2. In the majority of such cases (80%) the mutation has been narrowed down to two tumor suppressor genes, both part of the mTOR pathway 3,13: Tuberous sclerosis has a significant number of manifestations, involving many organ systems. Some will also use Inversion Recovery and not use contrast on a routine base. Patients die within 10 years of the onset of symptoms. DNET in an 11-year old boy presenting with refractory partial seizures. 1991;156 (5): 1081-6. Magnetic resonance imaging of the brain and spine. Neuroradiology 2010 52:479-487. by Bien CG, et al Some patients have lymphangioleiomatosis, a cystic lung disease seen in women. Tuberous Sclerosis Complex, Genes, Clinical Features and Therapeutics. 4. It is inherited in an autosomal dominant fashion and is characterized by an increased predisposition to hamartoma formation. J Child Neurol 2002; 17:373-384, by Hanefeld F, Kruse B, Holzbach U, Christen HJ, Merboldt KD, Hanicke W, Frahm J. LAM is a rare lung disease that results in a proliferation of smooth muscle throughout the lungs resulting in the obstruction of small airways leading to pulmonary cyst formation and pneumothorax. Pregnancy may exacerbate disease. The most common radiographic manifestations are: Cutaneous lesions are present in ~95% of cases, but are rarely appreciated radiographically 8: Treatment of seizures is essential and depending on the degree of intellectual disability, supportive care may be required. 5. JNR 2004 Jun-Jul;25(6):916-26, by Tortori-Donati P, Rossi A Lippincott Williams & Wilkins. The radial bands sign. Identical pulmonary changes seen in 1% of patients with tuberous sclerosis (predominant involvement of young men). Braffman BH, Bilaniuk LT, Naidich TP, et al. Heinrich Vogt (1875-1936) was a German neurologist that is notable by establishing the three pathognomonic clinical signs for tuberous sclerosis that became known as "Vogt triad”. In these infants there is greater perfusion to the apex of the gyri than to the cortex at the depth of the sulci. Intracranial subependymal tubers in this neonate exhibit increased signal intensity on short TR images. CT in a patient with Sturge-Weber shows huge cortical and subcortical tram-track calcifications involving the left posterior hemispere. Patients have seizures and hemiparesis, which is proportional to the size of the cleft and are more common in the open-lip type. Tuberous sclerosis or Bourneville's disease is an inherited condition characterized by the presence of hamartomas in many organs including angiomyolipoma of the kidney, cardiac rhabdomyoma and cortical and subependymal tubers in the brain. INTRODUCTION. Logue LG, Acker RE, Sienko AE. T2* and susceptibility weighted imaging (SWI) markedly increase the sensitivity of MRI to detect small cavernomas. {"url":"/signup-modal-props.json?lang=us\u0026email="}, {"containerId":"expandableQuestionsContainer","displayRelatedArticles":true,"displayNextQuestion":true,"displaySkipQuestion":true,"articleId":2224,"mcqUrl":"https://radiopaedia.org/articles/tuberous-sclerosis/questions/1624?lang=us"}. When patients do not meet these criteri… Hemimegalencephaly is a rare disease characterized by hamartomatous growth of one cerebral hemisphere or part of it. skin, eyes, and nervous system). The T1W-images show a comparison between normal lamination and sulcation on the left and polymicrogyria on the right (arrow). In 2011, the European Respiratory Society published guidelines for the diagnosis and management of lymphangioleiomyomatosis, which has established the following diagnostic criteria 9: 1. definite LAM 1.1. characteristic or compatible lung HRCT and lung biopsyfitting the pathological criteria for LAM or 1.2. characteristic lung HRCT and any of the following 1.2.1. renal angiomyolipoma 1.2.2. thoracic or abdom… This is typical for a DNET or dysembryoplastic neuroepithelial tumor, which we will discuss in a moment. Note large cyst with enhancement of mural solid tissue. Neuroradiology 1990; 31:492-497, by Montenegro MA et al Venous stasis and calcifications are best seen on the SWI. Calcification is common in ganglioglioma and is an important distinguishing factor from DNET and pleomorphic xanthoastrocytoma. It is defined as cerebral cortex scarring due to perinatal ischemia. On axial slices mesial temporal sclerosis is commonly overlooked. Tuberous Sclerosis Giant Cell Astrocytoma. Rasmussen's encephalitis is a progressive hemispheric atrophy of unknown origin. 2013;49:243-254. Since FLAIR may show false-positive results due to artefacts, the abnormalities should be confirmed on T2WI. T2* or SWI Mutations in the TSC1 and TSC2 genes, important regulators of the mammalian target of rapamycin (mTOR) signaling pathway, result in the development of tumors involving multiple organ systems. There is an open-lip type on the right and a closed-lip type on the left (red arrow). This is a tumor that develops from a subependymal nodule near the foramen of Monro. Treatment will be dictated by individual manifestations (e.g. These tumours share the following characteristics: Ganglioglioma is the most common tumor associated with temporal lobe epilepsy. Typically presents as cyst with enhancing mural nodule, but may be entirely solid, May be wedge shaped and point towards the ventricle, Supratentorial cyst with enhancing mural nodule which abuts the peripheral meninges, Non-enhancing enlargement of the tuber cinereum of the hypothalamus, Enlarged hemisphere with ipsilateral ventriculomegaly, Progressive atrophy of the involved hemispere, Anomalous venous drainage in areas of polymicrogyria. Radiology … They are also considered by some to be variants of ependymomas, with which they may co-exist (see below). MR will shows tissue loss and gliosis underneath a shrunken cortex. Subependymal nodules are small lesions protruding into the lateral ventricles. The etiology is unknown, but there is a relationship between MTS and prolonged febrile seizures earlier in life, complicated delivery and developmental processes. Helpful when searching for haemoglobin breakdown products as in posttraumatic changes and cavernomas, or to look for calcifications in tuberous sclerosis, Sturge-Weber, cavernomas and gangliogliomas. Tuberous sclerosis is a complex disorder which has multisystem involvement and varied clinical manifestations. This is called dual pathology. When meningeal involvement is not present, than a pleiomorphic xanthoastrocytoma is indistinguishable from a ganglioglioma. … Tuberous Sclerosis: subependymal tubers, intraventricular giant cell astrocytomas, ependymomas von Hippel Lindau: hemangioblastomas; Many non-tumorous diseases like small vessel disease, infections (septic emboli, abscesses) or demyelinating diseases like MS can also present as multifocal disease. Notice subcortical hyperintensity extending to the right ventricle indicating transmantle sign (blue arrow). Journ Clin Imag Sci 2011; 1(2):1-11, by Urbach H et al 28 (7): e32. CT of a patient with Tuberous Sclerosis shows multiple cortical and subcortical calcifications. Atlas SW. It is seen in infants presenting with seizures and precocious puberty. The FLAIR image on the right shows the subcortical hyperintensity. Closed-lip schizencephaly is characterized by cleft walls in apposition to each other. MRI in patients with Sturge-Weber can show: Coronal MR-images of a patient with Sturge-Weber show leptomeningeal enhancement in the right posterior hemispere. Two genetic loci for tuberous sclerosis have been identified so far. Identical clinical, radiologic, and pathologic pulmonary changes are seen in about 1% of patients with tuberous sclerosis. Small cystic ganglioglioma with a small enhancing nodule. 1998;13 (12): 624-8. ADVERTISEMENT: Supporters see fewer/no ads, Please Note: You can also scroll through stacks with your mouse wheel or the keyboard arrow keys. Imaging characteristics of tuberous sclerosis. Therefore, diagnostic criteria have been developed to aid the diagnosis of tuberous sclerosis. J. As many as 80% of patients with TSC … Subependymal giant cell astrocytoma (SEGA) In patients with a first ever seizure imaging will mostly show no brain-abnormalities, because the seizure is provoked by fever, drugs, dehydration or sleep deprivation. Multilocular cystic RCC is uncommon and discussed here. by Abdel Razek AA et al. There is no gender or race predilection and most symptomatic patients are aged 15-40 years 4. Sometimes the hyperintensity is seen extending from the subcortical area to the margin of the ventricle. Notice the hypoplastic left temporal lobe with cortical thickening (arrow) and atrophy of the white matter. In patients with multiple small black dots the differential diagnosis is: Diffuse axonal injury (DAI) AUTHORS. They usually start in the temporal lobe. The CT features included subependymal nodules in 25 of 26 patients (96%) and calcifications in 23 of 26 (88%). Most patients die within 10 years of the onset of symptoms. Lymphangiomyomatosis occurs only in women, usually of child-bearing age, between 17 and 50 years. 12. 9-y-old girl with refractory nocturnal epilepsy. The research team has developed an innovative strategy that will allow them to screen for antibody fragments specific for … In 15% of patients another developmetal abnormality can be found, mostly focal cortical dysplasia. Brain 2002; 125:1751-1759. by Bien CG et al The table shows a dedicated epilepsy protocol. Hamartomatous lesion-Astrocyte proliferation appears like candle stick drippings in ventricles Angiomyolipoma in kidney Rhabdomyoma in heart 100 % predictive of tuberous sclerosis. AlRayahi J, Zapotocky M, Ramaswamy V et-al. If a partial seizure spreads from one hemisphere to the other this will give rise to a secondarily generalised seizure. Therefore, diagnostic criteria have been developed to aid the diagnosis of tuberous sclerosis. AM Larson, SS Hedgire, V Deshpande,et al. MRI shows overgrowth of the left cerebral hemisphere. Heterotopic Grey Matter results from an arrested migration of normal neurons along the radial path between the ventricular walls (ependyma) and the subcortical regions. Adrenal incidentalomas are common and seen in about 3% of abdominal CT's, increasing up to 10% in elderly patients [1,2,3].The issue is to differentiate benign adrenal tumors from metastases or primary malignant masses without unnecessarily exposing the majority of patients to the burden of clinical workup, interventions and imaging follow-up. DWI shows diffusion restriction due to cytotoxic edema in the acute stage of the status epilepticus. The five black dots in the left cerebral hemisphere on the T2* are also cavernomas and are not visible on the T2WI. 2009 Jan;30(1):4-11, by Barkovich AJ. The coronal T2WI and FLAIR images show right-sided mesial temporal sclerosis. TUBEROUS SCLEROSIS. First study the images and then continue reading. Heterotopia present as nodular foci of grey matter intensity on all sequences. The cleft is lined by polymicrogyric gray matter.Open-lip schizencephaly is characterized by separation of the cleft walls. Typically seen in adolescents and young adults. Tuberous sclerosis is a genetic disorder affecting cellular differentiation and proliferation, which results in hamartoma formation in many organs (eg, skin, brain, eye, kidney, heart). AJR Am J Roentgenol. It is the second most common neurocutaneous disease. They do not enhance. 13. Show signs of chronicity, such as bone remodeling and scalloping of the adjacent skull. Cortical and glial scars usually result from meningitis or birth injury. In medication refractory epilepsia the most common location of the epilectogenic lesion is temporal lobe (60%), frontal lobe (20%) and parietal lobe (10%), periventricular (5%) and occipital (5%). See tuberous sclerosis diagnostic criteria 2. A complex partial seizure affects a larger part of the hemisphere and the person may lose consciousness. In simple partial seizures the person remains conscious. Patients present with early seizures, macrocrania and severe developmental delay with contralateral hemiparesis. A complete hemosiderin rim surrounds the lesion, but not when there is a recent bleeding. The high signal in the hippocamous reflects gliosis. T2*-images show multiple hemosiderin depositions at the interface between grey and white matter, consistent with diffuse axonal injury (DAI). Nov 5, 2017 - AKA tuberous sclerosis complex, is an uncommon genetic disorder that causes noncancerous (benign) tumors—unexpected overgrowths of normal tissue—to develop in many parts of the body. The tumor shows a characteristic bubby appearance and there is subtle scalopping of the skull. Pediatr Neurol . Notice the hemosiderin coating of the precentral gyrus consistent with superficial siderosis due to prior hemorrhage of the cavernoma (red arrowheads). AJNR. (2018) Radiographics : a review publication of the Radiological Society of North America, Inc. 38 (7): 2102-2122. In hemimegalencephaly it is important to exclude contralateral abnormalities, as these form a contraindication to hemispherectomy. All patients underwent CT; 16 patients underwent both. Most patients with uncontrollable seizures have complex partial seizures. The images demonstrate cortical and subcortical signal abnormalities on T2WI and FLAIR in the left temporal lobe indicating focal cortical dysplasia. The deeper layers of the cortex form multiple small gyri with derangement of the normal lamination and sulcation. Springer 2005, by Woermann FG, Vollmar C This is called the transmantle sign. 35-year-old patient with refractory temporal lobe epilepsy. As a group, they are characterized by widespread abnormalities often with characteristic appearances. 48. Axial unenhanced CT scan of brain shows numerous calcified subependymal nodules. Cavernoma in the postcentral gyrus on T1WI, T2WI and SWI. Notice the popcorn appearance with peripheral rim of hemosiderin on the T2WI. The shrunken cortex is best appreciated on a 3D-T1WI because of its high resolution and the superior delineation of the cortex, while FLAIR will show the hyperintensity associated with the gliosis. Pediatr Neurol . Eye abnormalities in a 4-year-old boy with Sturge-Weber syndrome. The thickened cortex may show a wide spectrum of abnormalities, such as lissencephaly, pachygyria or polymicrogyria. Another case of heterotopia with typical subcortical nodules (arrows). In the late stage, the involved hemisphere may atrophy due to constant seizure acitivity. Images of a typical subependymal heterotopia. Mesial temporal sclerosis may occur in association with other pathology, especially focal cortical dysplasia. A 46 year old biker presented with seizures after being hit by a car. E3513 Upstate University Hospital 750 East Adams Street Syracuse, NY 13210 315 464-3439 Polymicrogyria is a malformation due to an alteration of the cortical development in the late stage of neuronal migration. However, children with severe mental disability or uncontrollable seizures often need lifelong assistance. RCC is associated with hereditary syndromes, such as von Hippel-Lindau, tuberous sclerosis and Birt-Hogg-Dubé. The person will become unconscious and may have a tonic clonic seizure. Wiley-VCH. Subependymal giant cell tumors in tuberous sclerosis complex. Case 21: with subependymal calcified hamartomas, Case 22: with calcified subependymal nodules and sclerotic bone lesions, Case 25: prenatal cardiac rhabdomyomas in tuberous sclerosis (prenatal and neonatal findings), Case 27: with cortical tubers and radial bands, subependymal giant cell astrocytomas (SGCA), multifocal micronodular pneumocyte hyperplasia (MMPH), central nervous system manifestations of NF1, Sturge-Weber syndrome (encephalotrigeminal angiomatosis), basal cell nevus syndrome (Gorlin-Goltz syndrome), progressive facial hemiatrophy (Parry-Romberg syndrome), seizures: absent in one-quarter of individuals, intellectual disability: up to half have normal intelligence, adenoma sebaceum: only present in about three-quarters of patients, 88% are associated with calcification, although calcification absent in early childhood, visible within the first six months of age, variable signal, frequently high T1 and iso to high T2, enhancement is variable and is not a useful feature in distinguishing them from subependymal giant cell astrocytomas (SGCA); only serial growth is reliable, variable appearance, with nodular, ill-defined, cystic and band-like lesions seen, infarcts (due to occlusive vascular disorders), tuberous sclerosis accounts for 20% of all angiomyolipomas, angiomyolipomas are seen in 55-75% of patient with tuberous sclerosis, tend to grow and require surgical treatment, as the probability of hemorrhage is proportional to the size, 18-53% of patients with tuberous sclerosis, although rates of renal cell carcinoma are the same as in the general population, in patients with tuberous sclerosis, renal cell carcinoma tends to occur at a younger age, histologically identical to pulmonary LAM, chylous ascites, enlarged lymph nodes, dilatation of the thoracic duct, some studies have described a lymphangiomyomatosis-like change to be present in 25-40% of female patients with tuberous sclerosis, characterized by multicentric well-demarcated nodular proliferation of type II pneumocytes, benign striated muscle tumor characterized by the presence of spider cells, seen in 50-65% of patients with tuberous sclerosis, 40-80% of patients with cardiac rhabdomyomas have tuberous sclerosis, occur before the age of 1 year (75% of cases), typically regress before birth with spontaneous regression in 70% of children by age 4, thoracic duct and aortic/pulmonary artery aneurysm, hyperostosis of the inner table of the calvaria. Tuberous sclerosis complex diagnostic criteria update: recommendations of the 2012 Iinternational Tuberous Sclerosis Complex Consensus Conference. About 4 percent of all people will have at least one seizure during their lifetime. Patients usually present with exertional dyspnea and recurrent episodes of pneumothorax are common 8. Pediatr Neurol 27(4):282-8,2002. by Maria BL, et al There are two types of heterotopia: subependymal and subcortical. 1999;212 (3): 761-2. Patient develop an increasing frequency of seizures and progressive hemiplegia. The most common subtype of RCC is clear cell carcinoma, followed by papillary and chromophobe RCC. 27-year-old woman with bilateral renal angiomyolipoma in tuberous sclerosis. About 60 percent of patients with epilepsy can be controlled with antiepileptic drugs. Takanashi J, Sugita K, Fujii K et-al. 7. Seizure surgery in TSC is contemplated if a particular tuber can be implicated in seizure activity, or if a subependymal giant cell astrocytomas obstructs the foramen of Monro causing hydrocephalus. dual pathology. It results from mutations in the genes TSC1 and TSC2 and is known for causing neurological disorders including epilepsy and intellectual disability. Sagittal T1WI post contrast shows a giant cell astrocytoma in the right foramen of Monro. Unenhanced CT may show a hyperdense nodule or calcification, but in 50% of cases cavernomas will be occult on CT. T2WI and T2* gradient echo show multiple cavernomas. This patient has a bilateral schizencephaly. Images of a 27-year-old male with refractory occipital lobe epilepsy. Mesial temporal sclerosis is the most common cause of intractable epilepsy. TSC is a multisystem disorder, affecting many organs, most frequently the brain, skin, eyes, heart, kidneys, and lungs. Partial seizures - also called focal seizures - are seizures which affect only a part of the brain at onset. Cavernoma is also known as cavernous malformation or cavernous angioma. This differs from the signal characteristics of subependymal tubers in older patients. It is a vascular malformation with capillary venous angiomas in the face (port-wine stain), choroid of the eye and leptomeninges. The first gene maps to chromosome 9, specifically 9q34 (TSC1); the second gene maps to chromosome 16, specifically 16p13 (TSC2). AJR Am J Roentgenol. J Child Neurol 1998; 13:606-618. by Martin N, et al Kharrazi 1, Morteza Sanei Taheri 1, * 1 Assistant Professor, Department of Radiology, Shohada-e-Tajrish Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Using a dedicated MRI-protocol, it is possible to detect an epileptogenic lesion in 80 percent of these patients. Phakomatoses are a group of neurocutaneous disorders characterized by the involvement of structures that arise from the embryonic ectoderm (thus central nervous system, skin, and eyes). Overwater IE, Bindels-de Heus K, Rietman AB, et al. Axial T2WI and T1WI-CE show a giant cell astrocytoma at the level of the left foramen of Monro causing obstructive hydrocephalus. Another case of focal cortical dysplasia. In Sturge-Weber a vascular malformation of the choroid of the eye is seen. Coronal FLAIR and axial T2WI show T2-hyperintense cortical thickening and high signal in cortex and subcortical region. Axial FLAIR, axial DWI and coronal T2WI demonstrate a hyperintense hippocampus with a slightly compressed temporal horn of the lateral ventricle consistent with hippocampal edema. Evaluation of newly diagnosed tuberous sclerosis patients should include a personal and family history and a clinical examination, including funduscopy, cranial imaging (eg, MRI, nonenhanced CT scanning), renal ultrasonography, and echocardiography in infants. Tuberous sclerosis or Bourneville's disease is an inherited condition characterized by the presence of hamartomas in many organs including angiomyolipoma of the kidney, cardiac rhabdomyoma and cortical and subependymal tubers in the brain. Notice the asymmetric skull and slightly enlarged lateral ventricle. 23 (1): 241-6. 11. Other organs may also be involved. Dr. Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder affecting approximately 2 million people globally. Bilateral mesial temporal sclerosis is difficult to detect due to the lack of comparison with the unaffected contralateral hippocampus. Computed cranial tomography scanning and MRI are performed not only in suspect cases but also in patients whose diagnosis is … 4-year-old boy with Sturge-Weber syndrome. Umeoka S, Koyama T, Miki Y et-al. Child Neurol. subependymal giant cell astrocytomas, or retroperitoneal hemorrhage from renal angiomyolipoma). The lesions are almost completely black on the gradient echo due to blooming artefacts. T1WISuperior for cortical thickness and the interface between grey and white matter. When patients do not meet these criteria, they are sometimes referred to as manifesting a forme fruste of the condition. Silvia Tresoldi, Alice Munari, Giovanni Di Leo et-al. Kwiatkowski DJ, Whittemore VH, Thiele EA. During the hourlong webinar, Dr. Rohini Coorg, assistant professor of pediatrics – neurology at Baylor and director of the Comprehensive Tuberous Sclerosis Clinic at Texas Children’s, will explain how TSC occurs, how it is diagnosed and how different conditions associated with the diagnosis may be treated. The bubbly cystic appearance is seen as small cyst-like intratumoral structures that are very hyperintense on T2WI. Ulegyria must be differentiated from microgyria. These patients present with buphthalmos (enlarged eye) due to increased intraocular pressure and hemianopsia. There is dysplastic thick cortex and ventricular dilatation on the affected side. Schizencephaly is a cleft in the brain that connects the lateral ventricle to the subarachnoid space. Neurol India 2010 May-Jun,58(3):361-70, by Demaerel P The perpetuation of this parasitic disease is related to poor sanitation and hygiene. Ulegyria typically affects full term infants. Mesial temporal sclerosis (MTS) is a specific pattern of hippocampal neuronal loss accompanied by gliosis and atrophy. Pediatric Brain Tumor Genetics: What Radiologists Need to Know. Pancreatic neuroendocrine tumors in patients with tuberous sclerosis complex. Focal cortical dysplasia is a congenital abnormality where the neurons fail to migrate in the proper formation in utero. DNET in typical cases present as a bubbly mass which expands the affected gyri. Same patient. 10. J Magn Reson Imaging 2008 aug,28(2):300-7, by Kim SJ et al. Ganglioglioma in a young child. The table also summarizes epileptogenic lesions that are detected in patients with uncontrollable seizures. Hippocampal hyperintensity without volume loss is seen in: Status epilepticus Transmantle sign seen in another patient with focal cortical dysplasia. Tuberous sclerosis or Bourneville's disease is an inherited condition characterized by the presence of hamartomas in many organs including angiomyolipoma of the kidney, cardiac rhabdomyoma and cortical and subependymal tubers in the brain. Epilepsy in children with tuberous sclerosis complex: Chance of remission and response to antiepileptic drugs. Notice the track of grey matter in the left hemisphere on the axial image. Tuberous sclerosis complex (TSC) is a genetic disorder that affects multiple systems. Check for errors and try again. Notice FLAIR-hyperintensity (red arrow) and excessive enhancement of the wall of the left globe (blue arrow) consistent with a diffuse choroidal hemangioma. Closely related to developmental malformations. The patient was succesfully treated with amygdalo-hippocampectomy on the left. John James Pringle (1855-1922) was a Scottish dermatologist that also studied this disease leading some books to refer to it as "Bourneville-Pringle disease”. Barkovich AJ. DNET mimicking mesial temporal sclerosis 75 percent occur as solitary sporadic lesions and 10-30 percent occur as multiple lesions. The most common subtype of RCC is clear cell carcinoma, followed by papillary and chromophobe RCC. Notice atrophy of the left posterior cerebral hemisphere with leptomeningeal enhancement and thickening. 8. Axial T2WI shows hyperintense, but enlarged hippocampus with a bubbly appearance. MRI findings may be very subtle or may even be negative, therefore a high index of suspicion is mandatory! These findings are typical for focal cortical dysplasia. Pleomorphic xanthoastrocytoma (PXA) is a rare cause of temporal lobe epilepsy. The unenhanced CT shows a small calcification in the right lentiform nucleus. MR shows subtle hyperintensity of the left hippocampus on the axial FLAIR (blue arrow) and atrophy of the left hippocampus on coronal images (yellow arrow). Clinical findings: Majority of patients present with dyspnea. Roach ES, Gomez MR, Northrup H. Tuberous sclerosis complex consensus conference: revised clinical diagnostic criteria. Cavernomas consist of locules of variable size that contain blood products in different stages of evolution which produces a popcorn appearance. Myocardial Fatty Foci in Adult Patients with Tuberous Sclerosis Complex: Association with Gene Mutation and Multiorgan Involvement. Article by Sheena Melanie. the world of radiology is the world of magic and gessing. The most common clinical presentation is intractable seizures. JBR-BTR 2008 Nov-Dec;91(6):254-7, by Flores-Sarnat L Notice popcorn appeance and blooming artefact. The CT shows that most of the lesions are calcified.