Spasmophilia

SPASMOPHILIA, more correctly childhood tetany, is a syndrome based on increased excitability of the nervous system in children. A latent and a manifest period of the disease are distinguished. The latent period (latent tetany) is characterized by the absence of convulsions and the presence of signs of increased mechanical and galvanic excitability of the peripheral nervous system. The manifest period (manifest tetany, tetania manifesta) manifests in the form of attacks of clonic convulsions (convulsions, eclamptic convulsions—eclampsia infantum), prolonged tonic convulsions of the limbs ('proper tetanic convulsions', carpopedal spasm), laryngospasm and respiratory disorders, spasm of the sphincters and smooth musculature, cardiac activity disorders, and vegetative symptoms. Etiology and pathogenesis. Spasmophilia is a disease of early childhood, predominantly from 3 months to 2 years of age, the same age period in which we also have the highest incidence of rickets. Moreover, at the present time, the etiological and pathogenetic connection of childhood tetany with rickets is considered proven, and the existence of forms of non-rachitogenic tetany in infancy and early childhood is generally disputed. Forms of tetany occurring in older childhood (puerile tetany) are predominantly non-rachitogenic. In almost every case of spasmophilia in a child, it is possible to establish rickets of greater or lesser severity. In the etiology of childhood tetany, the type of feeding is of greater importance than in rickets: tetany in breast-fed children is encountered extremely rarely; switching bottle-fed children to breastfeeding leads to a noticeable weakening and disappearance of spasmophilic symptoms. The adverse effect of cow's milk is attributed to the influence of the mineral composition of milk serum and is primarily attempted to be explained by the high content of phosphates in milk (Freudenberg). The latter possibly supports the phosphate retention characteristic of tetany: in pronounced rickets not complicated by tetany, the content of inorganic phosphorus in the blood is lowered, whereas in cases complicated by tetany, the phosphorus content in the blood is normal or even elevated (see Rickets). An increase in the phosphorus content in the blood is accompanied by a decrease in the calcium content. Such a fluctuation in calcium content under the influence of phosphate retention is characteristic of mineral metabolism in manifest tetany. Ca in the blood in manifest tetany is 5–8 mg%, in latent tetany 8–9.5 mg% against 10–12 mg% in normal; Ca/P in tetany is 1.2, in rickets 3.5, in a healthy child 1.95. Tetany is a seasonal disease, showing maximum incidence in the pre-spring and spring months, and minimum in the summer and autumn months. This must also find its explanation in the corresponding seasonal fluctuations and shifts in the ratio of mineral components of the blood and tissue fluids, caused by changes in endocrine correlation—'hormonal spring crises'. In direct connection with this fact is the appearance of signs of manifest tetany in cases of overly rapid anti-rachitic treatment and the hyperphosphatemia with simultaneous hypocalcemia caused by it. The tetanogenic effect of fever is also associated with the change in metabolism and ionic constellation under its influence. The appearance of attacks of clonic and tonic convulsions at the beginning of an infectious disease as a manifestation of spasmophilia can be linked to the alkalotic shift in metabolism occurring at the beginning of the febrile period. The combination of alkalosis with hypocalcemia characterizes the disturbed metabolism in tetany. Hypocalcemia in the presence of acidosis does not cause a tetanoid reaction, just as alkalosis does not cause it without a corresponding shift in the ionic constellation. The opposite state of certain aspects of metabolism in rickets and tetany—hypophosphatemia and acidosis in rickets, hyperphosphatemia, hypocalcemia, and alkalosis in tetany—is currently viewed as a biphasic metabolic disorder caused by a deficiency of vitamin D (see Rickets). The ionic constellation specific to tetany occurs in a child whose entire pathological state is based on vitamin D deficiency. This position is primarily proven ex juvantibus by the favorable therapeutic effect of vitamin D and anti-rachitic treatment in children with spasmophilia (see treatment below). All these data, however, do not remove the great importance of hereditary-constitutional factors in the etiology and pathogenesis of childhood tetany. Individual predisposition is of such great importance in the genesis of spasmophilia that it is still interpreted by many as a congenital constitutional anomaly, as a diathesis—spasmophilic diathesis. Some note the hereditary-familial character of spasmophilia (Thiemich, Finkelstein), others believe that the important role is played not so much by direct transmission as by general neuropathy and the degenerative state of ancestors (Maslov). While acknowledging the great importance of constitutional factors, it is difficult, however, based on all the data presented above regarding the pathogenetic links between spasmophilia and rickets, to classify childhood tetany in the group of diatheses (see Diatheses). Puerile tetany, spontaneous tetany of adults, as well as experimental forms (phosphate tetany, bicarbonate, hyperventilation, guanidine, parathyreoprivic), while representing an etiologically and pathogenetically heterogeneous group, nevertheless provide material for elucidating the mechanism of the origin of childhood tetany as well. The study of the question of guanidine and parathyreoprivic tetany has clarified the essential difference between the clinical picture and genesis of these forms and childhood tetany (see). Symptomatology. Latent spasmophilia. Mechanical hyperexcitability: 1) Chvostek's facial phenomenon (Chvostek). When tapping the cheek in the region of the fossae caninae (pes anserinus) between the zygomatic arch and the corner of the mouth with a finger or a hammer, lightning-fast contractions of the musculature of the mouth, nose, and eyelid innervated by the n. facialis appear. The same contraction of the eyelid and lip musculature can be induced by tapping in the region of the eyelid or the corner of the mouth. 2) Peroneal phenomenon of Ibrahim, Lust (Ibrahim, Lust). When tapping with a hammer directly below the head of the fibula with relaxed muscles (supporting the lower leg with the left hand), an elevation of the outer edge and abduction of the foot appear. 3) An analogous symptom is noted upon mechanical excitation of the nn. radialis, ulnaris, femoralis. Electrical hyperexcitability (Erb's sign, Thiemich-Mann's sign) is a more constant sign of latent tetany. Increased galvanic excitability is especially noted upon opening the electrodes. In a normal child, contraction upon opening the cathode is obtained at a current strength above 5 mA; in spasmophilic children, at figures below 5 mA—at 4.3 and even 1.5. For a spasmophilic patient, the appearance of AnOC at a lower current strength than AnCC (Pirquet) is also characteristic. Trousseau and Schlesinger signs (Trousseau, Schlesinger) are noted in cases bordering between latent and manifest tetany. Trousseau's sign—spasmodic contraction of the fingers in the form of an obstetrician's hand upon irritation of the nerves in the sulcus bicipitalis by pressure of a finger or the application of an elastic tourniquet. An analogous symptom on the leg (Schlesinger) occurs upon strong flexion of the leg at the hip joint with extension at the knee. Respiratory phenomenon in response to peripheral irritation (Maslov)—spasmodic contraction of the respiratory muscles upon applying irritation to the child in the form of a prick. A normal child, when recording respiration with a pneumograph after irritation in the form of a prick, reacts with an increase in respiratory rate and a change in depth; in spasmophilia, however, not only with an increase in respiratory rate, but also with an arrest (apnea). Manifest tetany. 1) Eclamptic convulsions (eclampsia infantum)—attacks of general and localized convulsions with loss of consciousness, very much resembling convulsions that are both epileptic and caused by organic and functional brain disease (tumors, hemorrhages, hydrocephalus, syphilis, intoxications, etc.). The eclamptic attack is sometimes preceded by psychic excitement, loud crying, and general restlessness. A mild attack is often limited only to stupor and pallor of the face, twitching of the eyelids; for the most part, convulsions of the facial muscles are noted, often of all limbs, which frequently begin on one side. Consciousness is lost from the very beginning of the attack; the pupils do not react to light. The eclamptic attack may be preceded by prolonged tonic convulsions of the limbs (often in combination with laryngospasm). When attacks follow one after another, children lie in a state of convulsive contraction of the whole body with a face that alternately takes on a mask-like appearance and is distorted by rapid twitching, with foam, with a blood-stained mouth (if teeth are present), and with upturned eyes. The fontanelle is tense; the pulse is rapid and irregular. The duration of an eclamptic attack is 1/2–2 minutes, less often 3–5 minutes; when attacks follow one after another, status eclampticus may develop. In the overwhelming majority of cases, eclamptic convulsions occur without a significant increase in temperature; where there is fever, one must think of a coincidental occurrence with another disease or an infectious disease that caused the transition of the latent period of spasmophilia into the manifest one.

In very rare cases, with status eclampticus, by analogy with status epilepticus, significant hyperthermia may occur, caused by a disturbance in the function of the thermoregulatory centers. 2) Tetanic convulsions, a tetanoid state in the proper sense of the word—prolonged tonic convulsions of the limbs (carpopedal spasm, arthrogryposis); the distal parts of the hand affected by convulsions often lead to the position of the hand in the form of a paw, an obstetrician's hand, or tonic adduction of the thumb of the hand and foot is noted. Such tonic contractures of the limbs sometimes last for hours; often, swelling and congestive edema develop on the dorsal surface of the hands and feet during them. Along with carpopedal spasms, tonic convulsions of the rest of the musculature are often noted. Tonic convulsions of the facial muscles give the face a peculiar anxious, tense expression ("tetanic face"). In rare cases, opisthotonic or emprosthotonic curvature of the spine is noted. 3) Laryngospasm, spasmus glottidis, is a typical and frequent manifestation of Spasmophilia, occurring under the influence of a whole range of conditions that increase the excitability of the respiratory center: screaming, crying, coughing, waking from sleep, fever, overfilling of the stomach. A mild spasm of the glottis is expressed by a sonorous, moaning inhalation during crying or screaming. A spasm attack is expressed by sudden difficulty in inhalation; after a pause, a prolonged, moaning inhalation is heard. With a pronounced spasm, complete cessation of breathing (inspiratory apnea) is noted, exophthalmos, cyanosis, and loss of consciousness often occur; cardiac activity slows down and can reach 20 beats per 1 minute. Another, more dangerous type of inspiratory apnea attack is also encountered—"tetanus apnoicus," which is expressed in lightning-fast tonic convulsions of the entire musculature, cessation of breathing, asphyxia, and fainting. "Expiratory apnea" represents a cessation of breathing during exhalation, during which the child suddenly, without a moaning inhalation, turns blue, falls into an unconscious state, the pulse becomes thready, and if breathing is not restored, the child dies—"death during the first attack." In favorable cases, breathing is restored, often with a moaning inhalation. All these types of respiratory disorders, like laryngospasm, are apparently connected with the hyperexcitability of the respiratory center. 4) The involvement of smooth muscle is expressed in spasm of the bladder sphincter with urinary retention, spasm of the esophagus, the ciliary muscle, and specific narrowing of the pupil. A spastic state of the stomach and intestines is described, at the basis of which lies increased excitability of the autonomic nervous system. A rarely observed spastic state of the muscles of the bronchial walls—"bronchotetany"—is dangerous. Sudden death of a spasmophilic child, including that occurring during an attack of laryngospasm and apnea, should be considered as death from a disorder of cardiac activity ("tetany of the heart"). Course, duration, and outcome. The listed forms of manifestation of Spasmophilia are encountered in the most diverse combinations in children. In each individual case of Spasmophilia, one or another symptom comes to the fore: some cases proceed under the guise of laryngospasm, sometimes in combination with eclampsia, others with the picture of carpopedal spasms, in third cases, only attacks of eclampsia are noted among the obvious signs. In children of the first half-year of life, convulsions and laryngospasm are predominantly noted. Carpopedal spasms are encountered more often in children around 1 year of age and older; they are also encountered in children at an older age (puerile tetany). Puerile tetany, however, is not connected with spasmophilia, i.e., tetany of early childhood, and is not considered pathogenetically rachitogenic. The maximum frequency of the disease of spasmophilia falls on the age of 9-12 months of life. During the course of spasmophilia, as with rickets (see Rickets), periods of improvement and relapse are noted; the latter are noted in the late winter and spring months, often manifesting with the onset of an infectious disease. One or another manifestation can sometimes be traced up to the 3rd and 4th year of the child's life. Indications of the development of convulsions in epileptics even in infancy force one to consider these convulsions an early manifestation of epilepsy, and not Spasmophilia. In rare cases, a combination of eclampsia with epilepsy is encountered, but there is never a transition of eclampsia into epilepsy. Among the signs of latent Spasmophilia in older children, the Chvostek facial phenomenon is especially common, which, however, in view of its high frequency at this age (especially in children 8-14 years old), is evaluated by many not as a symptom specific to Spasmophilia, but rather as a symptom of neuropathy. Subsequent observations of children who have undergone Spasmophilia in infancy and early childhood have established a large percentage of children with hypereflexia, tics, speech defects, urinary incontinence, and other signs of neuropathy and psychopathy. Along with this, many also note general physical retardation (infantilism, asthenia) and various signs of functional disorders of circulation and internal organs (Finkelstein). All this especially emphasizes the significance of the constitutional factor in the etiology and pathogenesis of Spasmophilia. Differential diagnosis. Carpopedal spasms are so characteristic that they do not present diagnostic difficulties. In the eclamptic form, one can confuse the noted general convulsions with epileptic convulsions, with convulsions caused by intoxication during an incipient infectious disease, with convulsions on the basis of organic brain damage. Important for differential diagnosis in these cases is the presence of characteristic symptoms of mechanical and galvanic hyperexcitability (facial phenomenon, peroneal Trousseau, Erb's sign) and hypocalcemia. It is necessary to test for increased excitability some time after the convulsion attack, since in the period immediately adjacent to the convulsions, symptoms of hyperexcitability may be absent. In cases with early eclampsia in the 2nd and 3rd month of life, especially in premature infants, phenomena of peripheral hyperexcitability are often absent, but hypocalcemia is always noted in them. Characteristic laryngospasm is difficult to confuse with congenital stridor, false croup, or reprises in whooping cough. A picture resembling the apnea of spasmophilics is noted in psychopathic children as a reaction of dissatisfaction and irritation towards others ("holding one's breath"). Latent symptoms of hyperexcitability of the peripheral nervous system and reduced calcium content in the blood (see above) are important for differential diagnosis. Prevention and therapy. Prevention of Spasmophilia, in view of its close etiological and pathogenetic connection with rickets, boils down to the same measures (see Rickets). The especially protective significance of breast milk brings breastfeeding to the fore, as well as a correct hygienic regimen, sufficient use of fresh air, light, and the administration of vitamin D. With artificial feeding, the main task is to protect the child from nutritional disorders, achieving correct weight gain and tissue building. In all forms of Spasmophilia, transferring the child to natural feeding has a favorable effect. With artificial feeding, the diet is constructed without a scheme, depending on the degree of manifestation of Spasmophilia and the nutritional state of the child. In overfed spasmophilic children, in the absence of attacks of eclamptic convulsions, one can limit oneself to a general reduction in the amount of food and the prescription of a contrast diet: with excessive milk feeding, significantly limit the amount of milk, increase the amount of carbohydrates; with one-sided carbohydrate feeding, introduce milk. The presence of pronounced attacks of eclampsia and especially laryngospasm serves in such children as an indication for emptying the intestines, a tea diet for 6-12 hours, followed by a milk-free diet. Diet therapy is especially difficult in cases of Spasmophilia in children with chronic nutritional disorders, complicated and uncomplicated by diarrhea. The main task in these cases is the elimination of digestive and nutritional disorders; with an improvement in the child's general condition and weight gain, spasmophilic phenomena also improve. Drug treatment, used alongside dietetic treatment, boils down to the administration of: 1) narcotic and antispasmodic agents as symptomatic remedies for eclampsia—chloral hydrate 0.25-0.5 in an enema in 30.0-50.0 of water, luminal-sodium 0.1 in the form of a 10% solution, urethane 1-2 g per os, 1.5-3 in an enema; 2) calcium salts and acidotically acting preparations, e.g., ammonium chloride 0.6 per 1 kg of weight (3-6 g pro die) in a 10% solution, hydrochloric acid milk, according to Scheer, acid ammonium phosphate, etc. The effect of the latter agents, including calcium salts (5-6 g pro die Calc. chlorati sicci in the form of a 5-10% solution 5 times a day at 10.0, due to the unpleasant taste—in milk), is ensured only with the simultaneous implementation of energetic antirachitic treatment. The implementation of antirachitic treatment is the basis of all therapy for spasmophilia: mercury-quartz lamp, Vigantol, cod liver oil (cod liver oil with phosphorus—a long-recommended remedy: Phosphor. 0.01, 01).

Jecoris Aselli 100.0, two to three times a day, 5.0 each). When using a mercury-quartz lamp, one can expect an exacerbation of spasmophilic phenomena after the first sessions (see above - pathogenesis). This is avoided by the simultaneous use of calcium salts or an acidifying agent—ammonium chloride, hydrochloric acid milk, etc. It is considered inexpedient to begin anti-rachitic treatment only after a certain period of symptomatic treatment; the most rational approach is combined anti-rachitic and symptomatic treatment. In mild cases of latent tetany, one may proceed to anti-rachitic treatment, best of all with vitamin D preparations, without simultaneous symptomatic treatment (see Rickets).