TUBEROUS SCLEROSIS

     TUBEROUS SCLEROSIS

Also k/as- Bournville’s dis

AD

Triad -  Epilepsy

              - MR

              -Skin lesions- Adenoma sebaceum

                                  - Ash-leaf spots

                                  - Shagreen patch

                                  - Depigmented naevi

                                  - Fibroma molluscum

                                  -Fibrous plaques on forehead

                                  -Subungal hamartoma

- CNS- Ependymomas

         -Astrocytomas

-OCULAR-

*  Retinal astrocytomas-50%

# white semitransparent or mulberry appearing tum in the superficial retina.

*  Hypopigmented patches on iris & ret

*  Papilloedema & VI N palsy

INV- 

CBC

.CT and MRI-BRAIN

EEG

Echocardiography

X ray chest

CT scan –abdomen.

Rx-

Genetic councelling

Retinal astrocytomas require no t/t