STURGE-WEBER SYNDROME

Overview
Log In For Videos
Give Feedback
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

STURGE-WEBER SYNDROME

Imaging

Imaging for optimized detection of Sturge-Weber syndrome:

While Sturge-Weber syndrome may be detected on skull Xray and CT, especially when there is established calcification and atrophy, MRI is the imaging of choice for assessing detail. MRI should include thin slice volumetric T1-weighted images with contrast, and axial and coronal T2-weighted and FLAIR images. In addition, orbital involvement can be assessed with orbital MRI.

Imaging characteristics of Sturge-Weber syndrome:

On CT imaging, gyral subcortical calcification is seen, with a "tram-track" appearance, unilateral cerebral atrophy can also be appreciated.
On MR imaging, T1-weighted imaging with contrast shows leptomeningeal enhancement, this may not be seen in older patients. Other findings include cerebral atrophy, white matter signal abnormality, and an enlarged unilateral choroid plexus.
Angiography is not routinely performed, but is abnormal in most cases, showing absent superficial cortical veins, with dilated and tortuous deep veins.

CAUTION imaging findings may be absent in infants, as calcification and atrophy may be absent at this age.

Imaging of Sturge-Weber syndrome

The two CT images below show subcortical calcification in the left occipital lobe (arrows).

Sturge Weber 1

Sturge Weber 2

Imaging of Sturge-Weber syndrome

The image below is a T2 weighted image, showing atrophy of the right hemisphere, enlargement of the choroid plexus (arrow 1), a thickened overlying skull and subcortical calcification (hypointense / dark signal in the subcortical region - arrow 2).