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.     

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.     

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.     

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.     

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.     

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.     

Empty sella in children as a key for diagnosis

We performed magnetic resonance imaging of the pituitary gland in 354 children who were under the care of our pediatric neurological outpatient department. Among them, an empty sella was recognized in 11 children, who all showed normal hormonal results. The frequency of an empty sella was significantly high in idiopathic intracranial hypertension (4/4; P<0.0001) and nevoid basal cell carcinoma syndrome (3/5; P<0.0001). The ratio of an empty sella in children, excluding patients with idiopathic intracranial hypertension and nevoid basal cell carcinoma syndrome, is estimated to be 4/345 (1.2%), which is markedly lower than that in adults. Magnetic resonance imaging of the pituitary gland in children could provide important clues for the diagnosis of idiopathic intracranial hypertension or nevoid basal cell carcinoma syndrome.     

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.     

Endocrinology of pseudotumor cerebri

This article will review the endocrine function of obese young women with pseudotumor cerebri and primary empty sella syndrome. The literature can be difficult to interpret. Much of it predates the era of CT scanning. Often, cases of primary and secondary empty sella syndrome are comingled. The author reviews specific endocrinologic disorders that have sometimes been associated with increased intracranial pressure excluding pituitary adenomas.     

Cerebrospinal fluid pressure and prolactin in empty sella syndrome

In 58 female patients with the primary empty sella (PES) syndrome, a study of the CSF dynamics was done by evaluating both the absorptive reserve by a lumbar infusion test at constant rate, and/or the ICP increase occurring during REM phase of nocturnal physiological sleep. In 33, prolactin (PRL) dynamics were also investigated evaluating both the response to sequential stimulating test with thyrotropin-releasing hormone (TRH) and metoclopramide (MCP) and/or the circadian variation of PRL levels. Impairment of CSF dynamics was found in the 84% who had a hormonal pattern characterized by an increase of the PRL response to TRH and MCP and a decrease of the PRL circadian variation. Twenty-one patients with impaired CSF dynamics underwent CSF shunting procedures with disappearance of the signs of intracranial hypertension. They also had restoration of normal PRL dynamics but the endocrine alterations improved only moderately. Altered CSF dynamics play a role in the pathogenesis of the PES syndrome. A correlation between elevated ICP and the hypothalamo-hypophyseal control of PRL secretion may exist.     

Does bilateral transverse cerebral venous sinus stenosis exist in patients without increased intracranial pressure?

Objective

Transverse cerebral venous sinus stenosis (TSS) is common among patients with idiopathic intracranial hypertension. TSS likely also exists among individuals with normal intracranial pressure (ICP) but the prevalence is unclear. The goal of this study was to identify patients with incidental TSS and normal ICP and describe their characteristics.

Methods

Among 240 adult patients who underwent brain magnetic resonance imaging (MRI) with magnetic resonance venography (MRV) with contrast at our institution between September 2009 and September 2011, 44 had isolated TSS without further substantial imaging abnormality. Medical records were reviewed for symptoms of increased ICP, papilledema, cerebrospinal fluid (CSF) constituents and opening pressure (OP), and reason for brain imaging. Of these, 37 were excluded for confirmed or possible idiopathic intracranial hypertension. Of the remainder, 5 had CSF-OP ≤25 cmH₂O without papilledema, and 2 did not have measured ICP, but had no papilledema or symptoms of increased ICP. Imaging was re-interpreted to assess for signs suggestive of elevated ICP and to characterize the TSS further.

Results

All patients were women (mean age: 41, mean BMI: 37.1). CSF contents were normal, but OPs were at the upper limit of normal (22–25 cmH₂O). Indications for MRI/MRV included query pituitary abnormality (1), migraine (4), and anomalous-appearing optic nerves (2). All had bilateral TSS. Six had short TSS and an empty sella; 1 had long TSS and no empty sella; 1 had flattening of the posterior sclera; 2 had prominence of peri-optic nerve CSF.

Conclusion

Asymptomatic bilateral TSS exists in patients with ICP ≤ 25 cmH₂O, but is likely uncommon. CSF-OP was at the upper limit of normal in our patients, who also had other radiologic signs suggestive (but not specific) of chronically-raised ICP. Findings of bilateral TSS on imaging should prompt funduscopic examination for papilledema.     

侵袭性催乳素腺瘤治疗后海绵窦残留或伴空蝶鞍综合征

目的 探讨侵袭性催乳素(PRL)腺瘤经治疗后海绵窦残留伴空蝶鞍综合征的临床特征和治疗策略.方法 18例侵袭性PRL腺瘤(Knosp分级Ⅲ或Ⅳ级)经治疗后出现海绵窦肿瘤持续残留,首选药物治疗者9例,首选手术治疗结合药物和(或)放疗9例.结果 平均随访55个月,8例PRL水平正常,7例视力改善.14例因鞍内肿瘤吸收遗留空蝶鞍,7例PRL仍升高,其中5例有不同程度的视交叉下疝(P<0.05).PRL水平正常、无临床症状的7例患者采用低剂量溴隐亭治疗达到PRL水平和肿瘤体积的长期控制.结论 PRL腺瘤治疗后海绵窦肿瘤残留伴发空蝶鞍,这一现象应该引起高度重视,尤其对伴有视交叉下疝的病例,长期的密切随访是必需的.无内分泌和压迫症状的海绵窦残留病例可以采用低剂量的药物治疗达到长期的肿瘤控制和内分泌控制.     

Idiopathic intracranial hypertension without papilledema

We describe 10 patients with idiopathic intracranial hypertension who did not have papilledema. Idiopathic intracranial hypertension without papilledema, although rarely reported, may well be a clinically important headache syndrome. Historical and demographic features of patients with idiopathic intracranial hypertension without papilledema are similar to those of patients with papilledema. Obese women with chronic daily headache and symptoms of increased intracranial pressure, pulsatile tinnitus, history of head trauma or meningitis, an empty sella on imaging studies, or a headache that is unrelieved by standard therapy should have a diagnostic lumbar puncture. Findings from laboratory and neurologic investigations are normal in most patients with idiopathic intracranial hypertension without papilledema. Initial management should include removal of possible inciting agents, weight loss if applicable, and standard headache therapy. Lumbar puncture and diuretic therapy should precede a trial of corticosteroids. Surgery (lumboperitoneal or ventriculoperitoneal shunt or perhaps optic nerve sheath fenestration) may be indicated for prolonged incapacitating headache that is not responsive to medical management or lumbar puncture.     

Growth hormone-secreting pituitary adenoma confined to the sphenoid sinus associated with a normal-sized empty sella.

We present a case of growth hormone (GH)-secreting ectopic pituitary adenoma confined to the sphenoid sinus associated with a normal-sized empty sella. It has been well known that acromegaly is sometimes associated with an empty sella. However, such a case usually has a macroadenoma and an empty sella that is large. The authors considered the possible mechanisms of the association between a normal-sized empty sella and an ectopic pituitary adenoma in the sphenoid sinus as the following. Primary empty sella existed originally, and the pituitary adenoma developed later. The adenoma extended into the sphenoid sinus because of the pulsatile intracranial cerebrospinal fluid pressure.     

Simultaneous recording of cerebrospinal fluid pressure and middle cerebral artery blood flow velocity in patients with suspected symptomatic normal pressure hydrocephalus.

CSF pressure (intracranial pressure, in one patient lumbar pressure) was monitored continuously for one night in 23 patients with suspected symptomatic normal pressure hydrocephalus (NPH) to identify patients who might benefit from subsequent shunt surgery. In 20 patients middle cerebral artery (MCA) blood flow velocity by means of transcranial Doppler sonography (TCD) and CSF pressure were recorded simultaneously. In three patients transcranial Doppler signals were insufficient. Spontaneous changes in CSF pressure always paralleled changes in the TCD signal. Equivalents of B-waves as well as intermediate waves (in between B- and A-waves), and C-waves could be identified easily and always appeared in phase. The Doppler signal, however, could not be used to evaluate the absolute changes in CSF pressure. Fast Fourier Transform of the Doppler signal was a useful tool to indicate the relative frequency of B-wave equivalents. In five patients the injection of 10ml saline into the ventricle raised intracranial pressure considerably, but hardly affected the MCA blood flow velocity. Continuous TCD monitoring might be useful as a noninvasive screening procedure in patients with suspected symptomatic NPH before continuous invasive CSF pressure measurements are performed.     

Normal pressure hydrocephalus, parkinsonism, and primary empty sella--coincidence or cause-effect?

The triad of normal-pressure hydrocephalus, parkinsonism, and primary empty sella appeared in a 70-year-old woman. There was dramatic resolution of the extrapyramidal signs and symptoms on treatment with levodopa-carbidopa and ventriculojugular shunt. The syndrome may be the result of several mechanisms, but an alteration of cerebrospinal fluid (CSF) pressure dynamics may be the sole etiology for this triad.     

Empty sella, hypogonadism and hypopituitarism secondary to moyamoya disease.

We report the case of a young male who presented at the age of 34 years with intracranial hemorrhage due to moyamoya disease. He was later diagnosed with hypogonadism and hypopituitarism. Chromosomal evaluation revealed a normal karyotype. The results of further neuroradiological studies led to the diagnosis of empty sella syndrome.     

Relationship between chronic raised intracranial pressure and empty sella presenting hormonal disturbances

The role of intracranial pressure in the development and maintenance of the primary empty sella has been pointed out in the literature previously. The hormonal changes and clinical features have been evaluated in a 30 year-old female patient examined for a convexity meningioma and a 20 year-old patient examined for chronic hydrocephalus caused by cured meningitis. Histories and investigations revealed an empty sella turcica associated with primary amenorrhoea and delayed puberty. The removal of the convexity meningioma resulted in loss of amenorrhoea and a rise in plasma gonadotrophins. The establishment of a ventriculo-peritoneal shunt did not bring about any changes in hormonal values and clinical features except the subjective headache disappeared. The importance of consideration of intracranial causes in patients who have delayed puberty or absence of menstrual history is briefly emphasized in light of the literature. Our data also demonstrated a correlation between an increase in intracranial pressure and a deficiency of hormonal secretion by the hypophysis.     

Relationship between chronic raised intracranial pressure and empty sella presenting hormonal disturbances

The role of intracranial pressure in the development and maintenance of the primary empty sella has been pointed out in the literature previously. The hormonal changes and clinical features have been evaluated in a 30 year-old female patient examined for a convexity meningioma and a 20 year-old patient examined for chronic hydrocephalus caused by cured meningitis. Histories and investigations revealed an empty sella turcica associated with primary amenorrhoea and delayed puberty. The removal of the convexity meningioma resulted in loss of amenorrhoea and a rise in plasma gonadotrophins. The establishment of a ventriculo-peritoneal shunt did not bring about any changes in hormonal values and clinical features except the subjective headache disappeared. The importance of consideration of intracranial causes in patients who have delayed puberty or absence of menstrual history is briefly emphasized in light of the literature. Our data also demonstrated a correlation between an increase in intracranial pressure and a deficiency of hormonal secretion by the hypophysis.     

Brain MRI as a predictor of CSF tap test response in patients with idiopathic normal pressure hydrocephalus

In this study, our objective was to identify the characteristic morphological features of brain MRI associated with a positive cerebrospinal fluid (CSF) tap test in patients with idiopathic normal pressure hydrocephalus (iNPH). Patients diagnosed with clinical suspected iNPH were evaluated. All patients underwent a mini-mental state examination, a brain MRI, and a CSF tap test. The severities of clinical symptoms were rated before and after the CSF tap test. Characteristic brain MRI findings including frontal convexity narrowing, parietal convexity narrowing, upward bowing of the corpus callosum, empty sella, narrowing of the CSF space at the high convexity, marked dilatation of the Sylvian fissure, and disproportion between narrowing of the CSF space at the high convexity and dilatation of the Sylvian fissure (“mismatch” sign) on T1-weighted or FLAIR image were analyzed. Forty-three patients (33 males/ten females, mean age 76.9 ± 6.9 years) with possible iNPH participated in this study. The presence versus absence of empty sella (52.4 vs. 14.3%, OR 6.6, 95% CI 1.5–29.4, p = 0.02) and “mismatch” sign (45.5 vs. 9.5%, OR 7.9, 95% CI 1.5–42.5, p = 0.02) were associated with positive CSF tap test responses. The sensitivity, specificity, positive predictive value, and negative predictive value of the presence of either of these two MRI features in the prediction of CSF tap response were 72.7, 81, 80, and 73.9%, respectively. Specific brain MRI features can be used as markers for the identification of potential CSF tap test responders in iNPH patients. These features may serve as supplemental evidence in the diagnosis of iNPH patients.