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.

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.

Rathke's cleft cyst--an uncommon cause of headache in a young boy.

Developmental disorders of the pituitary can present like a mass lesion in the region of the pituitary and produce pressure effects. Rathke's cleft cyst is one such developmental disorder of the pituitary which presents in early life producing pressure effects namely visual disturbances due to its proximity to optic chiasma, headache, and hormonal imbalance due to pituitary malfunction. In this case report a 19 years boy presented with symptoms of headache and gradual loss of vision over one year period. Neuro-imaging study demonstrated a cystic lesion in the region of the pituitary. Trans-sphenoidal hypophysectomy was performed. Histopathology of the biopsied material was suggestive of Rathke's cleft cyst. Following surgery the boy had a good visual recovery. So early diagnosis and removal of a Rathke's cleft cyst has a good prognosis as compared to a craniopharyngioma which is a very close differential diagnosis.

Early destructive lesions in the developing brain: clinical and electrographic correlates

OBJECTIVE: Early brain insults can cause cavitary lesions including porencephaly (POR) and multicystic encephalopathy (MCE). The objective of this study was to investigate clinical and electrographic correlates associated to these types of destructive brain lesions. METHOD: Patients with POR and MCE were selected and submitted to clinical and Video-EEG monitoring. The following variables were analyzed: demographic data, type of lesion, presence of gliosis, perinatal complications, epilepsy, brain atrophy, and presence and frequency of epileptiform discharges. RESULTS: Twenty patients were included, 65 percent males, 35 percent females, ages ranging from 1 to 40 years, 14 with MCE and 6 with POR. Eighteen patients had hemiparesis, 19 had epilepsy (current or in the past), seven of them had refractory seizures, and 16 had epileptiform discharges. All patients with MCE had gliosis while only 2 with POR had it. CONCLUSIONS: No correlation was observed between type of lesion and clinical and electrographical outcome. However, a positive correlation was observed between frequency of discharges and presence of brain atrophy, and between MCE and gliosis.

OBJETIVO: Insultos cerebrais precoces podem causar lesões cavitárias incluindo porencefalias (POR) e encefalomalacias multicisticas (EMC). O objetivo deste estudo foi investigar correlatos clínicos e eletrográficos associados a estes dois tipos de lesões destrutivas. MÉTODO: Pacientes com POR e EMC foram selecionados e submetidos à avaliação neurológica e monitorização vídeo-eletrencefalográfica, analisando-se as seguintes variáveis: dados demográficos, tipo de lesão, presença de gliose, complicações perinatais, epilepsia, atrofia cerebral, presença e freqüência de descargas epilépticas. RESULTADO: Vinte pacientes foram incluídos, sendo 65 por cento do sexo masculino, 35 por cento do feminino, idades entre 1 e 40 anos, sendo 14 com EMC e 6 com POR. Dezoito pacientes tinham hemiparesia, 19 tinham ou tiveram epilepsia (7 deles refratários ao tratamento medicamentoso) e 16 deles tinham paroxismos epileptiformes. Todos com MCE tinham gliose associada, contra apenas 2 dos pacientes com POR. CONCLUSÃO: Não houve correlação entre tipo de lesão e evolução clínica e eletrográfica. Houve, entretando, correlação positiva entre freqüência de descargas epilépticas e presença de atrofia cerebral, e entre lesão do tipo EMC e presença de gliose.

Spinal intramedullary neuroepithelial (ependymal) cyst. A rare cause of treatable acute para paresis.

An 8-yr-old female child presented with acute onset of severe pain in the lower limbs and difficulty in walking. Spine MRI showed hyperintense signals on T2 weighted images at T2-T3 level, which was intramedullary in location. The patient was operated and histopathology reported as neuroepithelial cyst. Spinal intramedullary neuroepithelial cysts are rare. Spinal cord compression due to the cyst is very uncommon and because of its rarity the present case is being reported. The clinical features, embryogenesis and literature were reviewed briefly.

Alterações visuais decorrentes do cisto da fenda de Rathke: relato de caso / Visual alterations due to Rathke's cleft cyst: case report

Os cistos da fenda de Rathke são lesões para-selares, geralmente assintomáticas, presentes em 12 a 33 por cento das autópsias de pacientes com hipófise normal. Ocasionalmente, os cistos podem aumentar de volume a ponto de comprimir estruturas supra-selares e intra-selares, levando ao aparecimento dos sintomas. Clinicamente, os pacientes referem escurecimento visual, além de apresentarem defeitos campimétricos. M.A.S.R., 47 anos, sexo feminino, branca, natural de Pelotas/RS com queixa de embaçamento progressivo da visão do olho direito há 2 meses. Ao exame, constatou-se baixa visual e hemianopsia bitemporal. A tomografia computadorizada evidenciou imagem hipodensa arredondada, de bordos nítidos, em topografia selar, determinando remodelação e alargamento da sela túrcica. A ressonância magnética mostrou lesão expansiva cística localizada na sela túrcica com crescimento supra-selar. A referida lesão apresenta obliteração da cisterna supra-selar e importante compressão sobre o quiasma óptico. A abordagem cirúrgica confirmou a presença de lesão expansiva cística comprimindo o quiasma óptico e o exame de anatomia patológica do material diagnosticou cisto da fenda de Rathke. O exame oftalmológico, três meses após a cirurgia, mostrou melhora da acuidade visual e recuperação total dos defeitos do campo visual. O cisto da fenda de Rathke deve ser incluído no diagnóstico diferencial dos tumores para-selares passíveis de causar compressão da via óptica nervosa e ressalta-se a importância do diagnóstico e tratamento precoce no intuito de prevenir danos estruturais com perdas irreversíveis da função visual, bem como os distúrbios endócrinos.

Megalencephalic leukoencephalopathy with subcortical cysts.

Megalencephalic leukocncephalopathy is rare disorder seen in India in patient belonging to Agarwal community. Many of the patients may have a mild clinical course with gradual worsening of neurological disability. A case is being reported who was followed for 17 years and paradoxically showed radiological and clinical improvement.

Transient Isolated Trochlear Nerve Palsy Associated with Rathke's Cleft Cyst

We report the case of a 52-year-old woman who developed vertical diplopia of 1-days duration. Neuro-ophthalmological testing revealed left trochlear nerve palsy, and sellar MRI revealed a 1.5 cm-sized pituitary mass lesion, a Rathke's cleft cyst. The diplopia disappeared spontaneously after 6 days.

Suprasellar arachnoid cyst presenting with bobble-head doll movements: a report of 3 cases.

We report a series of three patients with suprasellar arachnoid cysts who presented with a rare 'bobble-head doll' syndrome. The abnormal head movements improved after surgical evacuation of the cysts in all the three cases. Various pathophysiological mechanisms involved in the bobble-head doll syndrome are discussed. The literature on suprasellar arachnoid cysts is briefly reviewed.

A Case of Rathke's Cleft Cyst Inflammation Presenting with Diabetes Insipidus

Rathke's Cleft Cyst (RCC), which is located at the intrasellar region, is considered to be the distended remnants of Rathke's pouch, an invagination of the stomodeum. Lined with columnar or cuboidal epithelium of ectodermal origin, RCC usually contains mucoid material and it is found in 13-22% of normal pituitary glands. The cyst rarely leads to the development of symptoms but, when it does, the most common presenting symptoms are headache, visual impairment, hypopituitarism and hypothalamic dysfunction. However, in some cases it presents symptoms of diabetes insipidus, decreased libido and impotence. Recently we experienced a case of RCC inflammation presenting with diabetes insipidus and treated with transsphenoidal surgery. To our knowledge, this is the first report of RCC presenting with symptoms of diabetes insipidus in Korea.

Congenital destructive hemispheric lesions and epilepsy: clinical features and relevance of associated hippocampal atrophy

We studied the clinical, EEG and MRI findings in 19 patients with epilepsy secondary to congenital destructive hemispheric insults. Patients were divided in two groups: 10 with cystic lesions (group 1), and 9 with atrophic lesions (group 2). Seizure and EEG features, as well as developmental sequelae were similar between the two groups, except for the finding that patients of group 2 more commonly presented seizures with more than one semiological type. MRI showed hyperintense T2 signal extending beyond the lesion in almost all patients of both groups, and it was more diffuse in group 2. Associated hippocampal atrophy (HA) was observed in 70 percent of group 1 patients and 77.7 percent of group 2, and it was not correlated with duration of epilepsy or seizure frequency. There was a good concordance between HA and electroclinical localization. The high prevalence of associated HA in both groups suggests a common pathogenesis with the more obvious lesion. Our findings indicate that in some of these patients with extensive destructive lesions, there may be a more circumscribed epileptogenic area, particularly in those with cystic lesions and HA, leading to a potential rationale for effective surgical treatment