Thyrotoxic Periodic Paralysis: A Peculiar Case with Unusual Dystonic Behavior and Variable Relations of Paralysis to Serum Potassium Levels TUNESUKE KUSAKABE, MASAKO YOSHIDA, AND MASAKI NISHIKAWA Section of Endocrinology, Department of Medicine, Kyoto City Hospital, Mibu, Kyoto, Japan 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 KC1 also provoked attacks of paralysis with main d'accoucheur or of main daccoucheur. The disease could be of the normokalemic variety of periodic paralysis with fluctuations 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. (J Clin Endocrinol Metab 43: 730, 1976)

ABSTRACT. This report describes a male patient, aged 49, with thyrotoxic periodic paralysis. The patient had had episodes of main daccoucheur 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 NaCI infusion, but also by oral KC1 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


ATIENTS with periodic paralysis have been classified into three groups on the basis of their serum potassium levels during the attack, and on the basis of their response to an administration of potassium and sodium. The differences and the relationship among hypokalemic, hyperkalemic, and normokalemic forms of periodic paralysis have been discussed (1-6). Although thyrotoxic periodic paralysis and hereditary hypokalemic periodic paralysis differ in several respects, there are many similarities (7,8). The present study concerns a patient with thyrotoxic periodic paralysis. The attacks of paralysis were normokalemic, and were associated with main d'accoucheur•, diplopia, and lid-lag. Observations on spontaneous and provoked attacks were made both prior to and after treatment of the thyrotoxicosis, and, after the remission of the latter, during the administration of desiccated thyroid. Received April 1, 1975.

Case History Patient N.O. was a 49-year-old man at the time of admission in November, 1973. His paternal and maternal grandparents were distantly related. There was no history of periodic paralysis, myotonia, or goiter in the family. In January 1967, when he was 43, he first noticed stiffness of the hands. When outside in winter, or when he washed his hands in cold water, his hands became stiff from several minutes to a halfhour. After August 1972, he had palpitations, excessive sweating, and increased appetite. In October 1972, he began to have episodes of weakness of both legs, usually in midafternoon or in the evening. Attacks of weakness were almost always associated with stiffness of the limbs, particularly the hands, and diplopia. The attacks appeared every two or three days, lasting from thirty min to eighteen h. On admission, he was of medium stature; height, 1.67 meters; weight 65.5 kg. He had a slightly enlarged, diffuse soft thyroid. Exophthalmus, Dalrymple's sign, or Graefe's sign were lacking. There was no muscular hypertrophy or wasting, and tendon reflexes were normal. The 131I-triiodothyronine resin sponge uptake


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THYROTOXIC PERIODIC PARALYSIS was 48.2%, the serum thyroxine iodine 24.0 /ng/100 ml, the serum triiodothyronine 7.1 ng/ml, and the thyroid 131I uptake at 24 h 76.6%. Antihuman thyroglobulin autoantibodies were present by hemagglutination. The results of a glucose tolerance test were within normal limits. The serum parathyroid hormone was 0.6 ng/ml (normal range: 0.5-1.0 ng/ml). The plasma renin activity was 3.0 ng/ml/h, (normal range 2.0-5.0 ng/ml/h), and the plasma aldosterone 10 ng/100 ml (normal range: 5-20 ng/100 ml). During free intervals, the patient had no symptoms excepting those of thyrotoxicosis: he had no difficulty in moving his arms, legs, head, and eyes, or in the relaxation of his grip. He had no problem with speech, respiration, or swallowing. There was neither lid-lag nor percussion myotonia of tongue, thenar, wrist extensor, or face muscles. In spontaneous attacks, the patient had stiffness and weakness of the limbs and diplopia. The attacks usually began one h after meals, especially if the patient ate much either in the daytime or in the evening, and lasted one to two h. When the experienced moderate or severe stiffness of his hands, the characteristic position of carpal spasm known as main daccoucheur was observed. In attacks, stiffness of the hands and legs tended to appear early and to fade as flaccid paralysis supervened, and, on occasion, reappeared immediately before recovery from the attacks. Attacks of weakness consisted of flaccid paralysis of the limbs. This was more severe in

the legs than in the arms. He could not hold his legs and arms elevated from the bed, although he was able to grip his hands. Throughout the attacks, he frequently complained of diplopia. Occasionally during severe attacks, his speech was slurred, but there was no dyspnea or dysphagia. During the attacks of paralysis, constant and moderate lid-lag, and definite but inconstant percussion myotonia of the thenar, hypothenar, and extensor digitorum communis were present, while lingual myotonia or Chvostek's sign could not be definitely elicited. Inflating a cuff around the upper arm sometimes provoked the main d'accoucher in that limb. Spontaneous attacks, stiffness of the hands, diplopia, and lid-lag were present, but there was no paralysis of the limbs. In provoked attacks, the symptoms and signs were similar. Methods and Results A. Initial


I. Spontaneous attacks (Table 1, A). Levels of serum sodium (Na), potassium (K), calcium (Ca), phosphorus (P), immunoreactive insulin (IRI), and blood sugar during free intervals were within normal range. At the onset of attacks, levels of blood sugar and IRI were always elevated because the attacks occurred after meals. There was no significant change in serum Na, K, Ca, and P before attacks, during attacks, or after recovery.

TABLE 1. Levels of serum electrolytes, blood sugar, and serum immunoreactive insulin (IRI) during free intervals and spontaneous attacks Levels of Period A. Before treatment 1. Before an attack During an attack After recovery 2. During an attack After recovery C. During thyroid therapy after remission of thyrotoxicosis Before an attack During an attack After recovery





Blood sugar t


4.3 4.0 4.1 3.7 4.5

148 142 143 148 149

4.9 4.8 4.9 — —

5.1 4.8 4.9 4.3 4.9

123 168 138 162 96

100 230 130 112 16

4.6 3.8 3.7

137 139 139

4.8 4.8 4.8

4.0 3.7 3.3

105 159 145

51 222 182

* mEq/liter. t mg/100 ml.


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JCE & M • 1976 Vol 43 • No 4

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FIG. 1. Electromyogram of the left adductor pollicis muscle. A: Post-contraction persistent potentials (v.c. = voluntary contraction). B and C: Spontaneous potentials.

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

Thyrotoxic Periodic Paralysis: A Peculiar Case with Unusual Dystonic Behavior and Variable Relations of Paralysis to Serum Potassium Levels TUNESUKE K...
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