Idiopathic hypoparathyroidism and paroxysmal dystonic choreoathetosis

Abstract

Micheli and associates [l) in a recent report in Annals described a patient with basal ganglia calcification and sporadic paroxysmal dystonic choreoathetosis without hypoparathyroidism. In a Letter to the Editor, Yamamoto and Kawazawa [ 2 ] pointed out that they had previously reported a similar case but with hypocalcemia.We are aware of an earlier report on idiopathic hypoparathyroidism [ 3 ] in a 13-year-old boy with curious attacks while playing football. He was found to have low serum calcium levels, and on skull roentgenogram "patchy cerebral calcification" was observed "situated in the region of the basal ganglia." By description his attacks were not ictal phenomena per se but episodes of dystonia with risus sardonicus. Several years ago we saw a 12-year-old boy with dizzy spells. These occurred upon standing up or upon initiation of movement such as beginning to run or walk rapidly. His mother had witnessed several episodes; she said he developed a strange smile on his face after which the right hand and arm drew up slowl!~ in the flexed position, and he bowed his head. Episodes lasted 15 to 30 seconds and could occur up to 20 times a day. O n neurological examination there were no abnormalities. When the patient was asked to sit for a minute and then suddenly stand up and walk about the chair in a clockwise ciirection, an attack was precipitated. There was no overt loss of consciousness. An electroencephalogram in the waking state revealed normal background activity with occasional low voltage spike and spike-slow wave complexes emanating from the right frontal region. The diagnosis of "reflex movement epilepsy" was made and phenytoin was prescribed. The frequency of episodes decreased dramatically. After several weeks, he developed massive myoclonic spasms of the legs that would throw him to the ground without loss of consciousness. Because of this "atypical response," a workup was undertaken and it was found that his serum calcium level was 5.6 and his serum phosphorus 11.0 mg/dl. The computed tomographic scan (Fig) revealed bilateral calcification of the basal ganglia and calcification of both frontal regions and the right cerebellar hemisphere.Ergocalciferol, 25,000 units twice a day, calcium lactate, and basal jel were prescribed. Serum calcium concentration increased to 7.4 mg/dl and all attacks ceased. Six months later he was doing well.It is likely that the enigmatic condition of "sporadic paroxysmal dystonic c:noreoathetosis" in association with hypocalcemia, hypoparathyroidism, and basal ganglia calcification represents a distinct syndrome. It should be distinguished from familial paroxysmal choreoathetosis originally

Paroxysmal choreoathetosis in a patient with idiopathic basal ganglia calcification, chorea, and dystonia

“…This patient first experienced paroxysmal attacks at age 37 (2), whereas our patient was 61 years of age at symptom onset. In contrast to our case, other patients from the literature with P D C and basal ganglia calcification show additional idiopathic hypoparathyroidism (6,7).…”

Paroxysmal hemiballism and idiopathic hypoparathyroidism

“…Paroxysmal appearance of her involuntary movements in our patient questions that calcium deposits represent the basis of explanation for the persistent extrapyramidal signs in hypoparathyroidism (chorea, parkinsonism). Moreover, we ob-served a complete disappearance of ballistic movements after the normalization of hypocalcaemia, the phenomenon already described in other cases with hypoparathyroidism associated with involuntary movements [3,7].…”

Paroxysmal kinesigenic dyskinesia in pseudohypoparathyroidism: Is basal ganglia calcification a necessary finding?

“…Some previous reports regarding PKD have described the causal relationship between hypocalcemia [4] and BG calcification. Interestingly, some authors, moreover, compared the prevalences of BG calcification between idiopathic hypoparathyroidism and pseudohypoparathyroidism [5].…”