Successful Electroconvulsive Therapy in Idiopathic Intracranial Hypertension With Mood Disorder and Partial Empty Sella Syndrome.

ABSTRACT: Patients with idiopathic intracranial hypertension (IIH) often experience significant burden from psychiatric comorbidities. Mood disorders are present in up to half of all patients with IIH, and they are often refractory to treatment by psychopharmacologic agents. Electroconvulsive therapy (ECT) is the criterion standard for treatment of patients with the most severe psychiatric burden but has relative contraindications in those possessing pathologies that raise intracranial pressure (ICP). There is a growing body of literature that a multidisciplinary care model would allow for patients with elevated ICP to receive ECT safely. Despite the high prevalence of mood disorders in patients with IIH, there are only 2 published case reports describing ECT delivery to patients from this cohort. We report our own case of a patient with IIH and major depressive disorder who received 38 bitemporal treatments with a positive response and no change in baseline ICP. Her positive response, along with the absence of elevation of ICP, aligns with the prior reports; however, her IIH symptoms have not responded as reported in the 2 cases-despite receiving more than 4 times the amount of treatments. Moreover, our patient possessed unique imaging for a partial empty sella syndrome, which has recently been found to be the only significant finding in patients who had a mood disorder before IIH diagnosis, versus a mood disorder developing after IIH diagnosis. This case serves to provide evidence of the safety and success of ECT in patients with IIH, relying on multidisciplinary care from psychiatry, neurology, and neuro-ophthalmology.

Clinical presentation, evaluation and case management of primary empty sella syndrome: a retrospective analysis of 10-year single-center patient data.

BACKGROUND: Primary Empty Sella (PES) syndrome is an increasingly common disorder, mostly diagnosed as an incidental finding during brain imaging scans. We intended to review the clinical management and hormonal profile of patients with PES. METHODS: The study included ten-year retrospective analysis of registry containing PES cases in the period 2007 to 2017, from a single tertiary care center. The keyword 'primary empty sella' was used to retrieve patient details from the radiology unit. The clinical and biochemical profile of PES patients was analyzed. Case management of PES patients and their rate of referral to endocrinologists was explored. RESULTS: The registry had 765 cases with a male: female ratio of 1:3.8 suggesting female predominance by almost four times. Although not significant, the onset of disease was earlier for males [Mean ± standard deviation (SD) (46.7 years ±17.3 vs 48.8 years±14.1), p = 0.110]. Almost 79% of the cases were found as an incidental finding during Magnetic Resonance Imaging. Of the total PES cases, only 20% were referred to the endocrinologists and the rest were handled by general physicians. Only 1-2.5% of the cases were evaluated for gonadal, growth and adrenal hormones by the general physicians. The hormonal evaluation by the endocrinologists was also found to be sub-optimal. Headache and visual disturbances were the most common presenting complaints followed by menstrual abnormalities. Endocrine abnormalities like thyroid dysfunction, hyperprolactinemia, hypogonadism and hypocortisolism were highly prevalent among those assessed. CONCLUSION: There is a gross under-evaluation of hormonal assessment and minimal case-referral to Endocrinologists. PES is associated with varying degrees of hormonal dysfunction, and hence early assessment and management is needed. Establishing a standard protocol for diagnosis and case management is essential with the involvement of a multidisciplinary team consisting of endocrinologists, neurologists, primary care phys icians and ophthalmologists.

Partial empty sella in a woman with cerebral venous sinus thrombosis: A rare presentation of polycythaemia rubra vera.

We report the case of a 59 year old woman who presented with a six week history of worsening bifrontal headache. On CT brain the only abnormal finding was a partially empty sella potentially indicative of increased intracranial pressure. MRI found a large cerebral venous sinus thrombosis in the superior sagittal sinus. Blood tests and a bone marrow biopsy revealed a diagnosis of JAK2 positive primary polycythaemia rubra vera. The lack of sensitivity and specificity of CT in the diagnosis of CVST should engender a low threshold for MRI in patients with risk factors and/or non-diagnostic abnormalities on initial CT. Management of this dual pathology involves both the immediate treatment of the thrombus with heparin bridging to warfarin and the long treatment for polycythaemia involving repeat venesections and cytoreductive therapy.

DIAGNOSIS OF ENDOCRINE DISEASE: Primary empty sella: a comprehensive review.

Primary empty sella (PES) is characterized by the herniation of the subarachnoid space within the sella, which is often associated with variable degrees of flattening of the pituitary gland in patients without previous pituitary pathologies. PES pathogenetic mechanisms are not well known but seem to be due to a sellar diaphragm incompetence, associated to the occurrence of upper sellar or pituitary factors, as intracranial hypertension and change of pituitary volume. As PES represents in a majority of cases, a neuroradiological findings without any clinical implication, the occurrence of endocrine, neurological and opthalmological symptoms, due to the above describes anatomical alteration, which delineates from the so called PES syndrome. Headache, irregular menses, overweight/obesity and visual disturbances compose the typical picture of PES syndrome and can be the manifestation of an intracranial hypertension, often associated with PES. Although hyperprolactinemia and growth hormone deficit represent the most common endocrine abnormalities, PES syndrome is characterized by heterogeneity both in clinical manifestation and hormonal alterations and can sometime reach severe extremes, as occurrence of papilledema, cerebrospinal fluid rhinorrhea and worsening of visual acuity. Consequently, a multidisciplinary approach, with the integration of endocrine, neurologic and ophthalmologic expertise, is strongly advocated and recommended for a properly diagnosis, management, treatment and follow-up of PES syndrome and all of the related abnormalities.

Primary empty sella with isolated ACTH deficiency and microprolactinoma.

The empty sella turcica is defined as the herniation of the subarachnoid space within the sella with displacement of the pituitary towards the posteroinferior wall. By autopsy studies, the incidence in the general population is around 20%. The association of prolactinoma and empty sella has been coincidental & infrequently reported. As such for microadenoma, visual field testing and screening for hypopituitarism is not needed, but if it is associated with empty sella, both visual field testing and screening for hypopituitarism is necessary.

[Primary empty sella: symptoms, physiopathology, diagnosis and treatement]. / Silla turca vacía primaria: clínica, fisiopatología y tratamiento.

Bibliographical review on the primary empty sella: concept, symptomatology, diagnosis, physiopathology and management.

Silla turca vacía primaria: clínica, fisiopatología y tratamiento / Primary empty sella: symptoms, physiopathology, diagnosis and treatement

En este artículo se revisa el concepto, clínica,fisiopatología y manejo de la silla turca vacía primariay los distintos problemas que plantea, a la luz de labibliografía que se ha considerado más relevante sobreel tema (AU)

Bibliographical review on the primary empty sella:concept, symptomatology, diagnosis, physiopathologyand management

Hypopituitarism in the elderly: two case-reports with heterogeneous presentation.

Hypopituitarism (HYPO) is an under-investigated disease in older patients. Symptoms, which may be life-threatening, are aspecific and often attributed to aging and/or related morbidities. We describe here the cases of two elderly patients who were ultimately diagnosed as having HYPO, the clinical presentations and etiologies of which were different. In the first patient, an invasive non-secreting pituitary macroadenoma causing HYPO was detected on brain morphological evaluation for head trauma secondary to falling. Glucocorticoid replacement therapy was started. Thyroid function evaluation showed hyperthyroidism (due to a known toxic multinodular goiter), which was already on treatment with methimazole; ultimately, after withdrawal of the antithyroid drug, secondary hypothyroidism occurred. The patient underwent non-radical pituitary surgery. Cognitive function, gait impairment and falling, which had previously been attributed only to aging, improved markedly after cortisone acetate and L-thyroxine replacement therapy. The second patient was hospitalized one month after the onset of hypotension, edema and pain in the legs, and progressive psychomotor impairment, which ultimately resulted in an intermittent soporous state. She was diagnosed as having global anterior HYPO secondary to empty sella, which caused severe multifaceted clinical and biochemical abnormalities (hyponatremia, pancytopenia, rhabdomyolysis). After treatment with i.v. fluids and hydrocortisone, the patient's clinical condition and biochemical alterations improved, and normalized over time with oral cortisone acetate and Lthyroxine combination therapy. Several protean symptoms that impair the quality of life of elderly patients are usually ascribed to aging. Such symptoms should be investigated with regard to possible HYPO, treatment of which can result in significant clinical benefit.

[A 36-year old female patient presenting with hypoglycemic coma]. / Hypoglykämisches Koma bei einer 36-jährigen Patientin.

A 36-year-old female was admitted to the emergency department with a hypoglycemic shock of unknown origin. Initial diagnostic procedures could exclude diabetes mellitus, postprandial hypoglycemia, insulinoma, tumor, drug side effects, and exogenous insulin application. Detailed evaluation of the patient's history revealed that the patient had not been able to lactate after an uncomplicated pregnancy and delivery 3 years ago. Endocrinological evaluation showed a complete adrenocorticotropic insufficiency caused by a Sheehan syndrome. Concomitantly, an empty sella syndrome was visible in MRI. Even after an uncomplicated delivery and even with normal laboratory values, Sheehan-Syndrome can become manifest in a hypoglycemic shock due to hypopituitarism with insufficiency of the adrenocorticotropic axis.

Silla turca vacía primaria. Diagnóstico, tratamiento y seguimiento / Primary empty sella syndrome. Diagnosis, treatment and follow-up

La silla turca vacía es una entidad neurorradiológica que se caracteriza por la herniación del espacio subaracnoideo dentro de la silla turca, con la consiguiente compresión de la hipófisis contra el suelo selar. Se denomina silla turca vacía primaria (STVP) cuando no existen antecedentes de procesos patológicos hipofisarios ni tratamientos previos de la hipófisis. Debido al uso generalizado de la tomografía computarizada y la resonancia magnética craneal, el diagnóstico de STVP es cada vez más frecuente. Aunque clásicamente se ha considerado que la función hipofisaria casi siempre es normal, en los últimos estudios publicados se ha demostrado que es una entidad en la que se encuentran con frecuencia alteraciones de la función hipofisaria (hipopituitarismo total o parcial hasta en el 50% de los pacientes, hiperprolactinemia en el 10%), y que en ocasiones es preciso recurrir a tratamientos neuroquirúrgicos ante los síntomas de hipertensión intracraneal. Se describe el caso de una paciente con STVP y se analizan los aspectos relacionados con su epidemiología, clínica, diagnóstico, tratamiento y seguimiento (AU)

Empty sella syndrome refers to a neuroradiological entity characterized by a cerebrospinal fluid-filled sella, with a small pituitary gland compressed to the rim of the fossa. The term primary empty sella syndrome (PESS) is used to refer to the condition when it is unrelated to previous surgical, pharmacological, orradiotherapeutic treatment of the sellar region. Due to the widespread use of computed tomography and magneticresonance imaging techniques, diagnosis of PESS has become increasingly frequent. Although normal pituitary function has traditionally been considered to be preserved in most cases of PESS, the latest studies have reported frequent pituitary abnormalities (partial or total hypopituitarism in up to 50% of patients and hyperprolactinemia in 10%). Moreover, neurosurgical treatment is mandatory in patients with symptoms of intracranial hypertension. We report a case of PESS and describe the epidemiological and clinical features, diagnosis, treatment and follow-up of patients with PESS (AU)

Transient pituitary dysfunction, empty sella, pseudotumor cerebri in a morbidly obese adolescent.

We report a 16-year-old morbidly obese African-American female who developed pseudotumor cerebri, partially empty sella syndrome and transient central hypothyroidism. After reduction of the increased intracranial pressure by lumbar puncture, normalization of the thyroid function occurred. The mechanism of the transient pituitary dysfunction in relation to the pseudotumor cerebri and empty sella is discussed.

Primary empty sella.

CONTEXT: The term primary empty sella (PES) refers to a number of endocrine and/or neurological disturbances that may be caused by the herniation of subarachnoid space within the sella. SETTING: The records of all patients with a diagnosis of empty sella between 1985 and 2002 seen at the Catholic University of Rome and University of Brescia were examined retrospectively. PATIENTS: We have observed 171 female and 42 male patients affected by PES (over 4:1 sex ratio). The mean age at diagnosis in our subjects was 51.8 +/- 2.1 yr. Mean body mass index was 27.3 +/- 3.5 kg/m2. MAIN OUTCOME MEASURE: All the patients have been analyzed first either with sellar computed tomography scan or magnetic resonance imaging. All patients underwent neurological, ophthalmological, and baseline endocrine evaluation (appropriate stimulation tests were performed when hypopituitarism was suspected). RESULTS: In the overall population, 40 of 213 patients had documented endocrine abnormalities, specifically 31 females and nine males. Twenty-two patients (10.3% of total patients; 18 women, 10.5% of all women, with a mean age of 38.6 +/- 1.1 yr and four males, with a mean age 46.5 +/- 3.52 yr) presented with hyperprolactinemia. Global anterior hypopituitarism was confirmed in nine patients. Eight patients presented an isolated GH deficiency. One hundred thirty-eight of our patients presented a so-called partial empty sella at computed tomography scan/magnetic resonance imaging, and 75 had total PES. CONCLUSIONS: PES may be associated with variable clinical conditions ranging from mild endocrine disturbances to severe intracranial hypertension and rhinorrhea. The need for treatment of hyperprolactinemia as well as for replacement hormone therapy must be assessed in PES. Symptomatic intracranial hypertension makes cerebrospinal fluid shunting procedures necessary.

Diagnosis, treatment, and outcome of pituitary tumors and other abnormal intrasellar masses. Retrospective analysis of 353 patients.

We reviewed the clinical features, essential laboratory data, pituitary imaging findings (computerized tomography and magnetic resonance imaging), management, and outcome of 353 consecutive patients with the presumptive diagnosis of pituitary tumor investigated from January 1984 through December 1997 at University Hospital, Lausanne, Switzerland. In 18 cases primary empty sella turcica was diagnosed, and in 13 cases of pseudacromegaly there were no endocrine abnormalities. The remaining 322 patients disclosed abnormal pituitary masses, including 275 pituitary adenomas, 18 craniopharyngiomas, 6 cases of primary pituitary hyperplasia, 6 intrasellar meningiomas, 6 cases of distant metastases, 4 intrasellar cysts, 2 chordomas, 1 primary lymphoma, and 1 astrocytoma. Biologic data and immunohistochemical analysis of the excised tissues demonstrated that prolactinomas and nonsecreting adenomas (NSAs) were the most frequent pituitary tumors (40% and 39%, respectively), followed by somatotropic adenomas with acromegaly (11%) and Cushing disease (6%). In contrast with the vast majority of NSAs, which significantly expressed glycoprotein hormones in tissue without secreting them, there was a small group of glycoprotein hormone-secreting adenomas (2%), which had a more severe clinical course after surgery. Thirty-eight pituitary masses were incidentally discovered, most of them NSAs. The expansion of pituitary adenomas into the right cavernous sinus was twice as frequent as to the left cavernous sinus. For the differential diagnosis of hyperprolactinemia, basal prolactin (PRL) levels above 85 micrograms/L, in the absence of renal failure and PRL-enhancing drugs, and a PRL increment of less than 30% after thyrotropin-releasing hormone (TRH) accurately ruled out functional hyperprolactinemia due to NSA, and were typical of prolactinomas. For screening and follow-up of acromegaly, basal growth hormone (GH) and insulin-like growth factor 1 (IGF-1) levels, as well as the paradoxical GH response to TRH (present in 2/3 acromegalic patients), could be used as convenient tools, but the most accurate test for diagnosis and prediction of outcome after therapy was GH (lack of) suppression during oral glucose tolerance test. In Cushing disease, single evening plasma cortisol was as good as the overnight dexamethasone suppression test for screening, and a combined dexamethasoneovine corticotropin-releasing hormone (oCRH) test was as accurate as the long dexamethasone suppression test to confirm the diagnosis. Bilateral inferior petrosal sinus catheterization coupled with oCRH test confirmed the pituitary origin of excess adrenocorticotropic hormone (ACTH) in all patients, including those with normal pituitary on magnetic resonance imaging (50% of the cases). However, this procedure failed to predict tumor localization correctly within the pituitary in 21% of patients. Pituitary cysts, meningiomas, and craniopharyngiomas with an intrasellar component were correctly diagnosed based on pituitary imaging in 75%, 67%, and 44% of cases, respectively. The remainder, as well as the cases of pituitary hyperplasia, metastases, and other less frequent pathologies, were initially diagnosed as NSAs or as masses of unknown nature. When surgery was indicated, pituitary adenomas and other intrasellar masses were operated on by the transsphenoidal route, with the exception of 100% of meningiomas, 83% of craniopharyngiomas, and 10% of NSAs, which were operated on by the transcranial route. Favorable late surgical outcome of prolactinomas could be predicted by a restored PRL response to TRH. However, dopamine agonist (DA) therapy, usually resulting in satisfactory control of PRL levels and in tumor shrinkage, progressively displaced surgery as primary treatment for prolactinomas throughout the study period. After full-term pregnancy, the size of prolactinoma decreased in 7 of 9 patients, and PRL was normal in 2. Surgery was the first treatment for NSAs, with a tumor rela

18p monosomy with GH-deficiency and empty sella: good response to GH-treatment.

A patient with 18p monosomy and GH deficiency due to pituitary hypoplasia, who showed an excellent response to GH-treatment, is described. Patients with this syndrome should be considered for endocrine evaluation, as they can benefit from hormonal substitution.

[The syndrome of benign intracranial hypertension]. / Sindrom dobrokachestvennoi vnutricherepnoi gipertenzii.

Eighty patients with benign intracranial hypertension (BIH) (76 females, 4 males, age range 15-54 years) were studied. Endocrine changes (pregnancy-17, obesity-16, dysmenorrhea-15, hyperthyroidism-7, etc.) were the most common cause of BIH (72.5%-58 from 80). Besides general sings of intracranial hypertension (papilledema-80, headache-76, nausea-47, dizziness-43, obnubilations-39), 36 (45%) patients had visual problems. After treatment complete recovery took place in 48 patients, non-significant residual changes persisted in 16 patients, main symptoms of BIH persisted in 16 patients. Papilledema regressed completely in 52 patients, post-papilledematous discoloration of optic disks or optic atrophy were discovered in 12 patients.

[Empty sella syndrome].

An empty sella is defined as a sella which, regardless of its size, is completely or partly filled with cerebrospinal fluid (CSF). Empty sella is occasionally found as a normal anatomical variation, which is referred to as primary empty sella. On the other hand, empty sella is also seen after surgery, irradiation or medical treatment of pituitary adenoma, which is called secondary empty sella. Magnetic resonance imaging (MRI) is useful in diagnosing empty sella. Primary empty sella is usually free from clinical symptoms but sometimes associated with headache, obesity, visual disturbance, non-traumatic CSF rhinorrhea and pituitary insufficiency. These associated findings constitute the empty sella syndrome. CSF rhinorrhea, visual disturbance and severe increased intracranial pressure are the indications for surgical treatment. Non-symptomatic cases require no treatment but periodical follow up is necessary.

[Angiographic occult vascular abnormalities in the area of the pons. Two case reports]. / Angiographische okkulte Gefässmissbildungen im Gebiet des Pons. Zwei Fallberichte.

On the basis of two case reports of occult vascular malformation of the pons that had been followed since the first clinical manifestation, clinical picture, diagnosis using modern imaging methods (especially magnetic resonance imaging--MRI) and therapy of this disease are discussed. In addition to a precise localization diagnosis based on typical scans of varying signal intensity, particularly after bleeding, MRI also allows a preoperative qualitative identification of the lesion and provides important information about the dynamics of the process. Further experience with this uncommon clinical category will improve the early diagnosis and selection of an adequate therapy because the technique of stereotactic radiotherapy (gamma-knife) appears to be most suitable.