Tuberous sclerosis

Case contributed by Abhishek Bhargava
Diagnosis certain

Presentation

Seizures since 1 month.

Patient Data

Age: 1 year
Gender: Male
mri
This study is a stack
Axial
T2
This study is a stack
Axial
T1
This study is a stack
Axial
FLAIR
This study is a stack
Coronal
T2
This study is a stack
Sagittal
T2
This study is a stack
Axial
DWI
This study is a stack
Axial
ADC
This study is a stack
Axial
Gradient Echo
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Info

Multiple subependymal nodules are present in both the lateral ventricles which appear isointense to brain parenchyma on all imaging sequences. No evidence of calcification in these subependymal nodules.

 Multiple T2 hyperintensities are seen in cortical and subcortical white matter of both cerebral hemispheres - likely represent cortical tubers.

Case Discussion

Cortical and subcortiucal tubers are seen in most of the cases and occurs due to enlarged atypical and disoragnized neuronal and glial elements with astrocytosis. Cortical tubers are typically benign pathologic entities. However, the extent of cerebral dysfunction, including intractable epilepsy and mental retardation, may be related to the burden from cortical tubers, especially when there is bilateral hemisphere involvement. In above case it acted as epileptogenic focus.

Subependymal nodules are similar to cortical tubers but have a tendency to calcify with age. It may convert into subependymal giant cell astrocytomas and occur near foramen of monro. It may result in obstructive hydrocephalus and patient requires survelliance every 1-3 years to rule out SEGA's.

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