HYPOTHALAMIC HAMARTOMA

Overview
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Seizure Classification
Generalized onset seizure
- Motor Onset
- Non-Motor Onset
Focal Onset Seizure
- By Onset Feature
- Focal to Bilateral Tonic-Clonic
- Hemispheric lateralization
- Lobar localization
  - Frontal
  - Temporal
  - Parietal
  - Occipital
Unknown Onset Seizure
Neonatal Seizure
Epilepsy Classification
Generalized Epilepsy
Focal Epilepsy
Generalized and Focal Epilepsy
Unknown Epilepsy
Epilepsy Syndromes
Neonate/Infant
- Self-limited
- With developmental and epileptic encephalopathy
Childhood
- Self-limited focal epilepsy syndromes
  - Remission expected in all cases by adolescence
    - Self-limited epilepsy with centrotemporal spikes
    - Self-limited epilepsy with autonomic seizures
  - Remission expected in most cases by adolescence
    - Childhood occipital visual epilepsy
    - Photosensitive occipital lobe epilepsy
- Genetic generalised epilepsy syndromes
  - Epilepsy with eyelid myoclonia
  - Epilepsy with myoclonic absence
- Idiopathic generalised epilepsy syndrome
  - Childhood absence epilepsy
- With developmental and/or epileptic encephalopathy
Variable Age
- Idiopathic generalised epilepsy syndromes
- Focal epilepsy syndromes
- Combined generalised and focal epilepsy syndrome
  - Epilepsy with reading-induced seizures
- With developmental and/or epileptic encephalopathy or progressive neurological deterioration
  - Progressive myoclonus epilepsies
  - Rasmussen syndrome
Epilepsy Etiologies
Genetic Etiologies
- Chromosomal abnormalities
- Gene abnormalities
Structural Etiologies
- Malformations of cortical development
- Vascular malformations
- Hippocampal sclerosis
- Hypoxic-Ischemic
- Traumatic brain injury
- Tumors
- Porencephalic Cyst
Metabolic Etiologies
Immune Etiologies
Infectious Etiologies
Unknown Etiologies
Encephalopathy
Epilepsy imitators

HYPOTHALAMIC HAMARTOMA

GENETICS

PATTERN OF INHERITANCE

95% de novo acquired somatic gene abnormality
5% familial inheritance - autosomal dominant (Pallister-Hall syndrome)

KNOWN GENES

GLI3 gene abnormality is responsible for Pallister-Hall syndrome; and GLI3 pathogenic variants are found in approximately 20% of sporadic hypothalamic hamartoma cases.

FAMILY HISTORY

May be present (see above).

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