Extensive apoptosis in a case of intractable infantile status epilepticus

Koshi A Cherian1, Karen Weidenheim, Alan D Legatt

  • 1Saul R. Korey Department of Neurology, Albert Einstein College of Medicine and Montefiore Medical Center, Bronx, NY 10467, United States. Kcherian@montefiore.org

Epilepsy Research
|May 26, 2009
PubMed

Insights

A young girl experienced severe seizures (status epilepticus) where brain cells died via apoptosis, not necrosis. This finding suggests new treatments targeting apoptosis could help children with prolonged seizures.

Area of Science:

  • Neuropathology
  • Pediatric Neurology
  • Epileptology

Background:

  • Intractable focal seizures with secondary generalization can progress to status epilepticus in infants.
  • Epilepsy surgery is a treatment option for refractory cases.
  • Understanding the underlying neuropathology is crucial for developing targeted therapies.

Observation:

  • An 8 1/2-month-old infant presented with intractable seizures and status epilepticus.
  • Neuropathologic examination revealed extensive apoptosis (programmed cell death) as the primary finding.
  • No specific etiology for the apoptosis or seizures was identified.

Findings:

  • This case represents the first documented instance of apoptosis, without concurrent necrosis, in a developing human brain associated with intractable status epilepticus.
  • The observed pattern of cell death suggests a specific mechanism in prolonged seizures.

Implications:

  • The findings suggest that apoptosis plays a significant role in the neuropathology of prolonged status epilepticus in infants.
  • Developing therapeutic strategies aimed at inhibiting apoptosis may offer a novel treatment approach for pediatric status epilepticus.
  • Further research into the mechanisms of apoptosis in the developing brain is warranted.

Related Concept Videos

Epilepsy and Seizures: Overview01:24

Epilepsy and Seizures: Overview

Epilepsy is a chronic neurological disease marked by recurrent, unpredictable seizures. These seizures are caused by abnormal electrical discharges in the brain, leading to behavior, sensation, or consciousness alterations. They can also cause transient impairment of awareness, interfering with daily activities.
Various factors can trigger epilepsy, including genetic factors, brain damage, metabolic causes, and unknown etiology. Diagnosis of epilepsy involves electroencephalography (EEG), which...
Seizures l: Introduction01:20

Seizures l: Introduction

Understanding seizures and epilepsy relies on key definitions that help in recognizing, classifying, and managing these disorders. These definitions provide a framework for recognizing, classifying, and managing seizure disorders.DefinitionsA seizure is a sudden, abnormal burst of electrical activity in the brain that can cause changes in awareness, movement, sensation, or behavior, depending on the area involved. Epilepsy is a chronic condition characterized by recurrent, unprovoked seizures,...
The Extrinsic Apoptotic Pathway01:17

The Extrinsic Apoptotic Pathway

The extrinsic apoptotic pathway is initiated when extracellular death-inducing signals, such as specific cytokines, activate the death receptors expressed on the cell surface. The immune cells involved in this pathway are natural killer cells (NK cells) and cytotoxic T-lymphocytes. NK cells are critical in innate immune response, while cytotoxic T-lymphocytes are associated with adaptive immune response. These cells recognize specific receptors expressed on the altered cells and activate...