Moyamoya disease associated with pituitary adenoma--report of two cases.

Moyamoya disease associated with prolactin (PRL)-producing pituitary adenomas occurred in two females with elevated blood PRL levels (285 and 120 ng/ml). Computed tomography revealed cystic tumors extending from the sella turcica to the suprasellar cistern. Carotid angiography demonstrated stenoses or obstructions of the bilateral internal carotid arteries at their end point and development of bilateral basal moyamoya vessels. Histological diagnosis in one case was PRL-producing chromophobe adenoma. No stigmata of neurofibromatosis or any history of irradiation was found. Compression of carotid arteries by the tumor was unlikely. These cases should therefore be classified as moyamoya disease accompanied by brain tumor, a very rare occurrence. The hypothalamic disturbance caused by moyamoya disease may have induced the hyperprolactinemia, resulting in secondary prolactinoma.     

家族性激素敏感性肾病综合征四例二家系报告

根据临床表型和对激素治疗的敏感性,家族性特发性肾病综合征可分为两种类型:激素抵抗件(FSRINS)和激素敏感性(FSSINS).后者在全世界各地、各种族均见报道, 但报道不多,其发病的遗传背景不详.     

Familial pineoblastoma. Report of two cases

Two cases of pineoblastoma are presented. One occurred in a 12-year-old girl and the other, 20 months later, in her 43-year-old mother. A possible genetic basis and mutual exposure to an environmental factor responsible for the expression of a tumor gene are postulated in the discussion.     

Familial meningiomas. Report of two cases

The Authors report a case of intracranial meningioma in two sisters, with no evidence of neurofibromatosis. A study of the karyotype has been performed; in one patient we have found a pericentric inversion of one chromosome 9. The possible role of the genetical factors in the develop of familial meningiomas is discussed.     

Familial chordoma. A report of two cases

We have treated 175 patients with a chordoma over a ten-year period. Only two had a family history of the condition and we describe these in this paper. In one patient the tumour was at the craniocervical junction and in the other the lesion affected the sacrum. We have undertaken a literature review of familial chordoma and have identified chromosomal abnormalities associated with the condition.     

Familial arteriovenous malformations. Report of four cases in one family

Familial arteriovenous malformations (AVM's) are uncommon entities, with only seven reported cases in the English literature. Some have been associated with hereditary telangiectasia. A family in which AVM's were found in four male members of two generations is reported. In addition, one patient had a large cyst associated with his AVM without previous evidence of acute hemorrhage, which is an uncommon presentation. The family is discussed and a brief review of the literature is presented.     

Familial gliomas: report of four families, with chromosome studies

Four families having multiple family members with cerebral gliomas are presented. Genetic studies were done in some, but no chromosomal abnormalities were found in this group of patients or their families. The authors recommend that careful attention be given to the family history of all glioma patients and that more extensive genetic studies be done. The formation of a registry to report cases of familial gliomas is also suggested.     

Familial idiopathic intussusception: a report of two families

The authors report on two families with 5 out of 10 and two of two siblings who presented with idiopathic ileocolic intussusception. This may suggest hereditary predisposition as an etiologic factor.     

Familial Hirschsprung's disease--a report of 22 affected siblings in four families

Twenty-two infants, belonging to four families from Gaza, presenting with typical features of Hirschsprung's disease are reported. Eight patients died at home shortly after birth, prior to 1974, due to low intestinal obstruction. Fourteen patients were referred to Hadassah University Hospital after 1974 and diagnosed, by rectal biopsy, as having Hirschsprung's disease. Thirteen underwent laparotomy and multiple intestinal biopsies. Ten had total intestinal aganglionosis, two had total or near total aganglionosis, and in only one patient was the common rectosigmoid aganglionosis found. One patient died prior to surgery. This unusual familial disease, involving very long aganglionic segments, raises the possibility that these infants suffered from a unique variant of Hirschsprung's disease.     

Familial idiopathic granulomatosis: sarcoidosis and Crohn''s disease in two Indian families.

The coexistence of sarcoidosis and Crohn's disease in different members of the same family is rare and only two instances are on record. Two Indian families showing this association have been studied. In one a brother and sister are affected, and in the other seven and possibly eight persons in two generations have been affected. The familial occurrence of both these conditions supports the view that a transmissible agent may be concerned in the genesis of both diseases in genetically susceptible individuals.     

Familial forms of intracranial cavernous hemangioma. Apropos of 5 cases in 2 families

The authors report five cases of cavernous hemangioma belonging to two families. In the first family, an 8 year old child was operated on in 1970 for a left frontal location. His mother, aged 39, was operated on in december, 1981, with stereotaxic approach, for two locations: one was frontal location, the other one was in the pineal region. Among the second family, a 21 year old woman was operated on in december 1976, for a cerebellar cavernous hemangioma. Her sister, aged 18, presented with subarachnoid hemorrhage in january 1980. C.T. scan showed a right temporal high attenuation area. This patient was not operated on. The aunt to those two sisters was operated on in october, 1981, for a left temporal cavernous hemangioma. Those five cases represent about half of the intracranial cavernous hemangiomas operated on in the Lille Neurosurgery Department since 1967. Even, unusual, the familial occurrence of this affection must be admitted. It shows a nosological interest, but also allows prevention by searching and surveying the asymptomatic cases, or genetic evaluation of disease risk.     

Metastatic carcinoma to pituitary adenoma. Report of two cases

The authors present two patients with symptomatic carcinomas which have metastasized to pituitary adenomas. Despite the advanced age of these patients, the preoperative clinical and radiologic evaluations were consistent with pituitary adenoma. There was no previous history of malignancy in either patient, and the symptomatic pituitary metastases were responsible for the initial presentations. A review of the literature is presented with emphasis on the incidence and possible factors responsible for tumors metastasizing to distant neoplasms.     

Familial myxomas in four siblings.

Familial myxomas of the heart are very rare, with only two previous reports noted in the literature. This report documents four siblings with myxomas, and three of these patients had two or more myxomas. One sibling had four myxomas excised. The clinical findings, history, examination, and surgical treatment are described.     

Familial occurrence of moyamoya disease with intracranial hemorrhage--report of two cases.

The authors report an instance of familial moyamoya disease involving a 47-year-old female and her 18-year-old daughter, both of whom initially presented, within a 1-year period, with intracranial hemorrhage. Cerebral angiography showed the typical features of moyamoya disease. The mother, whose disease was more advanced, underwent superficial temporal-middle cerebral artery bypass, with satisfactory results. Human leukocyte antigen typing was performed in both cases. The pertinent literature is reviewed.     

Mucinous cysts of the pituitary stalk. Report of two cases

This report describes two cases of a mucinous cyst (Rathke's cleft cyst) in the pituitary stalk: the first was found in a 29-year-old woman 5 years following pregnancy and the second in a 30-year-old woman 6 years after pregnancy. The presenting symptoms are analyzed and the diagnosis is discussed, with emphasis on the role of magnetic resonance imaging.