Echocardiographic evaluation of patients with subacute sclerosing panencephalitis

Abstract

Objective: Subacute sclerosing panencephalitis is a slowly progressive, inflammatory and neurodegenerative disease caused by virus infection in the central nervous system. Since there are a limited number of studies in the literature evaluating the cardiovascular functions of patients with SSPE, the present study evaluates the patients with SSPE using tissue Doppler echocardiography and compares them between the control group in order to shed some light on the subject. Methods: The study is a prospective observational study. 49 patients (17 female, 32 male) with SSPE were included in the study. Patients were divided into two groups: Stage 2 (n=29) and Stage 3 (n=20). Echocardiographic data were compared with a control group of 26 which is the same average age. All children underwent a detailed echocardiography, which contained an M-mode, pulse Doppler and tissue Doppler imaging. Results: Sinus tachycardia ( >100 beats/min in children) was detected in nineteen (38.7%) patients. There were not significant differences between parameters of systolic and diastolic function of the heart. Stage 2 group, EF: 69.9±6.4; SF: 39.2±5.58; and MPI (mitral): 0.38±0.03 and MPI (tricuspid): 0.39±0.10. And in the Stage 3 group, EF: 68.5±7.0, SF: 37.8±5.34, MPI (mitral): 0.37±0.09 and MPI (tricuspid): 0.38±0.12. In the control group EF:70.96±5.54; SF:39.96±5.05 and MPI(mitral): 0.35±0.06 MPI (tricuspid):0.36±0.04 and statistically meaningful differences were not found between patients and control groups (p >0.05). Conclusion: Cardiac functions may be preserved and cardiac functions constitute no significant risks of mortality in the advanced stages of patients with Subacute sclerosing panencephalitis, which is a group of chronic and bedridden patients.

Keywords

• Subacute Sclerosing Panencephalitis, echocardiography, cardiac function

Subakut sklerozan panensefalitli hastaların ekokardiyografik değerlendirilmesi

Abstract

Amaç: Subakut sklerozan panensefalit (SSPE), santral sinir sisteminin yavaş virüs enfeksiyonu sonucu ortaya çıkan kronik, progressif dejeneratif bir hastalığıdır. Bu hasta grubunun kalp fonksiyonları ile ilgili çalışmalar kısıtlı olduğu için bu konuya ışık tutmak amacıyla farklı evrelerdeki hastaların kalp fonksiyonları değerlendirilmiştir. Yöntemler: Çalışma prospektif bir çalışma şeklinde olup, Çocuk Nörolojisi polikliniğinde takip edilen 49 SSPE olgusu (17 kız, 32 erkek) çalışmaya alındı. Hastalar evre 2(n=29) ve evre 3(n=20) olarak iki gruba ayrıldı. Ekokardiyografik veriler yaş ortalaması aynı olan 26 kontrol grubu ile karşılaştırıldı. M- mode, pulse Doppler ve doku Doppler ekokardiyografik incelemeler ve gruplara uygulandı. Bulgular: Sinüs taşikardisi (100 atım/dak üzeri)19 hastada ( %37,8) mevcuttu. Kalbin sistolik ve diyastolik fonksiyon parametreleri arasında anlamlı farklılık yoktu. Evre 2 SSPE grubunda, EF: 69.9±6.4, FS: 39.2±5.58, MPI(mitral):0.38±0.03, MPI(triküspid): 0.39±0.10, Evre 3 grubunda, EF: 68.5±7.00, FS: 37.8±5.34, MPI(mitral):0.37±0.09, MPI(triküspid):0.38±0.12, Kontrol grubunda EF:70.96±5.54, FS:39.96±5.05, MPI(mitral):0.35±0.06, MPI (triküspid):0,36±0,04 olarak bulundu. Farklı evrelerde bakılan hastaların ekokardiyografik verileri kontrol grubu ile karşılaştırıldığında istatistiki olarak anlamlı farklılık bulunmamıştır (p > 0,05). Sonuç: Bu çalışma kalp fonksiyonlarının ileriki dönemlere kadar korunabileceğini, kronik ve yatağa bağımlı bir hastalık olan SSPE de bu durumun mortalite açısından önemli bir risk oluşturmayacağını göstermektedir.

Keywords

• Subakut sklerozan panensefalit, ekokardiyografi, kardiyak fonksiyon

References

1. Dyken PR. Subacute sclerosing panencephalitis. In : Swaiman KF, ed. Pediatric neurology. Principles and Prac- tice. 1989 St. Louis: The C. V Mosby Company 499-501.
2. Malhotra HS, Garg RK, Naphade P. Cluster of partial mo- tor seizures heralding the onset of hemimyoclonic subacute sclerosing panencephalitis. Mov Disord 2012;27:958-959.
3. İrdem A, Ecer S, Özbek MN, et al. Subakut Sklerozan Pan- ensefalit hastalarının epidemiyolojik özellikleri. Dicle Tıp Dergisi 2004;1:31-35.
4. Yakub BA. Subacute sclerosing panencephalitis. Early diag- nosis prognostic factors and natural history. J Neurol SCI 1996;139:227-234.
5. Khare S, Kumari S, Verghese T. Subacute sclerosing panen- cephalitis in Delhi. J Trop Pediatr 1994;40:326-328.
6. Risk WS, Haddad WS Chemali S. Substantial spontaneous long-term improvement in subacute sclerosing panenceph- alitis: six cases from the Middle East and a review of the literature. Arch Neurol 1978;35:494-502.
7. Meyer S, Lindinger A, Löffler G, et al. Mechanisms of dis- ease/hypothesis: Neurogenic left ventricular dysfunction and neurogenic pulmonary oedema. Wien Med Wochen- schr 2009;159:342-345.
8. Talman WT. Cardiovascular regulation and lesions of the central nervous system. Ann Neurol 1985;18:1-12.

9. Aydın OF, Karakurt C, Senocak F, et al. Heart rate variabil- ity and autonomic dysfunction in SSPE. Pediatr Neurol 2005;32:184-89.
10. Cui W, Roberson DA. Left ventricular Tei index in children: comparison of tissue Doppler imaging pulsed wave Dop- pler, and M-mode echocardiography normal values. J Am Soc Echocardiogr 2006;19:1438–1445.
11. Harada K, Orino T, Yasuoka K, et al. Tissue Doppler imag- ing of left and right ventricles in normal children. Tohoku J Exp Med 2000;191:21–29.
12. Eidem BJ, McMahon CJ, Cohen RR, et al. Impact of car- diac growth on Doppler tissue imaging velocities: a study in healthy children. J Am Soc Echocardiogr 2004;17:212– 221.
13. Meenakshi-Sundaram S, Taly AB, KamathV, et al. Auto- nomic dysfunction in Wilson’s disease-a clinical and elec- trophysiological study. Clin Auton Res 2002;12:181-189.
14. Tham EBC, Silverman NH. Measurement of the Tei index: a comparison of M-mode and pulsed Doppler methods. J Am Soc Echocardiogr 2004;17:259–265.
15. Sadaniantz A, Miller G, Hadi BJ, Parisi AF. Effects of left ventricular systolic function on left ventricular diastolic filling patterns in severe mitral regurgitation. Am J Cardiol 1997;79:1488-1492.
16. Oh JK, Seward JB, Tajik AJ. Assessment of diastolic func- tion and diastolic heart failure. In: Oh JK, Seward JB, Ta- jik AJ, eds 2006 The echo manual: Lippincott Williams Wilkins 121-142.
17. Harada K, Tamura M, Yasuoka K, Toyono M. A comparison of tissue Doppler imaging and velocities of transmitral flow in children with elevated left ventricular preload. Cardiol Young 2001; 11:261–268.
18. Lakoumentas JA, Panou FK, Kotseroglou VK, et al. The Tei index of myocardial performance: applications in cardiol- ogy. Hellenic J Cardiol 2005;46:52-58.
19. Tei C, Ling LH, Hodge DO, et al. New index of combined systolic and diastolic myocardial performance: a simple and reproducible measure of cardiac function a study in normal and dilated cardiomyopathy. J Cardiol 1995;26:357–366.