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Electrical activity induced by ischemia in the skeletal muscle of patients with spasmophilia.

The incidence of spontaneous electrical activity during ischemia was studied in normal and spasmophilic subjects with a Bronk electrode inserted in the first dorsal interosseous muscle. Subjects were of both sexes, aged 16 to 65 years. The diagnosis of spasmophilia was established electromyographically by a variant of the Alajouanine test. Ischemia induced in many cases an electrical activity consisting of discharge potentials that have the amplitude, duration and morphology of motor unit potentials. The number of discharging motor units varied, sometimes leading to an electrical Trousseau associated to a carpal spasm. The discharge of individual motor units was irregular and practically uninfluenced by passive manipulations on the muscle or voluntary contraction. The activity started usually 30 sec to 1 min after the onset of ischemia, reached a maximal intensity (as to the number of motor units and frequency of discharge) in about 1 min and declined gradually in the following minutes. This activity was encountered in 55.06% of 770 cases of spasmophilia and only in 11.66% of 283 normal subjects, with a tendency to more intense discharge in spasmophilia. Its incidence in the spasmophilic group was closely related to the intensity of spontaneous repetitive discharges appearing electromyographically after the activation test, reaching 78.63% in most severe cases. The behaviour was interpreted as indicating a higher susceptibility in spasmophilia of axons to depolarization induced by hypoxia. This higher susceptibility appears as an important component of the excitability alterations leading to the complex neuromuscular disorders occurring in spasmophilia.

Adolescent↗

Pathophysiological aspects of calcium metabolism in spasmophilia.

In order to clarify the pathophysiological mechanisms of spasmophilia, 34 subjects (31 females and 3 males) with spasmophilia were studied. The diagnosis of spasmophilia was based on a specific clinical protocol and electromyographic criteria. In the study, markedly reduced plasma ionized calcium and serum magnesium concentrations were observed together with slightly and non-significantly reduced plasma calcium and phosphate levels. An impairment of intestinal radiocalcium absorption was also noticed. Parathyroid secretion did not show any significant disturbance, but circulating calcitonin levels were found to be significantly lower than in normal subjects. The mean value of serum 25OHD was within the normal range, while a slight reduction in bone Gla protein, an index of osteoblastic activity, was detected. No difference between patients with spasmophilia and normal subjects was observed concerning 47Ca kinetics in red blood cells. The studies indicated that an impaired intestinal calcium transport together with low levels of circulating calcitonin represent the most important pathophysiological determinants of spasmophilia.

Adult↗

Voluntary multiplet discharge after the activation test for spasmophilia as an electromyographic sign of a mild form of this disease.

The electromyographic (EMG) diagnosis of spasmophilia is based on the appearance in a susceptible muscle of spontaneous burst (multiplet) discharges after an activation procedure consisting usually in 10 minutes ischemia followed by a pause of a length that varies with the author but does not exceed 5 minutes, and finally by a hyperventilation, again not exceeding 5 minutes. In investigations carried out many years ago we observed that a number of subjects with symptoms of spasmophilia showed no postischemic spontaneous bursts, but responded with bursts to voluntary contraction. In this work we present some findings leading to the conclusion that voluntary burst discharge after the activation procedure can represent a sign of spasmophilia and not a mere manifestation within normal limits. Thus, in most cases with no symptoms of spasmophilia, voluntary burst response was absent. On the other hand, most subjects with such an EMG alteration suffered of troubles attributable to spasmophilia. Crisis of tetania with partial or generalized contracture could be encountered in such cases with a frequency close to that of cases with mild spontaneous burst discharge, being, on the contrary, exceptional in cases with normal EMG. In a previous work we showed the existence of a close correlation between the increase above normal of the incidence of ischemic discharge and the intensity of postischemic spontaneous burst (multiplet) discharge. A significant increase of ischemic discharge was also found for the cases showing only voluntary bursts (34.29% of 487 cases as compared to 14.28% of 602 normals). In addition, the period of abnormal burst responses of motor units to voluntary command was significantly prolonged for the cases with more intense ischemic discharge. Finally, there is no gap between spontaneous and voluntary burst discharge, since not rarely a short contraction could trigger a rhythmical selfmaintained burst discharge entirely similar to the spontaneously occurring one. One may conclude that voluntary burst discharges, if rather persistent, can represent a sign of spasmophilic alteration of excitability that must be always tested EMG in suspected cases that give no spontaneous burst discharge to the facilitation procedures. It represents a change in excitability less severe than the spontaneous burst discharge.

Action Potentials↗

[Magnesium balance in patients with spasmophilia. Relation to results of electromyography].

BACKGROUND: The pathophysiological basis of spasmophilia is frequently magnesium deficiency and the therapeutic administration of magnesium salts has usually a favourable effect. However the parameters of magnesium balance are not always consistent with the results of electromyography. The objective of the present work was to test and interpret the relationship of results of these two basic diagnostic procedures indicated when spasmophilia is suspected. METHODS AND RESULTS: Thirty-three subjects (9 men and 24 women) with suspected spasmophilia were examined by non-invasive electromyography, using the technique of surface electrodes. All subjects had concurrently biochemical examinations: serum calcium and ionized calcium, serum magnesium (S-mg), magnesium in erythrocytes (ery-Mg) and magnesium in the blood haemolysate (H-Mg). In 29 patients and oral magnesium loading test was made with evaluation of the urinary Mg excretion after a constant Mg load (U-Mg). Statistical evaluation of the investigated parameters of the magnesium balance revealed a highly significant relationship between ery-Mg and U-Mg and H-Mg and ery-Mg (p < 0.005). A less close relationship was found between H-Mg and S-Mg (p < 0.05). Total and ionized calcium was in all examined subjects within the range of the arbitrary normal range. The EMG finding was positive (the finding of two and more multiplets in the ischaemic and hyperventilation test resp.) in 30 instances, i.e. in 91% of the examined subjects. In 72% there was agreement of the positivity of the EMG and magnesium deficiency (i.e. reduced values of ery-Mg and U-Mg), positivity of EMG combined with normal parameters of the Mg balance was recorded in 18%. In 6.1% of the examined subjects magnesium deficiency was confirmed combined with a normal EMG finding. CONCLUSIONS: Concurrent positivity of EMG and magnesium deficiency in 72% justifies the therapeutic administration of magnesium. In patients with a normal magnesium deficiency and positive EMG another cause of spasmophilia must be taken into consideration, incl. technical errors of interpretation of EMG results. A negative EMG associated with magnesium deficiency can suggest the central form of tetany, where magnesium treatment is also unequivocally indicated.

Adolescent↗

[The role of paraclinical studies in the diagnosis of hypomagnesemia spasmophilia].

Assuming that Ca+2 and Mg+2 play an important role in the cell (especially the neuronal and muscular ones) membrane excitability, this paper presents the place that the paraclinical specific investigations have in the proper diagnose of normal calcium serum level hypo-magnesium spasmophilia. A number of 52 patients presenting significant clinical spasmophilia, of age between 21 and 55 years old, from different professions and social classes, were selected for this study. Paraclinical investigations (spectrophotometry) showed a normal serum calcium level (an average of 9.22 mg%); also, a decrease of Mg+2 serum level (an average of 1.41 mg%) and normal levels for Na+ (3.04 g/l) and K+ (0.21 g/l). The electrophysiological investigations (EMG, EEG, VCN m, EKG) performed showed, on the EMG recordings, in 85% of the cases the presence of the typical spasmophilic repetitive discharges, increased when using compression ischemia and 3 minutes hyperventilation, while on the EEG recordings, most patients demonstrated the presence of diffuse or mainly frontal lobe cortical bioelectrical activity alteration, either in spontaneous conditions or after specific stimulation (3 minutes hyperventilation or SLI). The muscle bioelectrical activity was analyzed by mean of EMG recordings--a decrease of VCN m was detected on the SPE nerve (34.4 m/s) and also on the SPI nerve (34.2 m/s) The EKG recordings performed showed in 5% of the cases the presence of the ventricular repolarization disturbances (flat T waves). After receiving a proper treatment which included oral administration of calcium and magnesium gluconolactate, magnesium gluconolactate with B6 vitamin or Trimagant, there were important changes of all the studied parameters and so, as a specific therapy, we recommend the use of Mg+2 compounds, because it demonstrates a significant improvement of the clinical symptoms in spasmophilia and has no danger to precipitate in the body as Ca+2 exceeding does.

Adult↗

Neuromotor microalterations in subjects with spasmophilia symptoms: a preliminary study.

Spasmophilia is a relatively unknown condition characterized by perturbations of the neuromuscular system. We hypothesized that spasmophilia may negatively affect neuromotor functions in subtle ways. Three tests including tremor, rapid pointing movements, and alternating movements were quantified in a group of subjects with spasmophilia symptoms (SS) (n = 10) and a healthy control group (n = 10). Most of the characteristics used to evaluate motor functions in these three tests revealed no significant differences between the two groups except for two characteristics in alternating movements and two characteristics in rapid pointing movements. In terms of variances, a dissociation between voluntary movements and involuntary movements was observed for the two groups. Control subjects had significantly higher variances in involuntary movements such as tremor, while subjects with SS had significantly higher variances in voluntary movements such as alternating and rapid pointing movements. A significant increase in asymmetry in hand laterality was also noted for some characteristics in subjects with SS.

Humans↗

[Mitral valve prolapse and spasmophilia in children and adolescents].

71 cases of mitral valve prolapse (MVP) in the child and adolescent with an age range of 3 to 20 years were reviewed. The clinical symptoms, electrocardiography and catheter and angiographic data of this mitral abnormality were analysed. A systematic study of the electrocardiogram showed a tendency to spasmophilia in 68% of cases. The serum calcium, phosphate and magnesium levels were normal in 90 to 95% of patients. A reduced erythrocytic magnesium level seems to have been found in some patients. It is interesting to observe the similarity between the clinical signs in MVP and in spasmophilia. These clinical, biochemical and electrocardiographical results justify the systematic investigation of children and adolescents with MVP, with a view to the diagnosis of spasmophilia.

Adolescent↗

[Mitral valve prolapse and spasmophilia in the adult].

75 cases of mitral valve prolapse (MVP) for which no cause was found underwent electromyography (EMG). In 64 cases (85.3%) EMG showed changes suggestive of spasmophilia. The symptoms observed were those already described in this condition. The specific clinical signs of spasmophilia were often elicited with a positive Chvostek sign in 20 out of 30 cases (73.3%). Radiological, echocardiographical and haemodynamic studies underlined the hyperkinetic state of the left ventricle. Biochemical investigations showed a high incidence of low erythrocytic magnesium levels. Chest pain suggestive of angina pectoris, mitral valve prolapse and spasmophilia are frequently associated. The role of the low erythrocyte magnesium on left ventricular hyperkinesis and the production of MVP is discussed.

Adult↗

[Comparison of periodic syndrome and spasmophilia].

Two casual clinical observations here reported allow the Authors to discuss the possible relationships between periodic syndrome and spasmophilia. Particularly they stress how these common causes of recurrent symptoms (abdominal pain, headache, growing pains, anxiety, irritability) can be the same clinical entity. This suspicion is confirmed by EMG investigation: 25 children with periodic syndrome (8 males, 17 females; 8-13 years) and 10 normal subjects (6-11 years) participate in this study. Post-ischemic EMG shows signs of neuromuscular hyperexcitability as in spasmophilia in 21 children (84%) of the first group and only in 3 of the second group. Such findings should be regarded as the first step towards a better definition of the relationships between periodic syndrome and spasmophilia.

Adolescent↗

[25 OH CC (25-hydroxycholecalciferol in spasmophilia) (author's transl)].

The level of 25 OH CC in plasma was evaluated in spasmophilia (126 cases) comparatively with normal persons. This level is significantly decreased in spasmophilia. Therapeutic by 25 OH CC of the calcipenic symptoms was tested with good clinical results. Some precautions are necessary when spasmophilia is associated with idiopathic hypercalciura.

Calcium↗

[Clinical manifestations of spasmophilia in developing age].

82 children (47 male and 35 female), aged between 2 and 12 years, with signs and symptoms consistent with spasmophilia, were investigated with an emg technique to better define boundaries of tetanic syndrome. In all subjects an ischaemic test was performed to the upper limb and the spontaneous emg activity from thenar eminence was recorded both during and after ischaemia. Moreover a clinical examination, with particular attention to objective signs of spasmophilia, were performed and serum electrolytes determined. Electromyographic spontaneous activity was noticed in 46 children (24 female, 22 male), 31 of them were hypocalcemic. The frequency of the various clinical manifestations is reported and correlated with emg and humoral findings. Significant difference between sexes in the incidence of tetanic syndrome where not noticed. More frequent symptoms for spasmophilia are tensive headache and cramps, abdominal spasms, psycomotos instability and anxiety; Chvostek's sign is frequent but no specific. The other symptoms assume relevance only if present in association. The role of electromyographic criteria in the detection of tetanic syndrome is stressed.

Abdomen↗

[Katacalcin levels in healthy children and children with spasmophilia].

The calcitonin (CT) gene encodes at least 3 peptides: CT, the 21-aminoacid carboxyl-terminal flanking peptide (katalcin or PDN-21) and CT-gene related peptide. Normal thyroid C-cells as well as malignant ones co-secrete CT and PDN-21 in response to hypercalcemia, so assay of PDN-21 may be an usefull method to assess C-cells secretion. Because of our knowledge no data are available on PDN-21 values in children, we measured this peptide in healthy children and in spasmophilia (Sp), a disease that has been related to CT deficiency. We studied 16 healthy children (9 males, 7 females; aging from 3.0 to 11.6 years) and in 21 patients with diagnosed Sp (8 males, 13 females, aging from 4.6 to 13.0 years). PDN-21 were assayed in whole serum by RIA using synthetic human PDN-21 for standards, 125I-PDN-21 for tracer and specific antiserum. CT was measured the serum of the same subjects by RIA using an ultrasensitive methods. In healthy children PDN-21 serum values were 12.3 +/- 2.0 pg/ml and no significant differences were found between males and females. Children with Sp showed slow higher PDN-21 concentrations (14.0 +/- 1.4 pg/ml) than normals, but the difference was not statistically significant. Also CT values were not significantly different between normal children (21.4 +/- 3.7 pg/ml) and patients with spasmophilia (22.5 +/- 1.8 pg/ml). A high significant positive relation was found between katalcacin and CT levels in normals as well as in spasmophilics. The physiological effects of PDN-21 are actually unknown.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Spasmophilia: a clinical neurophysiopathological and biochemical study.

Spasmophilia is a problem much debated as regards its nosographic location. Its paroxysmal onset in a "neurotic" patient has confined spasmophilia within a psychiatric syndromic complex for many years. In the present study, strict clinical criteria used in selecting a population of spasmophilic patients included clinical, neurophysiological, biohumoral and psychological evaluations, providing further information about the real organic features of this syndrome. Moreover, on the basis of an organic pathogenetic hypothesis, all patients were administered medical therapy which resulted in clinical amelioration in a significant number of cases.

Adolescent↗

[Limping and muscle hypertonia concentrated in the calves. Relation to spasmophilia. Clinical and electromyographic study and treatment plan].

The authors report several cases of patients who presented with pain in one or both calves and a limping gait. Clinical examination and the EMG confirmed that these disorders were of musculo-tendinous origin, maximal at the level of the gemellus tendon, frequently forming a tendinitis. The syndrome consists of a hypertonia of the calves which is maximal on waking and which improves on stretching the muscles. Most of the subjects examined suffered from a syndrome of restless legs and presented alterations of the autonomic nervous system and spasmophilia (simultaneous EMG recording of the left and right soleus muscles). Examination of the pathogenesis reveals the possibility of a phenomenon of tonic muscular training synchronous with variations in an elevated and unstable autonomic tone. The authors discuss a possible relationship with spasmophilia and algodystrophy. Finally, they propose a plan of treatment.

Adult↗