No full-text available
To read the full-text of this research,
you can request a copy directly from the authors.
Hyponatremia-induced metabolic encephalopathy caused by Rathke's cleft cyst: A case report
Abstract
Rathke's cleft cysts are sometimes associated with aseptic meningitis or metabolic encephalopathy due to hyponatremia. We treated such a case manifest by lethargy, fever and electroencephalographic abnormalities. A 68-year-old man was admitted to our ward after experiencing general malaise, nausea and vomiting and then high fever and lethargy. On admission, he was drowsy and had nuchal rigidity and Kernig's sign. Physically, he was pale with dry, thickened skin. He had lost 5.0 kg of body weight in the last month. His serum sodium was 115 mEq/l. He had a low serum osmotic pressure (235 mOsmol/l) and a high urine osmotic pressure (520 mOsmol/l). His urine volume was 1200-1900 ml/24 h with a specific gravity of 1008-1015. The urine sodium was 210 mEq/l. He did not have an elevated level of antidiuretic hormone. Electroencephalograms showed periodic delta waves over a background of theta waves. With sodium replacement, the patient become alert and symptom free, and his electroencephalographic findings normalized. However, the serum sodium level did not stabilize, sometimes falling with a recurrence of symptoms. Magnetic resonance imaging clearly delineated a dumbbell-shaped intrasellar and suprasellar cyst. The suprasellar component subsequently shrunk spontaneously and finally disappeared. An endocrinologic evaluation showed panhypopituitarism. The patient was given glucocorticoid and thyroxine replacement therapy, which stabilized his serum sodium level and permanently relieved his symptoms. A transsphenoidal approach was performed. A greenish cyst was punctured, and a yellow fluid was aspirated. The cyst proved to be simple or cubic stratified epithelium, and a diagnosis of Rathke's cleft cyst was made. The patient was discharged in good condition with a continuation of hormonal therapy. Rathke's cleft cyst can cause aseptic meningitis if the cyst ruptures and its contents spill into the subarachnoid space. Metabolic encephalopathy induced by hyponatremia due to salt wasting also can occur if the lesion injures the hypothalamus and pituitary gland.
... 48/F Xanthogranulomatous hypophysitis Ruptured RCC Polyuria Nakayama et al. 1999 [33] 68/M Panhypophysitis Coexistent RCC Hyponatremia Roncaroli et al. 1998 [39] ...
Herein, we report on a lymphocytic hypophysitis related to a ruptured Rathke's cleft cyst which is not associated with pregnancy. A 45-year-old woman initially presented with headache and temporary double vision followed by amenorrhea. Preoperative imaging showed an intra- and suprasellar cystic mass. Complete resection of the tumor mass was performed via a transnasal, transseptal approach. Pathological examination displayed lymphocytic infiltrates within fibrotic tissue and residual pituitary cells accompanied by epithelial tissue of a Rathke's cleft cyst. The strongest inflammatory reaction was observed at the site of disrupture of the cyst integrity, suggesting that high protein levels from ruptured Rathke's cleft cyst might have triggered a lymphocytic hypophysitis. Our review of the literature provides further insights regarding the clinical behavior and different histological types of the lesions as well as the inflammatory changes that can occur in Rathke's cleft cysts.
Objective:
Hyponatremia is a known but underrecognized presentation of sellar lesions. Herein, we present a series of patients who presented with single or multiple episodes of hyponatremia.
Methods:
Over 5 years, patients undergoing endonasal surgery for a de novo sellar mass with hyponatremia as an initial presentation were included. Pathology, sodium levels, pituitary hormonal status, and treatment course were documented.
Results:
Of 282 patients, 16 (5.7%) (9 males, 7 females, age 32 to 84 years) presented with severe hyponatremia, with a mean serum sodium level of 115 ± 6 mmol/L (range, 101 to 125 mmol/L), and 3 patients had 2 or more episodes. Severe hyponatremia was a presenting sign in 0, 4.1, 14.3, and 37.5% of patients with craniopharyngiomas (n = 10), pituitary adenomas (n = 243), Rathke's cleft cysts (RCCs) (n = 21), and sellar arachnoid cysts (n = 8), respectively (P<.01). Half of the patients presenting with hyponatremia, including 6 of 10 patients with adenomas and 2 of 3 patients with RCCs, had pituitary apoplexy or cyst rupture. All patients had anterior pituitary gland dysfunction, including 81% with hypoadrenalism and 69% with hypothyroidism. Following surgery, hormonal status was unchanged or improved in 15 patients (median follow-up, 14 months). No patient had tumor/cyst recurrence or recurrent hyponatremia.
Conclusion:
Severe hyponatremia was a presenting sign in 5.7% of patients with sellar pathology, most frequently in patients with arachnoid cysts, RCCs, and pituitary apoplexy. Patients with new-onset severe hyponatremia and no obvious pharmacologic or systemic cause should undergo pituitary hormonal evaluation and brain imaging. Surgical resection and correction of hormonal deficiencies are associated with resolution of recurrent hyponatremic episodes.
Patients with acute alteration in mental status from encephalopathy because of underlying metabolic-toxic or endocrine abnormalities are frequently seen in the acute hospital setting. A rapid diagnosis and correction of the underlying cause is essential as a prolonged state of encephalopathy portends a poor outcome. Correct diagnosis and management remain challenging because several encephalopathies may present similarly, and further laboratory, imaging, or other testing may not always reveal the underlying cause. EEG provides rapid additional information on the encephalopathic patient. It may help establish the diagnosis and is indispensable for identifying nonconvulsive status epilepticus, an important possible complication in this context. The EEG may assist the clinician in gauging the severity of brain dysfunction and may aid in predicting outcome. This review summarizes the current knowledge on EEG findings in selected metabolic and endocrine causes of encephalopathy and highlights distinct EEG features associated with particular etiologies.
Rathke’s cleft cyst is a non neoplastic lesion remnant of the Rathke’s poutch. Symptomatic cysts must be surgically treated. There are no specific neuroradiological features for this lesion. We present a series of 14 symptomatic Rathke’s cleft cysts treated with transphenoidal endoscopic technique, and we correlate the MRI intensity images with the intraoperative findings, the pathological analysis, the growth pattern and the recurrence tendency, to understand wether there is a better way to recognize these lesions, especially the asyntomatic ones, that, despite other neoplastic cysts, colud only be observed with a MRI follow up.
Abstract We report an 18-year-old Japanese male with a lack of secondary sex characterization and growth failure caused by a rare association between Rathke's cyst and hypophysitis. He was referred to us because of delayed secondary sex characterization. Endocrinological examination showed panhypopituitarism, and the replacement of hydrocortisone, levothyroxine, and desmopressin acetate (DDAVP) was initiated. Brain magnetic resonance imaging (MRI) showed a suprasellar region and a swollen pituitary stalk. The mass was partially resected using the transsphenoidal approach. The pathological diagnosis was hypophysitis and Rathke's cyst. Follow-up MRI performed 1 year after surgery showed that the size of sellar region had not changed. After surgery, in addition to pre-operative hormonal replacement, growth hormone and testosterone were initiated. Two years later, the size of sellar region remains unchanged. In conclusion, while an association between Rathke's cyst and hypophysitis is rare, we suggest that this condition should be included in differential diagnosis of the sellar region, even in adolescents.
Rathke's cleft cysts are cystic sellar and suprasellar lesions, characteristically lined by a single layer of ciliated cuboidal or columnar epithelium.
We report 2 patients who presented with gastrointestinal symptoms and were initially investigated for dyspepsia. However, attention was subsequently drawn to persistent hyponatraemia that led to the diagnosis of panhypopituitarism due to Rathke's cleft cyst.
Transsphenoidal surgery followed by drainage of the cyst and partial excision of the cyst wall in both patients.
No recurrence of the lesions over a mean follow-up of 16 months. There has been an improvement of the hypothalamo-pituitary-adrenal axis in 1 patient and the hypothalamo-pituitary-thyroid axis and visual fields in the other.
Symptomatic Rathke's cleft cysts are rare and can occasionally cause panhypopituitarism. Ideal management of these cysts is unclear, but aspiration followed by partial excision of the cyst wall seems the best initial option.
An endoscopic endonasal approach was performed to remove an intra-suprasellar Rathke's cleft cyst. Rathke's cleft cyst are benign lesions, rarely diagnosed because they are often asymptomatic. To the best of our knowledge, at least 475 cases of Rathke's cleft cysts have been reported. They seem to arise from remnants of Rathke's pouch, an invagination of the stomodeum. A 52-year-old woman, complaining of bilateral frontal headaches, was operated on by using an endoscopic endonasal approach, for an intra-suprasellar tumor. The pre-operative diagnosis was non-functioning pituitary adenoma. Intra-operatively a creamy-coloured viscous tissue was found. After the removal of the cyst contents and of the capsule, the suprasellar structures were seen well. The chiasmatic cistern, the chiasm, the pituitary stalk and the pituitary gland were visualized with 0 and 30 degree endoscopes. The pathological findings showed a well-differentiated cuboidal epithelium. The diagnosis was Rathke's cleft cyst. No post-operative complications were observed. The endoscopic technique was particularly suitable in this case, both for the Rathke's cleft features and for an excellent outcome. The Rathke's cleft cyst was easily removed by suction and the cyst wall was entirely removed with curettes and pituitary punches. The hypophysis was distinguished from the cyst and was preserved. The surgical manoeuvres were all done under direct visual control. The absence of nasal packing and of breathing difficulties made comfortable the post-operative outcome. Thus, the endoscopic endonasal approach can be considered the favourite technique in case of either intra- and/or suprasellar Rathke's cleft cysts.
Rathke's cleft cyst is a non neoplastic lesion remnant of the Rathke's poutch. Symptomatic cysts must be surgically treated. There are no specific neuroradiological features for this lesion. We present a series of 14 symptomatic Rathke's cleft cysts treated with transphenoidal endoscopic technique, and we correlate the MRI intensity images with the intraoperative findings, the pathological analysis, the growth pattern and the recurrence tendency, to understand wether there is a better way to recognize these lesions, especially the asyntomatic ones, that, despite other neoplastic cysts, colud only be observed with a MRI follow up.
Stimulation of the region antero-ventral to the third cerebral ventricle (AV3V) by a cholinergic drug, carbachol, and lesions of the AV3V have been demonstrated in previous studies to either augment or decrease sodium excretion, respectively. Atrial natriuretic peptide (ANP) dramatically increases renal sodium excretion and has been localized to brain areas previously shown to be involved in control of sodium excretion. Consequently, to evaluate a possible role of brain ANP in evoking the changes in renal sodium excretion that follow stimulations or lesions of the AV3V, we determined the effect of injection of carbachol into the AV3V of rats on the concentration of plasma ANP and its content in several neural tissues, the pituitary gland, lungs, and atria. Conversely, the effect of lesions in the AV3V on plasma ANP and the content of the polypeptide in the various organs was determined. Injection of carbachol into the AV3V produced the expected natriuresis, which was accompanied within 20 min by a dramatic rise in the plasma ANP concentration and a rise in ANP content in the medial basal hypothalamus, the neurohypophysis, and particularly the anterior hypophysis but without alterations in the content of ANP in the lungs or the right or left atrium. Conversely, there was a dramatic decline in plasma ANP at both 24 and 120 hr after the AV3V lesions had been placed. This was accompanied by a slight decline in the content of the peptide in the lungs. There was no change in its content in the right atrium at 24 hr after lesions, but there was a significant increase at 120 hr. There was a small decline in the content in the left atrium at 24 hr, followed by a rebound to slightly elevated levels at 120 hr. These small changes contrasted sharply with the dramatic decline in content of the peptide in the medial basal hypothalamus, median eminence, neurohypophysis, choroid plexus, anterior hypophysis, and olfactory bulb. These declines persisted or became greater at 120 hr; except in the olfactory bulb in which the decline was no longer significant. The dramatic increase in plasma ANP after carbachol stimulation of the AV3V that was accompanied by marked elevations in content of the peptide in basal hypothalamus and neuro- and adenohypophysis suggests that the natriuresis resulting from this stimulation is brought about at least in part by release of ANP from the brain. Conversely, the dramatic decline in plasma ANP after AV3V lesions was accompanied by very dramatic declines in content of ANP in these same structures, which suggests that the previously shown decrease in sodium excretion obtained after these lesions may be at least in part due to a decrease in release of ANP from the brain. In view of the much larger quantities of the peptide stored in the atria, it is still possible that changes in atrial release may contribute to the alterations in plasma ANP observed after stimulation or ablation of the AV3V region; however, these results suggest that the dramatic changes in plasma ANP that followed these manipulations may be due to altered release of the peptide from brain structures as well as the atria and lungs.
: A 53-year-old man presented with polydipsia, polyuria, lightheadedness on standing, and syncope. Visual field deficits suggesting left optic tract compression and pituitary dysfunction consisting of diabetes insipidus, hyperprolactinemia, and anterior pituitary insufficiency were diagnosed. On the computed tomography scan, an isodense, ring-enhancing, parasellar mass was localized primarily in the suprasellar region and also extended into the sella. It was believed that the pituitary hormone hypersecretion and hyposecretion were due to hypothalamic dysfunction from the mass which was initially believed to be a craniopharyngioma. At surgery a Rathke's cleft cyst was resected. The unusual presentation of this Rathke's cleft cyst presenting as a hypothalamic lesion is discussed. (Neurosurgery 24:424-428, 1989) Copyright (C) by the Congress of Neurological Surgeons
We report a case of extremely severe hyponatraemia which led to the discovery of anterior hypophysis insufficiency associated with empty sella turcica in a dysimmune context. This type of hyponatraemia is consecutive to corticotropic and thyreo-tropic hormones deficiency and to inappropriate secretion of arginine vasopressin. These endocrine abnormalities are of suprapituitary origin. They can be explained by a hypothalamo-hypophyseal dysregulation of little known mechanism, or by a lesion of the hypothalamus.
We have experienced with 50 cases of parasellar tumors, four cases of which had persistent thirst, polydipsia, polyuria, and reversible temporary hyponatremia secondary to hypernatriuresis. The mechanism of the syndrome in these four cases could not be explained by either that of the syndrome of hypernatremia or of the so-called SIADH. We tentatively named this syndrome as "CEREBRAL POLYURIC HYPONATREMIA" and the criteria of this syndrome as as follows: 1) persistent thirst, polydipsia polyuria, 2) reversible temporary hyponatremia secondary to hypernatriuresis, 3) exception of the following items--administration of uretic drugs, renal and adrenal dysfunction, hyperglycemia, hyperlipemia, overadministration of water, and poor administration of NaCl. The mechanism of this syndrome is presumed as follows: 1) compression by a tumor or surgical attack to the anterior hypothalamus, 2) disturbance of the mechanism of ADH secret-on, 3) extrasecretion of natriuretic factor possibly produced in the anterior hypthalamus, and 4) preservation of the thirst center.
Following subarachnoid hemorrhage, the plasma concentration of atrial natriuretic factor is elevated and appears to be independent of atrial stretch. While the hypothalamus and circumventricular organs contribute to sodium and intravascular volume regulation, their influence on atrial natriuretic factor is not known. We tested the hypothesis that, following subarachnoid hemorrhage, suprasellar cisternal blood, intraventricular blood, or ventricular enlargement would be associated with elevated plasma levels of atrial natriuretic factor. Computed tomograms of 26 patients performed less than or equal to 3 days after hemorrhage were analyzed to determine the presence of suprasellar or intraventricular blood and enlargement of the third or lateral ventricle. These results were correlated with the plasma atrial natriuretic factor and serum sodium concentrations. The initial atrial natriuretic factor concentration was elevated and was higher in patients with suprasellar or intraventricular blood than in those without (suprasellar: 131 +/- 20 and 54 +/- 10 pg/ml, respectively; intraventricular: 137 +/- 25 and 84 +/- 31 pg/ml, respectively). The atrial natriuretic factor concentration remained higher over the week following hemorrhage in patients with suprasellar blood than in those without (127 +/- 16 and 68 +/- 12 pg/ml, respectively). The atrial natriuretic factor concentration was not correlated with hyponatremia (125-134 meq/l) or age-corrected ventricular size. Hyponatremia did not correlate with the presence of intraventricular or suprasellar blood. Our data suggest that suprasellar and intraventricular blood disturb hypothalamic function, resulting in an elevated plasma atrial natriuretic factor concentration. The presence of a direct relation between atrial natriuretic factor and hyponatremia remains unclear.
The clinical, radiographic, and pathological findings in 155 patients with symptomatic Rathke's cleft cysts are discussed. Eight patients were treated by the authors and 147 were collected in a review of the literature. This lesion occurred more often in female than male patients by a 2:1 margin, and the mean age at presentation was 38 years. The average patient had been symptomatic for nearly 3 years at the time of treatment, with the most common symptoms and signs being pituitary dysfunction, visual disturbances, and headaches. Affected children generally were pituitary dwarfs. The sella was enlarged in 80% of cases, and the cyst was situated in both an intrasellar and a suprasellar location in 71%. Computerized tomography revealed a low-density cystic mass with capsular enhancement in one-half of the cases. A variable appearance was seen with magnetic resonance imaging. Partial excision and drainage of the cyst by the transsphenoidal approach is the recommended treatment, as the recurrence rate is low. Most symptoms and signs improved or resolved following surgery with the exception of hypopituitarism and diabetes insipidus. The cyst lining was usually composed of ciliated cuboidal or columnar epithelium. Theories as to the origin of Rathke's cleft cysts are also discussed.
We report detailed data on 10 patients who underwent transsphenoidal microsurgical management of histopathologically confirmed Rathke's cleft cysts. Preoperatively, pituitary dysfunction was present in 90%, headaches in 80%, hyperprolactinemia in 70%, and visual interference in 40%. Computed tomography and magnetic resonance imaging had 90% and 100% sensitivity, respectively, in disclosing the lesion. The mean follow-up duration was 22 months. There was no mortality. The only morbidity was sustained diabetes insipidus in one case. Resolution or improvement in preoperative dysfunction occurred in the majority of patients: headaches in 100%, visual deficits in 75%, normalization of hyperprolactinemia in 83%, and reversal of panhypopituitarism in 33%. We conclude that Rathke's cleft cysts can be managed safely and effectively with transsphenoidal drainage and partial excision of the wall.
A 53-year-old man presented with polydipsia, polyuria, lightheadedness on standing, and syncope. Visual field deficits suggesting left optic tract compression and pituitary dysfunction consisting of diabetes insipidus, hyperprolactinemia, and anterior pituitary insufficiency were diagnosed. On the computed tomography scan, an isodense, ring-enhancing, parasellar mass was localized primarily in the suprasellar region and also extended into the sella. It was believed that the pituitary hormone hypersecretion and hyposecretion were due to hypothalamic dysfunction from the mass which was initially believed to be a craniopharyngioma. At surgery a Rathke's cleft cyst was resected. The unusual presentation of this Rathke's cleft cyst presenting as a hypothalamic lesion is discussed.
Atrial natriuretic factor (ANF) is a diuretic natriuretic peptide hormone produced by both the heart and brain which has been postulated to play a role in the hemodynamic and sodium instability that frequently follows subarachnoid hemorrhage (SAH). Levels of ANF were measured in 12 patients with nontraumatic SAH and nine control patients with unruptured cerebral aneurysms. At surgery, the mean plasma ANF level (± standard deviation) of the SAH group was significantly higher than that of the control group (158.1 ± 83.8 vs. 57.8 ± 45.3 pg/ml, respectively; p = 0.01). There was no significant difference in serum sodium concentration, blood pressure, or central venous pressure between these groups. Nine patients with SAH due to aneurysm rupture had plasma ANF levels similar to those in three patients with SAH due to other causes. Four patients with moderate to severe SAH had significantly higher mean cerebrospinal fluid (CSF) ANF values (17.7 ± 12.8 pg/ml) than five patients with minimal SAH (0.6 ± 0.9 pg/ml) or the control group of nine patients (3.7 ± 1.3 pg/ml) (p < 0.05). Five patients with moderate to severe SAH had significantly higher plasma ANF values (202.6 ± 72.2 pg/ml) than five with minimal SAH (86.8 ± 29.2 pg/ml) or the control group (57.8 ± 45.3 pg/ml) (p < 0.05). Plasma ANF values were substantially higher than CSF ANF content in the SAH group (p < 0.01) and in the control group (p = 0.05).
From these data it is concluded that: 1) plasma ANF is elevated significantly after SAH; 2) this rise appears unrelated to the cause of hemorrhage, serum sodium concentration, blood pressure, or central venous pressure, but is related to the extent of the hemorrhage; 3) ANF concentrations in the CSF are significantly lower than in plasma, and are elevated after moderate to severe SAH; and 4) the source of CSF ANF is probably the plasma, and the source of plasma ANF is likely the heart.
Myocytes in the atria contain a prohormone that gives rise to atrial natriuretic peptides (ANP), which have intrinsic hemodynamic regulatory activity. Little is known about mechanisms regulating ANP release. In rats with indwelling catheters, acute blood volume expansion with 5% (wt/vol) dextrose increases the amount of circulating immunoreactive ANP by a factor of 2-3, as determined by radioimmunoassay. Pithing, which both removes neurogenic influences and interrupts humoral influences of the brain and pituitary gland on the heart, completely blocked stimulus-induced release of ANP. Because our studies using pharmacological blockade of the autonomic nervous system had suggested that neurogenic mechanisms do not play a major role in ANP release, we sought a humoral mechanism involved in ANP secretion. Basal and stimulated release of ANP were significantly blunted in hypophysectomized rats (8 days after operation) but were completely restored when the resected anterior pituitary was reimplanted under the kidney capsule. This suggests that hormones of anterior pituitary origin are required for ANP secretion in response to acute volume loading.
Hyponatremia is common following aneurysmal subarachnoid hemorrhage and has been linked to the syndrome of inappropriate secretion of antidiuretic hormone. However, the demonstration of volume depletion and natriuresis in some patients has suggested that salt wasting is a more likely etiology. Atrial natriuretic factor appears to play a role in both central and peripheral regulation of sodium homeostasis. To investigate the behavior of circulating atrial natriuretic factor following subarachnoid hemorrhage, we studied 25 patients with intracranial aneurysms: 21 after acute subarachnoid hemorrhage and four without evidence of recent rupture. Atrial natriuretic factor was measured by radioimmunoassay of extracted plasma (normal value, 20.8 +/- 24.6, mean +/- 3 SD). Mean +/- SEM plasma atrial natriuretic factor concentration was elevated to 84 +/- 25 pg/ml on Day 1, rose to 134 +/- 29 pg/ml on Day 3, and fell to 86 +/- 17 pg/ml by Day 7 after subarachnoid hemorrhage (p less than 0.01). In two patients (9.5%) who developed hyponatremia after aneurysm rupture, plasma concentrations were no different from that in the group as a whole; concentrations in patients with no evidence of recent subarachnoid hemorrhage were not elevated. Neither fluid administration nor timing of surgery could account for the elevated concentrations. We conclude that concentrations of circulating atrial natriuretic factor are elevated after subarachnoid hemorrhage but do not solely account for the accompanying hyponatremia.
Die Autoren berichten über einen Fall einer symptomatischen Zyste der Rathke'schen Tasche (RCC). Der einzige klinische Hinweis war Kopfschmerz. Der Zysteninhalt wurde durch einen transsphenoidalen Zugang abgelassen. Der postoperative Verlauf was komplikationlos. Die Seltenheit symptomatischer RCC (70 Fälle wurden in der Literatur beschrieben, davon 30 Patienten operiert) macht den Vergleich aller Daten, die in den verschiedenen Publikationen festgehalten sind, erforderlich, um so den richtigen therapeutischen Weg zu finden.
Es scheint, daß das chirurgische Vorgehen auf die Entleerung der Zyste von einem transsphenoidalen Zugang aus beschränkt werden sollte. Dieses Vorgehen ist günstiger als durch eine Kraniotomie. Auf diese Weise wird vermieden, daß der Zysteninhalt in den Supraarachnoidalraum gelangt und zu einer postoperativen aseptischen Meningitis führt. Einige Autoren diskutieren die Möglichkeit der postoperativen Röntgenbestrahlung, obgleich lediglich 6 Patienten ein Rezidiv viele Jahre nach der Operation hatten und der therapeutische Effekt dieses Vorgehens nicht gesichert erscheint.
We studied the sodium balance and changes in plasma volume by an isotope dilution technique in the first week after an aneurysmal subarachnoid hemorrhage in 21 patients. In 11 of the patients, the plasma volume decreased by more than 10%. This was accompanied by a negative sodium balance and hyponatremia in 6 patients, a negative sodium balance without hyponatremia in 4 patients, and a positive sodium balance in 1 patient. Together with a decrease in plasma volume, blood urea nitrogen content increased and body weight decreased. Three patients developed hyponatremia without a decrease in plasma volume. Serum vasopressin was measured in 14 of the 21 patients. The values were elevated on admission and declined in the first week, regardless of the presence of hyponatremia. These findings indicate that natriuresis and hyponatremia in aneurysmal subarachnoid hemorrhage reflect salt wasting and not inappropriate secretion of antidiuretic hormone and that these changes should be corrected by fluid replacement rather than by fluid restriction.
Three cases of Rathke cleft cyst are presented. The clinical symptoms were disturbances of visual acuity and visual fields, and a functional disorder of the hypophysis. In two cases, there were associated meningeal signs. This meningitis was considered to be aseptic and ascribable to leakage of the cyst contents into the subarachnoid space. In one patient, the cyst extended to the suprasellar cistern; a computed tomography scan revealed an enhanced capsule, high-density spotting in a low-density area inside the cyst, and enhanced areas. In another patient, the tumor was localized in the sella turcica; high-density spotting was seen. Based on this finding, we suggest that computed tomography scanning facilitates differentiation from other sellar lesions to some degree. Electron microscopic studies of a cyst with cilia revealed the presence of a coating material around cells with microvilli. On the basis of this finding, Rathke cleft cysts may be considered to be namely, endodermal, inclusions from the upper respiratory tract.
Two unusual cases of symptomatic Rathke's cleft cysts are reported. In one, the cyst was entirely suprasellar with a normal sella turcica. The gross visual failure, hypothalamic disturbances, and hypopituitarism of the patient resolved after a transfrontal aspiration and partial excision of the wall of the cyst. The second patient was a pituitary dwarf with hypothalamic dysfunction, but with normal vision. The patient had a large intrasellar cyst that exhibited marked suprasellar extension with calcification of the capsular rim. After transsphenoidal aspiration and partial excision of the wall of the cyst, the patient achieved a satisfactory recovery. The differential features between Rathke's cleft cyst and craniopharyngioma are highlighted.
Two unusual cases of symptomatic Rathke's cleft cysts are described. The first patient was a 47-year-old man who presented with recurrent aseptic meningitis in addition to visual symptoms, panhypopituitarism, and hydrocephalus. The second patient was a 41-year-old man presenting with increasing headache, and a large, nonenhancing sellar cyst showing suprasellar extension on computerized tomography. The similarities between Rathke's cleft cysts and craniopharyngiomas are discussed.
Patients with intracranial disorders are prone to develop hyponatremia with inability to prevent the loss of sodium in their urine. This was originally referred to as "cerebral salt wasting," but more recently is thought to be secondary to the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). Blood volume determinations were made in 12 unselected neurosurgical patients with intracranial disease who fulfilled the laboratory criteria for SIADH. Ten of the 12 patients had significant decreases in their red blood cell mass, plasma volume, and total blood volume. The finding of a decreased blood volume in patients who fulfill the laboratory criteria for SIADH is better explained by the original concepts of cerebral salt wasting than by SIADH. The primary defect may be the inability of the kidney to conserve sodium.
We report a case of extremely severe hyponatraemia which led to the discovery of anterior hypophysis insufficiency associated with empty sella turcica in a dysimmune context. This type of hyponatraemia is consecutive to corticotropic and thyreo-tropic hormones deficiency and to inappropriate secretion of arginine vasopressin. These endocrine abnormalities are of suprapituitary origin. They can be explained by a hypothalamo-hypophyseal dysregulation of little known mechanism, or by a lesion of the hypothalamus.
A case of symptomatic Rathke's cleft cyst, showing symptoms due to hyponatremia, is reported. The patient was a 68-year-old woman with complaints of vomiting, diarrhea and somnolence. She had severe hyponatremia (109 mEq/l). Magnetic resonance image showed a cystic mass in the intra- and suprasellar region. A right fronto-temporal craniotomy was performed and the cyst was partially resected through the pterional approach. The cyst compressed the chiasma and anterior hypothalamus upward. In the cyst, there was xanthomatous fluid and hematoma. On histopathological examination, the cyst epithelium was diagnosed as Rathke's cleft cyst with craniopharyngioma component. These findings suggested that rapid compression to the anterior hypothalamus by intracystic hemorrhage caused hyponatremia.
Pituitary hyposecretion and hypersecretion produced by a
- M Wezel
- M Salcman
- Da Kristt
- Fe Gellad
- Kapcala
- Lp
Wezel M, Salcman M, Kristt DA, Gellad FE, Kapcala LP. Pituitary hyposecretion and hypersecretion produced by a
Symptomatic Rathke’s cleft cysts. Report of two cases
- Steinberg
Symptomatic Rathke’ s cleft cyst presenting with hyponatremia: a case report
- Muroi
MRI of pituitary cystic lesions: a study on cases with spontaneous regression
- Saeki