Fatal Outcome in Hemiconvulsion-Hemiplegia Syndrome

Abstract

Hemiplegia-hemiconvulsion-epilepsy syndrome is characterized by prolonged unilateral clonic seizures in a child followed by the development of hemiplegia. Focal status epilepticus results in unilateral cerebral edema of the epileptic hemisphere in the acute phase followed by cerebral hemiatrophy. Literature in the last 5 years does not describe malignant cerebral edema or resultant death. We report a case of a 3-year-old girl with hemiplegia-hemiconvulsion-epilepsy syndrome who died due to malignant cerebral edema and temporal lobe herniation. The first indication of worsening of clinical status after being seizure free was voltage suppression on continuous electroencephalography (EEG). We describe neuroimaging, EEG findings, and neuropathologic findings at autopsy and review pertinent literature. We also evaluate the evolving role of continuous EEG monitoring in the pediatric intensive care unit.

Survival following decompressive hemicraniectomy for hemiconvulsion-hemiplegia-epilepsy syndrome: case report

“…Pathological examination revealed neuronal shrinkage, cytoplasmic eosinophilia, and nuclear pyknosis, consistent with pathological findings in our case. 9 Similar to our case, both of these patients were initially treated with anticonvulsant medications and experienced prolonged status epilepticus that was difficult to abate. Also, each child experienced neurological decline resulting in pupillary dilation and herniation.…”

“…3 In the patient described by Jayakody and Joshi, a head CT was obtained on admission that revealed right hemispheric edema and a possible left small subdural hematoma, and thus the patient was transferred to a larger facility for further treatment. 9 Findings on a repeat CT scan obtained 2.5 hours later were unchanged, and brain MRI performed 4 hours later showed right hemispheric restricted diffusion with gyral edema involving 3 vascular territories with normal MRA findings of the head and neck. This patient's pupillary change was noted directly following attenuation of all EEG activity (about 31 hours after initial presentation) and this prompted repeat imaging that led to emergent right hemicraniectomy.…”

“…This patient's pupillary change was noted directly following attenuation of all EEG activity (about 31 hours after initial presentation) and this prompted repeat imaging that led to emergent right hemicraniectomy. 3,9 In the current case, the patient also underwent imaging on admission, as well as 24 hours after admission; however, there was no evidence of herniation on those evaluations. The patient underwent imaging again directly following the pupillary change, 48 hours after admission, and herniation was confirmed.…”

“…To date, there are only a few reported pediatric cases of HHE that have required surgical treatment of cerebral herniation in the form of a decompressive hemicraniectomy. 3,9 We present a report on the successful use of decompressive hemicraniectomy for the treatment of cerebral herniation in HHE and advocate close monitoring for development of intracranial hypertension in these patients. …”