Biphasic Clinical Course and Early White Matter Abnormalities may be Indicators of Neurological Sequelae after Status Epilepticus in Children

Abstract

Clinical course and serial neuroimaging findings are not fully described in children who have had neurological sequelae following status epilepticus. We found four patients who had neurological sequelae out of 42 children with status epilepticus in 2004. MRI studies were reviewed with specific attention to diffusion-weighted images (DWI) and the apparent diffusion coefficient (ADC). Proinflammatory cytokines, including tumor necrosis factor-alpha and interleukin-6, were measured in the cerebrospinal fluid (CSF) (3 patients). The clinical course showed biphasic; initial status epilepticus and neurological exacerbation along with seizure recurrence four to five days after onset. Within three days after initial status epilepticus, CT (all patients) and MRI (2 patients) did not show any abnormalities. From four to ten days after onset, MRI demonstrated diffuse hyperintensity in the cerebral white matter on DWI and hypointensity on ADC maps in all patients. Diffuse brain atrophy progressed thereafter. Tumor necrosis factor-alpha or interleukin-6 was elevated in all patients. A biphasic clinical course may be a specific feature for neurological sequelae. The preferential white matter involvement on MRI and elevated CSF cytokines indicate that glial dysfunction may play an important role in the pathophysiology of status epilepticus-associated cerebral damage.

Differences of Clinical Manifestations According to the Patterns of Brain Lesions in Acute Encephalopathy with Reduced Diffusion in the Bilateral Hemispheres

“…In this study, central-sparing lesions were not observed in any patient within the first 3 days after onset, consistent with several previous reports. 3,[12][13][14]17 In contrast, frontal occipital lesions were recognized within the first 3 days after onset and were followed by diffuse lesions. Second, the distribution of brain lesions is different in the 2 situations.…”

“…3 Several studies on acute encephalopathy have also reported a biphasic clinical course. [11][12][13][14] Onset is often marked by a prolonged seizure followed by improved consciousness. However, clustered seizures, signs of frontal lobe dysfunction, and worsening of consciousness become apparent at 3-4 days after onset.…”

Etiology is the key determinant of neuroinflammation in epilepsy: Elevation of cerebrospinal fluid cytokines and chemokines in febrile infection‐related epilepsy syndrome and febrile status epilepticus

“…Sakuma et al showed an elevation of CXCL1, CXCL9, CXCL10, IL‐6, and IL‐8 in the CSF of 14 patients with FIRES, compared to other inflammatory neurological diseases, and showed a correlation of chemokines with a poor prognosis. The literature on FRD (AESD and HHE) is scarce; elevation of IL‐6 was reported in the CSF in case reports with AESD …”

Early predictors of status epilepticus-associated mortality and morbidity in children

“…AEIMSE subtypes such as hemorrhagic shock and encephalopathy syndrome (HSES), acute necrotizing encephalopathy (ANE), acute encephalopathy with biphasic seizures and late reduced diffusion (AESD), and clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) were classified according to previous reports [5,7]. Idiopathic hemiconvulsionhemiplegia syndrome, characterized by long-lasting febrile seizures followed by hemiplegia, has clinical and imaging similarities to AESD [2,3,8]; therefore, it was included in the AESD category as well.…”