Tuberous Sclerosis Complex: The Role of Neuroradiology

Griffiths, PD; Martland, Timothy

doi:10.1055/s-2007-973708

Cited by 49 publications

(31 citation statements)

References 0 publications

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“…While it has been shown that MRI fails to detect SENs in up to a third of those individuals who show SENs on CT (a more sensitive modality for detecting calcified SENs), this increased sensitivity is of no known prognostic value. 22 MRI remains the test of choice for screening in patients with TSC.…”

Section: Discussion How Does a Sgct Differ From A Sen?mentioning

confidence: 99%

Subependymal giant cell tumors in tuberous sclerosis complex

Goh¹,

Butler²,

Thiele³

2004

Neurology

236

145

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Subependymal giant cell tumors in patients with TSC appear to be of mixed glioneuronal lineage, and, therefore, the current practice of classifying these tumors as astrocytomas merits revision. The clinical diagnosis of SGCT should be made for subependymal lesions in TSC that are associated with symptoms, papilledema, or radiologic evidence of hydrocephalus or interval growth. A diagnosis of probable SGCT should be made when a lesion has the potential to cause obstruction based on size or location. Annual screening by MRI with or without contrast is indicated until at least 21 years of age even if subependymal nodules are absent on initial imaging. A diagnosis of SGCT or probable SGCT warrants more frequent monitoring or surgical intervention.

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Section: Discussion How Does a Sgct Differ From A Sen?mentioning

confidence: 99%

Subependymal giant cell tumors in tuberous sclerosis complex

Goh¹,

Butler²,

Thiele³

2004

Neurology

236

145

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“…Why some of the lesions enlarge is not well understood. Contrast-enhanced MR imaging is more sensitive than CT for tumor identification [1, 2]. A new focal neurological deficit, increased intracranial pressure, changes in motion or uncontrolled seizures may be signs of a growing tumor.…”

Section: Discussionmentioning

confidence: 99%

“…Although there are variable pathological findings in multiple systems, the main cause of morbidity and mortality and the most prominent feature of this disease are neurological disturbances. In the cerebral parenchyma, subependymal hamartomas and cortical tubers occur by the overgrowth of astrocytes [1]. …”

Section: Introductionmentioning

confidence: 99%

An Unusual Tuberous Sclerosis Case Presenting with Fibrillary Astrocytoma

Eren¹,

Polat²,

Erman

2002

Pediatr Neurosurg

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Tuberous sclerosis (TS) is an autosomal dominant disorder. It is characterized by hamartomatous lesions in various organs such as the brain, skin, kidneys and heart. Subependymal and parenchymal nodules occur in the cerebrum. Although giant cell astrocytoma may develop from these subependymal nodules, tumor formation is very rare in the cortical nodules. We present the clinical, computed tomography and magnetic resonance imaging findings of a fibrillary astrocytoma originating from a cortical tuber in a 4-year-old female with TS.

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“…Up to now, no valid explanation has been proposed, while 88% to 95% of patients with TSC present SENs, [9,45] and about one child with TSC out of ten will develop SGCT(s). Exceptionally, SGCTs have been described in other ventricular compartments than the foramen of Monro, such as the lateral ventricle wall [2] or the temporal horn. [49] Elsewhere, extremely rare cases of neonatal SGCTs have contributed to our incomprehension of tumorigenesis and call in question again the supposed fi liation SEN-SGCT.…”

Section: Filiations Sen-sgctmentioning

confidence: 99%

“…TSC, also known historically as Bourneville's disease (1880), has been initially described by von Recklinghausen in 1862, while Vogt (1908) reported the typical and characteristic clinical triad of the disease associating facial angiofi bromatosis, epilepsy and mental retardation. [1,2] TSC is one of the more common single gene disorders, with an incidence of one in 5800 to 6000 live births, a prevalence of one in 30 000 and a frequency of one per 150 000 live births. [3,4] There is neither ethnic linking nor differences according to gender.…”

Section: Introductionmentioning

confidence: 99%