Tuberous sclerosis complex

Article section 13 of 17.  Previous  Next

By Hema R Murali MBBS and Narayana S Murali MD

Renal cysts may be associated with renal failure, and renal angiomyolipoma may cause occasional massive intra-abdominal bleeding.

Unfavorable prognostic factors for epilepsy include onset in infancy, presence of several seizure types, and occurrence of new EEG foci during evolution of the disease (Curatolo et al 1991). Prognostic markers of intellectual disability include cortical tubers in the frontal and temporal regions (Raznahan et al 2007), TSC2 mutations, and refractory seizures. Obstructive hydrocephalus caused by a subependymal giant cell astrocytoma was an important cause of death before contemporary neuroimaging and neurosurgical techniques.|{picture:tsss3.jpg}{caption:Subependymal giant cell astrocytoma}{label:This gadolinium-enhanced, T1-weighted MRI shows the enhancing intraventricular tumor. (Contributed by Dr. Sherman C Stein.)}| Bleeding within a subependymal giant cell astrocytoma is a rare complication that may be fatal. Adequate seizure control and evaluation for hydrocephalus or tumor are the first considerations in tuberous sclerosis complex patients with behavioral or cognitive decline. In many, epileptiform activity is not apparent clinically and is revealed only by video-EEG monitoring.

In This Article

Historical note and nomenclature
Clinical manifestations
Clinical vignette
Pathogenesis and pathophysiology
Differential diagnosis
Diagnostic workup
Prognosis and complications
References cited