Chagas Disease and Stroke: A neglected disease and a social dilemma
By Dr Vinícius Viana Abreu Montanaro MD MsC PhD, Neurologist, SARAH Network of Rehabilitation Hospitals, Brasilia, Brazil
Chagas disease is a vector-borne disease with multi-organ involvement, these include cardiac, gastrointestinal, and cerebral manifestations1. It is still considered a neglected disease worldwide1. Carlos Chagas originally described the disease in 1909 while investigating a malaria surge in Lassance, a province in Minas Gerais state, Brazil1.
The disease can initially manifest as an acute febrile illness characterized by fever, headache, facial edema, and the classic Romaña sign, which can last between 6 and 12 weeks2. A chronic form of infection can occur in approximately 40% of cases. This form is characterized by cardiac involvement including arrhythmias and cardiomyopathy2.
The disease is caused by the infection of the parasite Trypanosoma cruzi. Over 16 million people are infected worldwide3. There is growing concern regarding Chagas disease prevalence, especially in the developed world, considering the changes in migration flows. It is estimated over 300,00 people are infected in the United States alone4.
There is a well-described correlation between Chagas Disease and ischemic stroke, although large epidemiological data and treatment guidelines, especially secondary prevention, are still lacking5. This is a particular problem, considering most cases are of cardioembolic origin, due to direct infestation of the cardiac tissue by the parasite, and the fact that the disease has no approved specific treatment for its chronic form, which usually is the period when the strokes happen6.
Despite the majority of cardioembolic strokes, cases of IS in patients without cardiac involvement have also been reported. It has been recently found that the SSS/CCS TOAST classification is superior in identifying the cardioembolic etiologies4. Individual factors such as the initial modified Rankin scale show a significant correlation with increased mortality and recurrence6. Also, recent recommendations of secondary prophylaxis have been questioned due to conflicting evidence, such as the embolic score IPEC\FIOCRUZ, which was proven not to be effective in adequately identifying the patient at higher risk of recurrence of IS due to cardioembolism, which would warrant anticoagulation as secondary prophylaxis4.
A recent machine learning algorithm was deemed the best tool for the etiological classification of these cases5. Although acute treatment does not change due to this particular etiology, neurovascular physicians need to recognize these neglected patients due to the need for improvement of management for secondary prevention (anticoagulation) and continuous cardiologic follow-up.
The presence of epidemiological and clinical data suggestive of CD should always alert the possibility of the diagnosis7. This usually happens in patients with a reduced systolic function with no apparent cause in a patient from an endemic region. Other cardiac abnormalities include arrhythmias(atrial fibrillation or flutter), segmental hypokinesis, and cardiac insufficiency syndrome with no known cause7.
1 Chagas C (1909) New human trypanosomiasis: studies in the morphology and the evolutionary cycle of the Schizotrypanum cruzi n. Gen., n. SP Etiologic Agent of a new morbid entity in man. Mem Inst Oswaldo Cruz 1:159–218
2 F.J. Carod-Artal, J. Gascon J, Chagas disease and stroke, Lancet Neurol, (2010) 533–542, 9
3 Montanaro VVA, Hora TF, Da Silva CM, et al. Epidemiology of concurrent Chagas disease and ischemic stroke in a population attending a multicenter quaternary rehabilitation network in Brazil. Neurol Sci 2019;40:2595-2601.
4 Montanaro VVA, Da Silva CM, De Viana Santos CV, et al. Ischemic stroke classification and risk of embolism in Chagas disease. J Neurol 2016;263:2411-2415.
5 Montanaro VVA, Hora TF, Guerra AA, et al.Artificial Intelligence-Based Decision for the Prediction of Cardioembolism in Patients with Chagas Disease and Ischemic Stroke. Journ of stroke and cerebrovasc dis. Vol. 30, No. 10 (October), 2021: 106034
6 Montanaro VVA, Hora TF, Da Silva CM, et al. Mortality and stroke recurrence in a rehabilitation cohort of patients with cerebral infarcts and Chagas disease. Eur Neurol 2018;79:177-184.
7 Oliveira-Filho J, Viana LC, Vieira de Melo RM, Faiçal F, Torreão JA, Villar FA, Reis FJ (2005) Chagas disease is an independent risk