51) with rheumatic diseases were included in the study. Each patient was his or her own control and was given a single intramuscular injection of either 50 mg of indomethacin or a placebo. A placebo controlled, double blind experimental design was used. Patients were evaluated before and 30, 60, and 75 minutes after the injection. Seventy five minutes after injection, indomethacin gave a 54% decrease in the amplitude of the nociceptive reflex, whereas the placebo produced a decrease of only 12%. This suggests that indomethacin exerts a depressive effect on the amplitude of the nociceptive reflex and affects the central nervous system as part of its analgesic action.

Adult↗
PubMed1992

Contrast sensitivity is increased in a case of nonparkinsonian freezing gait.

We measured spatiotemporal contrast sensitivity and gait variables in a 60-year-old man who had spontaneous episodes of freezing gait without any sign of rigidity or tremor. One major factor triggering freezing was the characteristics of visual space, as freezing episodes occurred during spontaneous walking and passing through apertures and never during walking with eyes closed. Initiation and maintenance of locomotion were greatly facilitated when transverse stripes with optimal intervals were placed on the ground in front of the patient. On the other hand, contrast sensitivity to low-to-intermediate spatiotemporal frequencies was increased in this patient when compared with that of elderly controls. These data suggest that hypersensitivity to visual stimulation and to modifications of the visual environment, associated with abnormal ocular motor behavior, can be one factor leading to the freezing gait phenomenon, which could be called "hypersensitivity braking" and be considered as a sensorimotor disorder.

Aged↗
PubMed1992

31P NMR spectroscopy and ergometer exercise test as evidence for muscle oxidative performance improvement with coenzyme Q in mitochondrial myopathies.

Two patients with mitochondrial encephalomyopathy due to complexes I and IV deficiencies received 150 mg/d of coenzyme Q10 (CoQ). We studied them with a bicycle ergometer exercise test and 31P NMR spectroscopy before and after 10 months of treatment. Before treatment, we observed a low phosphocreatine/inorganic phosphate (PCr/P(i)) resting value along with abnormally high resting lactate concentration. During exercise, there was a pronounced acidosis with delayed kinetics of postexercise recovery for blood lactate, pH, PCr, and PCr/P(i) ratio. Oxygen uptake during exercise was reduced while the lowering of the ventilatory threshold indicated an early activation of glycolysis. After treatment, the bicycle ergometer exercise test indicated a significant improvement with a decrease in resting blood lactate level, an increase in oxygen consumption during exercise, and an increase in the kinetics of lactate disappearance during the recovery period. A shift of the ventilatory threshold to higher workload was present. 31P NMR spectroscopy confirmed the improvement, showing a significant increase in the PCr/P(i) ratio at rest and in the kinetics of recovery for pH, PCr, and PCr/P(i) ratio following exercise in patient 1. For patient 2, we observed a less pronounced acidosis correlated with a lesser amount of Pi produced during exercise. These observations indicate an improvement of mitochondrial function and a shift from high to low glycolytic activity in both patients consequent to CoQ treatment.

Adolescent↗
PubMed1992

[Nociceptive threshold and Parkinson disease].

It has now become possible to measure pain thresholds in man by the threshold or amplitude of the leg flexion nociceptive reflex (RIII reflex). These parameters accurately reflect the pain levels perceived by the patients. The aim of the present study was to assess pain thresholds in Parkinsonian patients, using the RIII reflex. Painful phenomena are often mentioned in Parkinson disease, but the perceived pain threshold level can be difficult to assess because of the depressive symptoms which are often associated with Parkinson disease. In 8 cases out of 10, the pain threshold was found to be higher in patients with Parkinson disease than in a control population of the same age: in 2 cases, naloxone injection led to recovery of the normal pain threshold level.

Aged↗
PubMed1992

[Polysaccharide amylopectin-type storage myopathy].

We report a late onset form of polysaccharide myopathy with progressive limb girdle muscles weakness, without cardiomyopathy. Muscle biopsy showed a vacuolar myopathy in type 1 fibres. The PAS positive diastase resistant deposits were made of filamentous material at electron microscopy similar to long chain glycogen. Muscle glycogen levels and glycogen metabolism enzymes were normal. Numerous abnormal mitochondrial with paracrystalline inclusions were observed around the storage material. Twelve patients with polysaccharide amylopectin-like storage myopathy have previously been reported. This disease must be distinguished from other diseases with polysaccharide accumulation such as branching enzyme deficiency and some cases of phosphofructokinase deficiency. In other disorders, no deficient enzymes in the glycogen pathway was found. Some of them show systemic storage (Lafora disease, adult polyglucosan body disease). Corpora amylacea, Bielchowsky bodies and basophilic degeneration of the myocardium represent localised depositions. A few inclusions can also be observed in hypothyroid myopathy. In polysaccharide myopathy allosteric inactivation of phosphofructokinase by a mitochondrial dysfunction is considered by analogy with cases of polysaccharide storage related to phosphofructokinase deficiency.

Aged↗
PubMed1992

[Chronic polyradiculoneuritis. 25 cases].

Twenty-five patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) were studied in order to define the clinical, biological, electrophysiological and pathological features of this disease. There were 11 men and 14 women ranging in age from 15 to 82 years. The average follow-up was 43 months. Patients fulfilled the criteria laid down by Dyck et al. (1975), except that progression of weakness was at least 2 months and not 6. Fourteen patients had a progressive course and 11 a relapsing one. Weakness was almost constant (24/25), sensory impairment was present in 22/25 with deep sensation predominantly impaired. Areflexia was observed in all patients. A history of previous infection or other possible precipitating event was given by 7 patients. Cerebrospinal fluid examination showed a raised total protein count in 22 cases. Electrophysiological examination revealed a slowing down of nerve conduction velocities to an extent compatible with a demyelinating process in 23 cases; 2 patients only had prolongation of F-waves. Sural nerve biopsy was less informative: inflammatory process--the most specific finding--was observed in only 3 out of 20 biopsies. Six patients had benign forms or spontaneous remission, the others were treated with corticosteroids or with immunosuppressive drugs. Most patients (64%) recovered very well. Only one died during the time of study. Eight other patients were treated with high-dose intravenous immunoglobulins and 4 of them improved with administrations at regular intervals to maintain the benefits observed. The occurrence of CIDP in association with other conditions is reviewed and we discuss its nosological position among the acquired demyelinating neuropathies.

Adolescent↗
PubMed1992

[Inclusion body myositis and neuromuscular diseases with rimmed vacuoles].

A retrospective study of 40 patients with various neuromuscular disorders and more than 3 muscle fibers with rimmed vacuoles has been performed. Two subgroups of patients were distinguished according to the presence or absence of inflammatory exudates. In the first group (14 patients), inflammatory exudates were observed and numerous fibers showed partial invasion. Abnormal filamentous inclusions (16-18 nm in diameter) were found by electron microscopy in muscle fibers cytoplasm and/or nuclei. The diagnosis of inclusion body myositis (IBM) was made in these cases. They presented with insidious proximal muscle weakness and were not improved by immunosuppressive therapy. Immunohistological studies demonstrated T lymphocytes predominance, only few natural killer and B lymphocytes. The number of T8 lymphocytes was high in endomysial sites while T4 were more numerous in perivascular exudates. Abnormal membranous expression of class I MHC antigens was observed on muscle fibers lying near the inflammatory exudates. In the second group of cases (26 patients), no inflammatory exudate was observed. This group of neuromuscular diseases with rimmed vacuoles was heterogeneous. In 10 cases, abnormal filamentous inclusions (16-18 nm in diameter) were observed in rimmed vacuoles. However, this ultrastructural feature did not help in distinguishing subgroups. Various neuromuscular disorders were observed in this group: oculopharyngeal muscular dystrophy (12 cases with IBM like filaments in 4 cases), chronic spinal atrophy (5 cases with IBM like filaments in 3 cases), post poliomyelitis syndrome (2 cases with IBM-like filaments in one), muscle glycogenosis with IBM like filaments (2 cases), hereditary limb girdle myopathy or distal myopathy (3 cases) and 1 patient clinically presenting with polymyositis and another with cramps and myalgias. No abnormal sarcolemmal expression of class I MHC was found in this group. The pathogenesis of IBM is discussed. Besides T cell mediated cytotoxicity, denervation may be involved. The nature of the abnormal 16-18 nm filamentous inclusions remains unknown. These filaments are not IBM specific.

Adult↗
PubMed1992

[Axonal neuropathy and salazosulfapyridine: slow-acetylator phenotype].

We report a case of an axonal sensorimotor neuropathy involving salazosulphapyridine in a slow-acetylator patient with ulcerative colitis. Rather than hypersensitivity the mechanism of the neuropathy can be assumed to be toxicity. The role played by the respective different metabolites in the occurrence of this uncommon side effect is uncertain.

Acetylation↗
PubMed1992

[Congenital indifference and congenital insensitivity to pain].

Congenital indifference to pain is often mistaken for congenital insensitivity. It is characterized by the occurrence since childhood of lesions, mainly cutaneous and osteoarticular secondary to strictly painless traumas. However, despite the lack of pain, the patient is able to discriminate a painful stimulus. Autopsy shows no abnormality of the nervous system. A dysfunction of the central endomorphinic systems has been suggested. Congenital analgesia is associated with anhidrosis in Swanson's syndrome (in which Lissauer the tractus is absent in the spinal cord) and with dysautonomia in Riley-Days's disease (in which there is a lack of amyelicinic fibres). On account of these data, some authors refuse the autonomy of congenital indifference and classify it in the group of the various autonomic and sensory neuropathies. However it seems justified to acknowledge the congenital analgesia with two varieties: congenital indifference in which there is no sensation of pain but normal sensory pathway and tonic function of endomorphinic system, congenital insensitivity in which the painful stimulus is not transmitted to the central nervous system.

Humans↗
PubMed1992

[Brucella osteomyelitis of the upper end of the humerus: contribution of magnetic resonance imaging].

The authors report a case of brucella osteomyelitis of the upper end of the humerus in a 77-year-old patient, presenting as a large swelling of the soft tissues of the shoulder, present for a year. The diagnosis was made by isolation of the organism and specific serology. The authors stress the value of magnetic resonance imaging, which provided confirmation of the diagnosis of osteomyelitis as well as an accurate topographic assessment.

Aged↗
PubMed1992

[Inflammatory myalgic syndrome and muscular mitochondrial abnormalities: 4 cases].

Histologic and biochemical anomalies of muscle mitochondria were identified in four patients with predominantly rhizomelic myalgia clinically suggestive of an inflammatory disease but inconsistent biologic evidence of inflammation. This clinical pattern was initially suggestive of atypical polymyalgia rheumatica and could not be ascribed to any other disease. To explain this combination of anomalies, several hypotheses can be put forward, including coincidence, aging, and nonspecific mitochondrial anomalies resulting from immunologic or inflammatory disease. The speculation that these patients have an autonomous syndrome cannot be outruled but should be considered with caution. A therapeutic trial with coenzyme Q is under way.

Aged↗
PubMed1992

[Post-poliomyelitis syndrome: 29 cases].

The post-polio syndrome refers to new neuromuscular symptoms developed by some patients many years after recovery from acute poliomyelitis. Several groups were separated: musculo-skeletal symptoms (different from a new spinal cord disease), infraclinical signs (EMG), post-polio muscular atrophy (new lower motor neuron objective signs) with several subgroups: cramps and fasciculations, benign focal weakness and atrophy (in previously affected muscles or in unaffected muscles), progressive spinal muscular atrophy. The following examination were performed in some cases, but not all, in this retrospective study: muscle CT scan, conventional electromyography (EMG), quantifying-EMG, macro-EMG and single-fiber EMG. The serum titers of neutralising antibodies to polio virus type 1, type 2 and type 3 were negative. No oligoclonal bands were found in the CSF from 6 patients screened by electrophoresis immunoelectrophoresis. Serum creatine kinase or aldolase was high in 6 patients. The same unusual features in this syndrome were observed on muscle biopsies: muscular hypertrophy and interstitial eosinophils; two patients had rimmed vacuoles in the muscle fibers.

Adult↗
PubMed1992

[Rademaker and Garcin syndrome associated with pallidal calcifications].

We report gait abnormalities with exaggerated support against gravity in a 76 year-old woman. This exaggeration, and its demonstration by ankle dorsiflexion in supine position, was first described by Rademaker and Garcin (1933) as probably resulting from cerebellar and brainstem lesions. In the present case, quantitative analysis of locomotion showed a strong reduction in both stride duration and stride length. There was an increased variability between two consecutive locomotor cycles and a large increase in both stance duration and relative double-support duration. Electromyography clearly showed leg hypertension following ankle dorsiflexion, contrasting with normal leg mobility in the supine patient. This suggested that reflexive antigravity support abnormalities could explain this pattern of locomotor disabilities, differentiating them from other kinds of "marche à petits pas". The present case was associated with bilateral pallidal calcifications, suggesting an involvement of the basal ganglia in support reaction against gravity and in control of adequate postural muscle tone necessary for locomotion.

Aged↗