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[Chorea as the presenting form of progressive systemic sclerosis]
M Seijo Martínez1, M Castro del Río, A Losada Campa
1Servicio de Neurología, Complexo Hospitalario de Pontevedra. mseijom@meditex.es
Insights
This study reports a rare case of hemichorea, a movement disorder, caused by ischemic stroke in a patient with progressive systemic sclerosis. Findings suggest primary cerebrovascular damage may be a direct complication of this condition.
Area of Science:
- Neurology
- Rheumatology
- Vascular Medicine
Background:
- Neurologic complications in progressive systemic sclerosis (PSS) are infrequent and can stem from various factors.
- Cerebrovascular disease has been occasionally reported in PSS, but its association with movement disorders remains undocumented.
Observation:
- A 66-year-old male with Raynaud's phenomenon and polyarthralgias presented with new-onset right-sided hemichorea.
- Clinical examination revealed skin changes consistent with PSS, elevated anticentromere antibody titer, and capillary abnormalities.
- Neurological imaging identified an infarct in the left caudate nucleus and internal capsule.
Findings:
- The patient's hemichorea was attributed to ischemic stroke, a previously unreported presentation of PSS.
- Brain SPECT revealed bilateral hypoperfusion, predominantly on the left side, correlating with the infarct location.
- The case supports the hypothesis that PSS can directly cause primary cerebrovascular damage.
Implications:
- This case highlights the potential for PSS to manifest with significant neurological deficits, including movement disorders.
- It underscores the importance of considering cerebrovascular complications in patients with PSS, even in the absence of typical vascular risk factors.
- Further research into the mechanisms of cerebrovascular involvement in PSS is warranted to improve patient outcomes.
Abstract:
We present an exceptional case of a patient with hemichorea of ischemic origin as a form of presentation of progressive systemic sclerosis. The neurologic complications, both central and peripheral, of this condition are considered infrequent and may be due to coexisting or iatrogenic factors, or may be a direct complication of progressive systemic sclerosis. There have been occasional reports of cerebrovascular disease in progressive systemic sclerosis, and this apparent rarity may be related to structural and morphological variations in brain arteries. Recently, there have been reports of primary cerebrovascular changes and vascular calcifications in progressive systemic sclerosis. There are no reported cases associated with movement disorders. The patient is a 66-year-old ex-smoker, and no other vascular risk factors, with chronic Raynaud's phenomenon affecting both hands, and polyarthralgias. Three months previously he began to complain of choreic movements affecting the right-sided limbs without any other neurological manifestations. The abnormal movements responded to haloperidol. There were skin changes suggestive of progressive systemic sclerosis, and capillaroscopy showed abundant avascular zones with dilated capillary loops. Anticentromere antibody titer was elevated to 1/1280. Brain arteriography was normal, and the brain SPECT showed bilateral hypoperfusion bilaterally, predominantly on the left side. Brain MR imaging confirmed an infarct in the left caudate and adjacent internal capsule. The study of this case supports the hypothesis of a direct primary cerebrovascular damage in progressive systemic sclerosis.