Signs and Symptoms of Tuberous Sclerosis Complex

Image

Tuberous Sclerosis Complex (TSC) is a multisystem disorder. Described in 1908 as a VOG tries, including adenoma sebaceum, epilepsy and mental retardation. Because TSC aوٴects no two people in the same way, there is no sure way to predict where and how the disorder will manifest. 57 patients at the Child and Youth Health Care Institute of Vojvodina were diagnosed with TSC on the basis of hypomelanotic skin lesions and epileptic seizures (45.61%), hypomelanotic skin changes, cardiac rhabdomyomas and epileptic seizures (21.05%), hypomelanotic skin changes and neuroimaging (3.51%), epileptic seizures and neuroimaging (10.53%) and epileptic seizures and kidney changes (1.75%).

Several types of brain c may be seen in patients with TSC, including cortical tubers, Sub Ependymal Nodules (SEN), and SEGA tumors. Some individuals will have all of these changes, whereas others will have none. Нe vast majority of individuals with TSC, however, will have one of these abnormalities. Abnormal neurological findings result from their location, size, and growth.

Seizures occur in 60-90% of children with ТSС, in three quarters of infants when they are manifested as epileptic spasms. 57.9% of children having TSC have the ٽrst attack during within first 6 months of life, 75% within the first year and 6.4% aіer the age of Multifocal or focal EEG changes are found in over 70% of patients with TSC. It is believed that the development of epilepsy aіer the first attack in TSC is 100%. Mental retardation depends on the presence of epileptic seizures and the development of epileptic encephalopathy, as well as on the secondary damage to the localization of nodules, astrocytoma, or SEGA tumor or it can be a result of hydrocephalus.

Skin changes

Нe best recognised TSC cutaneous manifestation are hamathoma: Adenoma sebaceum, angiobroma. They appear in early childhood and show progression during puberty and adolescence. Other skin lesions presents as: Hypomelanotic macules, ungual and gingival fibromas shagreen patch or forehead and face patches.