Bilateral Temporal Arachnoid Cysts Associated With Tuberous Sclerosis Complex

Abstract

The association between tuberous sclerosis complex and intracranial abnormalities such as hemimegalencephaly, schizencephaly, intracranial arterial aneurysms, and corpus callosum agenesis/dysplasia has been reported in the recent literature. However, the association between tuberous sclerosis complex and bilateral temporal arachnoid cysts has not been reported. A case of an 8-year-old boy with a mental impairment and epileptic seizures that began 5 years earlier is presented. The neuro-radiological findings are consistent with tuberous sclerosis complex associated with bitemporal arachnoid cysts. His epileptic seizures were treated with antiepileptic medications. To the authors' knowledge, this is the first reported case of tuberous sclerosis complex associated with bilateral arachnoid cysts of the temporal region. In such cases, arachnoid cysts may exacerbate the clinical picture.

Type 1 neurofibromatosis associated with asymptomatic cystic malformations of central nervous system (CNS)

“…A few patients with neurofibromatosis were revealed to have arachnoid cysts and only one case with bilateral temporal arachnoid cysts, as in our second patient, was reported 5,9 . Also one with tuberous sclerosis complex was found to have the bilateral temporal arachnoid cysts 10 . As these reports are in keeping with cortical developmental abnormalities, Tatli et al 10 .…”

“…Arachnoid cysts are nontumoral intra‐arachnoid fluid collections that may appear throughout the cerebrospinal axis 5,9 . Among the intracranial space‐occupying lesions, the reported incidence of arachnoid cyst forms only 1%, and they are nearly always sporadic and single, although cysts are very rarely bilateral 10 . A few patients with neurofibromatosis were revealed to have arachnoid cysts and only one case with bilateral temporal arachnoid cysts, as in our second patient, was reported 5,9 .…”

“…Also one with tuberous sclerosis complex was found to have the bilateral temporal arachnoid cysts 10 . As these reports are in keeping with cortical developmental abnormalities, Tatli et al 10 . suggested that genetic factors are responsible for this association.…”

Less common manifestations in TSC

“…Arachnoid cysts have been occasionally reported in TSC (Itoua et al, 1999;Tatli & Guzel, 2007) and they seem to be more frequent in TSC than in general population. A review of brain MR scans of 220 TSC patients found arachnoid cysts in 12 patients (Figure 2a,b; 5.5%; general population: 0.5%), and 10 were males, suggesting that males with TSC may be at higher risk for the presence of arachnoid cysts.…”

Gonadotropin-Dependent Precocious Puberty: Neoplastic Causes and Endocrine Considerations

“…Furthermore, arachnoid cysts have also been described in association with hypothalamic hamartomas and tuberous sclerosis [110, 111]. Neurologic and visual field disturbances are common.…”