Chiasmal syndrome due to intrasellar abscess

A 43-year-old woman had diabetes insipidus and amenorrhea. There was panhypopituitarism on endocrine testing and visual field examination showed inexorably progressive loss to bitemporal hemianopsia. All radiographic findings were normal, but craniotomy disclosed the cause of this chiasmal syndrome to be an intrasellar abscess which, on culture, grew a Gram-positive anaerobe, Peptococcus. Intrasellar or pituitary abscess is rare, but it must always be considered in the differential diagnosis of the chiasmal syndrome, since loss of pituitary and visual function may occur much more rapidly than with the tumors most often responsible for this syndrome and since surgical cure is possible.     

New standards for the assessment of sella turcica volume in children

New standards for the assessment of sella turcica volume were derived from studies in normal children. Use of these standards facilitates the diagnostic evaluation of children with short stature, since many patients with idiopathic hypopituitarism have abnormally small sella turcica volumes. The sellar volume should be estimated early in the evaluation of the short child and, if found to be small, should serve as a stimulus for prompt, thorough evaluation of pituitary function.     

Nelson's syndrome: frequency, prognosis, and effect of prior pituitary irradiation

Previous reports differ regarding the frequency and course of pituitary tumors occurring after adrenalectomy for bilateral adrenal hyperplasia (Nelson's syndrome). In this report, 120 patients who were adrenalectomized for bilateral adrenal hyperplasia were followed for 2 to 20 years. Nine of the 120 developed Nelson's syndrome (8%), the tumors appearing 6 months to 16 years after adrenalectomy. In the majority of cases, the course was benign; seven patients are living an average of 9.7 years after discovery of their tumors. Finally, contrary to previous reports, pituitary irradiation before adrenalectomy did not prevent Nelson's syndrome. Twenty of 120 patients had pituitary irradiation as the initial treatment for bilateral adrenal hyperplasia and two subsequently developed pituitary tumors. Thus, after adrenalectomy for bilateral adrenal hyperplasia, all patients, regardless of previous pituitary irradiation, should be followed indefinitely with periodic X rays of the sella turcica for the possible occurrence of Nelson's syndrome.     

The accuracy of CT and MR evaluation of the sella turcica for detection of adrenocorticotropic hormone-secreting adenomas in Cushing disease

PURPOSE: To document the accuracy of CT and MR of the sella turcica for detecting adrenocorticotropic hormone-secreting adenomas in Cushing disease. METHODS: The radiologic findings of the sella turcica prior to transsphenoidal surgery are reviewed in 141 patients who had biochemical evidence of pituitary-dependent Cushing disease. Axial thin-collimation CT scans with sagittal and coronal reformations before and after contrast enhancement were obtained in 125 patients. Seventy-eight patients had MR examinations with a 1.5-T superconducting magnet. In 11 of the patients gadolinium-enhanced MR scans were also obtained. The preoperative interpretation of the imaging studies was correlated with the surgical findings and patients follow-up. RESULTS: The sella turcica was enlarged in 43 cases (30%). In 125 patients reformatted or direct coronal thin-collimation CT scans were available. Seventy-eight of the patients had MR. In the 12 patients with pituitary macroadenomas, the accuracy of CT (n = 10) and MR (n = 10) in respect to detection of the lesion was 100%. Of the 98 microadenomas assessed by CT, 47 (48%) were directly depicted as distinct hypodense lesions. In only 31 of 73 cases (42%), however, could CT predict the precise anatomic location and extent of the lesions. Only patients in whom the hypercortisolism was corrected by later surgery were considered for the correlation analysis. Of the 52 microadenomas assessed by MR, 28 (53%) were directly depicted as distinct lesions of reduced signal intensity on T1-weighted images, and in only 21 of 41 cases (52%) did MR show good correlation to the surgical findings. Some degree of partially empty sella was found in 22% of the patients. CONCLUSIONS: Although both the sensitivity and the diagnostic accuracy of imaging methods of the sella turcica have been considerably improved in comparison with previous reports, they still provide only a minor contribution to the diagnosis and differential diagnosis of Cushing syndrome.     

Utility of magnetic resonance in the etiologic diagnosis of hypopituitarism

The diagnosis of hypothalamic-pituitary disorders relies on a combination of clinical and biochemical data and imaging techniques. During the last decade, computed tomography (CT) has been the best technique for the evaluation of the hypothalamuspituitary region, but in recent years magnetic resonance (MR) has improved the diagnostic efficiency of CT. We retrospectively review the clinical records of 40 hypopituitary patients from the endocrinology unit of our hospital. The aim of the present study was to establish the role of MR in the etiologic diagnosis and anatomic definition of hypopituitarism, when compared with CT. Secondarily, we studied the different pituitary hormones in this condition. The diagnoses were: 12 postsurgical hypopituitarism, 10 empty sella turcica, 7 Sheehan's syndrome, 5 idiopathic hypopituitarism, 3 pituitary disgenesis, 2 craniopharyngioma and 1 macroprolactinoma. GH was the most commonly affected hormone, followed by gonadotrophins, corticotrophin and thyrotrophin (100%, 94%, 76% and 68% respectively). In 24 patients both MR and CT studies were performed. MR was diagnostic in 22 patients, and CT in 15 patients (p < 0.05). MR offered improved diagnostic or anatomical data in 16 patients of the 24 in whom both techniques were performed (p < 0.05). We conclude that MR allows a better definition of the hypothalamus-pituitary region than CT, contributing to the etiologic diagnosis and improving the anatomical findings. Empty sella turcica should be considered a common cause of hypopituitarism.     

Optic neuropathy in familial dysautonomia

Optic atrophy, which is indicative of a CNS disorder, is a rarely described manifestation of familial dysautonomia (Riley-Day syndrome). As these patients are now living longer, the prevalence of optic neuropathy also may be increasing. We present a man with familial dysautonomia and visual loss resulting from optic atrophy and visual field defect suggestive of chiasmal pathology.     

Effect of size-heterogeneity of denatured T7 DNA molecules on their renaturation

We noted occasional diagnostic confusion with papilledema and chiasmal compression when its true congential nature was not recognized in 12 patients with a benign ocular syndrome. Retinoscopy, fluorescein angiography, ocular ultrasonography, and histopathology suggested that associated visual field defects were related to a localized staphylomatous ectasia of the posterior globe.     

The primary empty sella an endocrine study on 12 cases

Twelve patients (10 women and 2 men) with a primary empty sella turcica were studied. Endocrine function tests were performed as follows: growth hormone (GH) was measured after insulin-induced-hypoglycaemia, luteinizing hormone (LH) and follicle-stimulating hormone (FSH) after LH-releasing hormone, thyrotrophin (TSH) and prolactin after thyrotrophin-releasing hormone; pituitary reserve of adrenocorticotrophin (ACTH) was determined by measurement of plasma cortisol after lysine-vasopressin and 11 deoxycortisol after metyrapone. Five of the patients (group A) had no endocrine disturbance. Seven patients (group B) had a hypothalamo-pituitary disorder. Two of them had panhypopituitarism which appeared in one case after meningoencephalitis and in the other after a severe cranial trauma. In two cases an amenorrhoea-galactorrhoea syndrome with increased prolactin level (68 and 230 ng/ml) led to a diagnosis of a prolactin producing adenoma, which was confirmed by surgery. Finally three cases of amenorrhoeagalactorrhoea, with normal prolactin level, and/or diabetes insipidus remained unexplained. However, no causal relationship could be demonstrated between the pituitary disturbance and the "empty sella". Primary empty sella turcica is therefore a neuroanatomical and neuroradiological entity with no endocrine implication. A pituitary disorder might suggest a microadenoma or an incidentally associated disease.     

Cryotherapy in the treatment of spring conjunctivitis

A 15-year-old boy had growth failure and failure of sexual development. The probable onset was at age 10. Endocrine studies showed hypopituitarism with deficiency of growth hormone and follicle-stimulating hormone, an abnormal response to metyrapone, and deficiency of thyroid function. Luteinizing hormone level was in the low-normal range. Posterior pituitary function was normal. Roentgenogram showed a large sella with some destruction of the posterior clinoids. Transsphenoidal exploration was carried out. The sella was empty except for a whitish membrane; no pituitary tissue was seen. The sella was packed with muscle. Recovery was uneventful, and the patient was given replacement therapy. On histologic examination,the cyst wall showed low pseudostratified cuboidal epithelium and occasional squamous metaplasia. Hemosiderin-filled phagocytes and acinar structures were also seen. The diagnosis was probable rupture of an intrasellar epithelial cyst, leading to empty sella syndrome.     

Surgical indications for and limitations of staged transsphenoidal surgery for large pituitary tumors

Surgical indications and limitations of transsphenoidal surgery and intentionally staged transsphenoidal surgery for large pituitary tumors were investigated by retrospective analysis of the surgical and neuroradiological findings of 22 patients with large tumor and suprasellar extension. Twelve patients underwent intentionally staged transsphenoidal surgery, and 10 underwent combined transsphenoidal and transcranial surgeries. Successful removal by intentionally staged surgery required descent of the suprasellar part following the first transsphenoidal surgery. Analysis showed that a smooth suprasellar extension and/or soft tumor are indications for intentionally staged transsphenoidal surgery even in patients with small sella. However, lobulated suprasellar extension, fibrous and firm tumor without descent, and small sella with normal pituitary gland located at the bottom of the sella are contraindications for staged transsphenoidal surgery. The first transsphenoidal surgery is always indicated to achieve histological confirmation and decompression of optic nerves. Transcranial surgery should be attempted instead of repeated transsphenoidal surgeries when the residual tumor does not descend following the first transsphenoidal surgery.     

Ventilation-perfusion lung images in the Swyer-James syndrome

Ventilation-perfusion lung imaging appears to be a significant nonivasive means of supporting the clinical diagnosis of Swyer-James syndrome. Three cases are presented which display characteristic delayed washout of xenon from a small or normal-sized, underperfused hyperlucent lung.     

Empty sella and headache

Empty sella is an anatomical condition caused by herniation of the subarachnoid space into the pituitary fossa through an incompetent sellar diaphragm. Headache seems to be one of the most frequently reported symptoms. We studied 13 headache patients (12 females, 1 male) presenting with primary empty sella (PES) on CT scan. The characteristics of the headache were analyzed and plasma levels of pituitary hormones or cortisol assessed. We confirmed the nonspecific nature of the headache in PES even though the majority of our patients complained of daily headache, mostly localized anteriorly. Hormone plasma levels were within normal range in all the patients assessed. However, four patients reported earlier endocrine disorders and more than half of the patients presented with obesity. In our opinion, PES should be suspected in middle-aged overweight women with daily headache even in the absence of endocrine symptomatology.     

Human fetal pituitary gland in holoprosencephaly and anencephaly

The normal prenatal development of the human pituitary gland and the gland-supporting sella turcica has recently been investigated. The sella turcica area constitutes a developmental boundary area in the cranial base. Posterior to the area the cranial base has developed close to the notochord, and anterior to the region the cranial base development is dependent chiefly on neural crest cell migrations. In the present study the sella turcica region was analyzed in two fetuses with holoprosencephaly (cyclopia and median cleft) and four fetuses with anencephaly combined with rachischisis in the neck region (GA 16-20 weeks). The sella turcica region was investigated radiologically and histologically. Adenohypohyseal gland tissue was localized by immunohistochemical hormonal marking. In both types of malformation an open craniopharyngeal canal was seen in the base of the sella turcica with adenohypophyseal glandular tissue located in the sella turcica, in the canal, and in the pharyngeal connective tissue at the external side of the cranial base. In conclusion, severe malformations of the pituitary gland occur in both holoprosencephaly, which is a polytopic field defect located anterior to the sella turcica, and in anencephaly associated with notochordal insufficiency posterior to the sella turcica. This might indicate that the sella turcica area, bounding different developmental fields, is involved in various craniofacial malformations. It is consequently recommended that examination of the pituitary gland should become a part of the routine autopsy of prenatal material when malformations in the face, brain, and cranial base occur.     

Computed tomography, ultrasound and lymphography in the diagnosis of malignant lymphoma

Lymphographic findings were compared with the results at histopathology in 42 laparotomized patients with malignant lymphoma. Of 12 patients with lymphographically suggested lesions in normal-sized lymph nodes, only one had pathologic changes at histologic examination. Computed tomography gave a better idea of the extent of the disease than lymphography. Moreover, CT had an equal diagnostic accuracy as lymphography for demonstrating enlarged para-aortic lymph nodes. CT should therefore be able to replace lymphography in examination of malignant lymphoma. Ultrasound had the same diagnostic accuracy as CT regarding the upper and middle abdomen but not in the lower abdomen. Ultrasound is of special value in thin patients and when guided needle biopsy is required.     

Decrease of the sella turcia to normal size, a major sign of the effectiveness of pituitary tumour irradiation

In this preliminary paper, the authors review 20 cases of pituitary tumours, with sella reduction to normal size, associated to the biological recuperation and social rehabilitation of most patients, after conventional radiation therapy given in high-dose courses. It is evaluated that sella reduction to normal size, whenever encountered, can be considered as the major anatomico-radiological sign of the effectiveness of different therapeutical methods applied in the case of secretory and non-secretory tumours.     

Sequence of a 42-kb mouse region containing the imprinted H19 locus: identification of a novel muscle-specific transcription unit showing biallelic expression

We have followed four patients with Bartter syndrome for a mean of 25.4 years (range 21.5-28.8 years) after diagnosis. All patients received non-steroidal anti-inflammatory drugs (NSAID). In all patients, various degrees of renal dysfunction were noted to be temporally associated with NSAID therapy. In two patients, renal dysfunction resolved after discontinuing NSAID therapy, while maintaining other chronic medications such as potassium-sparing diuretics. Renal dysfunction persisted after NSAID withdrawal in two patients. We report these cases as a warning that NSAID should be considered an important cause of either reversible or irreversible renal dysfunction in Bartter syndrome.     

Pyogenic liver abscesses: a comparison of older and younger patients

The purpose of the present study was to analyze the morphology of the sella turcica in children born with myelomeningocele. Profile radiographs from 16 children (nine females and seven males) born with myelomeningocele were analysed. The contour of the anterior wall of the sella turcica in myelomeningocele patients, instead of following the normal cranio-caudal direction, was always in an obliquely antero-posterior direction. The sella turcica thus appeared broad cranially with a diverging anterior wall, or with both diverging anterior and posterior walls. This appearance gave and impression of a wide sella turcica in myelomeningocele with less depth than normal. The investigation has drawn attention to the fact that congenital malformations in the axial skeleton, even though, as in the case of myelomeningocele, they are located far from the cranial base, may have manifested themselves in the cranial base as well. The pathogenetic relationship between these manifestations is to be found in the early embryonic structure, the notochord. With the concept of embryological developmental fields, defined as areas with a common developmental origin, such as the notochordal field involved in myelomeningocele, new ways seem to be emerging for an improvement of aetiologically based diagnosis and treatment.     

Presaccadic omnidirectional burst activity in the basal interstitial nucleus in the monkey cerebellum

The endocrine function and pituitary imaging in Sheehan's syndrome more than 30 years after causative events were evaluated. Magnetic resonance imaging (MRI), a combined anterior pituitary test, plasma vasopressin-to-osmolality adaptation study, and antithyroid and antipituitary cell antibody measurement were performed in 6 women with Sheehan's syndrome. The interval from delivery to the onset of symptoms of hormonal deficiency ranged from 3 to 32 years. Since clinical onset, all had received glucocorticoid and thyroid replacement therapy. Cranial MRI examination showed an "empty sella" in 5 cases. Among these, 2 of 5 (40%) demonstrated panhypopituitarism and the other 3 (60%) maintained gonadotropin response. The pituitary gland was normally discernible but with a low-intensity lesion on T1-weighted images in a patient who maintained PRL and gonadotropin responses. Posterior pituitary function was abnormal in 3 of 6 (50%) despite the absence of polyuria. No antipituitary antibodies were detected in any of the cases. Thyroid peroxidase antibody was negative in all cases, but antithyroglobulin antibody was detected in 2 of 6 (33%). Thyroid-stimulating antibody was not detected, but one case had an anti-TSH antibody. Thirty years after the initial events, most patients with Sheehan's syndrome showed signs of an empty sella on MRI, all having noticeable suppression of anterior and/or posterior pituitary hormones with no related autoimmunity.     

The long-term changes after the implantation of silicone breast prostheses

For a large scaled test 52 patients with anterior transposition of the ulnar nerve and 62 patients with elbow injuries were examined by questionnaire, physical examination and electroneurography. In conformity with existing literature 24 patients out of 52 suffering from cubital tunnel syndrome had an elbow trauma previously. To our great surprise in seven patients out of 46 with elbow injuries a cubital tunnel syndrome could be found for the first time. The cubital tunnel syndrome appears to be a frequent complication of elbow injuries. Besides the well known fractures of the medial epicondyle and pericondylar fractures leading to cubital tunnel syndrome, in our study fractures of the head of the radius and processus coronoideus were found quite often. Patients having typical anamnesis and complaints should be checked by electroneurography in order to permit a quick operation and to prevent incurable damages of the nerve. The diagnosis leading to operation of the elbow should however be made with greatest care. In case the operation is unavoidable, the ulnar nerve should be thoroughly checked and anterior transposition should be carried out.     

Pathology of the eye in sarcoidosis

Comprehensive neurologic and ophthalmologic examinations of 100 patients with morphologically verified sarcoidosis showed the most frequent involvement of optic nerves and less incident lesions of the eye proper and its appendages. Vision disorders were detected in 39.0 patients, chiasmal disorders in 12.0%. Fundus oculi abnormalities were found in 27.0% of patients. A specific feature of vision disorders in sarcoidosis was that almost half of the patients did not feel them, which may be explained by a predominantly chronic latent pattern of these disorders and by the fact that, despite manifest disorders of peripheral vision, central vision with correction remained normal for a longer time.