[Atypical form of Miller Fisher syndrome].
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Ataxia↗
Biomedical subjects
J P Azulay
Publications and source records attributed to J P Azulay.
Nervous system, muscles and systemic diseases--11th International Meeting on Neuromuscular Diseases, in Marseille, France, September 12-14, 1992.
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Humans↗
Emery-Dreifuss muscular dystrophy: linkage to markers in distal Xq28.
Emery-Dreifuss muscular dystrophy (EMD) is characterised by (1) early contractures of the Achilles tendons, elbows, and postcervical muscles, (2) slowly progressive muscle wasting and weakness with a predominantly humeroperoneal distribution in the early stages, and (3) cardiomyopathy with conduction defects and risk of sudden death. Inheritance is usually X linked recessive but can be autosomal dominant. Family linkage studies have mapped X linked EMD to the distal long arm of the X chromosome but precise genetic localisation has been hampered by the rarity of this condition. We report three new families with X linked Emery-Dreifuss muscular dystrophy studied with DNA markers from Xq27-qter and three previously published families typed for additional markers. No recombination was observed with the red/green cone pigment locus, RGCP (lod score, Z = 2.46), the factor VIII coagulant gene locus, F8C (Z = 6.39), or with DXS115 (Z = 4.94). Two recombinants were observed which mapped EMD distal to DXS15 (DX13) and DXS52 (St14) respectively. Multipoint linkage analysis gave odds exceeding 200:1 for EMD being distal to these markers. A multipoint analysis incorporating published data gave the map cen-DXS304-9cM-DXS15-3cM-DXS52-2 cM-(RGCP,EMD)-3cM-F8C-2cM-DXS115 with odds of 120:1 in favour of a location for EMD between DXS52 and F8C as compared to the next best position distal to F8C.
Adult↗
Regression of allopurinol-induced peripheral neuropathy after drug withdrawal.
A patient experienced an axonomyelinic peripheral neuropathy during a long-term allopurinol treatment. The symptoms and signs regressed after drug withdrawal, and the nerve conduction velocities and distal latencies improved. The incidence of allopurinol-induced peripheral neuropathy is very low. Thus, facilitatory factors have to be sought.
Allopurinol↗
[Association of post-radiation focal muscular atrophy and hypertrophy].
We report a 48 year old woman who had radiotherapy for uterine carcinoma and who developed amyotrophy and muscle hypertrophy in one lower limb. Very few cases of post-radiation monomelic amyotrophy have been reported. On the other hand denervation hypertrophy was presumed to be well known. The seat of the lesions was presumed to be radicular and spinal. The mechanism of atrophy and hypertrophy is discussed.
Adenocarcinoma↗
[New methods of analysis of gait in the evaluation of the action of anti-inflammatory drugs. Apropos of naproxen].
Two nontraumatizing and reproducible tools for quantitative gait analysis as part of the objective evaluation of nonsteroidal antiinflammatory drugs are reported. The Bessou gait analyzer is a simple mechanical device which measures spatiotemporal and kinematic parameters (stride length; duration of stride, weight-bearing, and forward swing; speed and peak speed of swing; and overall speed of walking). The ELITE system is a considerably more complex instrument for analyzing three-dimensional motions through high-speed shape recognition. Computerized data management allows not only to determine the spatiotemporal parameters listed above but also to study displacements of selected landmarks in several planes and to derive joint angles and angular speeds. Coupling of the ELITE system to a force platform and to telemetric transmission surface electromyography substantially expands the range of data provided. The mechanical gait analyzer was used to evaluate the effects of naproxen in 11 patients with osteoarthritis of the knee; another similar study in 13 patients with osteoarthritis of the hip is ongoing. Gait was recorded before and after daily administration of naproxen for seven days. After treatment, walking speed was significantly increased as a result of increased stride length and decreased stride duration. Durations of monopodal and bipodal weight-bearing decreased concomitantly with the increase in speed, whereas duration of forward swing remained unchanged. These data demonstrating improved kinematic gait parameters show that naproxen is effective in reducing the adverse mechanical effects of pain. This study also demonstrates the usefulness of gait analysis for evaluating drugs.
Aged↗
[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↗
[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↗
[Non hypertrophic amyloid myopathy with muscular inflammation in plasma cell dyscrasia].
The case of a 41 year old woman with amyloid myopathy is reported. Clinical involvement consisted of limb girdle muscle weakness, mild scapular muscle atrophy and dysphagia. In contrast with the published cases, abnormal firmness, pseudohypertrophy of the musculature and macroglossia were absent. Muscle biopsy showed endo- and perimysial amyloid deposits but also inflammatory infiltrates. Inflammatory cells typing was studied by immunocytochemical methods and revealed a predominant T-helper cell infiltration. Free kappa light chains were present in serum and urine. Serum immunoglobulin levels were reduced. Bone marrow examination revealed mild plasmocytosis without abnormal cells. Immunofluorescence and immunoperoxidase techniques for identification of the type of amyloid fibrils showed positivity with antisera to kappa light chains. A 4-year follow-up revealed a progressive worsening of muscle weakness despite immunosuppressive treatment. No malignant plasmocytosis occurred. The unusual inflammatory muscle infiltration observed in this case may suggest an associated polymyositis.
Adult↗
Apomorphine-induced yawning in migraine patients: enhanced responsiveness.
Migraine patients usually exhibit hypersensitivity to dopaminergic agonists, but the tests used (such as the so called "piribedil test") preferentially assess the sensitivity of the peripheral dopaminergic receptors. Apomorphine-induced yawning may be useful in the evaluation of central dopaminergic receptor sensitivity. Nine migraine patients were included in an age- and sex-matched control study. They had been without treatment for 1 month and free of a migraine attack for at least 7 days. Apomorphine hydrochloride (5 micrograms/kg) was administered subcutaneously, and yawning was recorded by two observers. The cumulative number of yawns as a function of time was studied. The comparison of the regression graphs showed a significant difference between migraine patients and controls. Migraine patients exhibited enhanced responsiveness to apomorphine challenge. This finding supports the hypothesis of a constant central dopaminergic hypersensitivity.
Adult↗
Differential activation of motor units in the wrist extensor muscles during the tonic vibration reflex in man.
1. Single motor unit activity was recorded in the extensor carpi radialis longus and extensor carpi radialis brevis muscles of five healthy human subjects, using metal microelectrodes. 2. Motor units were characterized on the basis of their twitch contraction times and their force recruitment thresholds during voluntary imposed-ramp contractions. 3. The discharge patterns of forty-three motor units were studied during tonic vibration reflex elicited by prolonged (150 s) trains of vibration (30 Hz) applied to the distal tendons of the muscles. The temporal relationships between the individual small tendon taps of the vibratory stimulus and the motor unit impulses were analysed on dot raster displays and post-stimulus time histograms. 4. After tendon taps, the impulses of motor units with long twitch contraction times (mean +/- S.D., 47.2 +/- 10.7 ms) and low recruitment thresholds (0.88 +/- 0.6 N) formed a single narrow peak (P1) with a latency (22.7 +/- 1.4 ms) which was comparable to that of the tendon jerk in the extensor carpi radialis muscles. These motor units were named 'P1 units'. On the other hand, the response of motor units with shorter twitch contraction times (31.1 +/- 3.3 ms) and higher recruitment thresholds (3.21 +/- 1.3 N) showed two peaks: a short latency (23.4 +/- 1.3 ms) P1 peak similar to the previous one and a P2 peak occurring 9.4 +/- 1.2 ms later. These motor units were named 'P1-P2 units'. 5. When the reflex contraction increased slowly, the P1 peaks of 'P1-P2 units' were clearly predominant at the beginning of the contraction, during the rising phase of the motor unit discharge frequency, while the P2 peaks became predominant when the units had reached their maximal discharge frequency. 6. Increasing the tendon vibration frequency (35, 55, 75, 95 Hz) did not modify the 'P1 unit' discharge pattern. Due to interference between vibration period and peak latencies, increasing the vibration frequency caused the P1 and P2 peaks of 'P1-P2 units' to overlap. 7. Superficial cutaneous stimulation of the dorsal side of the forearm during tendon vibration noticeably decreased the P1 peaks in both types of motor units. In the P2 peaks it could result in either a decrease or an increase but the average effect was a slight increase. 8. When applied 10 s before tendon vibration, cutaneous stimulation considerably suppressed the tonic vibration reflex.(ABSTRACT TRUNCATED AT 400 WORDS)
Adult↗
Apomorphine-induced blinking and yawning in healthy volunteers.
Yawning and spontaneous blink rate (SBR) are two physiological reflexes which have been incompletely examined but one neurobiological step of these two behaviours seems, at least in part, dopamine-dependent. The reference dopaminergic agonist, apomorphine hydrochloride (0.5, 1, and 2 micrograms kg-1 s.c.), was compared with a placebo in a double-blind latin-square design, and was shown to induce yawning and increase SBR in a population of eight healthy volunteers. These two behavioral effects were not dose-related. The individual SBR differences were correlated with the individual number of yawns for all the four treatments at the 10-30 min interval. Thus, parallel yawning and SBR behaviour suggests a similar pharmacological mechanism. Apomorphine-induced yawning and blinking may be therefore of use in the evaluation of central dopaminergic pathways in man.
Adult↗
[Neurological disorders disclosing auricular myxoma: 3 cases].
We report 2 cases (n. 1 and 2) of cerebral infarction caused by a left atrial myxoma, and 1 case (n. 3). of dementia associated with a large left atrial myxoma. Cerebral emboli and neurological symptoms which can occur prior to cardiac obstructive and/or systemic signs require echocardiography. The cardiac post-operative condition was dramatically improved in cases revealed by an hemiplegia.
Aged↗
[Yawning. Physiopathology and neuropharmacology].
Yawning is a common physiological phenomenon, induced by hunger, sleepiness or impersonation. Its behavioral aim seems to be an effort to vigilance. There are numerous pathological reasons explaining yawning, as for example global (infectious, metabolic and toxical) or focal neurological diseases. The modification of yawning in pathology where a dopaminergic dysfunction is to be found, as well as some data of animal experimentation, support when it takes place the dopaminergic system's implication. The pre-synaptical receptor's role had been suggested for a long time but actually yawning seems to be linked with a D1-D2 cooperation. The cholinergic system probably control a final effective pathway. We also imply the peptidergic, serotoninergic, androgine and opiace systems. If the localization of a putative center of yawning is impossible, the nigrostriatal dopaminergic pathway, the paraventricular nucleus of hypothalamus and the pituitary gland play a major role. Yawning can be used as a functioning index of the dopaminergic system in vivo in the human being.
Animals↗
[Immunoallergic vasculitis: a new adverse effect of guanidine?].
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Adult↗
[Late-onset multiple sclerosis and serum monoclonal gammopathy: an incidental association?].
Four patients older than 45 years with a central nervous system demyelinating disease associated to a monoclonal gammopathy are reported. The neurologic disease met the diagnostic criteria of multiple sclerosis, with the particularity of a late onset. Monoclonal paraproteinemia was also present in the cerebrospinal fluid, associated with an intrathecal immunoglobulin synthesis. The clinical data and the course of the disease were comparable to the previous reports of late onset multiple sclerosis. A causal link between the dysglobulinemia and the neurological disease may not be demonstrated. However, such an association may underline the role of humoral processes in multiple sclerosis.
Age Factors↗