[Tetany as a form of presentation of adult celiac disease.] / Tetania como forma de presentación de enfermedad celiaca en el adulto.

Introduction: celiac disease is an autoimmune disease with symptoms involving multiple organs. The forms of presentation vary considerably, which makes it difficult to diagnose. The objective is to present an atypical case of celiac disease. Methodology: descriptive, retrospective, cross-sectional study of a case of an adult woman who presented with tetany Results: investigating the case, the diagnosis of tetany was reached secondary to an intestinal malabsorption Conclusion: celiac disease can occur atypically as a tetany

Introducción: Introducción: la enfermedad celiaca es una enfermedad autoinmune con síntomas que involucran a múltiples órganos. Las formas de presentación varían de modo notable lo que dificulta su diagnóstico. El objetivo es presentar un caso atípico de enfermedad celiaca. Metodología: estudio descriptivo, retrospectivo, de corte transversal de un caso de mujer adulta que se presentó con tetania Resultados: investigando el caso se llegó al diagnóstico de tetania secundaria a un síndrome de malabsorción intestinal debida a enfermedad celiaca, confirmada por histología. Conclusión: la enfermedad celiaca puede presentarse atípicamente como una tetania.

Pseudohypoparathyroidism type 1B - a rare cause of tetany: case report.

Pseudohypoparathyroidism (PHP) is a rare group of disorders characterised by end-organ resistance to the parathyroid hormone (PTH). A 16-year-old boy presented with a 2-year history of involuntary dystonic movements involving mainly the left hand, initially after writing and later during physical exercise. Serum calcium was 1.37 mmol/L (2.20-2.69), phosphate 2.1 mmol/L (0.8-1.45) and PTH 302 ng/L (12-88). CT scan of the head demonstrated multiple subcortical and diffuse basal ganglia calcifications. Genetic analysis confirmed a methylation defect in the GNAS cluster on chromosome 20q13.32 which established the diagnosis. Treatment with calcitriol and calcium carbonate led to complete remission of symptoms. Causes of hypocalcaemia should be considered in evaluating patients with movement disorders. The diagnosis of PHP-1B is challenging but the overall prognosis is excellent.

[Electrophysiological analysis of functional state of spinal cord motoneurons in rats with parathyroprivous tetany].

In normal rats and of those with parathyroprivous (hypocalcemic) tetany the comparative analysis of background activity (BA), tetanic and posttetanic increase and decrease of frequency of spinal cord (SC) motoneurons (MNs) responses under high-frequency (50, 100Hz) stimulation (HFS) of flexor (G) and extensor (P) hind-limb nerves have been conducted. The on-line selection and program analysis of the spikes was produced. On the 3-7 and 21-22 days of development of acute and chronic tetany, respectively, the significant tetanic and posttetanic changes of MNs activity without meaningful changes in BA was registered. Along with the abrupt increase of excitatory manifestation of activity to HFS in a period of development of acute tetany was observed their relative weakening in animals with chronic tetany. Simultaneously the weakening or total disappearance of depressor reaction, especially expressed in the period of development of acute tetany was noted. It was concluded on the causal dependence of the parathyroprivous convulsions due to disturbances of correlation of inhibitory-excitatory processes in SC MNs.

Myokymia and neuromyotonia in veterinary medicine: a comparison with peripheral nerve hyperexcitability syndrome in humans.

Involuntary muscle hyperactivity can result from muscle or peripheral nerve hyperexcitability or central nervous system dysfunction. In humans, diseases causing hyperexcitability of peripheral nerves are grouped together under the term 'peripheral nerve hyperexcitability' (PNH). Hyperexcitability of the peripheral motor nerve can result into five different phenotypic main variants, i.e. fasciculations, myokymia, neuromyotonia, cramps and tetany, each with their own clinical and electromyographic characteristics. This review focuses on the most commonly described expressions of PNH in veterinary medicine, i.e. myokymia and neuromyotonia, in particular in young Jack Russell terriers. Data from 58 veterinary cases with generalized myokymia and neuromyotonia were analyzed, including unpublished treatment and follow-up data on eight Jack Russell terriers from a previous study and seven additional Jack Russell terriers. A dysfunction of the potassium channel or its associated proteins has been found in many human syndromes characterized by PNH, in particular in generalized myokymia and neuromyotonia, and is suspected to occur in veterinary medicine. Potential pathomechanisms of potassium channel dysfunction leading to signs of PNH are broad and include genetic mutations, antibody-mediated attack or ion channel maldistribution due to axonal degeneration or demyelination. A more accurate classification of the different PNH syndromes will facilitate a more rapid diagnosis and guide further research into natural occurring PNH in animals.

Selected blood chemistry values in mobility-impaired broiler breeder hens with suspected calcium tetany using the i-STAT handheld clinical analyzer.

Calcium tetany is a poorly defined disease of broiler breeder hens that results from acute hypocalcemia. It is characterized by impaired mobility, increased mortality, and absence of gross lesions that would explain the impaired mobility. To evaluate if hens with impaired mobility had calcium tetany or other abnormalities, blood values from normal and affected hens were determined using the i-STAT handheld clinical analyzer. Three flocks were evaluated weekly prior to peak production (range 25-30 wk of age) comparing normal hens to hens with clinically apparent calcium tetany. Calcium tetany suspect (CaTS) hens from four additional flocks were also evaluated. Significant hypocalcemia (P < 0.001) was observed in CaTS hens (average = 1.14 mmol/L ionized calcium [iCa]) compared to normal hens (average = 1.53 mmol/L iCa) in only one of three flocks sampled weekly. Clinically affected hens from one of the other four flocks also had hypocalcemia. Blood value abnormalities in mobility-impaired hens without hypocalcemia included hypernatremia. Findings in this study indicate calcium tetany is one cause of impaired mobility in breeder hens, but mobility impairment without hypocalcemia can also occur. Calcium tetany should be confirmed by finding significantly decreased levels of iCa in the blood, as diagnosis based on clinical presentation and necropsy results can be inaccurate. The i-STAT handheld clinical analyzer is an efficient, relatively low-cost method to determine iCa and other blood chemistry values that may be associated with impaired mobility in broiler breeder hens.

Overexpression of HSP10 in skeletal muscle of transgenic mice prevents the age-related fall in maximum tetanic force generation and muscle Cross-Sectional Area.

Skeletal muscle atrophy and weakness are major contributors to frailty and impact significantly on quality of life of older people. Muscle aging is characterized by a loss of maximum tetanic force (P(o)) generation, primarily due to muscle atrophy, to which mitochondrial dysfunction is hypothesized to contribute. We hypothesized that lifelong overexpression of the mitochondrial heat shock protein (HSP) HSP10 in muscle of mice would protect against development of these deficits. P(o) generation by extensor digitorum longus muscles of adult and old wild-type and HSP10-overexpressing mice was determined in situ. Muscles were subjected to damaging lengthening contractions, and force generation was remeasured at 3 h or 28 days to examine susceptibility to, and recovery from, damage, respectively. Muscles of old wild-type mice had a 23% deficit in P(o) generation and a 10% deficit in muscle cross-sectional area compared with muscles of adult wild-type mice. Overexpression of HSP10 prevented this age-related fall in P(o) generation and reduction in cross-sectional area observed in muscles of old wild-type mice. Additionally, overexpression of HSP10 protected against contraction-induced damage independent of age but did not improve recovery if damage occurred. Preservation of muscle force generation and CSA by HSP10 overexpression was associated with protection against the age-related accumulation of protein carbonyls. Data demonstrate that development of age-related muscle weakness may not be inevitable and show, for the first time, that lifelong overexpression of an HSP prevents the age-related loss of P(o) generation. These findings support the hypothesis that mitochondrial dysfunction is involved in the development of age-related muscle deficits.

Ultrastructural changes accompanying development of fatigue in frog twitch skeletal muscle fibres.

OBJECTIVE: The aim of the present study was to characterise and compare alterations in the ultrastructure of the functionally identified isolated twitch skeletal muscle fibres of the frog after repeated tetanic stimulation and under experimental conditions which modified their fatigability. METHODS: Single isolated twitch muscle fibres of m. iliofibularis of adult frogs Rana temporaria were subjected to intermittent tetanic stimulation. Fibres at specified degree of fatigue were processed for electron microscopic observation and ultrastructural examination. RESULTS: The fatigue-resistant (FR) fibres that developed 90% of the control tetanic tension after 10 min stimulation in ordinary Ringer's solution showed regions with dilated intermyofibrillar spaces containing small vesicles and swollen mitochondria. In addition to the changes observed in FR fibres, the easily fatigued (EF) fibres that produced 50% of the original tension after 3 min stimulation showed small vacuoles in the sarcoplasm. In EF fibres that preserved 10% of the control tension after 10 min stimulation and showed swelling of the longitudinal sarcoplasmic reticulum, the central element of triads and mitochondria, large vacuoles were present. FR fibres exposed to low Ca2+ medium containing 0.02 mmol/l verapamil, lost their resistance to fatigue. Their contractile responses fell down to 20 % within 0.5 min of stimulation. Those fibres displayed large vacuoles and changes in mitochondria as observed in EF fibres after 10 min stimulation. CONCLUSION: These results suggest that morphological changes accompanying reduction of the contractile force (i) appear earlier than the reduction of the contractile ability, (ii) correlate with the degree of reduction of the contractile capacity but not with the duration of contractile activity, (iii) are not specific for the fatigue fibre type.

Grass tetany in a herd of beef cows.

Five cows in a herd of 15 cattle that had just been turned out onto lush pasture after having over-wintered on poor quality hay died suddenly. Biochemical profiles collected from the cadavers revealed reduced serum levels of magnesium, urea, and beta-hydroxybutycate. Classical grass tetany (hypomagnesemia) was diagnosed on postmortem examination.

Postmortem biochemical markers of experimentally induced hypomagnesaemic tetany in cattle.

Severe hypomagnesaemia and tetany were induced in 10 lactating cows by feeding them semi-synthetic low magnesium diets and the animals were used to study the stability of postmortem markers of hypomagnesaemic tetany. There were significant relationships between the concentrations of magnesium in either cerebrospinal fluid (CSF) or plasma and either aqueous or vitreous humour. The onset of hypomagnesamic tetany was also associated with low magnesium concentrations in plasma, CSF and aqueous and vitreous humour. Magnesium concentrations less than 0.25 mmol/litre in fresh aqueous humour may be indicative of severe hypomagnesaemia and possible tetany in lactating cows, but the concentration of magnesium in aqueous humour was unstable postmortem. The concentration of magnesium in vitreous humour was relatively stable and a concentration of less than 0.55 mmol/litre could be used as a diagnostic marker of tetany in cattle for up to at least 48 hours postmortem, at ambient temperatures typical of Northern Ireland.

[Recurrent tetany as the first symptom of late manifesting celiac disease]. / Rezidivierende Tetanien als Erstsymptom einer spätmanifestierten Zöliakie.

BACKGROUND: The diagnostic classification of a malabsorption is often made more difficult, particularly in oligosymptomatic forms. CASE REPORT: A female patient is presented, where the diagnosis of malabsorption syndrome was concluded because of recurring tetanias. This could be traced back to an oligosymptomatic celiac disease. Diagnostic course of action, differential diagnosis as well as further observation during therapy are discussed. CONCLUSION: Oligosymptomatic developments of celiac disease are common amongst adults and present a diagnostic challenge. The existence of a malabsorption should be considered, even if the momentary individual symptoms are unclarified.

Postoperative tetany in patients with Graves' disease: a risk factor analysis.

OBJECTIVE: There is little information regarding the clinical risk factors for postoperative tetany in patients with Graves' disease. We analysed the risk factors responsible for postoperative tetany by univariate and multivariate analysis in thyroidectomized patients with Graves' disease, and we discuss the mechanisms of hypocalcaemia and tetany after surgery. PATIENTS: The subjects were 1742 consecutive patients with Graves' disease who underwent subtotal thyroidectomy between 1992 and 1994. RESULTS: Univariate analysis of 15 possible risk factors demonstrated that 10 were significant: sex, required operation time, blood loss, preoperative serum calcium level, estimated weight of thyroid remnant, size of goitre, amount of excised thyroid tissue, serum alkaline phosphatase concentration, age at operation and TSH-binding inhibitory immunoglobin (TBII). In multivariate analysis in female patients, the risk factors were: preoperative lower serum calcium level, younger age, higher serum alkaline phosphatase concentration, larger size of goitre and higher value of TBII, in order of decreasing probability of significance. CONCLUSION: These results, combined with previous observations in which a significant decline in serum parathyroid hormone level was found in patients with Graves' disease who suffered postoperative tetany, suggests that postoperative tetany may occur during the period of bone restoration due to antithyroid drug therapy and be due to continuation of a calcium flux bone concomitant with transient hypoparathyroidism induced by surgery. Calcium supplements during preoperative period and/or after surgery may be appropriate for patients who are anticipated to develop postoperative tetany based on these risk factors.

Some observations on variations in filament overlap in tetanized muscle fibres and fibres stretched during a tetanus, detected in the electron microscope after rapid fixation.

Anomalous tension development which would not be predicted from the descending limb of the length-tension curve occurs during prolonged tetani and after stretch during a tetanus. Variations in filament overlap might account for all or part of the tension enhancement. Fibres isolated from frog skeletal muscle were rapidly fixed during a tetanus with mercuric chloride in ethanol and chloroform so that the correct alignment of the filaments in the overlap zones was preserved. The fibres were examined in polarized light with compensation, and in the electron microscope. There were variations in striation spacing along the length of the fibres, and severe shortening with contraction bands near the tendon insertions, confirming observations made by others on live fibres. Many variations in filament overlap which would not be detectable by light microscopy or laser diffraction were seen in the electron microscope. In a pilot study we measured differences in the width of the overlap zones between half-sarcomeres in a small area and within individual half-sarcomeres. In the latter case the variations were greater in a fibre which developed creep of tension and one which did not. Even greater variations were seen in three fibres stretched during a tetanus, and, in two of these, there were some grossly elongated half-sarcomeres in which the filaments had pulled out of the overlap zones, leaving gaps.

Variation of surface density and other parameters in remodeling of trabecular bone in various conditions.

To appreciate the remodeling of the trabecular bone, the static morphometric parameters of surface density (SV(TRAB/BONE] and volumetric fraction (VV(TRAB/BONE] of cancellous bone were measured and compared to remodeling parameters, i.e., the surface extents of active/inactive resorption, active bone formation and nonmineralized bone. Iliac bone biopsies from 28 subjects with spasmophilia, osteoporosis and primary hyperparathyroidism were studied by means of a Nachet-France NS 2000 automatic image analyzer and a Zeiss ocular integrator; the results obtained in each group showed comparable values for the two methods (r = .8 for each group, with P less than .01). The remodeling parameters measured by means of the ocular integrator were compared with the variation measurements of the trabecular surface density, SV(TRAB/BONE). The correlation between the inactive osteoid surface and the coefficient of variation of the mean (CVM of SV(TRAB/BONE] on sections was significant for the three pathologic groups. The average mean values and standard errors of the mean of this latter parameter for the spasmophilia and hyperparathyroidic groups were, respectively, 0.063 +/- 0.008 and 0.092 +/- 0.012. Analysis of the experimental data shows that the simple global measurement of CVM (SV(TRAB/BONE] by means of an automatic image analyzer supplies information on the skeletal state during tissue remodeling.

[Changes in the ultrastructure of synapses and neurons of the ventral horns of the spinal cord in cats with tetany as a result of the removal of the parathyroid glands]. / Izmeneniia ul'trastruktury sinapsov i dvigatel'nykh neironov ventral'nykh rogov spinnogo mozga u koshek s paratireoprivnoi tetaniei.

An electron microscopic investigation on ultrastructure of synapses and ventral horns of the lumbar thickening in the spinal cord of cats with parathyroprival tetany has been performed. When motor disorders are clearly seen on the 2d - 4th days after removal of the parathyroid glands, together with the changes demonstrating functional activity of the synapses, certain irreversible degenerative changes of the "dark" type are seen in the axodendritic and axosomatic synapses. A complex of structural-metabolic changes has been revealed in the motor neurons, they have both functional-compensatory and degenerative character. It is possible to suggest that the changes revealed at parathyroprival tetany result from a disturbed metabolism and increased afferent influences.

Study on static morphometric parameters for a diagnostic approach of osteoporosis.

The diagnostic value of the morphometric parameters Vv,Sv, dtrab of cancellous bone and relations between them were examined. Iliac bone biopsies of osteoporotic and spasmophilic subjects were studied by means of a Mop 1 semi automatic digitizer tablet joint to a C B M 3032 microprocessor. Both the average Sv and Vv values were significantly different between the two groups. Concordance of Sv values with the radiological diagnosis was closer than Vv ones. The global estimation of mean trabecular thickness is discussed. In conclusion the confrontation of experimental data shows that simple global measure of Sv completes and precise the knowledge of skeleton state.

[Death in a tetany attack]. / Tod im tetanischen Anfall.

A case of latent tetany in a 24-year-old woman is reported. She had suffered several attacks of tetany in the last 12 years, mostly triggered by external stimuli. One evening, the woman was found dead in the hall of her flat beside the telephone with the upper limbs in a tetanic convulsive attitude. The attack of tetany was triggered by a telephone conversation. The parathyroid glands were normal. Day-old purulent myocarditis could be demonstrated in the left ventricle under the light microscope. The two possible pathogenetic explanations of the causal chain leading to death are discussed. It was astonishing that a clinical symptom (tetanic convulsion) was fixed in rigor mortis.

Neurogenic benign fasciculations, pseudomyotonia, and pseudotetany. A disease in search of a name.

We studied two patients with abnormal spontaneous muscular activity. The first had widespread fasciculations, painful spasms, delayed muscular relaxation, and hyperhidrosis. Improvement occurred after several years. The second case had generalized paresthesia, mild stiffness, a positive result from Trusseau's test, and was relieved by administration of carbamazepine. Both patients had abnormal conduction velocity. Examination of muscle biopsy specimens disclosed fiber type grouping and increased collateral ramification of motor axons. These observations exemplify symptoms and signs that resemble those of myotonia and tetany and occasionally occur in partial denervation. they provide additional evidence of the neurogenic nature of Isaacs-Mertens syndrome.