Asymptomatic enlargement of the sella turcica.

Forty-six patients with enlarged sella turcica and pneumographic evidence of an intrasellar mass were initially untreated. The courses were variable, and 50% did not require subsequent treatment. All patients with initial visual involvement developed progressive visual impairment. Of patients with clinical evidence of pituitary insufficiency, 66% developed visual field defects and required treatment. Only one patient, whose sole symptom was headache, was subsequently treated, and no asymptomatic patient developed subsequent symptoms and required treatment. Asymptomatic patients with an enlarged sella turcica should have an air study to exclude an "empty sella" syndrome or primary hypothyroidism.     

腺垂体功能减退症临床特征变化分析

目的探讨腺垂体功能减退症患者的病因结构变化及临床表现。方法回顾性分析我院2013-01—2016-12住院及门诊78例腺垂体功能减退症患者的临床资料。结果男32例(41.03%),女46例(58.97%);诊断时年龄11~89岁,平均62.5岁;鞍区占位(包括术前及术后)52例(66.67%),席汉综合征8例(10.26%),空泡蝶鞍9例(11.65%),病因不明8例(10.26%),垂体-下丘脑发育不良1例(1.28%)。首次就诊科室:纳差厌食、恶心呕吐就诊于消化内科36例(46.15%)最常见。ACTH+TSH+Gn+G激素缺乏为19例最多,占24.36%,ACTH+TSH+Gn缺乏15例,占19.23%。结论腺垂体功能减退症病因结构发生变化,发病人群、首发症状及受累激素也不同,患者女性多于男性,发病年龄偏高,症状不典型,分布于临床多个科室,其中以低钠血症为首发临床表现就诊消化内科最多。     

Does pseudotumor cerebri cause the empty sella syndrome?

Eight patients had both the "primary empty sella syndrome," diagnosed by the finding of an air-filled sella turcica at pneumoencephalography, and pseudotumor cerebri, diagnosed by the finding of an elevated cerebrospinal fluid pressure in the presence of normal ventricular size and position on pneumonencephalography. All eight patients were obese women, and six were hypertensive. Six complained of headaches and menstrual irregularities, and two were asymptomatic. Three had visual symptoms and four had papilledema at the time of examination. These two clinical disorders appear to be frequently related, and when they are related, visual field defects and visual loss are more likely to occur than when either entity appears alone. Chronically increased intracranial pressure from pseudotumor cerebri may produce an empty sella if the diaphragma sella is incompetent and the subarachnoid space herniates into the sella turcica.     

Empty sella turcica in intracranial sarcoidosis. Pituitary insufficiency, primary polydipsia, and changing neuroradiologic findings

A 37-year-old man with visual loss was found to have hypopituitarism and primary polydipsia associated with sarcoidosis. Neuroradiologic studies demonstrated a dramatic evolution of CNS lesions, including a left thalamic infarct, an enhancing suprasellar mass, and ultimately an empty sella turcica. The patient has been clinically stable in spite of these changes. This case is likely to be the first reported of CNS sarcoidosis with an empty sella turcica documented by computed tomography.     

鼻咽癌放疗后垂体功能减退的临床研究

目的 探讨鼻咽癌放疗后垂体功能减退(RIH)的临床特点及影像学表现. 方法 回顾性分析自2000年至2010年在中山大学孙逸仙纪念医院就诊的鼻咽癌放疗后诊断为RIH的9例患者临床资料(男7例,女2例),分析其临床表现、激素水平、影像学表现及后期随访结果. 结果 9例患者均诊断为RIH,距离放疗结束的时间为10月～15年.5例患者发现1种以上垂体分泌激素的紊乱,其中甲状腺激素分泌减少和高催乳素血症是最常见的表现,其次是促肾上腺皮质激素分泌减少.2例患者影像学表现为垂体萎缩和空泡蝶鞍.5例患者经激素替代治疗后症状改善.结论 鼻咽癌放射治疗可导致垂体功能损害,大部分患者表现为亚临床症状并缺乏典型垂体改变,早期、定期的激素监测和影像学随访对于RIH的早期诊断和治疗尤为重要.     

Empty sella syndrome]

The authors present two middle-aged female patients with empty sella revealed at imaging studies (CT, MRI). Their main complaint was severe fronto-parietal and fronto-temporal headache. Physical examination showed obesity, hypertension, and local hypersensitivity on deep palpation and percussion in the above-mentioned regions, in both cases. Endocrine function of pituitary gland, visual fields and fundi were normal as was EEG. The CSF composition and pressure also showed no abnormalities. The diagnostic and therapeutic problems of empty sella syndrome are discussed.     

Magnetic Resonance Imaging in Empty Sella Phenomena

Of 500 consecutive unselected patients who underwent brain magnetic resonance imaging (MRI), 20 (12 females, 8 males; mean age, 41 yr) were found to have empty sella. The clinical and imaging findings were compared to those in 20 normal adult control subjects, and 20 patients of comparable age and sex from the group of 500 consecutive patients. Variations in the pituitary infundibulum, the optic chiasm, and the sellar floor did not correlate to symptoms. Little difference in clinical symptomatology between patients with empty sella and control subjects was found. It is concluded that empty sella is often an insignificant finding in adults.     

The 'empty' sella.

Records of all patients diagnosed as having an "empty" sella turcica at the University of Michigan Medical Center after 1972 were retrospectively reviewed. Nineteen patients satisfied the two criteria, absence of prior surgical or radiotherapeutic intervention and air within the sella turcica at pneumoencephalography. Associated clinical signs and symptoms were varied and nonspecific. Two patients had relative visual field deficits without increased intracranial pressure. Three patients had papilledema and were diagnosed as having had benign increased intracranial pressure. Increased intracranial pressure was not a consistent enough finding to be considered an etiology for the empty sella syndrome.     

Secondary partial empty sella syndrome in an elite bodybuilder.

The pituitary gland is a hormone-responsive gland and is known to vary in size depending on the hormonal status of the patient and the multifaceted positive and negative feedback hypothalamic-pituitary-gonadal axis. Partial empty sella syndrome with an atrophied pituitary gland is seen in primary neuroendocrinopathies such as growth hormone deficiency, primary hypothyroidism, central diabetes insipidus and hypogonadism. Partial empty sella has also been shown to occur in patients with elevations in intracranial pressure. Secondary partial empty sella syndrome with significant pituitary gland atrophy from negative feedback inhibition of long-term exogenous hormonal use has not been previously reported. We are reporting on a case of partial empty sella syndrome occurring in an elite bodybuilder with a long history of exogenous abuse of growth hormone, testosterone and thyroid hormone. The pathophysiological mechanisms of secondary partial empty sella syndrome from exogenous hormone use and the possibility for elevations in intracranial pressure contributing to this syndrome will be discussed.     

Treatment of empty sella syndrome with ventriculoperitoneal shunt.

A symptomatic empty sella developed in a female patient undergoing bromocriptine therapy for microprolactinoma. Placement of a ventriculoperitoneal shunt dramatically improved the symptoms of headache and blurred vision. The post-operative imaging showed resolution of the empty sella. She was able to resume bromocriptine therapy without recurrence of her previous symptoms and give birth to a baby 20 months later. An MRI 44 months after surgery and on bromocriptine therapy showed no recurrence of the empty sella. We conclude that ventriculoperitoneal shunt may be a simple, and durable treatment for drug induced empty sella and allows resumption of bromocriptine therapy for preexisting microprolactinoma.     

Secondary partial empty sella syndrome in an elite bodybuilder

Abstract

The pituitary gland is a hormone-responsive gland and is known to vary in size depending on the hormonal status of the patient snd the multifaceted positive and negative feedback hypothalamic-pituitary-gonadal axis. Partial empty sella syndrome with an atrophied pituitary gland is seen in primary neuroendocrinopathies such as growth hormone deficiency, primary hypothyroidism, central diabetes insipidus and hypogonadism. Partial empty sella has also been shown to occur in patients with elevations in intracranial pressure. Secondary partial empty sella syndrome with significant pituitary gland atrophy from negative feedback inhibition of long-term exogenous hormonal use has not been previously reported. We are reporting on a case of partial empty sella syndrome occurring in an elite bodybuilder with a long history of exogenous abuse of growth hormone, testosterone and thyroid hormone. The pathophysiologal mechanisms of secondary partial empty sella syndrome from exogenous hormone use and the possibility for elevations in intracranial pressure contributing to this syndrome will be discussed.     

Analysis of Empty Sella Secondary to the Brain Tumors

Objective

The definition of empty sella syndrome is ''an anatomical entity in which the pituitary fossa is partially or completely filled with cerebrospinal fluid, while the pituitary gland is compressed against the posterior rim of the fossa''. Reports of this entities relating to the brain tumors not situated in the pituitary fossa, have rarely been reported.

Methods

In order to analyze the incidence and relationship of empty sella in patients having brain tumors, the authors reviewed preoperative magnetic resonance imaging (MRI) of 72 patients with brain tumor regardless of pathology except the pituitary tumors. The patients were operated in single institute by one surgeon. There were 25 males and 47 females and mean patient age was 53 years old (range from 5 years to 84 years). Tumor volume was ranged from 2 cc to 238 cc.

Results

The overall incidence of empty sella was positive in 57/72 cases (79.2%). Sorted by the pathology, empty sella was highest in meningioma (88.9%, p = 0.042). The empty sella was correlated with patient''s increasing age (p = 0.003) and increasing tumor volume (p = 0.016).

Conclusion

Careful review of brain MRI with periodic follow up is necessary for the detection of secondary empty sella in patients with brain tumors. In patients with confirmed empty sella, follow up is mandatory for the management of hypopituitarism, cerebrospinal fluid (CSF) rhinorrhea, visual disturbance and increased intracranial pressure.     

Propionic acidemia associated with visual hallucinations

Propionic acidemia, an autosomal recessive disorder, is a common form of organic aciduria resulting from the deficiency of propionyl-CoA carboxylase. It is characterized by frequent and potentially lethal episodes of metabolic acidosis often accompanied by hyperammonemia. A wide range of brain abnormalities have been reported in propionic acidemia. We report recurrent visual hallucinations in 2 children with propionic acidemia. Four visual hallucination events were observed in the 2 patients. Three episodes were preceded by an intercurrent illness, and 2 were associated with mild metabolic decompensation. The 2 events in one patient were associated with a seizure disorder with abnormal electroencephalogram. Brain magnetic resonance imaging showed abnormal basal ganglia and faint temporo-occipital swelling bilaterally. This is probably the first report of visual hallucinations in propionic acidemia and should alert the treating clinicians to look for visual hallucinations in patients with organic acidurias, especially in an unusually anxious child.     

Empty sella syndrome in childhood

The empty sella syndrome is common in middle-aged women, usually presenting with headache, and only occasionally associated with endocrine or visual abnormalities. It is rare in childhood. Childhood cases tend to present either with endocrine disturbances, visual symptoms, or with craniofacial syndromes. We present three cases of complete empty sella with childhood onset, each discovered unexpectedly during evaluation of endocrine or visual dysfunction.     

Hyperprolactinaemia and the empty sella

Hyperprolactinaemia is an endocrine abnormality seen not infrequently in the population with empty sella; a radiological and anatomical diagnosis of a deformed and enlarged sella turcica. Often there is no associated pathology within the pituitary gland itself, lending to the hypothesis that the empty sella syndrome per se has a yet-to-be defined role in hyperprolactinemia. We report a patient who presented initially with non-specific symptoms of meningeal irritation and viral illness on a long background of galactorrhoea. The patient demonstrated elevated serum prolactin, and a diagnosis of empty sella was made on the basis of MRI findings.     

垂体腺瘤经蝶切除术后发生迟发性视功能减退原因及处理探讨

目的 探讨经蝶切除垂体腺瘤术后继发迟发性视功能减退的原因，尤其是其与空蝶鞍的关系，提出积极防治的意义。方法 分析了北京协和医院诊治过的4例经蝶手术切除垂体腺瘤术后继发不同程度空蝶鞍的迟发性视功能减退患者，并结合文献加以讨论。结果 这4例患者中2例再次经颅手术，1例再次经蝶手术，1例接受保守治疗，治疗后其视功能均有不同程度地改善或恢复正常。结论 结合文献复习，认为经蝶术后合并空蝶鞍的患者发生迟发性视功能减退与视神经和视交叉局部的手术后瘢痕组织机械性牵拉以及血运改变有关。综合治疗后4例患者的视功能均有不同程度的改善或恢复正常，这既说明了上述原因的存在，又显示了积极防治的意义。     

Psychiatric symptoms in a patient with isolated adrenocorticotropin deficiency: case report and literature review

We report a 59-year-old man with isolated adrenocorticotropin (ACTH) deficiency. The patient presented with sudden onset of delusions and hallucinations at the age of 54, which resolved gradually without treatment. Subsequently, the patient manifested stereotypy, wandering, hypobulia, and autistic symptoms, and was treated with antipsychotics for 1 year without any improvement. He suffered from neuroleptic malignant syndrome-like symptoms at the age of 59. A thorough endocrine assessment revealed isolated ACTH deficiency. After hydrocortisone supplementation, the physical and psychiatric symptoms improved dramatically. Clinicians should consider this rare disease when diagnosing patients with refractory psychiatric symptoms and unique physical symptoms of isolated ACTH deficiency.     

The Heidenhain variant of Creutzfeldt-Jakob disease: clinical, pathologic, and neuroimaging findings.

We report two patients who developed isolated visual symptoms and signs as initial manifestations of Creutzfeldt-Jakob disease (CJD). Both patients had normal conventional T1- and T2-weighted brain magnetic resonance (MR) images; in one patient, early cortical abnormalities were detected by diffusion-weighted and fluid attenuated inversion recovery (FLAIR) magnetic resonance imaging (MRI). Results from the cerebrospinal fluid assay for the 14-3-3 brain protein were also negative in one patient, despite pathologic confirmation of CJD at autopsy. The Heidenhain variant of CJD should be considered in all patients who present with isolated visual manifestations, including homonymous hemianopsia and normal conventional brain MRI. Diffusion-weighted and FLAIR MRI may demonstrate early cortical abnormalities in patients with CJD. The CSF assay for the 14-3-3 protein may be normal, even in pathologically confirmed cases.     

Pseudotumor cerebri. A theory on etiology and pathogenesis

Ten patients with pseudotumor cerebri were studied. The diagnosis was based on bilateral papilledema and absence of intracranial masses and hydrocephalus. Based on the radiological findings, three groups of patients emerged: (A) Five patients with an empty sella. Four had an enlarged sella; (B) Two patients with a pituitary adenoma; and (C) Three patients without sellar or pituitary pathology. The onset of symptoms was acute in four patients, two from group A and one each from groups B and C. One patient from group A had a lesion of the chiasma. Two patients from group B and two from group C had symptoms and signs suggesting engagement of structures in the cavernous sinus. It is suggested that pseudotumor cerebri may be caused by bilateral compression of the cavernous sinuses because of acute necrotic swelling of a preexisting pituitary adenoma.     

Empty sella syndrome in nevoid basal cell carcinoma syndrome

We reported the magnetic resonance imaging of four young patients (13 to 19 years) with nevoid basal cell carcinoma syndrome (NBCCS), which showed empty sella, agenesis of the corpus callosum and empty sella, an interhemispheric lipoma with callosal dysgenesis, and an arachnoid cyst in the posterior fossa, respectively. Calcification of the diaphragma sellae, which is a protective barrier against the pulsating action of the cerebrospinal fluid, may cause the empty sella in NBCCS.