Necrotizing Leukoencephalopathy Associated with Nonconvulsive Status Epilepticus and Periodic Short-Interval Diffuse Discharges: A Clinicopathological Study

Abstract

We describe the clinical, neuroimaging and neuropathological features of an immunocompromised patient diagnosed as having refractory nonconvulsive status epilepticus (NCSE), and whose consecutive electroencephalograms (EEGs) revealed persistent periodic short-interval diffuse discharges (PSIDDs). Prominent subcortical white matter lesions in keeping with the diagnosis of multifocal necrotizing leukoencephalopathy may be neuropathological substrate of NCSE with persistent PSIDDs.

Salzburg consensus criteria are associated with long-term outcome after non-convulsive status epilepticus

“…There is great controversy as to whether NCSE causes permanent brain damage [3,15,[24][25][26][27] The current rationale for aggressive treatment with intravenous anti-seizure medication is based on small reports with a cumulative number of approximately 500 cases of NCSE, including disabled children [28]. Neurological deficits in adult ambulatory patients with focal NCSE without coma and absence SE are rare, [3,[28][29][30][31][32][33][34] whereas NCSE in coma is associated with substantial morbidity [38]. The considerable neurological deficits and decreased independence in activities of daily living at discharge described in our study may partly be attributable to the large proportion of patients with NCSE in coma, representing about half of the study sample.…”

Multifocal Necrotizing Leukoencephalopathy Mimicking Sporadic Creutzfeldt-Jakob Disease

“…They have also recently been reported in an MNL case [6] . PSWC are presumably related to cortical disconnection from deep generators, as in diffuse axonal injury [7] .…”

Ambulatory non‐convulsive status epilepticus evolving into a malignant form

“…In two patients (Cases 5 and 6), CSF white blood cell count was 300/mm 3 and 20/mm 3 , respectively. In contrast, two patients (Cases 2 and 3) had a low CSF white blood cell count, but in one (Case 3) a brain biopsy was reported to be in keeping with non‐specific necrotising encephalitis (Fernández‐Torre et al , 2006). The presence of infective organisms was not confirmed for any of the patients.…”

“…Four patients died during the course of mNCSE; the clinical causes of death are mentioned in table 2. Post mortem examination revealed multifocal necrotising leukoencephalopathy in one (Case 3) (Fernández‐Torre et al , 2006). Unfortunately, autopsy was not authorised in two patients (Cases 1 and 4) and results were not available in another (Case 2).…”