Aims and objectives
To highlight the high utility of cross sectional imaging: Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) in assessing the diagnosis of cerebral tuberous sclerosis (TS).
To expose different imaging aspects of the disease.
Methods and materials
14 patients were eligible to participate in a retrospective study from April 2014 to October 2018.
Mean patient age was 17 (range,
12-20).
The sex-ratio was 1,33.
The diagnosis of tuberous sclerosis (TS) was suspected by a triad of clinical features (Vogt triad): mental retardation,
epilepsy,
and adenoma sebaceum (facial angiofibroma).
Neurologic manifestations were present in approximately all of patients with TS and were the primary cause of morbidity and mortality.
Seizures were present in most of these patients and often begin in the first...
Results
In our study,
we only illustrated central nervous system (CNS) involvement of TS.
Four common CNS abnormalities were found.
A patient could present more than one feature.
Cortical Tubers
Tubers (grossly akin to potato tubers),
after which the disease tuberous sclerosis is named,
are glioneuronal hamartomas composed of enlarged atypical and disorganized neuronal and glial elements with astrocytosis.
They were found in all patients and were multiple and bilateral in 8 patients (57,1%).
They were located in the frontal lobes in 10 patients (71,4%) although...
Conclusion
Tuberous sclerosis (TS) is an autosomal dominant,
neurocutaneous disorder that is characterized by development of hamartomatous tumors in multiple organs and neuronal migration abnormalities.
TS is a multisystem genetic disorder with specific intracranial manifestations such as cortical and cerebellar tubers,
subependymal nodules,
and SEGAs,
as well as abdominal and thoracic manifestations such as renal angiomyolipoma (AML) and lymphangioleiomyomatosis (LAM).
TS results from a mutation in the TSC1 or TSC2 genes,
with approximately one-third of cases being inherited.
The common underlying biochemical pathways have also been...
Personal information
Ghita Berrada,
[email protected],
Central Department of Radiology,
Ibn Rochd Universitary hospital,
Casablanca,
Morocco.
Kawtar Charef,
[email protected],
Central Department of Radiology,
Ibn Rochd Universitary hospital,
Casablanca,
Morocco.
Zineb Abbad El andaloussi,
[email protected],
Central Department of Radiology,
Ibn Rochd Universitary hospital,
Casablanca,
Morocco.
Hanae El Mrabet,
[email protected],
Central Department of Radiology,
Ibn Rochd Universitary hospital,
Casablanca,
Morocco.
Hayat El Hajoui,
[email protected],
Central Department of Radiology,
Ibn Rochd Universitary hospital,
Casablanca,
Morocco.
Samia El Manjra,
[email protected],
Central Department of Radiology,
Ibn Rochd Universitary hospital,
Casablanca,
Morocco.
Samira Lezar,...
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