THE MULTIFARIOUS CUSHING'S - LESSONS FROM A CASE SERIES.

Endogenous Cushing's syndrome is rare, with an incidence of 0.7-2.4 per a million people a year. Clinical presentation of Cushing syndrome can be pleomorphic, and establishing diagnosis can be difficult. Early recognition and rapid control of hypercortisolaemia are necessary to decrease morbidity and mortality in these patients. We report a series of 6 endogenous Cushing's syndromes of different etiologies (4 Cushing's disease and 2 adrenal Cushing's syndrome) assessed in our endocrine department over a decade (2009-2019). In order to highlight the diversity of clinical forms, diagnostic tools and specific management of this condition we labelled each case suggestively: the typical Cushing's disease, the Pseudo Cushing's, the elusive Cushing's disease, the mild autonomous cortisol hypersecretion, Cushing's syndrome in pregnancy and Cushing's disease with thromboembolism. We discussed their particularities which were revelatory for the diagnosis, such as dermatologic, cardiovascular, musculoskeletal, neuropsychiatric, or reproductive signs, reviewing literature for each manifestation. We also discuss the commonalities and differences in laboratory and imagistic findings. Therapeutic approach can also differ with respect to the particular condition of each patient and the multiple choices of therapy will be reviewed.

CRANIOPHARYNGIOMA - CLINICAL AND THERAPEUTIC OUTCOME DATA IN A MIXED COHORT OF ADULT AND PAEDIATRIC CASES.

BACKGROUND: Craniopharyngiomas are benign but locally invasive tumours of the sellar region that arise from ectopic embryonic remnants of Rathke's pouch, affecting both children (adamantinomatous type -aCP) and adults (papillary type -pCP) and associated with significant morbidity. OBJECTIVE: To study the clinical presentation of CRF as well as the posttreatment evolution of craniopharyngioma in children versus adults in a large mixed cohort. MATERIAL AND METHODS: We performed a retrospective review of CRF patients evaluated in the National Institute of Endocrinology in Bucharest between 1990 and 2016. RESULTS: A total of 107 patients (72 adults, 35 children) with a mean follow-up of 6.2 years were included. The presenting symptoms were mostly headache, visual impairment, symptoms of hypopituitarism, diabetes insipidus. Some symptoms or hormonal abnormalities were significantly more prevalent in the children group (p<0.05): nausea/ vomiting (47.8% vs 16.7%), photophobia (21.7% vs 5.6%), diabetes insipidus(28.5% vs 8.3%), GH deficiency (68.8% vs 17.1%). Impaired visual acuity (67.6%of cases) or visual fields (71.4%) were more frequent in adults compared to children (44.1%; 51.6%). The tumor dimensions were similar in both groups (3.05± 1.05 cm in children; 2.7± 1.07 cm in adults). Massive suprasellar extension reaching the third ventricle was frequently present in all cases. All cases underwent surgery but only a minority of those not cured received postoperative adjuvant radiotherapy. Frequent postoperative complications were: aggravation of the endocrine deficit (>80% of cases in both groups needed chronic replacement therapy), central diabetes insipidus (68.2% children, 34.3% of adults). CONCLUSIONS: Despite similar tumor dimensions and extension compared to adults, craniopharyngioma in children is more frequently associated with signs of intracranial pressure. The results and complications of treatment are similar in adults and children.

The multimodal management of GH-secreting pituitary adenomas: predictive factors, strategies and outcomes.

Object. The aim of this study was to analyze a series of 28 patients with acromegaly who underwent a multimodal surgical, medical and radiosurgical therapy, with a special attention to the advantages, complications, and predictive factors of a successful outcome. Methods. 28 consecutive cases of GH-secreting pituitary adenomas, who underwent transsphenoidal endoscopic or microscopic surgery, between 1 January 2014 and 31 December 2014 were retrospectively reviewed. Tumors were classified according to the diameter, measured on MRI, as micro- or macroadenomas, and parasellar (cavernous sinus) tumor extension was analyzed based on the Knosp grading score. The mean follow-up period was of 18.4 months. Criteria justifying the complete hormonal remission were preoperative basal serum GH < 2.5 µg/ L, preoperative nadirGH < 1 ng/ L after OGTT and normal preoperative IGF-I levels age and sex-matched. Results. An overall complete hormonal remission rate was achieved in 64.3% of the patients. The remission rate was higher in patients with microadenomas (77.8%) than in those with macroadenomas (57.9%). A number of predictive factors, which might have interfered with the hormonal remission rate from a statistical, clinical and paraclinical point of view, were identified: tumor size (r = 0.625), preoperative GH serum levels (r = -0.517), cavernous sinus extension was quantified according to Knosp grading score (r = 0.469) and the degree of tumor subtotal resection (r = 0.598). Conclusions. Favorable hormonal and visual remission rates can be achieved after transsphenoidal resection of GH-secreting pituitary adenomas; however, the management remains challenging, the increased surgical experience being important for higher cure rates. If a biochemical hormonal cure is not achieved postoperatively, adjuvant medical or radio surgical therapy can be recommended.

SPONTANEOUS CONCEPTION AND TERM DELIVERY IN A WOMAN WITH UNCONTROLLED ACROMEGALY AND HYPOGONADOTROPIC HYPOGONADISM.

BACKGROUND: Patients with acromegaly (caused by growth-hormone-secreting pituitary adenomas) are at increased risk of hypopituitarism, in particular hypogonadotropic hypogonadism, before and after multimodal therapy. In affected women of reproductive age, fertility is impaired and complex fertility treatments are needed to achieve conception. CASE PRESENTATION: We present the case of a young woman with acromegaly caused by a GH-secreting macroadenoma with suprasellar and bilateral cavernous sinus extension; hypogonadotropic hypogonadism and secondary hypothyroidism were present from the initial evaluation. Neurosurgical intervention was repeatedly recommended but the patient refused it initially; also she was non-compliant to the medical treatment of acromegaly. Transsphenoidal tumor debulking with adjuvant gamma-knife radiotherapy was eventually performed. Following treatment persistent active acromegaly and hypogonadotropic hypogonadism were diagnosed. Under chronic estroprogestative replacement therapy, the patient conceived and delivered a full-term healthy newborn without any complications. Possible mechanisms are discussed. CONCLUSIONS: Secondary hypogonadotropic hypogonadism in pituitary patients, even when considered permanent (after surgery and radiotherapy), can exceptionally allow spontaneous conception and normal course of pregnancy.

Diabetes mellitus, chronic complication in patients with acromegaly: case report and review of the literature.

UNLABELLED: Disturbances of glucose metabolism are frequently observed in patients with acromegaly. Excess amounts of GH and IGF1 interacts with metabolic regulation, and indeed, GH hypersecretion is associated with hepatic and peripheral insulin resistance; this and also other mechanisms are involved in the development of diabetes mellitus. It can quickly improve if the levels of GH decline after the therapy. CASE REPORT: We present a patientof 54 years old, admitted in the clinic in 2009, with diagnosis of acromegaly. MRI scan reveals an expansive pituitary tumor 15/16/17 mm. Values of GH, IGF1 and blood glucose were much above normal. Body mass index 27, 5 kg/m2. After 12 months of a medical treatment with somatostatin analogues, the evolution of blood parameters was favorable, but the patient has discontinued his treatment. At the hospitalization in 2012 there is a precarious control of the diabetes under oral antidiabetic agents. A new treatment has been applied: it was practiced surgical transsphenoidal ablation. At last admit, May 2014, the value IGF1 has been normal, GH has been below 1 ng/mL, and HbAlc was 5.27%. Dyslipidemic syndrome has been constantly present; the values of triglycerides and cholesterol should be a little more upper limit. CONCLUSIONS: Following successful treatment of acromegaly with surgery, glucose tolerance improves. Balancing type 2 diabetes, with return to normal HbA1c and the decrease in cholesterol and triglycerides values, represents the result of favorable normalization GH in our patients with acromegaly.

Brain abscesses: clinical experience and outcome of 52 consecutive cases.

BACKGROUND: Brain abscesses are still a very important pathology, with high mortality and morbidity, even with the advancement of imaging technologies and antibiotic treatment. In this study, we analyzed the relationship between clinical, biological and therapeutic features at admission and clinical outcome in patients with brain abscesses. MATERIAL AND METHOD: This is a retrospective study over 52 consecutive patients with brain abscesses treated in our Neurosurgical Department during 8 years, between January 2003 and December 2011. Laboratory tests, cerebral CT or MRI scans, blood and urine cultures were performed. Surgical treatment consisted of either CT guided stereotactic aspiration or craniotomy with the resection of the abscess. Materials from the brain abscess were cultured for aerobic and anaerobic bacteria. Parenteral antibiotic therapy usually lasted between 6 to 8 weeks. RESULTS: Clinical outcome was assessed at 6 months endpoint by using Glasgow Outcome Scale (GOS). The most common cause of brain abscess was hematogenous spread. There were 41 patients with solitary and 11 with multiple brain abscesses. Regarding microbiological findings, we obtained positive cultures from brain materials in 41 patients. Stereotactic aspiration was performed in 33 patients, surgical excision in 15 and medical therapy alone in 4 patients. As clinical outcome, 84.6% patients had a favorable outcome (GOS 5 and 4) and 15.4% had an unfavorable outcome (severe disability - 4 and death - 4). CONCLUSIONS: Early diagnosis, optimal surgical intervention and timely use of appropriate antibiotics are essential for a good outcome. No significant difference in outcome was found among various surgical treatment modalities. For mortality, initial Glasgow Coma Score (GCS) 8, associated predisposing factors and systemic infections were significant contributing factors.

[Severe pleuropericarditis induced by long-term bromocriptin therapy, report of a case and review of the literature]. / Pleuropéricardite sévère associée à un traitement par bromocriptine au long cours, revue de la littérature à propos d'un cas.

INTRODUCTION: Bromocriptin, member of the class of ergolines, is commonly prescribed as treatment of Parkinson's disease. Apart from vascular, digestif, neurologic and psychic disorders, the authors report cases of retroperitoneal fibrosis and pleural effusion, as adverse reactions related to the bromocriptin. SYNTHESIS: About 40 cases of skin, pleural, lung and retroperitoneal attacks were described after long term and high doses of bromocriptin. More ten years ago, the first case of constrictive pericarditis was cited in the medical literature, and the bromocriptin was incriminated as responsible. Since then, two other cases were cited. Our observation is a constrictive pericarditis, found in a 72 years old patient treated with bromocriptin for Parkinson's disease since five years (cumulative dose intake 73 grams). Investigations aimed to establish etiology were negative. Bromocriptin is suspected and the treatment is discontinued. As in the three other cases, cardiac and neurologic conditions markedly improved after bromocriptin's withdrawal. A pericardic thickening persists at the echography. CONCLUSION: The responsibility of bromocriptin in the etiology of constrictive pericarditis is seldom discussed, because it remains an exclusion diagnosis. Periodic chest X-ray and echocardiography should be considered in patients with long-term bromocriptin treatment.

[Cutaneous metastatic Crohn's disease]. / Manifestations métastatiques cutanées de la maladie de Crohn.

INTRODUCTION: Cutaneous metastatic Crohn's disease is a granulomatous inflammation of the skin that is noncontiguous to the gastrointestinal tract. CASE REPORT: A 42-year-old man with Crohn's disease is admitted for antibiotic resistant erysipela-like dermo-hypodermitis. The presence of granulomatous lesions on skin biopsy, the absence of any infectious agent identified despite extensive investigations and the dramatic improvement observed with corticosteroid eventually lead to the diagnosis of cutaneous metastatic Crohn's disease. CONCLUSION: During Crohn's disease, biopsy should be considered for every undiagnosed skin lesion as the characteristics of cutaneous metastatic Crohn's disease are not specific. The presence of a non caseous granulomatous dermal infiltration suggests the diagnosis and should make consider the initiation of corticosteroid and the discontinuation of others inappropriate therapeutics (i.e. surgical treatment, antibiotics).

The prognostic value of ventricular rupture in cerebral hemorrhage.

Analysis of 41 cerebral hemorrhage cases associated or not with intraparenchymatous hematoma and ventricular rupture shows the role played by these associated phenomena in the evaluation and prognosis of these patients. The death rate was 56.25% in simple cerebral hemorrhage, 100% in hemorrhage associated with hematoma, 30.76% in hemorrhage associated with ventricular rupture and 80% in hemorrhage associated with hematoma and ventricular rupture. The authors point out that the mere presence of ventricular rupture is not a really aggravating factor. A particular severity resulting from association of hemorrhage with hematoma seems to be due to the extension of the cerebral lesion produced by the two conditions associated.