A brain-heart interaction: Bickerstaff's brainstem encephalitis with takotsubo cardiomyopathy.

Abstract

Bickerstaff's brainstem encephalitis (BBE) is a neurological condition characterised 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 Chinese female with no significant medical history was admitted after a fall due to transient bilateral lower limb numbness. A day after her initial presentation, she developed altered sensorium and further deteriorated within hours - with bulbar weakness, increasing oxygen requirements requiring intubation and cardiogenic shock needing noradrenaline support. She also had rapid atrial fibrillation treated with amiodarone. Her cardiac enzymes were elevated (high sensitivity troponin I 2137pg/ml; upper limit 15.6pg/ml) and echocardiography demonstrated apical ballooning and dyskinesis with hypercontractility of the base in the left ventricle consistent with Takotsubo cardiomyopathy. Examination revealed bulbar weakness with poor gag and cough reflexes, ataxia and complex opthalmoplegia. Her reflexes were brisk and pupils mydriatic with sluggish pupillary response. Magnetic resonance imaging revealed a gadolinium-enhancing lesion in the brainstem on T1 sequence and anti-GQ1b antibody was positive. She was diagnosed with BBE and treated with intravenous immunoglobulin with good functional recovery. Repeat echocardiography demonstrated interval improvement in apical ballooning. This is the first described case of Takotsubo cardiomyopathy associated with BBE. The pathogenesis of Takotsubo cardiomyopathy in relation to BBE is postulated to be related to dysautonomia with sympathetic overactivity and excessive catecholamine release. In cases of hemodynamic instability in BBE, Takotsubo cardiomyopathy should be considered.