Abstract Acromegaly is a rare disease characterized by changes in the bone and soft tissue systems, induced by excess growth hormone and insulin-like growth factor type 1. Among the skin lesions associated with acromegaly is cutis verticis gyrata, an hypertrophic, and coarse folding of the skin of the scalp, an association of uncommon incidence and unknown prevalence. This case report describes the case of a patient diagnosed with acromegaly at age 60 with previously unidentified cutis verticis gyrata. This report aims to review the literature on cutis verticis gyrata and its unusual association with acromegaly.

ABSTRACT Objective: Dyslipidemia is prevalent among patients with hypopituitarism, especially in those with growth hormone (GH) deficiency. This study aimed to evaluate the response to statin therapy among adult patients with dyslipidemia and hypopituitarism. Subjects and methods: A total of 113 patients with hypopituitarism following up at a neuroendocrinology unit were evaluated for serum lipid levels. Dyslipidemia was diagnosed in 72 (63.7%) of these patients. A control group included 57 patients with dyslipidemia and normal pituitary function. The distribution of gender, age, weight, and dyslipidemia type was well balanced across both groups, and all participants were treated with simvastatin at doses adjusted to obtain normal lipid levels. Results: Patients with hypopituitarism and dyslipidemia presented deficiency of TSH (69%), gonadotropins (69%), ACTH (64%), and GH (55%) and had a similar number of deficient pituitary axes compared with patients with hypopituitarism but without dyslipidemia. All patients with dyslipidemia (with and without hypopituitarism) had lipid levels well controlled with doses of simvastatin ranging from 20-40 mg/day. The mean daily dose of simvastatin was not significantly different between patients with and without hypopituitarism (26.7 versus 23.5 mg, p = 0.10). Similarly, no significant variation in simvastatin dose was observed between patients with different causes of hypopituitarism, presence or absence of GH deficiency, number of deficient pituitary axes, prior pituitary radiation therapy or not, and presence or absence of obesity. Conclusions: Patients with GH deficiency without GH replacement showed good response to simvastatin at a mean dose equivalent to that used in individuals with dyslipidemia and normal pituitary function.

Resumo Os adenomas secretores de prolactina (PRL) estão associados à alta incidência de cefaleia. O papel da hiperprolactinemia no contexto da dor de cabeça não está claro, nem o efeito da redução dos níveis da PRL na cefaleia. Métodos: O presente estudo longitudinal avaliou pacientes hiperprolactinêmicos (69), quanto à presença e às características da cefaleia antes e após o tratamento da hiperprolactinemia. Resultados: Cefaleia foi relatada por 45 (65,2%) pacientes, independente da etiologia da hiperprolactinemia. O fenótipo de enxaqueca foi mais prevalente (66,6%). Os medicamentos usados no tratamento da cefaleia não foram alterados durante o estudo. A primeira linha de tratamento da hiperprolactinemia foram os agonistas dopaminérgicos. Na última reavaliação, o nível de PRL sob tratamento estava dentro da faixa de referência em 54,7% dos casos, observando-se resolução completa ou parcial da cefaleia em 75% dos casos. A mediana de PRL neste momento em pacientes com resolução completa da cefaleia foi de 17 ng/mL, nos que relataram recuperação parcial foi de 21 ng/mL, e naqueles em que a cefaleia não se alterou foi de 66 ng/mL, com uma diferença significativa entre o grupo com resolução completa da cefaleia versus o grupo com cefaleia inalterada (p=0,022). Nos casos com resolução completa da cefaleia, a queda mediana nos níveis de PRL foi de 89% e nos casos com resolução parcial de cefaleia de 86%, ambos significativamente diferentes (p<0,001) da queda nos casos com cefaleia inalterada. Conclusão: Os dados permitem concluir que, nesta série, na maioria dos casos, a redução do nível de PRL foi seguida pela cessação ou alívio da dor.

Abstract Prolactin (PRL) secreting adenomas are associated with high incidence of headache. The role of hyperprolactinemia in the headache context is not clear, nor is the effect of its treatment on headache. Methods: The present longitudinal study evaluated hyperprolactinemic patients (69), in terms of presence and characteristics of headache before and after hyperprolactinemia treatment. Results: Headache was reported by 45 (65.2%) patients, independent of the etiology of hyperprolactinemia. The migraine phenotype was the most prevalent (66.6%). Medications used in the treatment of headache not changed during the study. The first line of treatment of hyperprolactinemia was dopaminergic agonists. In the last reevaluation, PRL level under treatment was within the reference range in 54.7% of the cases, and it was observed complete or partial resolution of the headache in 75% of the cases. The median PRL at this time in patients with complete headache resolution was 17 ng/mL, in those who reported partial recovery was 21 ng/mL, and in those in whom the headache did not change was 66 ng/mL, with a significant difference between the group with complete headache resolution vs. the group with unchanged headache (p=0.022). In the cases with complete headache resolution, the median fall on PRL levels was 89% and in those cases with partial headache resolution 86%, both significantly different (p<0.001) from the fall in the cases with an unchanged headache. Conclusion: Data allow us to conclude that, in this series, in the majority of cases the reduction in the level of PRL was followe3d by cessation or relief of the pain.

Introduction: prolactinomas are pituitary adenomas that express and secrete prolactin. These patients are overweight and the mechanisms are being studied. Goals: assess nutritional and metabolic status of overweight patients with and without hyperprolactinemia caused by prolactinoma and compare them. Materials and methods: cross-sectional study, patients with body mass index (BMI) ≥ 25 kg/m² with and without prolactinoma: 1) 20 normoprolactinemic (NPrl) with prolactinoma; 2) 23 hyperprolactinemic (HPrl) with prolactinoma; 3) 28 controls without prolactinoma or alterations in prolactin levels. Evaluated through anthropometric, dietetics, and biochemical assessment. Results: of the 71 patients evaluated, most were obese women with macroprolactinomas. All three groups had diets with low caloric and monounsaturated fatty acid (MUFA) intake, the NPrl group had low carbohydrate (CHO) intake and high lipid (LIP) and saturated fatty acid (SFA) intake, and the NPrl and HPrl groups had appropriate intake of polyunsaturated fatty acids (PUFA). The HPrl group had elevated total cholesterol. HDL cholesterol was below the recommended threshold for most patients. No statistically significant differences were found in anthropometric and biochemical variables among the groups. Conclusions: most patients with prolactinomas and controls are obese and metabolically similar regardless of prolactin levels. All groups presented low caloric and MUFA intake. Protein, LIP, SFA, and cholesterol were significantly different among the groups, the NPrl group ingested less amount of protein and greater of fat. Snacking between meals and changes of food consumption on weekends was reported by most patients. This is the first study comparing patients with prolactinomas and controls, both with overweight, regarding food consumption and feeding behavior.

Introducción: los prolactinomas son adenomas hipofisarios que expresan y secretan prolactina. Estos pacientes tienen sobrepeso y el mecanismo está en estudio. Objetivos: evaluar el estado nutricional y metabolico de los pacientes con sobrepeso con y sin hiperprolactinemia causada por prolactinoma y compararlos. Materiales y métodos: es un estudio transversal con pacientes con índice de masa corporal (IMC) . 25 kg/m² con y sin prolactinoma: 1) 20 normoprolactinemicos (NPrl) con prolactinoma; 2) 23 hiperprolactinemicos (HPrl) con prolactinoma; 3) 28 controles sin prolactinoma o alteraciones en los niveles de prolactina. Evaluados a traves de estudios antropometricos evaluacion dietética y bioquímica. Resultados: de los 71 pacientes evaluados, la mayoria eran mujeres obesas con macroprolactinomas. Los tres grupos tenian dietas con baja ingesta de calorias y acidos grasos monoinsaturados (MUFA), el grupo NPrl tenía ingesta baja en carbohidratos (CHO) y alta en lipidos (LIP) y acidos grasos saturados (SFA), y los grupos NPrl y HPrl tenían ingesta apropiada de ácidos grasos poliinsaturados (PUFA). El grupo HPrl tenía el colesterol sérico por encima del valor recomendado, mientras el colesterol HDL estaba por debajo del valor recomendado en la mayoría de los pacientes. No se encontraron diferencias estadísticamente significativas en las variables antropométricas y bioquimicas entre los grupos. Conclusiones: la mayoría de los pacientes con prolactinomas y los controles son obesos y metabolicamente similares, independientemente de los niveles de prolactina. Todos los grupos presentaron baja ingesta de calorías y de ácidos grasos monoinsaturados. Proteinas, LIP, SFA y colesterol fueron significativamente diferentes entre los grupos, el grupo de NPrl ingiere menos cantidad de proteína y mayor de grasa. La mayoría de los pacientes manifiestan picar entre las comidas y cambios en el consumo de alimentos los fines de semana. Este es el primer estudio que compara a pacientes con prolactinomas y controles, ambos con sobrepeso, en cuanto a consumo de alimentos y comportamiento alimentario.

A cirurgia transesfenoidal é o procedimento cirúrgico mais utilizado para abordagem da região hipofisária, sendo por vezes associada a complicações oronasais. MATERIAL E MÉTODOS/OBJETIVO: Estudo prospectivo, através de questionário específico e avaliação clínica complicações oronasais crônicas não-diagnosticadas, em pacientes submetidos à cirurgia transesfenoidal convencional em diferentes serviços de neurocirurgia há mais de 6 meses. RESULTADOS: 49 pacientes, 37/45 com macroadenoma. 14/49 submetidos a mais de uma intervenção, em 2/5 por via transesfenoidal. Abordagem transesfenoidal 92,8% via sublabial. Nenhum apresentava queixa espontânea. Com o questionário específico, 63,2% apresentaram queixas. Um apresentava fístula oronasal, outro, estenose da área de válvula nasal com deformidade nasal externa. A rinoscopia detectou alterações em 77,5% e a endoscopia nasal em 87,7%. Perfuração septal presente em 10/12 pacientes com crostas e 2 com secreção purulenta. Todos 4 pacientes submetidos a 2 abordagens transesfenoidais apresentaram perfuração do septo e sinéquias nasais. Nos casos com abordagem endonasal observaram-se sinéquias2, alteração em meato médio1 e estenose em área de válvula nasal1. Apenas 2 pacientes apresentaram avaliação normal. CONCLUSÃO: Alta incidência de complicações nasais após abordagem transesfenoidal convencional, observadas (exame) e não referidas espontaneamente indicam a necessidad de investigação otorrinolaringológica complementada com endoscopia nasal sistemática nestes pacientes.

Transsphenoidal surgery is the most commonly used surgical procedure to handle the hypophyseal region, sometimes associated with oronasal complications. MATERIAL AND METHODS/AIM: To evaluate prospectively (specific questionnaire, clinical evaluation) undiagnosed chronic oronasal complications in patients submitted to conventional transsphenoidal adenomectomy surgery, operated at different neurosurgery services more than 6 months ago. RESULTS: 49 patients were evaluated, 37/45 presented macroadenoma. 28,5% were submitted to more than one intervention, 2/5 transsphenoidally. Transsphenoidal approach 92.8% through sublabial route. No patient had spontaneous complaint. With the specific questionnaire 63.2% presented complaints. One patient presented an oronasal fistula, 1 stenosis of the nasal valve area with external nasal deformity. Rhinoscopy detected alterations in 77.5%, nasal endoscopy in 87.7%. Septal perforation was present in 10/12 patients with scabs and 2 with purulent secretion. All 4 patients submitted to 2 transsphenoidal approaches presented septal perforation and nasal synechiae. In the endonasal, synechiae (2), alteration in medium meatus (1) and stenosis of the nasal valve area (1) were observed. Only two patients presented normal evaluation. CONCLUSION: A high incidence of nasal complications after conventional transsphenoidal surgery observed through examination and not reported spontaneously point to the need of otorhinolaryngological investigation complemented by nasal endoscopy in patients submitted to procedures through this route.

Neoplasias como câncer de próstata, mama e cólon estão relacionadas à acromegalia. Raras vezes foi mencionada a associação com melanoma. Descreve-se caso de paciente com acromegalia no qual foi identificada lesão melanocítica suspeita, com posterior confirmação de melanoma. A excisão cirúrgica da lesão levou à cura da neoplasia. Chama-se a atenção para a necessidade de exame cuidadoso da pele de pacientes com acromegalia.

Neoplasias such as prostate, breast, and colon cancer are commonly associated with acromegaly. However, the association of the latter with melanoma has been rarely mentioned. We describe the case of a patient with acromegaly in whom a suspicious melanocytic lesion was detected, and later confirmed to be melanoma by means of biopsy. Surgical excision of the lesion led to the cure of the neoplasia. More attention should be drawn to the need for careful skin examination of patients with acromegaly.

Os autores relatam o caso de uma paciente feminina, de 24 anos, com hiperprolactinemia, na qual a tomografia computadorizada de sela túrcica detectou uma calcificação na haste hipofisária. Uma vez excluídas outras etiologias possíveis, concluiu-se pela provável relação causal entre a presença da calcificação e a hiperprolactinemia, em consequência da interrupção do aporte dopaminérgico à glândula pituitária .

In this work, the authors report the case of a female patient with 24 years of age with hyperprolactinemia, who presented a pituitary stalk calcification as seen by CT scan. Once other possible etiologies were excluded, we concluded that the calcification was probably related to hyperprolactinemia caused by interruption of the input of dopamine to the pituitary gland.

Alterações eletrencefalográficas têm sido detectadas em crianças com pubarca precoce. Esse achado poderia ser o reflexo da presença atual ou passada de patologia do sistema nervoso central ou conseqüência do nível aumentado de esteróides sexuais. Com o objetivo de testar essa última hipótese, submetemos à avaliação eletrencefalográfica 10 pacientes (7F, 3M) com hyperplasia supra-renal congênita (HSRC) por deficiência da 21-hidroxilase, 4 deles com a forma clássica e 6 com a forma não-clássica da doença. O exame neurológico tradicional foi realizado em 7 pacientes, sendo normal em todos, enquanto o exame neurológico evolutivo diagnosticou distúrbio de hiperatividade e déficit de atenção em dois. Em outro paciente, dados de história foram compatíveis com retardo do desenvolvimento neuropsicomotor. A avaliação eletrencefalográfica, quantitativa com mapeamento cerebral nos pacientes com idade superior a 3 anos (n= 9), e tradicional no restante, foi considerada anormal para a idade em 8 pacientes (80%), sendo o traçado em 7 casos caracterizado como lento. A alta freqüência de alterações eletrencefalográficas nessa amostra, bem como na pubarca precoce, sugere que esses achados sejam secundários à presença de níveis androgênicos elevados em faixa etária inapropriada e alerta para a importância da avaliação c acompanhamento neurológico de crianças portadoras de HSRC.

Electrocncephalographic changes have been delected in children with premature pubarche. These findings might be the result from past or current central nervous system dysfunction, or from increased sex steroids. In order to test the last assertion we have performed clcctroencephalographic studies in 10 patients (7F, 3M) with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency, of whom 4 classic and 6 nonclassic forms of the syndrome. Traditional neurologic examination was performed in 7 patients, the result being considered normal in everyone, while evolutional neurologic examination detected hyperactive disturbance and attention deficit in two patients. In another patient, the clinical findings were compatible with delay of neuropsychomotor development. Quantitative electroencephalography with brain mapping in patients over three years-old (n= 9), and conventional EEO in lhe remaining patient, were considered abnormal for age in 8 patients (80%), the record being characterized as slow in 7 patients. The high rate of electroencephalographic changes in this sample, as well as in premature pubarche, suggests that these findings might result from high inappropriate for age androgenic levels, and warns about the importance of neurologic examination and follow-up in children with CAH.

Prolactinoma é raro na infância e adolescência. O diagnóstico mais precoce conhecido na literatura ocorreu aos 8 anos de idade. Aqui é relatado o achado de tumor selar em menina com 6 anos, embora só fosse procurado auxílio médico tardiamente, quando a paciente já apresentava atrofia parcial de papila. Nessa ocasião, foi constatada reserva diminuída de hormônio do crescimento e de cortisol, bem como hiperprolactinemia. Devido à rápida deterioração visual, a paciente foi submetida a craniotomia fronto-temporal com a hipótese diagnóstica de craniofaringioma. O exame imuno-histoquímico do tecido tumoral foi positivo para prolactina. Conclui-se que o prolactinoma deve ser considerado no diagnóstico diferencial de tumores selares na infância, por possibilitar terapêutica menos agressiva.

Prolactinoma is rare in cihldhood and adolescence. The earliest known diagnosis was reported in a patient at 8 years of age. We report the case of a sellar tumor found in a 6 years old girl. After a long symptomatic period she was brought for treatment. At this time she had partial papillar atrophy, hyperprolactinemia, and diminished reserve of growth hormone and Cortisol. Due to rapid visual deterioration, she was submitted to a frontotem-poral craniotomy for suspected craniopharyngioma. The tumor tissue immunohistochemistry was positive for prolactin. It is concluded that prolactinoma must be considered in differential diagnosis of sellar tumors in childhood since it benefits from a less aggressive therapy.