Case Report Herzschr Elektrophys 2015 · 26:300–302 DOI 10.1007/s00399-015-0380-8 Received: 26 May 2015 Accepted: 1 June 2015 Published online: 4 July 2015 © Springer-Verlag Berlin Heidelberg 2015
R. Rivinius1 · F.F. Darche1 · B. Campos2 · A. Unterberg2 · L. Schweizer3 · D. Thomas1 · H.A. Katus1 · P.A. Schweizer1 1 Department of Cardiology, Angiology and Pulmology, University of Heidelberg, Heidelberg, Germany 2 Department of Neurosurgery, University of Heidelberg, Heidelberg, Germany 3 Department of Neuropathology, Institute of Pathology, University of Heidelberg, Heidelberg, Germany
It’s all in your head: sinus node dysfunction secondary to a sphenoid wing meningioma Medical history A 57-year-old man presented at our clinic with recurrent episodes of dizziness, weakness of his legs, and presyncope without loss of consciousness. The symptoms had started 2 years ago and exacerbated during the weeks prior clinic admission. Medical history revealed no significant disorders, and the patient denied chest pain, shortness of breath, or palpitations. However, he complained of progressive fatigue and loss of physical capacity.
Observations The resting electrocardiogram (ECG) showed low heart rates (45–50/min) and 24-h Holter recording displayed pauses up to 4.3 s (. Fig. 1). Echocardiography revealed normal left ventricular ejection fraction and no structural or valvular heart disease. An exercise stress test exhibited regular chronotropic response without signs of ischemia. Medication included pantoprazole (reflux disease) and budesonide/formoterol (asthma) but did not comprise drugs that could explain the bradycardia. Pronounced limb weakness prompted neurological consultation. Cranial magnetic resonance imaging revealed a large right-sided medial intracranial tumor surrounded by edema adjacent to the medial sphenoid wing, suggestive of a meningioma (. Fig. 2).
300 | Herzschrittmachertherapie + Elektrophysiologie 3 · 2015
Fig. 1 9 Diagnosis of sinus node dysfunction by 24-h Holter recording. a Episode with recurrent pauses caused by intermittent sinus arrest. b Detail of a, showing a 4.3 s pause
Abstract · Zusammenfassung
Therapy and its course Surgical removal was recommended according to current guidelines, and the case was discussed in our interdisciplinary board conference with respect to the concomitant sinus node dysfunction (SND). Regarding the possibility that SND could be caused by the brain tumor, we decided to apply a transient cardiac pacemaker perioperatively, while decision upon permanent pacemaker implantation was postponed. Surgery was performed successfully, and the tumor could completely be removed without complications. Histopathology of several biopsies provided evidence of a meningothelial meningioma WHO grade I (. Fig. 3). Continuous postoperative ECG surveillance displayed regular sinus rhythm, and 24-h Holter recording 2 days later showed a normal rate profile without pauses. The transient pacemaker could be removed, and the patient was discharged from hospital after recovery. On follow-up 8 weeks later, the patient did not complain any discomfort and denied presyncope/syncope. An additional 24-h Holter recording confirmed the absence of SND.
Discussion Distinguishing cardiogenic or neurogenic etiology of syncope is often challenging and may be complicated by the notion that primary neurological conditions can affect cardiac rhythm disorders and vice versa. Here, we report on a patient with symptomatic SND, who would require permanent pacemaker implan tation in the absence of a reversible etiology. However, with respect to an additional limb weakness neurological assessment was undertaken and unraveled a large meningioma as underlying medical problem. Patients suffering from intracranial tumors usually present with diverse symptoms including headache, nausea, vomiting, seizures, or neurological deficits but also dizziness and presyncope/syncope [1]. Of note, sporadic episodes of bradycardia and cardiac asystole have been previously described in association with meningiomas in an animal study [2]. The central nervous system plays a pivot al role for the regulation of cardiac rate
Herzschr Elektrophys 2015 · 26:300–302 DOI 10.1007/s00399-015-0380-8 © Springer-Verlag Berlin Heidelberg 2015 R. Rivinius · F.F. Darche · B. Campos · A. Unterberg · L. Schweizer · D. Thomas · H.A. Katus · P.A. Schweizer
It’s all in your head: sinus node dysfunction secondary to a sphenoid wing meningioma Abstract Background. A 57-year-old man presented with recurrent episodes of dizziness, weakness of legs, and presyncope. The electrocardiogram showed a sinus bradycardia and recurrent sinus pauses. Results. Cardiac evaluation revealed a normal left ventricular ejection fraction without ischemic, structural, or valvular heart disease. Pronounced limb weakness prompted neurological consultation. Cranial magnetic resonance imaging showed a large right-sided intracranial tumor adjacent to the medial sphenoid wing. Surgical removal of the tumor was accomplished successfully after application of a transient cardiac pacemaker, while deci-
sion upon permanent pacemaker implantation was postponed. Histopathology provided evidence of a meningothelial meningioma. Postoperative assessment displayed the absence of sinus node dysfunction after tumor removal. Conclusion. Careful differential diagnostic assessment of patients with symptomatic bradycardias needs to rule out reversible causes before implantation of permanent devices. Keywords Sinus node dysfunction · Bradycardia · Meningioma · Cardiac pacemaker
Sekundäre Sinusknotendysfunktion verursacht durch ein Keilbeinmeningeom Zusammenfassung Hintergrund. Ein 57-jähriger Patient stellte sich mit wiederkehrendem Schwindel, Beinschwäche und Präsynkopen vor. Elektrokar diographisch zeigte sich eine Sinusbradykar die mit rezidivierenden Sinuspausen. Ergebnisse. Die kardiologische Abklärung ergab eine normale linksventrikuläre Ejektionsfraktion ohne Anhalt für eine ischämische, strukturelle oder valvuläre Herzerkrankung. Aufgrund der Beinschwäche wurde ein kraniales Magnetresonanztomogramm veranlasst, das einen rechtsseitig gelegenen intrakraniellen Tumor in Nachbarschaft zum medialen Keilbeinflügel zeigte. Der Tumor konnte unter perioperativer Versorgung mit einem passageren Herzschrittmacher erfolgreich
and rhythm and large, space-occupying meningiomas can compromise cerebral structures. Tumor-induced compression may lead to dysfunction of the autonomic nervous system, which can result in vari ous types of arrhythmias. Likewise tem poral or frontal lobe seizures have been associated with bradycardia and cardiac arrest and electrical stimulation of the cingulated gyrus or orbitofrontal cortex was reported to affect heart rate [3]. In our patient, SND was found prior to surgical removal of the meningioma but not after the procedure. Hence, we suggest that tumor
entfernt werden, die Entscheidung bezüglich eines permanenten Schrittmachersystems wurde zurückgestellt. Die histopathologische Analyse erbrachte die Diagnose eines meningothelialen Meningeoms. Postoperativ war keine Sinusknotendysfunktion mehr nachweisbar. Schlussfolgerung. Bei unklaren symptoma tischen Bradykardien ist eine sorgfältige dif ferentialdiagnostische Abklärung wichtig, um reversible Ursachen vor Implantation permanenter Geräte auszuschließen. Schlüsselwörter Sinusknotendysfunktion · Bradykardie · Meningeom · Herzschrittmacher
compression may have resulted in an increased vagal tone leading to a reversible form of SND. In this regard, Freeman et al. [2] described a meningioma in a dog associated with sinus bradycardia and sinus arrest. Return to normal heart rhythm could be achieved by intravenous administration of atropine. These findings point to an increased vagal tone that ultimately caused SND, providing an animal model for the underlying rhythm problem of our case.
Herzschrittmachertherapie + Elektrophysiologie 3 · 2015
| 301
Case Report
Fig. 2 9 Diagnosis of the intracranial tumor by magnetic resonance imaging. T2-weighted images reveal a hyperintense large right medial sphenoid wing meningioma characterized by a sharply defined tumor margin and surrounding edema. a coronal, b sagittal, and c axial views Acknowledgments. We are indebted to the patient who gave written informed consent for publication of the data.
Compliance with ethical guidelines Conflicts of interest. R. Rivinius, F.F. Darche, B. Campos, A. Unterberg, L. Schweizer, D. Thomas, H.A. Katus, and P.A. Schweizer state that there are no conflicts of interest. This article does not contain studies on humans or animals.
Fig. 3 9 Histopathology of the meningothelial meningioma WHO grade I. a The tumor cells form lobules, partly demarcated by thin collagenous septae, and demonstrate a pseudosyncytial growth pattern. The nuclei are uniform, round to oval shaped, and occasionally central clearing can be observed (Hematoxylinand-eosin stain; magnification × 200); b Proliferation is low, Antigen-Ki-67 is up to 3 % (Antigen-Ki-67 stain, magnification × 200)
Conclusion
Corresponding address
The present case illustrates the requirement for careful differential diagnostic assessment of patients with symptomatic bradycardias. Reversible causes need to be ruled out before implantation of permanent devices.
302 | Herzschrittmachertherapie + Elektrophysiologie 3 · 2015
P.A. Schweizer MD Department of Cardiology Angiology and Pulmology University of Heidelberg, Heidelberg [email protected]
References 1. Whittle IR, Smith C, Navoo P, Collie D (2004) Meningiomas. Lancet 363:1535–1543 2. Freeman KP, Monlux AW, Heald D, Sealock MC (1985) Bradycardia associated with meningioma in a dog. J Am Vet Med Assoc 187:838–839 3. Locatelli ER, Varghese JP, Shuaib A, Potolicchio SJ (1999) Cardiac asystole and bradycardia as a manifestation of left temporal lobe complex partial seizure. Ann Intern Med 130:581–583
R. Rivinius1 · F.F. Darche1 · B. Campos2 · A. Unterberg2 · L. Schweizer3 · D. Thomas1 · H.A. Katus1 · P.A. Schweizer1 1 Department of Cardiology, Angiology and Pulmology, University of Heidelberg, Heidelberg, Germany 2 Department of Neurosurgery, University of Heidelberg, Heidelberg, Germany 3 Department of Neuropathology, Institute of Pathology, University of Heidelberg, Heidelberg, Germany
It’s all in your head: sinus node dysfunction secondary to a sphenoid wing meningioma Medical history A 57-year-old man presented at our clinic with recurrent episodes of dizziness, weakness of his legs, and presyncope without loss of consciousness. The symptoms had started 2 years ago and exacerbated during the weeks prior clinic admission. Medical history revealed no significant disorders, and the patient denied chest pain, shortness of breath, or palpitations. However, he complained of progressive fatigue and loss of physical capacity.
Observations The resting electrocardiogram (ECG) showed low heart rates (45–50/min) and 24-h Holter recording displayed pauses up to 4.3 s (. Fig. 1). Echocardiography revealed normal left ventricular ejection fraction and no structural or valvular heart disease. An exercise stress test exhibited regular chronotropic response without signs of ischemia. Medication included pantoprazole (reflux disease) and budesonide/formoterol (asthma) but did not comprise drugs that could explain the bradycardia. Pronounced limb weakness prompted neurological consultation. Cranial magnetic resonance imaging revealed a large right-sided medial intracranial tumor surrounded by edema adjacent to the medial sphenoid wing, suggestive of a meningioma (. Fig. 2).
300 | Herzschrittmachertherapie + Elektrophysiologie 3 · 2015
Fig. 1 9 Diagnosis of sinus node dysfunction by 24-h Holter recording. a Episode with recurrent pauses caused by intermittent sinus arrest. b Detail of a, showing a 4.3 s pause
Abstract · Zusammenfassung
Therapy and its course Surgical removal was recommended according to current guidelines, and the case was discussed in our interdisciplinary board conference with respect to the concomitant sinus node dysfunction (SND). Regarding the possibility that SND could be caused by the brain tumor, we decided to apply a transient cardiac pacemaker perioperatively, while decision upon permanent pacemaker implantation was postponed. Surgery was performed successfully, and the tumor could completely be removed without complications. Histopathology of several biopsies provided evidence of a meningothelial meningioma WHO grade I (. Fig. 3). Continuous postoperative ECG surveillance displayed regular sinus rhythm, and 24-h Holter recording 2 days later showed a normal rate profile without pauses. The transient pacemaker could be removed, and the patient was discharged from hospital after recovery. On follow-up 8 weeks later, the patient did not complain any discomfort and denied presyncope/syncope. An additional 24-h Holter recording confirmed the absence of SND.
Discussion Distinguishing cardiogenic or neurogenic etiology of syncope is often challenging and may be complicated by the notion that primary neurological conditions can affect cardiac rhythm disorders and vice versa. Here, we report on a patient with symptomatic SND, who would require permanent pacemaker implan tation in the absence of a reversible etiology. However, with respect to an additional limb weakness neurological assessment was undertaken and unraveled a large meningioma as underlying medical problem. Patients suffering from intracranial tumors usually present with diverse symptoms including headache, nausea, vomiting, seizures, or neurological deficits but also dizziness and presyncope/syncope [1]. Of note, sporadic episodes of bradycardia and cardiac asystole have been previously described in association with meningiomas in an animal study [2]. The central nervous system plays a pivot al role for the regulation of cardiac rate
Herzschr Elektrophys 2015 · 26:300–302 DOI 10.1007/s00399-015-0380-8 © Springer-Verlag Berlin Heidelberg 2015 R. Rivinius · F.F. Darche · B. Campos · A. Unterberg · L. Schweizer · D. Thomas · H.A. Katus · P.A. Schweizer
It’s all in your head: sinus node dysfunction secondary to a sphenoid wing meningioma Abstract Background. A 57-year-old man presented with recurrent episodes of dizziness, weakness of legs, and presyncope. The electrocardiogram showed a sinus bradycardia and recurrent sinus pauses. Results. Cardiac evaluation revealed a normal left ventricular ejection fraction without ischemic, structural, or valvular heart disease. Pronounced limb weakness prompted neurological consultation. Cranial magnetic resonance imaging showed a large right-sided intracranial tumor adjacent to the medial sphenoid wing. Surgical removal of the tumor was accomplished successfully after application of a transient cardiac pacemaker, while deci-
sion upon permanent pacemaker implantation was postponed. Histopathology provided evidence of a meningothelial meningioma. Postoperative assessment displayed the absence of sinus node dysfunction after tumor removal. Conclusion. Careful differential diagnostic assessment of patients with symptomatic bradycardias needs to rule out reversible causes before implantation of permanent devices. Keywords Sinus node dysfunction · Bradycardia · Meningioma · Cardiac pacemaker
Sekundäre Sinusknotendysfunktion verursacht durch ein Keilbeinmeningeom Zusammenfassung Hintergrund. Ein 57-jähriger Patient stellte sich mit wiederkehrendem Schwindel, Beinschwäche und Präsynkopen vor. Elektrokar diographisch zeigte sich eine Sinusbradykar die mit rezidivierenden Sinuspausen. Ergebnisse. Die kardiologische Abklärung ergab eine normale linksventrikuläre Ejektionsfraktion ohne Anhalt für eine ischämische, strukturelle oder valvuläre Herzerkrankung. Aufgrund der Beinschwäche wurde ein kraniales Magnetresonanztomogramm veranlasst, das einen rechtsseitig gelegenen intrakraniellen Tumor in Nachbarschaft zum medialen Keilbeinflügel zeigte. Der Tumor konnte unter perioperativer Versorgung mit einem passageren Herzschrittmacher erfolgreich
and rhythm and large, space-occupying meningiomas can compromise cerebral structures. Tumor-induced compression may lead to dysfunction of the autonomic nervous system, which can result in vari ous types of arrhythmias. Likewise tem poral or frontal lobe seizures have been associated with bradycardia and cardiac arrest and electrical stimulation of the cingulated gyrus or orbitofrontal cortex was reported to affect heart rate [3]. In our patient, SND was found prior to surgical removal of the meningioma but not after the procedure. Hence, we suggest that tumor
entfernt werden, die Entscheidung bezüglich eines permanenten Schrittmachersystems wurde zurückgestellt. Die histopathologische Analyse erbrachte die Diagnose eines meningothelialen Meningeoms. Postoperativ war keine Sinusknotendysfunktion mehr nachweisbar. Schlussfolgerung. Bei unklaren symptoma tischen Bradykardien ist eine sorgfältige dif ferentialdiagnostische Abklärung wichtig, um reversible Ursachen vor Implantation permanenter Geräte auszuschließen. Schlüsselwörter Sinusknotendysfunktion · Bradykardie · Meningeom · Herzschrittmacher
compression may have resulted in an increased vagal tone leading to a reversible form of SND. In this regard, Freeman et al. [2] described a meningioma in a dog associated with sinus bradycardia and sinus arrest. Return to normal heart rhythm could be achieved by intravenous administration of atropine. These findings point to an increased vagal tone that ultimately caused SND, providing an animal model for the underlying rhythm problem of our case.
Herzschrittmachertherapie + Elektrophysiologie 3 · 2015
| 301
Case Report
Fig. 2 9 Diagnosis of the intracranial tumor by magnetic resonance imaging. T2-weighted images reveal a hyperintense large right medial sphenoid wing meningioma characterized by a sharply defined tumor margin and surrounding edema. a coronal, b sagittal, and c axial views Acknowledgments. We are indebted to the patient who gave written informed consent for publication of the data.
Compliance with ethical guidelines Conflicts of interest. R. Rivinius, F.F. Darche, B. Campos, A. Unterberg, L. Schweizer, D. Thomas, H.A. Katus, and P.A. Schweizer state that there are no conflicts of interest. This article does not contain studies on humans or animals.
Fig. 3 9 Histopathology of the meningothelial meningioma WHO grade I. a The tumor cells form lobules, partly demarcated by thin collagenous septae, and demonstrate a pseudosyncytial growth pattern. The nuclei are uniform, round to oval shaped, and occasionally central clearing can be observed (Hematoxylinand-eosin stain; magnification × 200); b Proliferation is low, Antigen-Ki-67 is up to 3 % (Antigen-Ki-67 stain, magnification × 200)
Conclusion
Corresponding address
The present case illustrates the requirement for careful differential diagnostic assessment of patients with symptomatic bradycardias. Reversible causes need to be ruled out before implantation of permanent devices.
302 | Herzschrittmachertherapie + Elektrophysiologie 3 · 2015
P.A. Schweizer MD Department of Cardiology Angiology and Pulmology University of Heidelberg, Heidelberg [email protected]
References 1. Whittle IR, Smith C, Navoo P, Collie D (2004) Meningiomas. Lancet 363:1535–1543 2. Freeman KP, Monlux AW, Heald D, Sealock MC (1985) Bradycardia associated with meningioma in a dog. J Am Vet Med Assoc 187:838–839 3. Locatelli ER, Varghese JP, Shuaib A, Potolicchio SJ (1999) Cardiac asystole and bradycardia as a manifestation of left temporal lobe complex partial seizure. Ann Intern Med 130:581–583
