Paulo Andrade de Mello

Nelson’s Syndrome: Complete Remission with Cabergoline but Not with Bromocriptine or Cyproheptadine Treatment

A woman affected by Cushing’s disease underwent bilateral adrenalectomy followed by radiotherapy of the hypothalamic-pituitary area when she was 18 years old. Thereafter, she used hydrocortisone acetate replacement therapy (35.5 mg divided into two daily doses). At the age of 26 years, the patient exhibited the clinical signs of the Nelson’s syndrome, i.e. skin and gingival hyperpigmentation accompanied by amenorrhea, and elevated ACTH plasma levels (2,850 pg/ml, normal range 15–80 pg/ml). The magnetic resonance imaging (MRI) analysis of the sellar region evidenced a pituitary macroadenoma, measuring 14 × 13 mm. The patient was initially treated with cyproheptadine hydrochloride (12 mg/day) for 18 months. There was a partial improvement of the symptoms, with a reduction of the ACTH plasma levels to 112 pg/ml, but without any modification of the tumor mass. Due to sleepiness and weight gain, the cyproheptadine treatment was interrupted and substituted by a cabergoline (0.5 mg twice a week) therapy. Soon after cabergoline was applied an improvement of the clinical symptoms and signs was observed such as a regression of the tumor mass and the normalization of the ACTH plasma titers (38 pg/ml). Later, cabergoline was substituted by bromocriptine (7.5 mg/day) and the plasma levels of ACTH increased again (247 pg/ml), and headache and cutaneous hyperpigmentation were recorded. When cabergoline was reintroduced there was a clinical improvement and normalization of ACTH plasma levels (64 pg/ml). The MRI analysis of the sella region demonstrated a complete remission of the pituitary adenoma. The results obtained show for the first time that a long-term treatment with cabergoline also brings about a complete remission of Nelson’s syndrome in the presence of a pituitary macroadenoma.

Expression of p53, Ki-67 and c-erb B2 in growth hormone-and/or prolactin-secreting pituitary adenomas

The subcellular events implicated on the formation and behavior of pituitary adenomas are not fully understood. In this study we investigated the presence of p53, Ki-67 and c-erb B2 in 38 pituitary adenomas with immunohistochemical positivity for GH and prolactin (n=26; 68.4%), for prolactin (n=9; 23.7%) and for GH (n=3. 7.8%). The analyses revealed the following results: 24 (63.2%) tumors expressed variable positivity for c-erb B2, 11 (28.9%) expressed p53 positivity and 11 (28.9%) tumors were variably positive for Ki-67. Our results demonstrated a high percentage of GH/prolactin-, prolactin- and GH-secreting tumors with immunohistochemical positivity for c-erb B2. Once this membrane receptor is related to growth factors EGF and TGFalpha and both have a definite effect on tumor growth, our data suggest a possible role for c-erb B2 on the evolution of these tumors.

Mean intrasellar pressure, visual field, headache intensity and quality of life of patients with pituitary adenoma

Intrasellar pressure (ISP) measurement technique has recently opened a new line of research in neuroendocrinology. The absolute and mean ISP values were investigated in 25 patients consecutively operated at the Brasilia University Hospital (DF). These data were correlated with serum prolactin levels, number of visual quadrant affected, tumor size, quality of life (measured through the SF-36 scale) and graded headache (measured through the HIT-6 scale). No correlation was observed. The p values were 0.887; 0.137; 0.892; 0.812 and 0.884; respectively. The HIT-6 values were inversely and moderately correlated with total RAND SF-36 and its mental and physical dimensions.

Clinical and laboratorial characterization and post-surgical follow-up of 87 patients with non-functioning pituitary macroadenomas.

OBJECTIVE It was to assess the main characteristics of patients undergoing pituitary tumor surgery. METHOD Eighty-seven patients (44 men; 44.8±13 years old) were included. RESULTS The main symptoms were visual alterations (87.3%), headache (70.1%), diminished libido (34.4%), galactorrhea (22.9%) and hair loss (19.5%). The axes affected were gonadotropic (72.6%), thyrotropic (48.4%) and corticotropic (38.7%), without significant changes after surgery. The average largest tumor diameter was 3.1 cm before surgery and 1.56 cm after surgery. The most frequent postoperative complications were hydro-electrolyte and acid-base disorders (12%), diabetes insipidus (9%), visual field alterations (9%), liquoric fistula (8%) and nasal obstruction (7%). The patients were affected by more than one complication. CONCLUSION Although a decrease in tumor volume was achieved through surgery, hormonal deficiencies persisted in most of the patients and new surgical approaches were necessary for dealing with tumor recurrence or persistence.

Fibroma condromixóide da coluna torácica: Relato de caso e revisão da literatura

A case of chondromyxoid fibroma (CMF) arising from the Sth right costovertebral junction and spreading into spinal canal causing spinal cord compression is presented. A myelotomography revealed a complete block at T5 level. The patient underwent a decompressive laminectomy with removal of an epidural tumor. This specimen was sent for pathological examination and interpreted as a CMF. The patient had a neurological improvement, post operative MRI revealed a spinal cord free of compression, and we decided on the follow up of the case. Two years later there was reccurrence of the tumor. A posterolateral access by costotransversectomy was made and the lesion was resected. The patient had a neurological improvement which persists on the follow up (two years, at present). Clinical, radiologic and histologic findings, surgical management and recurrence are discussed. The pertinent literature is reviewed.

Tratamento da neurocisticercose com praziquantel

Twenty seven patients with neurocysticercosis were treated with praziquantel in progressive doses reaching 50 mg/kg/day associated with dexamethasone for 21 days. The patients were followed during and after treatment and those followed up for one year repeated their immunological tests (indirect immunofluorescence and ELISA) at this time. Headache was the most frequent symptom during the treatment, occurring on 37% of patients. During the treatment 18.5% of patients had intracranial hypertension and one died. One year after treatment 72.2% of patients who finished treatment improved. The immunological tests became negative in 45.4% of patients sera and 42.8% of cerebrospinal fluids. There was no correlation between the clinical evolution and immunological, testis. In this study it is not possible to afirm that both negative immunological tests and good clinical evolution were consequents to the efficacy of praziquantel treatment. Due to the great frequency and seriousness of this treatment complications, the patients with neurocysticercosis must be individually evaluated to know the risks and the benefits of the treatment with praziquantel.

Stroke: bowel dysfunction in patients admitted for rehabilitation.

Aim : to assess the prevalence of diminished frequency of bowel movements, lumpy or hard stools, intestinal constipation, straining, incomplete evacuation, incontinence (bowel dysfunctions) in patients with brain injury resulting from cerebrovascular accident, either self-reported or reported by their caregivers; to describe the type and frequency of such dysfunctions; and the prevalence of laxative use both before and after stroke. Method : cross-sectional study with 98 hospitalized patients admitted for rehabilitation between December 2009 and May 2010. Results : the prevalence of bowel dysfunctions before stroke was 23.96% whereas after the lesion it was 55.21% (p<0.0001). As reported by patients/caregivers, the chances of developing bowel dysfunctions increase sevenfold after stroke, 95% CI (2.44-24.26). The most frequent dysfunctions before stroke were intestinal constipation (73.91%) and diminished frequency of bowel movements (17.39%). After stroke, constipation remains to be the most frequent dysfunction reported (50%), followed by diminished frequency of bowel movements (26.79%), incomplete evacuation (12.50%), and lack of privacy (5.36%). The use of laxatives was 19,15% after the lesion, but not statisticaly significant (p=0.0736). Conclusion : Bowel dysfunctions increases significantly after stroke. Therefore, further studies are needed to better understand and characterize such dysfunctions, which are scarcely described in the literature.

Constipação intestinal em pacientes admitidos em programa de reabilitação neurológica

OBJECTIVES: To determine the prevalence of constipation in patients admitted for rehabilitation and to verify the results of two intervention models for bowel retraining during hospitalization. METHODS: A longitudinal, analytical study, with 98 patients admitted to rehabilitation ward between December 2009 and May 2010. RESULTS: The prevalence of constipation was 57.1%, there was no correlation with gender, education, age, transportation assistance, language disorder, physical activity, diet and presence of constipation; the improvement in functional capacity was a predictor of progress in intestinal frequency; interventions introduced demonstrated an important role in improving bowel habits. CONCLUSIONS: Conduct studies may provide treatment options for constipation, improving the quality of life for these individuals.

Prognostic Value of Invasion, Markers of Proliferation, and Classification of Giant Pituitary Tumors, in a Georeferred Cohort in Brazil of 50 Patients, with a Long-Term Postoperative Follow-Up

Although some pituitary adenomas may have an aggressive behavior, the vast majority are benign. There are still controversies about predictive factors regarding the biological behavior of these particular tumors. This study evaluated potential markers of invasion and proliferation compared to current classification patterns and epidemiogeographical parameters. The study included 50 patients, operated on for tumors greater than 30 mm, with a mean postoperative follow-up of 15.2 ± 4.8 years. Pituitary magnetic resonance was used to evaluate regrowth, invasion, and extension to adjacent tissue. Three tissue biomarkers were analyzed: p53, Ki-67, and c-erbB2. Tumors were classified according to a combination of histological and radiological features, ranging from noninvasive and nonproliferative (grade 1A) to invasive-proliferative (grade 2B). Tumors grades 2A and 2B represented 42% and 52%, respectively. Ki-67 (p = 0.23) and c-erbB2 (p = 0.71) had no significant relation to tumor progression status. P53 (p = 0.003), parasellar invasion (p = 0.03), and classification, grade 2B (p = 0.01), were associated with worse clinical outcome. Parasellar invasion prevails as strong predictive factor of tumor recurrence. Severe suprasellar extension should be considered as invasion parameter and could impact prognosis. No environmental factors or geographical cluster were associated with tumor behavior.

Factors Associated With Intestinal Constipation in Chronic Patients With Stroke Sequelae Undergoing Rehabilitation.

The objective of this study was to define which stroke-related factors constitute independent variables in the incidence of intestinal constipation (IC) of chronic patients admitted to a hospital rehabilitation program. All patients consecutively admitted for rehabilitation were recruited for the study. In the Poisson multiple regression analysis using a hierarchical model, sociodemographic variables, comorbidities, medication, previous history of constipation, life habits, and stroke-related variables were considered for defining factors associated with IC. A 31% prevalence (95% confidence interval [CI]: 25.3–37.1) of IC was detected. Among the factors associated, female gender (adjusted prevalence ratio [PRadjusted] = 1.79; 95% CI: 1.20–2.68), intestinal complaints prior to stroke (PRadjusted = 3.71; 95% CI: 2.60–5.31), intake of less than 800 ml of fluid per day (PRadjusted = 1.72; 95% CI: 1.20– 2.45), age greater than 65 years at brain injury (PRadjusted = 1.67; 95% CI: 1.01–2.75), and partially impaired anterior brain circulation (PRadjusted = 3.35; 95% CI: 1.02–10.97) were associated with IC. Female gender, elderly, prior history of IC, low fluid intake, and partial impairment of anterior brain circulation were factors independently associated with IC in stroke survivors undergoing rehabilitation. These findings require further validation and may serve toward improving bowel retraining programs for this patient group.