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 »  Introduction
 »  Case report
 »  Discussion
 »  References

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Year : 2000  |  Volume : 48  |  Issue : 4  |  Page : 401-2

Sporadic paroxysmal exercise induced dystonia : report of a case and review of the literature.


Department of Neurology, Calcutta National Medical College and Hospital, 24, Gorachand Road, Calcutta, 700014, India.

Correspondence Address:
Department of Neurology, Calcutta National Medical College and Hospital, 24, Gorachand Road, Calcutta, 700014, India.

  »  Abstract

Sporadic paroxysmal exercise induced dystonia (PEID) is a rare condition. So far only fifteen cases have been reported in the world literature. It is characterised by dystonic posture on prolonged exercise, which gets relieved with rest. In general, these are refractory to medical treatment. We report one such case, along with review of the literature. The lower limbs were spared and only right shoulder was tucked up with the head turning to right side. The duration of exercise necessary to bring out the dystonic posture gradually diminished with time, a feature not reported previously.

How to cite this article:
Bhattacharyya K B, Basu S, Ray A D, Bhattacharya S. Sporadic paroxysmal exercise induced dystonia : report of a case and review of the literature. Neurol India 2000;48:401


How to cite this URL:
Bhattacharyya K B, Basu S, Ray A D, Bhattacharya S. Sporadic paroxysmal exercise induced dystonia : report of a case and review of the literature. Neurol India [serial online] 2000 [cited 2018 Nov 16];48:401. Available from: http://www.neurologyindia.com/text.asp?2000/48/4/401/1487




   »   Introduction Top

Paroxysmal exercise induced dystonia (PEID) is a clinical entity characterised by dystonic postures in parts of the body, most commonly affecting the lower limbs, following prolonged exercise, lasting 5 to 30 minutes and disappearing (in most cases), within minutes of cessation of the physical activity. This is in contrast to two other more commonly observed related conditions namely, paroxysmal kinesiogenic dystonia (PKD), where attacks are precipitated by sudden movement and are mostly short lived; and paroxysmal non kinesiogenic dystonia (PNKD), where the dystonic postures are spontaneous in nature and are prolonged. Other agents like, levodopa, trihexyphenidyl, or acetazolamide are often not useful. PEID can be familial and sporadic. The former is more commonly encountered. Sporadic PEID, on the other hand, is a rare entity and so far only fifteen cases have been reported, two of which have been documented from our country. We report one such patient of sporadic PEID.

   »   Case report Top

SH, a 45 year old male, complained of painful cramps in the neck and grotesque posturing of the head and right shoulder on walking for about 10 minutes, of two years duration. Initially it was mild, but later, started interfering with his daily activities. The length of time required to produce the abnormal posturing had been slowly decreasing. Now walking up and down for about hundred yards was adequate to bring out the movements. Cold shower and a moderate dose of alcohol did not bring out the bizarre posturing. The posture was never brought out by sudden movement and the family history was non-contributory. On examination, his right shoulder was tilted up, and the head was bent towards the right side. He was in considerable distress. The symptoms totally subsided after about ten minute's rest. The features were reproducible after he was asked to squat and stand up for about 30 times or when asked to pace up and down for about 3 minutes. The lower limbs were remarkably free from dystonic posturing. The neurological examination was otherwise unremarkable. Touching the chin or forehead seemed somewhat to relieve the problem suggesting thereby 'geste antagonistique', the phenomenon of recovery from dystonic posturing with the employment of a sensory trick. Laboratory investigations showed normal serum ceruloplasmin level (30 mg/dl). 24 hours urinary copper excretion (120 µ g in 24 hours) was within normal limits. There was no Kayser-Fleischer ring. CT scan of the brain and electroencephalogram did not show any abnormality.
Carbamazepine even upto 800 mg daily, did not produce any benefit. Levodopa -carbidopa preparation (250/25) upto four tablets a day and trihexiphenidyl (2 mg twice daily) and were also found to be equally ineffective. He could not afford tetrabenazine. He was lost to follow-up after one year.

   »   Discussion Top

Paroxysmal movement disorders have been traditionally named and classified as proposed by Lance.[1] Paroxysmal dystonic choreathetosis (PDC), a familial autosomal dominant entity is characterised by spells of long attacks of dystonia, lasting from 5 minutes to 4 hours, and which are often induced by alcohol, fatigue, stress and extremes of temperature. On the contrary, paroxysmal kinesiogenic choreoathetosis (PKC) is characterised by chorea, dystonia or both and is induced by brief (less than 5 minutes) movements, which sometime respond well to anticonvulsants. Lance also described three members in a family who developed dystonic postures lasting 5 to 20 minutes, after prolonged exercise. Plant et al[2] described another such family. Examples of sporadic cases of PEID are however rare and fifteen such cases have been reported so far, two of whom were of Indian origin.[3],[4],[5],[6] The largest series have been provided by Bhatia et al.[6] Lance's original classification[1] has undergone changes in recent times and the one proposed by Demirkiran and Jankovic,[5] modifying the previous one, seems more exhaustive and rational. They suggest that short attacks induced by sudden movement be termed paroxysmal kinesiogenic dyskinesia (PKD) and the ones that are spontaneous and prolonged be termed paroxysmal nonkinesiogenic dyskinesia (PNKD). They also bring to light a new entity, paroxysmal exercise induced dyskinesia (PEID), where attacks are precipitated by prolonged exercise.
The problems of the case under report were similar to the cases of PEID described in the literature. The abnormal movements were precipitated by prolonged exercise and were neither spontaneous not following sudden movement, thus differentiating the condition from PKD and PNKD. The movements were relieved with rest. Importantly, the family history was negative, thus attesting the sporadic nature of the condition.
The attack in the present case was always confined to one side, as seen in four out of the [eight] cases reported by Bhatia et al.[6] However, it was never brought out by cold shower as was reported by Wali.[4] There was no evidence of dyskinesia or dystonic writing tremor during the attacks. All the cases of sporadic PEID described so far, developed dystonic postures following prolonged exercise, and in two cases, following exposure to cold.[4],[6] Though some of the described patients developed generalised dystonia, all of them presented with leg dystonia as an integral feature. In only four cases, the neck and the shoulder were additionally involved.[6] However, the unique finding in our case was non involvement of lower extremities, a universal description in all the reported cases. Additionally, it was recorded that the time required for bringing out the dystonic posture was diminishing over a period of time, a feature that has not been described in any of the fifteen cases reported so far.
In conclusion, PEID is considered a distinct entity, different from PKD and PNKD. Sporadic cases of PEID are indeed rare, and the present case reported, demonstrates some unique features, not reported so far in the literature.
 

  »   References Top

1.Lance JW : Familial paroxysmal dystonic choreoathetosis and its differentiation from related syndromes. Ann Neurol 1977; 285-293.   Back to cited text no. 1    
2.Plant GT, Williams AC, Earl CJ et al : Familial paroxysmal dystonia induced by exercise. J Neurol Neurosurg Psychiatry 1984; 47 : 275-279.   Back to cited text no. 2    
3.Wali GM : Paroxysmal hemidystonia induced by prolonged exercise and cold. J Neurol Neurosurg Psychiatry 1992; 55 : 236-237.   Back to cited text no. 3    
4.Nardocci N, Lamperti E, Rumi V et al : Typical and atypical forms of paroxysmal choreoathetoris. Dev Med Child Neurol 1989; 81 : 670-674.   Back to cited text no. 4    
5.Demirkiran M, Jankovic J : Paroxysmal dyskinesias : Clinical features and classification. Ann Neurol 1995; 38 : 571-579.   Back to cited text no. 5    
6.Bhatia KP, Soland VL, Bhatt MH et al : Paroxysmal Exercise-Induced Dystonia : Eight new sporadic cases and a reviewof the literature. Mov Disord 1997; 12 : 1007-1012.   Back to cited text no. 6    

 

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