A brain–heart interaction: Bickerstaff’s brainstem encephalitis with takotsubo cardiomyopathy

Takotsubo cardiomyopathy should be considered in hypotensive patients with acute neurological diseases, such as Bickerstaff brainstem encephalitis. Early identification of this condition allows for institution of appropriate blood pressure support treatment and prompt initiation of IVIG. Outcomes of Takotsubo cardiomyopathy in neurological conditions are generally good. Bickerstaff’s brainstem encephalitis (BBE) is a neurological condition characterized by ataxia, opthalmoplegia and altered sensorium. Neurological diseases, such as Guillain-Barre syndrome, have previously been reported in association with Takotsubo cardiomyopathy. We describe a case of Takotsubo cardiomyopathy associated with BBE. A 62-year-old woman with no past medical history presented to our hospital with an unwitnessed fall precipitated by transient lower limb numbness. She also had coryzal symptoms in the week prior to admission. On examination, she was oriented and hemodynamically stable. Neurological examination was normal. Her electrocardiogram showed normal sinus rhythm with normal R progression. No Q waves were present to suggest underlying ischemic heart disease. A day after initial presentation, she developed altered sensorium and deteriorated rapidly within hours. She had bulbar weakness, increasing oxygen requirements necessitating intubation and hypotension requiring inotropic support. Her pupils were mydriatic at 5 mm bilaterally with a sluggish pupillary response. She had brisk reflexes, poor gag and cough reflexes, ataxia, bilateral ptosis and complex ophthalmoplegia. Cerebrospinal fluid analysis showed three white blood cells, normal protein and glucose levels and no cytoalbuminologic dissociation. Bacterial cultures and a viral encephalitis panel were negative. Magnetic resonance imaging revealed a gadolinium-enhancing lesion in the brainstem on T1 sequence (Figure 1). Anti-GQ1b antibody was positive. Electroencephalography did not show any epileptiform discharge and nerve conduction studies showed no features of demyelination. A diagnosis of Bickerstaff brainstem encephalitis was made based on positive magnetic resonance imaging findings and suggestive clinical presentation of altered sensorium, bulbar weakness and ataxia. Gadolinium-enhancing brainstem lesion on T1 supportive of BBE given clinical context (left); apical ballooning and akinesia of the left ventricular apex during end-systole (right). Gadolinium-enhancing brainstem lesion on T1 supportive of BBE given clinical context (left); apical ballooning and akinesia of the left ventricular apex during end-systole (right). A comprehensive work-up for hypotension was conducted. This included a cardiac evaluation. The patient’s troponin level was elevated at 2137 pg/ml (normal upper limit 15.6 pg/ml). An electrocardiogram showed atrial fibrillation with rapid ventricular response, and this was aborted with amiodarone. However, the hypotension persisted. A transthoracic echocardiogram showed a depressed ejection fraction of 25% and classical systolic ballooning and akinesia of the left ventricular apex (Figure 1) consistent with that of Takotsubo Cardiomyopathy. Not all cases of BBE present with the classical triad of ophthalmoplegia, ataxia and altered sensorium. Other possible BBE features include flaccid symmetrical tetraparesis, dysarthria and hyper-/hypo-reflexia. Takotsubo cardiomyopathy is characterized by transient regional systolic dysfunction resulting in left ventricular ballooning, in the absence of obstructive coronary artery disease. It has been increasingly reported in various neurological diseases including status epilepticus, subarachnoid hemorrhage and Guillian–Barré syndrome.1,2 While autonomic dysfunction remains an important differential of hemodynamic instability in patients falling under the anti-Qq1b spectrum, it ought to be differentiated from that of Takotsubo cardiomyopathy as the treatment management differs. About 10% of those with Takotsubo cardiomyopathy develop cardiogenic shock. It is postulated that sympathetic over-excitation of the brain in neurological diseases result in excessive catecholamines which drive the hyperdynamic basal contraction and apical systolic dysfunction of the heart.3 Thus, inotropes, such as adrenaline/noradrenaline are to be used with caution as they can potentially be counterproductive due to the underlying pathogenesis. Adequate filling pressures would need to be ensured. Alternative blood pressure supporting measures such to dobutamine, levosimendan, enoximone and an intra-aortic balloon pump should be considered.4,5 The patient was treated with a 5-day course of intravenous immunoglobulin (2 g/kg) with good functional recovery thereafter. Heart failure management with diuretics, angiotensin converting enzyme -inhibitors and beta-blockers was instituted. Interval echocardiography postintravenous immunoglobulin showed improvement in apical ballooning of the left ventricle. The prognosis of BBE is generally good with most patients making a complete recovery.6 While there are rare cases of Takotsubo cardiomyopathy resulting in lethal arrhythmias and acute heart failure, early recognition of BBE and its possible association with Takotsubo cardiomyopathy could prompt early echocardiography and early institution of IVIG as deemed necessary. Conflict of interest: None declared.