Table 2 Baseline manifestations of TSC reported in TOSCA
From: TuberOus SClerosis registry to increase disease Awareness (TOSCA) – baseline data on 2093 patients
Manifestations of TSC | Patients at baseline, n (%) |
|---|---|
Neurological | |
SEGA | 510 (24.4) |
Cortical tuber | 1721 (82.2) |
SEN | 1636 (78.2) |
Cerebral white matter radial migration lines | 429 (20.5) |
Renal | |
Renal angiomyolipoma | 987 (47.2) |
Multiple renal cysts | 477 (22.8) |
Polycystic kidneys | 73 (3.5) |
Impaired renal function | 43 (2.1) |
Renal malignancy | 24 (1.1) |
Pulmonary | |
Lymphangioleiomyomatosis | 144 (6.9) |
Cardiavascular | |
Cardiac rhabdomyoma | 717 (34.3) |
Dermatologic | |
≥ 3 hypomelanotic macules | 1399 (66.8) |
Facial angiofibroma | 1199 (57.3) |
Shagreen patch | 573 (27.4) |
Ungual or periungual fibromas | 350 (16.7) |
Forehead plaque | 295 (14.1) |
Confetti lesions | 179 (8.6) |
Ophthalmologic | |
Retinal hamartoma | 294 (14.0) |
Epilepsy | 1748 (83.5) |