Case Report: Takotsubo Cardiomyopathy in Bickerstaff Brainstem Encephalitis Triggered by COVID-19

Abstract

Takotsubo cardiomyopathy (TCM) is a stress-induced cardiomyopathy triggered by critical illness including severe neurological disorders. However, an association between TCM and Bickerstaff brainstem encephalitis (BBE) has rarely been described. During the current coronavirus disease 2019 (COVID-19) pandemic, growing evidence indicates that COVID-19 often leads to various neurological disorders, but there are few reports of an association between COVID-19 and BBE. Here we report a case of TCM associated with BBE triggered by COVID-19, which subsided with immunotherapy for BBE. Both transthoracic echocardiography and electrocardiography led to early and accurate diagnosis of TCM. Sustained hemodynamic instability due to TCM was immediately lessened with immunotherapy whereas additional plasmapheresis and immunotherapy were required to treat BBE. This case indicates that BBE might follow COVID-19 and TCM should be considered when hemodynamic status remains unstable in a patient with BBE.

Keywords: Bickerstaff brainstem encephalitis; Takotsubo cardiomyopathy; anti-GQ1b ganglioside antibody; coronavirus disease 2019; hemodynamic instability; intravenous immunoglobulin therapy; transthoracic echocardiogram.

Figure 1

Brain magnetic resonance imaging (MRI) and electrophysiological tests. (A) Chest computed tomography detected possible COVID-19 pneumonia on admission. (B) Axial brain MRI diffusion weighted imaging (DWI) and fluid-attenuated inversion recovery (FLAIR) images on admission. No abnormalities were detected. (C) Nerve conduction study on the 6th day from onset (i.e., hospital day 2). The motor nerve conduction study revealed a slight reduction in compound muscle action potential amplitudes and no conduction block with an apparent increase of stimulation threshold. The orthodromic sensory nerve conduction study showed a reduction in sensory nerve action potentials. F-wave examination exhibited no responses in the upper and lower extremities. CMAP, compound muscle action potential; SNAP, sensory nerve action potential. (D) Blink reflex test on day 7 (i.e., hospital day 3) showed bilateral loss of both R1 and R2 with bilateral trigeminal nerve stimulation. (E) Median nerve somatosensory evoked potential testing on day 6 (i.e., hospital day 2). P13/14 and N18 were normally evoked, whereas N20 was lost. These results suggested interruption of the somatosensory pathway in the brainstem. Ai, the ipsilateral earlobe. CPc and CPi, the centroparietal electrode contralateral and ipsilateral to the stimulation, respectively. C2S, the spinous process over the second cervical spine. Fz, the midline frontal electrode. NC, non-cephalic reference on the contralateral shoulder. (F) Standard 12-lead electrocardiography on admission (day 5) demonstrated inverted T-waves in leads I, II, aVF, and V1–V6. (G) Transthoracic echocardiography on admission (day 5). Images in end-diastole (left) and end-systole (right) revealed segmental wall motion abnormalities with apical akinesis (arrows) and hyperkinesis in the basal segments, which was compatible with Takotsubo cardiomyopathy.

Figure 2

Clinical course. Clinical and treatment course. The horizontal axis represents the day from onset. BNP, B-type natriuretic peptide; CK, creatine kinase; EOM, extraocular movement; GCS, Glasgow Coma Scale; IVIG, intravenous immunoglobulin therapy (400 mg/kg/day for 5 consecutive days); IVMP, intravenous methylprednisolone pulse therapy (1 g/day for 3 consecutive days); mRS, modified Rankin scale (0, no symptoms; 1, no significant disability; 2, slight disability, able to look after own affairs without assistance, but unable to carry out all previous activities; 3, moderate disability, require some help, but able to walk unassisted; 4, moderately severe disability, unable to attend to own bodily needs without assistance, and unable to walk unassisted; 5, severe disability, require constant nursing care and attention, bedridden, incontinent); NAd, noradrenaline administration; PE, plasma exchange; TnI, troponin I.