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Thyrotoxic periodic paralysis: a peculiar case with unusual dystonic behavior and variable relations of paralysis to serum potassium levels.

This report describes a male patient, aged 49, with tyrotoxic periodic paralysis. The patient had had episodes of main d'accoucheur for eight years. Since thyrotoxicosis had affected the patient last year, he had had attacks of flaccid paralysis of the limbs associated with main d'accoucheur. While the spontaneous attack was normokalemic and responded favorably to potassium, attacks similar to the spontaneous one were provoked not only by glucose infusion, carbohydrate feeding, and NaCl infusion, but also by oral KCl administration. Transition from hypokalemic to hyperkalemic type of paralysis occurred during potassium treatment of a sodium-induced attack, and that from hyperkalemic to hypokalemic type of paralysis occurred after glucose infusion given during a potassium-induced attack. Intra-arterial epinephrine injection caused prompt paralysis of the perfused hand. A main d'accoucheur was induced in one hand by cooling it in chilled water. The spontaneous attacks of flaccid paralysis disappeared after the patient returned to the euthyroid state, but reappeared when he was treated with high doses of desiccated thyroid. Even when the patient was relieved from thyrotoxicosis, he still experienced stiffness in his hands. The administration of glucose, insulin, and KCl also provoked attacks of paralysis with main d'accoucheur or main d'accoucheur. The disease could be of the normokalemic variety of periodic paralysis with fluctations in the potassium levels depending on the provocative tests employed. It seems that the unusual dystonic behavior of this patient is due not to myotonia, but to muscle spasm resulting from metabolic abnormalities. It could also be suggested that excess thyroid hormones have adverse effects on the development and syndrome of periodic paralysis by abetting a latent hereditary abnormality.

Blood Glucose

The light and electron microscopic changes in the skeletal muscles during paralysis in thyrotoxic periodic paralysis.

Light microscopy of the biopsied quadriceps muscles during paralysis in 17 patients with thyrotoxic periodic paralysis (TPP) showed no abnormalities in 23.5 per cent, sarcolemmal nuclear proliferation in 35.5 per cent, atrophy of muscle fibers in 29.4 per cent, central nuclei in 23.5 per cent, fatty infiltration in 17.6 per cent, vacuolation in 11.8 per cent, and sarcoplasmic masses in 11.8 per cent. The muscle specimens were also examined by electron microscopy in ten of these patients; the main changes observed were vacuolation (90 per cent), mitochondrial abnormalities (100 per cent), glycogen granules accumulation (100 per cent), disruption of the myofibers (50 per cent), and changes in the T-system (40 per cent). The light and electron microscopic changes in the skeletal muscles during paralysis were not well correlated with the severity of the muscle weakness of hypokalemia.

Adult

A new standardized and effective method of inducing paralysis without administration of exogenous hormone in patients with familial periodic paralysis.

A prolonged glucose loading test is described. When used in five patients with familial periodic paralysis it brought them into a state of massive hypopotassaemic paralysis at the first attempt, without the use of exogenous hormone. Three normal persons were subjected to the same glucose loading without exhibiting hypopotassaemia or muscular weakness. There were no essential differences in serum glucose levels between the two groups of individuals. The efficacy of previous methods of inducing paralysis, hormonal as well as non-hormonal, is reviewed, and the advantages of this new method are pointed out.

Blood Glucose

Clinical observations on 600 cases of recurrent laryngeal nerve paralysis.

A statistical review was made on 600 cases of recurrent laryngeal nerve paralysis who visited the author's clinic during the last 16 years. The yearly incidence, the age and sex distributions and the classifications of the causes of paralysis were presented and discussed. A special attention was given to the topics of associated laryngeal paralysis, bilateral recurrent laryngeal nerve paralysis and the so-called epidemic paralysis. A brief comment was also made on diagnosis and treatment of recurrent laryngeal nerve paralysis in general.

Adolescent

Auditory symptoms associated with herpes zoster or idiopathic facial paralysis.

Auditory symptoms (hyperacusis, tinnitus, decreased hearing) have long been recognized to accompany herpetic or idiopathic facial paralysis. Twenty-nine percent of 1,080 patients with idiopathic facial paralysis and 37 percent of 172 with herpes zoster oticus facial paralysis had auditory symptoms. Abnormal related sensori-neural hearing loss was documented in only 11 of these 377 patients with auditory complaints. All of the 11 had a diagnosis of herpes zoster oticus. Sensori-neural hearing loss occurs in only about 6.5 percent of patients with herpes zoster facial paralysis, and no confirmed case of such loss in idiopathic facial paralysis has been reported. In patients presenting with sensori-neural hearing loss accompanying facial paralysis believed to be idiopathic, herpes zoster should be suspected even in the absence of vesicles. Factors favorable for recovery of auditory function include age 64 years or younger, mild initial hearing loss, a cochlear pattern of hearing loss, and absence of vertigo. Recovery of auditory function does take place; however, a high-tone sensori-neural loss may persist except in younger patients.

Adolescent

[Recent developments in electrical tests applied to the study of facial paralysis].

We have already shown the spontaneous development over a period of time of idiopathic facial paralysis, in relation to the extent of the resulting disfigurement, by means of analytical clinical measurement representing the sum of 19 factors constituting the force of contraction, tonus, coordination and hemispasm. This spontaneous development takes place in 3 stages: --an initial period up to the 15th day; --an intermediate period during which there is clinically total facial paralysis; --a period of recuperation. The duration of this development in relation to the extent of sequelae enables a rough distinction to be made between: a) benign facial paralysis in which disfigurement is nil or very slight (1 to 6 p. 100), incomplete paralysis or complete paralysis in which recuperation starts about the 15th day at the earliest, or at about the 4th month at the latest; c) very serious facial paralysis with moderate (38 to 66 p. 100), or considerable (66 to 100 p. 100) disfigurement and in which recuperation starts at the end of the 3rd month at the earliest. The authors have attempted to indicate how effective very prompt quantitative electrical procedures are in helping to assess the seriousness of ultimate permanent disfigurement (see article).

Electrodiagnosis

Respiratory paralysis to improve oxygenation and mortality in large newborn infants with respiratory distress.

The nonsynchronous respiratory efforts of neonates with surgically correctable disorders may inhibit effective mechanical ventilation. The records of 25 infants treated with metocurine for muscular paralysis to improve mechanical ventilation were reviewed. All patients were greater than 35 (37.6 +/- 2.1) weeks gestation and 2.27 (2.98 +/- .47) kg. All required ventilatory support with an FiO2 of 100%. The mortality rate of this group of infants was 20% as compared with 73% (p < .001) in a similar group of 26 infants managed without paralysis. In 10 of the 25 infants treated with metocurine, pre- and 1 hr postparalysis paO2 values were available. The mean paO2 prior to paralysis was 62 (45--111) mm Hg and the mean post-paralysis paO2 was 144 (75--227) mm Hg, representing at 132% increase in paO2 (p < .001). The mean dosage for metocurine was 3.5 (1.45--6.79) mg/kg/day; however, those requiring paralysis for greater than 7 days showed a dramatically increasing requirement. These preliminary data suggest that respiratory paralysis reduces right-to-left shunting, improves paO2 and decreases mortality in large infants with severe respiratory distress requiring ventilatory support.

Diaphragm

A simple, single-injection method for inducing long-term paralysis in embryonic chicks, and preliminary observations on growth of the tibia.

A method for inducing paralysis in embryonic chicks is described. This involves single injections of the neuromuscular blocking agents, D-tubocurrarine Chloride or decamethonium iodide, into 10-day embryos. The dose which optimises survival and paralysis is determined along with the effect of the drugs on embryonic growth. Decamethonium iodide at a dose of 1 mg per embryo gave maximum survival and paralysis to 18 days of incubation. Paralysis was assessed by observation of treated embryos in ovo and by examination of embryos removed from their shells between 11 and 18 days of incubation. Embryos were completely paralysed 24 hours post-injection and remained paralysed until 18 days of incubation. Paralysed embryos failed to hatch. Development of the leg musculature was severely retarded in paralysed embryos. This method of inducing paralysis has considerable advantages over previous continuous infusion methods. The growth and collagen content of the tibia in the paralysed embryos was reduced and these results, and other applications of the method, are discussed.

Acetylcholinesterase

Electromyographic (EMG) study in thyrotoxic periodic paralysis.

Thyrotoxic periodic paralysis (TPP) is produced by a different metabolic disorder than familial periodic paralysis. In the familial disorder, a myopathic cause is well known, but although electromyography can be used to demonstrate the site of the lesion in TPP, such studies are rare in the medical literature and the results are conflicting. In this report, EMG studies were carried out on eight Chinese patients with thyrotoxic periodic paralysis during the attack and the results compared with studies repeated during a remission. Most cases showed a myopathic pattern during an attack of paralysis which disappeared during remission. The myopathic changes noted were a decrease in duration of muscle action potentials, an increase in polyphasic potentials, a satisfactory interference pattern with reduced amplitude and a reduced amplitude of the evoked muscle action potential on nerve stimulation. Peripheral nerve function was normal in the cases studied. It is concluded that the weakness in TPP is myopathic and that the peripheral nerve function during paralysis is normal.

Action Potentials