Epilepsia partialis continua in children with fulminant subacute sclerosing panencephalitis

Abstract

Various inflammatory diseases of central nervous system, including subacute sclerosing panencephalitis, could cause epilepsia partialis continua. Two boys with epilepsia partialis continua with onset in terminal phase of atypical subacute sclerosing panencephalitis have been reported. Children were not vaccinated against measles, and the second case had history of measles at an early age. In both cases, the onset of subacute sclerosing panencephalitis was characterized by altered behavior and cognitive decline with very fast mental and neurological deterioration. One boy was suffering from complex partial seizures and myoclonic jerks synchronous with periodic electroencephalographic pattern. Diagnosis was proved by increased titers of antimeasles antibodies in both serum and cerebrospinal fluid. In terminal phase of the disease, epilepsia partialis continua of localized group of the muscles was diagnosed, with good response to intravenous infusion of midazolam. Surface electroencephalographic recordings during epilepsia partialis continua did not show the epileptic discharges. During the terminal phase of the disease, no other type of seizures and movement disorders were recognized, except epilepsia partialis continua. In spite of the treatment, period from the onset of disease to death lasted less than 3 months, suggesting very fulminant course of subacute sclerosing panencephalitis.

Subacute Sclerosing Panencephalitis With Atypical Clinical and MRI Findings

“…Atypical features include unusual age of onset, visual loss, seizures and other focal symptoms as initial presentations, a lack of SSPE-specific EEG pattern, and atypical fast progression of disease [6]. While ophthalmological symptoms such as optic atrophy, chorioretinitis, and papilledema are commonly associated with SSPE [4][5][6][7]. However, the presentation of vision loss preceding neurological symptoms is rare [7] as in our patient.…”

“…Atypical form of SSPE occurs in about 10% of all patients and there are no defined stages in clinical presentation due to rapid course [5]. Atypical features include unusual age of onset, visual loss, seizures and other focal symptoms as initial presentations, a lack of SSPE-specific EEG pattern, and atypical fast progression of disease [6].…”

Cluster of partial motor seizures heralding the onset of hemimyoclonic subacute sclerosing panencephalitis

“…One boy was suffering from complex partial seizures and myoclonic jerks synchronous with a periodic EEG pattern. In both patients, the period from onset of disease to death lasted less than 3 months 12. Our patient presented with a cluster of partial motor seizures without changes in higher mental functions, followed by periodic hemimyoclonus.…”

“…Usually, SSPE presents with cognitive and behavioral abnormalities followed by myoclonic jerks and, subsequently, an akinetic‐mute state 2. Uncommonly, SSPE may manifest with different types of seizures, such as generalized‐tonic‐clonic seizure, complex‐partial seizure, and hemimyoclonus 4–12. A case with isolated facial twitching has also been described 13.…”

Subacute Sclerosing Panencephalitis in a Child with Recurrent Febrile Seizures

“…The disease may present with varying symptoms. Uncommonly, subacute sclerosing panencephalitis may manifest with different types of seizures, such as generalized tonic-clonic seizure and myoclonic atonic seizures [5, 6]. Here, we reported a patient who presented with a history of recurrent febrile seizures, followed by deterioration in cognitive function.…”