November 1996, Volume 46, Issue 11

Case Reports

Chorea - A Presenting Feature of Giant Cell Arthritis

Najib-ul-Haq  ( Department of Medicine, Khyber Medical College, Peshawar. )
Iftikhar Ali Shah  ( Department of Medicine, Khyber Medical College, Peshawar. )

Introduction

Chorea occurs as a hereditary or non-hereditary disor­der. Non- hereditary chorea most commonly occurs in rheumatic fever (Sydenham’s Chorea) but may also be encountered in other metabolic and systemic disorders. Green House and Albuquerque1 listed more than forty different causes of chorea. The etiology of non-rheumatic chorea may cause diagnostic difficulties, due to its rarity. We report a 75 years old patient with giant cell arteritis presenting as chorea. Introduction Chorea occurs as a hereditary or non-hereditary disor­der. Non- hereditary chorea most commonly occurs in rheumatic fever (Sydenham’s Chorea) but may also be encountered in other metabolic and systemic disorders. Green House and Albuquerque1 listed more than forty different causes of chorea. The etiology of non-rheumatic chorea may cause diagnostic difficulties, due to its rarity. We report a 75 years old patient with giant cell arteritis presenting as chorea.

Case Report

A seventy-five years old non-smoker male presented with malaise, bodyache, headache mid feeling unwell. He was treated with Tab. Diagesic (Dextropropoxyphene+Paraceta­mol). A month later, he complained of binning of vision in the right eye and abnormal movements on the left side of the body. History did not reveal any movement disorder in the family and he did not take any medications except Diagesic. There was no history of diabetes, hypertension or alcohol intake. On examination, the pulse was 86/min regular, all the pulses were intact and the temporal arteries had a granularfeel on palpation. Blood pressure was 170/90 mmHg. Chorea form movements were noted on the left side of the body. Right disc appeared pale and a formal ophthalmological opinion con­firmed Isehemic Papillitis on the right side. Left optic disc was normal. Respiratory system did not, reveal any abnormality and the rest of the systemic examination was also unreniark­able. Right temporal after biopsy was cell arteritis may suggest a ‘Malignant Course’ possibly due to extensive cerebral vessels involvement and the perforniedonthe same day and 80 rug prednisolone was given daily in divided doses investigations at this stage included: ESR, 96mm at the end of is hr. and haemoglobin 12 Gm%. Total and differential icucocyte counts were normal, Blood sugar 7m mol/L, se mm calcium 2.4 mmol/L. ASO titte, ANF, T3, T4 and TSH levels were normal. VDRL was negative. ECG and eehoeardiogra­phy revealed no abnormality. Chest and skull x-rays and CT sean of the skull were normal. Histological changes in temporal artery showed giant cell arteritis. Three weeks later, he developed choreiform movements on the right side as well, followed by complete blindness in the left eye. It was agam confirmed to be due to ischemic papillitis. The patient died two months later with disabling chorea.

Discussion

In 1960, Paradise2 suggested that chorea nay be expected though not documented in conditions like polyarteri­tis and deramatomyositis, i.e., conditions causing arteritis. Cherea has been reported in SLE, Henoch-Schonlen purpura and in association with central retinal artery occlusion3. Chorea occurring in cases of giant cell arteritis is unusual. Giant cell arteritis is a disorder resulting in systemic vasulitis particularly involving the cranial- vessels. The sudden onset and stepwise pattern of chorea in this patient was most likely due to cerebral vessels involvement. Giant cell artorizis does not affect the life span4 but in this patient the course was quickly progressive, inspitc of steroids. Thus chorea in giant disease may be resistant to conventional doses of steroids.

References

1. Green Home. A, H and Albuquerque, N M On chorea, Lupus Erythan- atosus and cerebral arteritis. Arch. ln(cm. Med., 1966-117:389-393.
2. Paradise. J.L. Sydenham\\\'s chores without evidence of rheumatic fever. N. Engl. J. Mcd., 1960;263;13:625-629.
3. Hearn, OW. and Roper-Hall, MJ. Obstruction of the central retinal artery associated with chorea. Br. Med. J., 1961;2:684-86.
4. Westhcrall, D.J., Ledingham, J.G.G. and Worrell, D A. Oxford Textbook of Medicine, Ed: 2 English Language Book Society. New York, Oxford University Press, 1987. pp. 16-153.

Journal of the Pakistan Medical Association has agreed to receive and publish manuscripts in accordance with the principles of the following committees: