The Temporal Lobe Agenesis Syndrome

Robinson, R. G.

doi:10.1093/brain/87.1.87

Cited by 137 publications

(51 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Sin embargo, 2 teorías sugieren que esta anomalía se produce también en el mismo periodo gestacional (7,8). Dicha entidad clínica consiste en colecciones de líquido benignas en la membrana subaracnoidea.…”

Section: Discussionunclassified

Quiste subaracnoideo y displasia del ala mayor del esfenoides en neurofibromatosis

Cortés-Arreguín

Palafox

Molina³

2015

Cir. plást. iberolatinoam.

View full text Add to dashboard Cite

ResumenLos quistes subaracnoideos representan menos del 1% de los tumores de ocupación intracraneal.Hay pocas publicaciones acerca de quistes subaracnoideos del lóbulo temporal con remodelación de la cavidad orbitaria, pero no existen en relación al tratamiento reconstructivo de la cavidad orbitaria en esta patología.Presentamos el caso clínico de un varón de 44 años de edad con diagnóstico de neurofibromatosis tipo 1, aumento progresivo de volumen del ojo y párpado izquierdos de 40 años de evolución y dolor retro-ocular intermitente desde hacía 10 años, al que se le diagnostica un quiste subaracnoideo del lóbulo temporal que invade la cavidad orbitaria con displasia del ala mayor del esfenoides.Realizamos drenaje y cistocisternostomía del quiste subaracnoideo, con reconstrucción de paredes orbitarias con malla de titanio y cobertura con un colgajo de pericráneo así como elevación y reposicionamiento del globo ocular mediante cantopexias, seguida de una osteotomía de la órbita para reducir el volumen de la misma.Palabras clave Neurofibromatosis, Quiste subaracnoideo, Remodelación orbitaria, Reconstrucción orbitaria. Nivel de evidencia científica 5 AbstractArachnoid cysts represent less than 1% of brain tumors. There are few reports of subarachnoid cysts of the temporal lobe with subsequent remodeling of the orbit, however there are no reports regarding the reconstructive management of the orbital cavity in this pathology.We report the case of a man of 44 years with diagnostic of neurofibromatosis type 1 who presented progressive increase in volume of the left eye of 40 years of evolution, with intermittent retro-ocular pain. A temporal lobe arachnoid cyst was diagnosed invading the orbital cavity and dysplasia of the greater wing of the sphenoid. Drainage and cystocisternostomy of arachnoid cyst was performed, with orbital wall reconstruction with titanium mesh and covered with a flap of pericranium as well as the lifting and repositioning of the eyeball by canthopexies and orbital osteotomy to decrease the volume of the cavity.

show abstract

Section: Discussionunclassified

Quiste subaracnoideo y displasia del ala mayor del esfenoides en neurofibromatosis

Cortés-Arreguín

Palafox

Molina³

2015

Cir. plást. iberolatinoam.

View full text Add to dashboard Cite

show abstract

“…The most frequent localization is in the middle fossa, corresponding to more than half the cases reported in the literature 2 . The origin of middle fossa arachnoid cysts is related to two theories: primary malformation of arachnoid (Starkman et al) 3 or secondary development due to partial agenesis of the temporal lobe (Robinson et al) 4 . Magnetic Resonance Imaging (MRI) demonstrated the presence of morphologic abnormalities in the temporal lobe and adjacent bone remodeling, which indicate a secondary origin of arachnoid cysts 1 .…”

mentioning

confidence: 99%

“…Magnetic Resonance Imaging (MRI) demonstrated the presence of morphologic abnormalities in the temporal lobe and adjacent bone remodeling, which indicate a secondary origin of arachnoid cysts 1 . Temporal lobe agenesis/hypogenesis is believed to be an important etiology of middle fossa arachnoid cysts 4,5 . The temporal region is quite important in epileptology, since temporal lobe epilepsy (TLE) is not only the most frequent form of epilepsy in adults, but also the refractory epilepsy with the best post-operative results 4 .…”

mentioning

confidence: 99%

“…Temporal lobe agenesis/hypogenesis is believed to be an important etiology of middle fossa arachnoid cysts 4,5 . The temporal region is quite important in epileptology, since temporal lobe epilepsy (TLE) is not only the most frequent form of epilepsy in adults, but also the refractory epilepsy with the best post-operative results 4 . In TLE the main feature is the hippocampal atrophy (HA), which can be associated with atrophy of the amygdala and the temporal lobe 5 .…”

mentioning

confidence: 99%

See 1 more Smart Citation

Temporal lobe hypogenesis associated with arachnoid cyst in patients with epilepsy

Kobayashi

Bonilha

et al. 2003

Arq. Neuro-Psiquiatr.

View full text Add to dashboard Cite

-Objective: To determine the frequency of temporal lobe hypogenesis (TLH) associated with arachnoid cysts (AC) in patients with epilepsy. Method: We retrospectively revised 655 consecutive MRI scans from patients followed in our epilepsy clinic. We identified patients with temporal AC and then performed careful visual analysis in a workstation. Patients with evident expansive or destructive lesions were excluded. Results: Only 4 (0.6%) patients had AC in the left temporal lobe, all associated with TLH. In addition, there were also ipsilateral dysgenetic characteristics in the ipsilateral hippocampus including abnormal shape and axis, and hyperintense T2 signal. In one patient this hippocampal abnormality was bilateral. Conclusion: AC with TLH was rarely found in our patients with epilepsy and it was always associated with hippocampal dysgenesis. Although volumetric reduction of the temporal lobe can be observed in patients with epilepsy and hippocampal abnormalities, the presence of adjacent AC points to a malformative etiology.KEY WORDS: hippocampal dysgenesis, cortical development malformation, partial epilepsy, seizures, magnetic resonance imaging.Hipogenesia de lobo temporal associada a cisto aracnóide em pacientes com epilepsia Hipogenesia de lobo temporal associada a cisto aracnóide em pacientes com epilepsia Hipogenesia de lobo temporal associada a cisto aracnóide em pacientes com epilepsia Hipogenesia de lobo temporal associada a cisto aracnóide em pacientes com epilepsia Hipogenesia de lobo temporal associada a cisto aracnóide em pacientes com epilepsia RESUMO -Objetivo: Determinar a freqüência de hipogenesia de lobo temporal (HLT) associada a cistos de aracnóide (CA) em pacientes com epilepsia. Método: Revisamos retrospectivamente 655 exames consecutivos de ressonância magnética de pacientes do ambulatório de epilepsia da UNICAMP. Identificamos os pacientes com CA na região temporal e então realizamos análise visual em uma estação de trabalho. Pacientes com lesões expansivas ou destrutivas evidentes foram excluídos. Resultados: Apenas 4 (0,6%) pacientes tinham CA na região temporal esquerda, todos associados a HLT. Além destas alterações, observamos características disgenéticas no hipocampo ipsilateral incluindo anormalidades no formato e eixo e hipersinal em T2. Em um paciente estas alterações hipocampais eram bilaterais. Conclusão: CAs associados a HLT foram raramente encontrados, e quando presentes, estavam sempre acompanhados de disgenesia hipocampal. Apesar da redução volumétrica do lobo temporal poder ser observada em pacientes com epilepsia e alterações hipocampais, a presença de um CA adjacente indica uma etiologia de malformação. PALAVRAS-CHAVE: disgenesia hipocampal, epilepsia parcial, convulsões, cistos de aracnóide, ressonância magnética. Arachnoid cysts are considered frequent and incidental findings in neuroimaging studies 1 . The most frequent localization is in the middle fossa, corresponding to more than half the cases reported in the literature 2 . The origin of middle fossa ...

show abstract

“…A origem do AC está provavelmente relacionada à divisão ou duplicação da aracnóide 17 . No passado acreditava-se que ocorria uma agenesia da região fronto-temporal no período fetal 14 . Estudos recentes de volume cerebral em pacientes com AC demonstram não haver diminuição do volume cerebral no hemisfério com AC, quando comparado com o hemisfério contralateral 19 .…”

unclassified

Associação singular de síndrome de Kallmann e cisto aracnóide da fossa média: relato de caso

Fernandes

Guerra

Lemos

et al. 1995

Arq. Neuro-Psiquiatr.

View full text Add to dashboard Cite

RESUMO -O hipogonadismo hipogonadotrófico pode resultar de diferentes anomalias do sistema nervoso central, apresentando sinais clínicos que dependem da idade de aparecimento, bem como do grau de deficiência gonadotrófica e de sua associação com outras deficiências hipofisárias. Relatamos o caso de um rapaz de 18 anos com atraso puberal, retardo de crescimento estatural a partir de 10 anos e história de dificuldade de aprendizado escolar. Ao exame apresentava discreto aspecto eunucóide, 162 cm de altura (z score = -2,17), estadiamento puberal GII, PII, testículos de 4 cm 3 . A avaliação complementar demonstrou níveis pré-puberais de testosterona, megateste com resposta normal, exceto ao teste de estímulo com GnRH agudo e prolongado. CT de crânio mostrou cisto aracnóide da fossa média esquerda com extensão supra-selar. Foi feito o diagnóstico de hipogonadismo hipogonadotrófico provavelmente secundário à compressão pelo cisto aracnóide e por isso, optou-se por derivação cisto-peritoneal. Após a cirurgia não houve retomada do desenvolvimento puberal, sendo então verificada anosmia bilateral e, portanto, diagnosticada Síndrome de Kallmann, que foi confirmada pelos achados da RNM, embora os resultados hormonais não sejam totalmente compatíveis com a referida síndrome. Não encontramos na literatura descrição da associação entre Síndrome de Kallmnann e cisto aracnóide e acreditamos que neste caso os resultados da avaliação hormonal possam ser devidos a tal associação que provocou adicionalmente uma disfunção hipofisária. PALAVRAS-CHAVE: hipogonadismo, cisto aracnóide, Síndrome de Kallmann.Singular association of Kallmann's syndrome and arachnoid cyst of middle fossa: case report SUMMARY -Hypogonadotrophic hypogonadism can result from different abnormalities in the central nervous system. The clinical picture depends upon the time of onset the deficiency, the magnitude of the gonadotropins deficiency and whether there are other pituitary hormone deficiencies as well. We report on a 18-year-old boy, who was investigated because of pubertal and growth delay. He also had learning disabilities. On physical examination he exhibited mild eunuchoid aspect, 162 cm height (z score = -2,17), pubertal development on stage G II, P II, and 4 cm 5 testis. Laboratory investigation revealed pre-pubertal levels of testosterone and normal results of the combined test of anterior pituitary function, except for in GnRH acute and prolonged test. Brain CT showed an arachnoid cyst on left middle fossa with expansion to suprasellar cisterna. He was diagnosed as having hypogonadotrophic hypogonadism secondary to compression by the cyst, and a cyst-peritoneal derivation was performed. After surgery there was no improvement of the pubertal state and bilateral anosmia was discovered, so Kallmann's syndrome was then diagnosed and was confirmed by MRI, even though the hormonal results are not totally matched with the refered syndrome. We did not find in the literature any description of the association between Kallmann's syndrome and arachnoid cyst ...

show abstract

The Temporal Lobe Agenesis Syndrome

Cited by 137 publications

References 0 publications

Quiste subaracnoideo y displasia del ala mayor del esfenoides en neurofibromatosis

Quiste subaracnoideo y displasia del ala mayor del esfenoides en neurofibromatosis

Temporal lobe hypogenesis associated with arachnoid cyst in patients with epilepsy

Associação singular de síndrome de Kallmann e cisto aracnóide da fossa média: relato de caso

Contact Info

Product

Resources

About