Acute encephalopathy with biphasic seizures and late reduced diffusion accompanied by Takotsubo cardiomyopathy.


Department of Neurology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Japan; Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan. Electronic address: [Email]


BACKGROUND : Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is characterized by biphasic seizures and impaired consciousness. Takotsubo cardiomyopathy (TTC), which is typically triggered by psychological or physical stress, is characterized by transient myocardial dysfunction affecting the left ventricular apex. Recent reports have suggested that seizures can also trigger TTC. However, no cases of TTC accompanied by AESD have been reported.
METHODS : A previously healthy 4-year-old girl was brought to a hospital with first-time febrile generalized tonic-clonic convulsions, which lasted approximately 40 min. After the seizure resolved, she was intubated due to respiratory deterioration. On the next day, her cardiac function deteriorated, and echocardiography revealed systolic apical ballooning of the left ventricle accompanied by hyperkinesis of the basal wall, which are typical in patients with TTC. Her condition gradually improved, and catecholamine support was tapered. However, 6 days after admission, she experienced a cluster of brief convulsions. Ten days after admission, head MRI revealed lesions with reduced diffusion throughout the cortex, except in the occipital lobe, as well as perirolandic sparing. Follow-up MRI 35 days after onset revealed whole-brain atrophy, following which she developed severe cognitive dysfunction.
CONCLUSIONS : Our patient developed TTC accompanied by features of AESD. Our findings may thus provide insight into the development of TTC and prompt further studies regarding the relationship between prolonged seizures and TTC.


Catecholamine,Creatine kinase,N-terminal pro-brain natriuretic peptide,Status epilepticus,Stress,Takotsubo syndrome,Troponin-T,