Description

A patient who has inherited the tuberous sclerosis complex may have one or more intracranial lesions.


 

A key finding is the cortical tuber, which are nodular areas of abnormal cortical development or "dysplasia").

(1) These may occur in the cerebrum and/or cerebellum.

(2) They may or may not have calcifications (calcified ones are easier to detect).

(3) They may be described as gyral cores or sulcal islands.

 

The total number of tubers is best determined using MRI (T2-weighted images) and contrast.

 

Other intracranial lesions in the tuberous sclerosis complex include:

(1) focal areas of cortical dysplasia

(2) subependymal nodules, typically in the walls of the lateral ventricles

(3) subependymal giant cell astocytoma(s)

(4) white matter migration lines or wedges

(5) white matter cyst-like lesions

 

The total cortical tuber count and other measures indicating extensive disease may correlate with neurological complications such as:

(1) severe seizures that are difficult to control

(2) mental retardation

(3) behavioral problems

However, some patients with extensive CNS involvement may be asymptomatic while symptomatic patients may have limited CNS disease on imaging studies.

 


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