TUBEROUS SCLEROSIS

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
Epilepsy Classification
Generalized Epilepsy
Focal Epilepsy
Generalized and Focal Epilepsy
Unknown Epilepsy
Epilepsy Syndromes
Neonatal/Infantile
- Self-limited neonatal seizures and Self-limited familial neonatal epilepsy
- Self limited familial and non-familial infantile epilepsy
- Early myoclonic encephalopathy
- Ohtahara syndrome
- West syndrome
- Dravet syndrome
- Myoclonic epilepsy in infancy
- Epilepsy of infancy with migrating focal seizures
- Myoclonic encephalopathy in non-progressive disorders
- Febrile seizures plus, genetic epilepsy with febrile seizures plus
Childhood
- Epilepsy with myoclonic-atonic seizures
- Epilepsy with eyelid myoclonias
- Lennox-Gastaut syndrome
- Childhood absence epilepsy
- Epilepsy with myoclonic absences
- Panayiotopoulos syndrome
- Childhood occipital epilepsy (Gastaut type)
- Photosensitive occipital lobe epilepsy
- Childhood epilepsy with centrotemporal spikes
- Atypical childhood epilepsy with centrotemporal spikes
- Epileptic encephalopathy with continuous spike-and-wave during sleep
- Landau-Kleffner syndrome
- Autosomal dominant nocturnal frontal lobe epilepsy
Adolescent/Adult
- Juvenile absence epilepsy
- Juvenile myoclonic epilepsy
- Epilepsy with generalized tonic-clonic seizures alone
- Autosomal dominant epilepsy with auditory features
- Other familial temporal lobe epilepsies
Any Age
- Familial focal epilepsy with variable foci
- Reflex epilepsies
- Progressive myoclonus epilepsies
Epilepsy Etiologies
Genetic Etiology
- Chromosomal abnormalities
- Gene abnormalities
Structural Etiology
- Malformations of cortical development
- Vascular malformations
- Hippocampal sclerosis
- Hypoxic-Ischemic
- Traumatic brain injury
- Tumors
- Porencephalic Cyst
Metabolic Etiology
Immune Etiology
- Rasmussen syndrome
- Antibody mediated
Infectious Etiology
Unknown Etiology
- Febrile infection related epilepsy syndrome
Epilepsy imitators

TUBEROUS SCLEROSIS

Imaging

Imaging for optimized detection of tuberous sclerosis:

MRI, with thin slice volumetric T1-weighted images, axial and coronal T2-weighted and FLAIR images.

Imaging characteristics of tuberous sclerosis:

cortical tubers (present in 80% or more patients, cerebellar tubers may also be present)
subependymal nodules (present in 95% or more patients)
subependymal giant cell astrocytoma (present in around 10-15% of patients)
white matter abnormalities and cysts

The pathological and imaging features of cortical tubers can be indistinguishable from those of focal cortical dysplasia (FCD IIb). Hemimegalencephaly has been reported in some patients with tuberous sclerosis.

Imaging in tuberous sclerosis

This is a T2-weighted image of a cortical tuber in an immature brain. There is a localized area of gyral expansion in the right frontal lobe, with abnormal low signal in the underlying white matter. The imaging features of this tuber can be similar to that of a focal cortical dysplasia (FCD type IIb).

TS1

Imaging in tuberous sclerosis

Both images below are from the same patient and show:

multiple cortical tubers - areas of gyral expansion with thickening of the overlying cortex and abnormal signal in the underlying white matter
subependymal nodules
white matter abnormality

TS2

TS3

Imaging in tuberous sclerosis

The image below is a T2-weighted image showing cortical tubers in the right and left frontal lobes (arrows).

TS4