Epilepsy in hypothalamic hamartoma: clinical and EEG features.
A Simon Harvey1, Jeremy L Freeman
1Children's Epilepsy Program, Children's Neuroscience Centre, Royal Children's Hospital, Parkville, Melbourne, Australia. simon.harvey@rch.org.au
Seminars in Pediatric Neurology
|June 5, 2007
Summary
Hypothalamic hamartoma (HH) can cause early-onset gelastic seizures and developmental delays. Effective surgical treatment targeting the HH can reverse or halt these neurological issues.
Area of Science:
- Neurology
- Developmental Neuroscience
- Pediatric Epilepsy
Background:
- Hypothalamic hamartoma (HH) is a congenital brain malformation.
- HH can lead to precocious puberty or seizures, particularly gelastic seizures.
- Diagnosis of HH and associated epilepsy is often delayed due to normal initial EEGs.
Purpose of the Study:
- To describe the clinical manifestations and progression of epilepsy in children with hypothalamic hamartoma.
- To highlight the diagnostic challenges associated with gelastic seizures in HH.
- To emphasize the potential for reversal of neurological deficits with surgical intervention.
Main Methods:
- Review of clinical cases with hypothalamic hamartoma.
- Analysis of seizure types, EEG findings, developmental trajectories, and behavioral issues.
- Correlation of electroclinical manifestations with disease progression.
Main Results:
- Gelastic seizures, often starting in infancy, are a common early symptom.
- Epileptic progression can occur, leading to complex partial and generalized seizures.
- Developmental slowing and behavioral problems often parallel seizure progression.
Conclusions:
- Early diagnosis and surgical treatment of hypothalamic hamartoma are crucial.
- Effective surgical intervention can arrest or reverse neurological and developmental impairments.
- Understanding seizure propagation mechanisms is key to managing HH-related epilepsy.
Related Concept Videos
01:22Epilepsy ll: Types
Recurrent seizures, stemming from abnormal electrical activity in the brain, are the defining characteristic of epilepsy, a chronic neurological condition. Because seizure features vary greatly, epilepsy is classified using two systems: by seizure type and by epilepsy syndromes. These classifications enable clinicians to describe seizure patterns and select suitable treatment strategies.I. Classification by Seizure Type1. Focal EpilepsyFocal epilepsy begins in one hemisphere of the brain.
01:24Epilepsy 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...
Various factors can trigger epilepsy, including genetic factors, brain damage, metabolic causes, and unknown etiology. Diagnosis of epilepsy involves electroencephalography (EEG), which...
01:13Seizures: Classification
Epilepsy is primarily characterized by unpredictable seizures, either provoked by an identifiable factor, such as injury or illness, or unprovoked, occurring spontaneously without apparent cause.
Seizures are typically classified into two main categories: focal and generalized seizures.
Focal Seizures
Focal seizures originate from specific regions of the brain. These seizures are further sub-classified into two types:
Seizures are typically classified into two main categories: focal and generalized seizures.
Focal Seizures
Focal seizures originate from specific regions of the brain. These seizures are further sub-classified into two types:
01:20Seizures 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,...
01:20Antiepileptic Drugs: Potassium Channel Activators
Ezocgabine or retigabine, an antiepileptic drug of remarkable efficacy, has revolutionized the management of seizures. It is a potassium channel activator, explicitly targeting the family of Q subtype potassium channels. It enhances the transmembrane potassium currents, regulating neuronal excitability. This action stabilizes the resting membrane potential, a pivotal factor in mitigating the hyperexcitability that characterizes epilepsy.
Ezogabine has gained approval as an adjunctive treatment...
Ezogabine has gained approval as an adjunctive treatment...
01:19Seizures ll: Types
Seizures are sudden bursts of abnormal electrical discharge in the brain that interfere with normal function. They are commonly divided into three groups: focal seizures, generalized seizures, and other types that do not fit neatly into either category.Focal SeizuresFocal seizures begin in a single brain region. When awareness is preserved, they are called focal aware seizures and may cause sensations such as tingling, unusual smells, or flashing lights. When awareness is impaired, they are...