Usefulness of Diagnostic Criteria of Tuberous Sclerosis Complex in Pediatric Patients

Jóźwiak, Sergiusz; Schwartz, Robert A.; Jänniger, Camila K.; Bielicka-Cymerman, Jolanta

doi:10.1177/088307380001501003

Cited by 214 publications

(189 citation statements)

References 14 publications

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“…The incidence of SEGA in tuberous sclerosis complex (TSC) varies from 5% to 14% [1][2][3] . Although this tumor is considered specific for TSC, there are reports of patients with SEGA who have none of the other stigmata of TSC.…”

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confidence: 99%

“…Although this tumor is considered specific for TSC, there are reports of patients with SEGA who have none of the other stigmata of TSC. Usually these tumors grow slowly, but enlarge enough to cause increased intracranial pressure, seizures, and focal neurological signs 3,4 . The computed tomography (CT) and magnetic resonance imaging (MRI) are useful in non-invasive investigation of these tumors.…”

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confidence: 99%

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Subependymal giant cell astrocytoma with high choline/creatine ratio on proton MR spectroscopy

Neto

Gasparetto

Bruck

2006

Arq. Neuro-Psiquiatr.

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-Objective: To report a case of subependymal giant cell astrocytoma (SEGA) in a patient with tuberous sclerosis, emphasizing the proton MR spectroscopy (MRS) findings. Case: A three year-old boy with tuberous sclerosis presented with a 3-month history of mild headache. The physical examination showed discrete mental retardation and multiple hypomelanic macules in the legs and back. MRI showed many cortical tubers and subependymal nodules. At the left foramen of Monro, there was a 15 mm nodule, with heterogeneous signal on T1 and T2-weighted images, and strong enhancement after contrast administration. MRS study TE=144ms) showed the following ratios: a) left foramen of Monro nodule N-acetylaspartate (NAA)/creatine (Cr)=0.93 and Choline (Cho)/Cr=1.6 and b) at the right Monro foramen NAA/Cr=1.56 and Cho/Cr=1.29. Conclusion: The MRS performed at the SEGA may show high Cho/Cr and low NAA/Cr ratios, similar to the other brain neoplasms. As a consequence, MRS may be a valuable tool for the early detection of neoplastic transformation of subependymal nodules near the foramina of Monro in patients with tuberous sclerosis.KEY WORDS: subependymal giant cell astrocytoma, proton magnetic resonance spectroscopy, tuberous sclerosis.Astrocitoma subependimário de células gigantes com alta relação colina/creatina à espectroscopia de prótons por ressonância magnética RESUMO -Objetivo: Relatar caso de astrocitoma subependimário de células gigantes (ASCG) em paciente com esclerose tuberosa, enfatizando os achados de espectroscopia de prótons por ressonância magnética (EPRM). Caso: Um menino de três anos de idade apresentou-se com cefaléia por três meses. O exame físi-co demonstrou retardo mental discreto e múltiplas máculas hipomelanóticas no dorso e pernas. A ressonân-cia magnética (RM) evidenciou múltiplos túberes corticais e nódulos subependimários. No forame de Monro esquerdo foi observado um nódulo de 15 mm, com sinal heterogêneo nas imagens ponderadas em T1 e T2, e realce intenso após a administração de contraste. A EPRM (multi-voxel PRESS, TE=144ms) demonstrou as seguintes relações: a) no nódulo no forame de Monro esquerdo N-acetilaspartato (NAA)/creatina (Cr)=0,93 e colina (Cho)/Cr=1,6 e b) no forame de Monro direito NAA/Cr=1,56 and Cho/Cr=1,29. Conclusão: A EPRM pode demonstrar relações altas de Cho/Cr e baixas de NAA/Cr em pacientes com ASCG, semelhante a outros tumores cerebrais. Sendo assim, a EPRM pode representar importante ferramenta diagnóstica na detecção precoce de transformação neoplásica de nódulos subependimários próximos ao forame de Monro em pacientes portadores de esclerose tuberosa. PALAVRAS-CHAVE: astrocitoma subependimário de células gigantes, espectroscopia de prótons por ressonân-cia magnética, esclerose tuberosa.Subependymal giant cell astrocytoma (SEGA) is a tumor that typically occurs in the lateral ventricles near the foramina of Monro. The incidence of SEGA in tuberous sclerosis complex (TSC) varies from 5% to 14% [1][2][3] . Although this tumor is considered specific for TSC, there are r...

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confidence: 99%

mentioning

confidence: 99%

Subependymal giant cell astrocytoma with high choline/creatine ratio on proton MR spectroscopy

Neto

Gasparetto

Bruck

2006

Arq. Neuro-Psiquiatr.

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“…We present a patient who presented three characteristic lesions of this rare disease: such as, cortical tubers, subependymal nodules, subependymal giant cell astrocytomas [1][2][3] , and meningioma, that not this considered like part of this disease, which was peculiar to this case. The association of the three lesions suggests a possible common connective-tissue defect.…”

Section: Discussionmentioning

confidence: 87%

“…Cortical tubers, subependymal nodules and subependymal giant cell astrocytoma are the characteristic intracranial lesions of TSC 1 . However, the full triad occurs in only 29% of patients 1,2 . The majority of patients with TSC have epilepsy, although the mechanisms underlying epileptogenesis remain unknown.…”

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confidence: 99%

Tuberous sclerosis complex and sphenoid meningioma

Castillón-Benavides

Salinas-Lara

Ponce-Guerrero

et al. 2010

Arq. Neuro-Psiquiatr.

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“…All subjects had TSC diagnosed by standard criteria 13 . An age and gender paired control group composed of fifteen healthy volunteers (11 females and 4 males; mean age 11.3 years, Sd 2.74) was also selected.…”

Section: Studied Populationmentioning

confidence: 99%

Proton MR spectroscopy of the foramen of Monro region in patients with tuberous sclerosis complex

Carvalho-Neto¹,

Bruck²,

Antoniuk³

et al. 2008

Arq. Neuro-Psiquiatr.

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-Purpose:To evaluate with 1H-magnetic resonance spectroscopy (MRS) the metabolites rations of the foramen of Monro's region in patients with tuberous sclerosis complex (TSC). Method: Twelve patients with TSC and an age and gender-matched control group underwent MR imaging at a 1.5T scanner, and 1H-MRS at the foramen of Monro level with a multivoxel acquisition. Similar volumes of interest were selected in each side of the foramen of Monro and in the basal ganglia (lentiform nuclei). The obtained N-acetylaspartate (NAA), creatine (Cr) and choline (Cho) peak amplitude values and ratios were studied. The statistical analysis was performed and p<0.05 was considered statically significant. Results: There was no significant difference between the NAA/Cr and Cho/Cr ratios near to the foramen of Monro and basal ganglia of the TSC patients compared with the controls (p>0.05). Conclusion: The NAA/Cr and Cho/Cr ratios near to the foramen of Monro and basal ganglia of TSC patients are similar to the rations obtained in the control group.Key woRdS: tuberous sclerosis complex, magnetic resonance imaging, proton spectroscopy. espectroscopia de prótons por rM da região do forame de Monro em pacientes com complexo esclerose tuberosaResumo -Objetivo: Avaliar através de espectroscopia de prótons as relações dos metabólitos da região do forame de Monro em pacientes com complexo esclerose tuberosa (CeT). Método: doze pacientes com CeT e um grupo controle pareado por sexo e idade realizaram RM em aparelho de 1,5T, e a espectroscopia de prótons foi obtida ao nível do forame de Monro com aquisição multi-voxel. Volumes de interesse similares foram posicionados em cada lado do forame de Monro e nos gânglios da base (núcleos lentiformes). os valores das relações e amplitudes de pico do N-acetilaspartato (NAA), creatina (Cr) e colina (Cho) foram estudados. A análise estatística foi realizada e valores de p<0,05 foram considerados estatisticamente significativos. Resultados: Não houve diferença significativa entre as relações NAA/Cr e Cho/Cr na região do forame de Monro e nos gânglios da base dos pacientes com CeT comparados com os controles (p>0,05). Conclusão: As relações NAA/Cr e Cho/Cr na região do forame de Monro e nos gânglios da base de pacientes com CeT são semelhantes àquelas obtidas no grupo controle. PAlAVRAS-CHAVe: complexo esclerose tuberosa, imagem por ressonância magnética, espectroscopia de prótons.

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Usefulness of Diagnostic Criteria of Tuberous Sclerosis Complex in Pediatric Patients

Cited by 214 publications

References 14 publications

Subependymal giant cell astrocytoma with high choline/creatine ratio on proton MR spectroscopy

Subependymal giant cell astrocytoma with high choline/creatine ratio on proton MR spectroscopy

Tuberous sclerosis complex and sphenoid meningioma

Proton MR spectroscopy of the foramen of Monro region in patients with tuberous sclerosis complex

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