Cerebral amyloid accumulation–related rapid mental deterioration presented with status epilepticus

Abstract

Listen

Translate article

AbstractBackgroundAmyloids are characterized by a fibrillar β‐sheet secondary structure. Cerebral accumulation of β‐amyloid peptide is proposed to be one of the toxic pathogeneses of dementia, most commonly seen in Alzheimer’s disease (AD) and cerebral amyloid angiopathy (CAA). Here we reported patients who presented with status epilepticus and rapid mental deterioration. Their amyloid PET imaging displayed diffuse deposition.MethodWe retrospectively reviewed clinical presentation, EEGs, and brain MRI from a patient group with an amyloid‐PET imaging study.ResultFour patients were admitted to the hospital with status epilepticus. Case 1, a 75‐year‐old woman, suffered an impairment of consciousness with focal jerks. Epileptic discharging from bilateral frontotemporal regions continued for days after jerking controlled. Noticeable decline in remembering and problem solving developed rapidly within two months. Case 2, a 79‐year‐old woman, had mild cognitive impairment before administered anti‐seizure medications for right leg weakness after repeated focal seizures. After that, she had a more severe decline of dementia. Case 3, an 81‐year‐old woman, got an unimprovable left hemiparesis for two weeks of a continuing left‐sided jerk. She had rapidly increasing forgetfulness. Case 4, a 61‐year‐old man, was treated as super‐refractory status epilepticus for periodical choreic movement and dystonic jerks. Medications suppressed generalized periodic epileptiform discharges temporally. After one month, the epileptic discharging turned to periodic sharp wave complexes. He expired one month later. MRIs of Case 1 and 3 showed cortical atrophy. On case 2’s MRI, corticosubcortical microhemorrahge was revealed. Case 4’s diffusion‐weighted MRI showed diffuse cortical ribbon. The amyloid PETs of Case 1, 2, and 3 demonstrated diffuse deposition with focal cortical maximum spatially responsible for generating focal ictal discharges. The nuclear imaging on Case 4 showed maximal amyloid deposition on basal ganglia.ConclusionHere we reported four patients with refractory status epilepticus before dementia. Two cases were diagnosed with AD. One patient was categorized into CAA. In Case 4, according to positive CSF‐14‐3‐3, MRI cortical ribboning, EEG features, and clinical course, probable Creutzfeldt‐Jakob disease was impressed. Amyloid deposition may contribute to the increase of cortical susceptibility to seizures. Moreover, medically refractory status epilepticus hastens mental deterioration.