Quiste de bolsa de Rathke e hiperprolactinemia, una causa infrecuente de amenorrea primaria / Rathke cleft cysts and hyperprolactinemia, an uncommon cause of primary amenorrhea

El quiste de la bolsa de Rathke es una lesión epitelial benigna de la región selar, formada a partir de remanentes embrionarios. La mayoría de los casos son asintomáticos, aunque pudiera presentarse con cefalea, disfunción hipofisaria y trastornos visuales, muy infrecuentemente como apoplejía hipofisaria. Se presenta el caso de una paciente que, habiendo presentado amenorrea primaria, se le realiza el diagnóstico de quiste de la bolsa de Rathke con hiperprolactinemia, logrando menarquia luego del tratamiento con cabergolina.

Rathke's cyst is a benign epithelial lesion of the sellar region, formed from embryonic remnants. Most cases are asymptomatic although it could present with headache, pituitary dysfunction and visual disorders, very infrequently as pituitary stroke. We present the case of a patient who, having presented primary amenorrhea, is diagnosed with Rathke's cyst with hyperprolactinemia, achieving menarche after treatment with cabergoline.

Hallazgo por neuroimaginología de microangiopatía cerebral retiniana con calcificaciones y quistes / Neuroimaging findings in cerebroretinal microangiopathy with calcifications and cysts

La microangiopatía cerebral retiniana con calcificaciones y quistes es una enfermedad poco frecuente, caracterizada por alteraciones cerebrales, retinianas y óseas, así como por predisposición al sangrado gastrointestinal. Existen pocos reportes de casos de esta condición, especialmente en adultos, en quienes la incidencia es baja. Los hallazgos por medio de neuroimágenes son característicos, con calcificaciones bilaterales y múltiples formaciones quísticas. El propósito de este artículo fue hacer una revisión bibliográfica e ilustrar dos casos cuyo diagnóstico fue posible con la ayuda de neuroimágenes.

Cerebroretinal microangiopathy with calcifications and cysts is a rare condition characterized by brain, retinal and bone anomalies, as well as a predisposition to gastrointestinal bleeding. There are few reported cases of this condition in adults, among whom the incidence is low. Neuroimaging findings are characteristic, with bilateral calcifications, leukoencephalopathy and intracranial cysts. The purpose of this article was to do a literature survey and illustrate two cases diagnosed with the aid of neuroimaging.

Cisto epidermoide da fossa posterior: relato de caso / Epidermoid cyst of the posterior fossa: a case report

Epidermoid cysts of the central nervous system are uncommon conditions, which are frequently located in the cerebellopontine angle and around the pons. They are covered with keratinized squamous epithelium and keratin lamella, which give its contents a soft, white-pearly appearance. Epidermoid cysts are mostly originated from malformations, presumably associated with surface elements of the nervous system ectoderm during the closure of the neural groove or formation of secondary cerebral vesicles. The authors describe a case of epidermoid cyst in the posterior fossa causing hydrocephalus and review morphologic and diagnostic criteria of this lesion.

Os cistos epidermoides do sistema nervoso central (SNC) são condições incomuns, estando localizados mais frequentemente no ângulo pontocerebelar e ao redor da ponte. Eles são revestidos por epitélio escamoso queratinizado e lamelas de queratina, tornando seu conteúdo branco-perolado e pastoso. Os cistos epidermoides são, na maioria das vezes, originados de malformações, possivelmente associados ao entremeio de elementos superficiais do ectoderma do SNC durante o fechamento da placa neural, ou formação das vesículas cerebrais secundárias. Os autores descrevem um caso de cisto epidermoide da fossa posterior determinando hidrocefalia e revisam critérios morfológicos e diagnósticos dessa lesão.

Quiste glioependimario congenito: reporte de caso / Glioependymal congenital cyst: case report

El Quiste Glioependimario es un tumor extremadamente raro y excepcionalmente pocos casos han sido reportados; se caracterizan por una cavidad cerrada, con una capa de tejido de epitelio ependimario o una mezcla de tejido ependimario y epitelio coloideo, con un lumen relleno por líquido cefalorraquídeo. Presentamos el caso de un paciente manejado en el servicio de neurocirugía de la Universidad de Cartagena con esta patología.

Rathke's cleft cyst and partial feet adactyly: an unusual association

A 53 year-old woman presented a recurrent bifrontal headache of 2 years duration and bilateral progressive visual disturbance. The clinical and neurological examination showed a bilateral feet adactyly and bitemporal hemianopsia. The brain MRI demonstrated a Rathke's cleft cyst. The patient was operated by a transnasal endoscopic approach. It seems that this unusual association has never been described before.

Mulher de 53 anos com história recorrente de cefaléia com duração de 2 anos bilateral e progressiva, acompanhada de distúrbios visuais. O exame clinico e neurológico mostrou uma adactilia dos pés e hemianopsia bitemporal. A ressonância nuclear magnética cerebral mostrou um cisto de Rathke. A paciente foi operada por via transnasal endoscópica. Aparentemente esta é a primeira vez que esta associação é descrita na literatura.

Leukodystrophies: Indian scenario.

The leukodystrophies are familial disorders with onset usually in infancy or childhood. The clinical features consist of motor dysfunction with varying degree of cognitive decline. Magnetic Resonance Imaging (MRI) has helped to identify and characterize these disorders. In some leukodystrophies, biochemical enzymatic and genetic defects have been identified. The commonest leukodystrophy seen in India is Megalencephalic Leukodystrophy with subcortical cysts. The essential features consist of large head, mild pyramidal and cerebellar dysfunction, and occasional seizures. MRI studies show extensive white matter changes with temporal cysts. It is common in the Agarwal community in India. An identical mutation in exon 2 of the MLC 1 gene has been identified in this community suggesting a founder effect.

Megalencephalic leukoencephalopathy with subcortical cysts.

Megalencephalic leukoencephalopathy with subcortical cysts is a rare disease first described in 1995. It is characterized by macrocephaly and early onset white matter degeneration. We report two siblings who were diagnosed to have this disease. This disease must be included in differential diagnosis of macrocephaly with early onset leukoencephalopathy.

Cistos de bolsa de Rathke: diagnóstico e tratamento / Rathke's pouch cysts: diagnosis and treatment

Estudamos retrospectivamente quatro pacientes com cisto de bolsa de Rathke. Chamamos a atençäo para a apresentaçäo clínica e para o tratamento destas lesões. Todos ocorreram em pacientes do sexo feminino. Os sintomas predominantes foram amenorréia, cefaléia e déficit visual. Revisamos várias séries publicadas de cistos de bolsa de Rathke. Concluimos que o tratamento cirúrgico é ainda a melhor conduta e que a via transesfenoidal é a abordagem mais indicada

Computed tomography versus magnetic reasonance imaging in diagnosis of congenital cystic intracranial lesions

Twenty one cases of congenital cystic intracranial lesions [having a range of age 24 days to 15 years] were subjected to CT and MRI examinations. The imaging findings were compared with the final diagnosis obtained by clinical data, operation and follow up. The lesions appeared mostly as hypodense nonenhancing masses of CSF density by CT, exerting mass effect according to the size and no surrounding edema. By MRI, they appeared hypointense in T1WI and hyperintense in T2WI. The pathological varieties encountered were; 2 cases of holoprosencephaly, 2 hydranencephaly, one porencephaly, 8 Dandy walker [DW] complex, 7 arachnoid cysts, and one epidermoid tumour. Both CT and MRI can give similar informations concerning the size, shape, rim thickness and other morphologic characteristics of cystic intracranial lesions. Additional data given by MRI are; superior visualization of cerebellar vermis in cases of Dandy Walker complex hence it could be classified into [type A] with absent vermis and [type B] with present vermis in the axial MRI cuts for the fourth ventricular level. MRI is better for detection of infection in an arachnoid cyst by the presence of high signal intensity inside the cyst, It is also able to predict the nature of the contents of the epidermoid cyst whether protein or triglycerides according to its signal intensity in both T[1] and T[2] WI. MRI can detect abnormal signal intensity in the cyst wall of a porencephalic cyst that helps differentiation from arachnoid cysts. Also MRI has a better performance specially in posterior fossa lesions as it is not affected by beam hardening artifact as CT. Other advantages of MRI include lack of ionizing radiation and direct visualization of blood flow, Muliplanar capability and high soft tissue resolution. CT is superior to MRI in the detection of calcification in certain lesions as epidermoid cyst