Not a stroke? A Pituitary Macroadenoma with an unusual presentation / ¿No es una trombosis cerebral? Un macroadenoma hipofisario con una presentación inusua

We present the case of a 66 year-old male, no medical history, presenting with facial palsy, dysphagia and right hemiparesis and hemihypostesia.Neurological examination also showed left eye ptosis. Stroke was first suspected. In head-CT and angio-CT scans image compatible with macroadenoma are seen. These kind of neurological defficits are not usual with theses lesions which present with headache and visual disturbances.Stroke diagnosis and treatment should be prompt. This shoudl’t however mislead us to be precipitous and ignore differencial diagnosis. Pituitarymacroadenomas usually present with headaches and visual disturbances but this is not always the case as this report shows us. (AU)

Perfil epidemiológico e clínico de uma amostra populacional de pacientes com acromegalia no estado de Alagoas / Clinical and epidemiological profile of a sample population of patients with acromegaly in the state of Alagoas

Objetivo: determinar o perfil epidemiológico e clínico de pacientes com acromegalia no Estado de Alagoas. Métodos: trata-se de um estudo descritivo, observacional, que foi realizado na Farmácia de Medicamentos Excepcionais do Estado de Alagoas, abrangendo 12 pacientes com acromegalia dessa instituição, correspondendo a 85% dos acromegálicos do Estado de Alagoas. Resultados: dos 12 pacientes estudados, a média de idade foi de 50,8 +/- 12,1 anos e, no momento do diagnóstico, uma média de idade de 44,5 +/- 11,6 anos. Foram realizados exames de imagem em 91,6% dos pacientes, em que se evidenciou macroademona hipofisário no momento do diagnóstico. A octreotida foi o medicamento principal, utilizado em 92% dos pacientes. Conclusão: O estudo corrobora, em boa parte dos dados, a literatura atual e revela a necessidade de maior precocidade de diagnóstico e início de terapêutica adequada, evitando o surgimento de comorbidades e complicações típicas da doença. (AU)

Objective: to determine the clinical and epidemiological profile of patients with acromegaly in the state of Alagoas. Method: This is a descriptive and observational study, which was held at the Exceptional Drug Pharmacy of the State of Alagoas. This study included 12 patients with acromegaly of this institution, corresponding to 85% of acromegaly in the State of Alagoas. Results: The mean age of the patients was 50.8 +/- 12.1 year and the mean age at diagnosis was 44.5 +/- 11, 6 years. Medical imaging exams were performed in 91.7% of patients, showing pituitary macroadenoma at the moment of diagnosis. Octreotide was the main medication, used in 91,7% of patients. Conclusion: The study corroborates, in most of the data, the current literature and reveals the need for earlier diagnosis and appropriate therapy, which avoids the appearance of typical comorbidities and complications of this disease. (AU)

Assessment of the extent of pituitary macroadenomas resection in immediate postoperative MRI. / Valoración del grado de resección de los macroadenomas hipofisarios en la resonancia magnética posquirúrgica inmediata.

OBJECTIVE: To evaluate if it is possible to determine the extent of pituitary macroadenomas resection in the immediate postoperative pituitary magnetic resonance imaging (MRI). MATERIAL AND METHODS: MRI of patient with pituitary macroadenomas from January 2010 until October 2014 were reviewed. Those patients who had diagnostic MRI, immediate post-surgical MRI and at least one MRI control were included. We evaluate if the findings between the immediate postsurgical MRI and the subsequent MRI were concordant. Cases which didn't have evolutionary controls and those who were reoperation for recurrence were excluded. The degree of tumor resection was divided into groups: total resection, partial resection and doubtful. All MRI studies were performed on a1.5T machine following the same protocol sequences for all cases. One morphological part, a dynamic contrast iv and late contrast part. RESULTS: Of the 73 cases included, immediate postoperative pituitary MRI was interpreted as total resection in 38 cases and tumoral rest in 28 cases, uncertainty among rest or inflammatory changes in 7 cases. Follow- up MRI identified 41 cases total resection and tumoral rest in 32. Sensitivity and specificity of 0.78 and 0.82 and positive and negative predictive value (PPV and NPV) 0.89 and 0.89 respectively were calculated. CONCLUSION: Immediate post-surgery pituitary MRI is useful for assessing the degree of tumor resection and is a good predictor of the final degree of real resection compared with the following MRI studies. It allows us to decide the most appropriate treatment at an early stage.

Apoplejía hipofisaria, presentación de dos casos clínicos y revisión del tema / Pituitary apoplexy, presentation of two clinical cases and review of the subject

Antecedentes: la apoplejía hipofisaria es un síndrome clínico agudo, potencialmente fatal, que acontece como complicación de un adenoma hipofisario; presenta una complejidad diagnóstica, y puede imitar numerosos procesos intracraneales. Objetivo: realizar la revisión bibliográfica del tema, y describir dos casos clínicos, dada la poca frecuencia de presentación de la enfermedad y su complejidad diagnóstica. Desarrollo: caso 1, paciente masculino, de 52 años con antecedentes patológicos de hipertensión arterial y macroadenoma hipofisario no funcionante. Durante su seguimiento por Neurocirugía se detectó hiperprolactinemia, por lo que se inició tratamiento con bromocriptina a dosis progresiva, lo cual desencadenó cuadro agudo no definido. Tres meses después presentó nuevo cuadro agudo, que se diagnosticó como apoplejía hipofisaria; caso 2, paciente masculino, de 49 años, con antecedente de hipertensión arterial con tratamiento irregular. Fue ingresado en servicio de terapia intermedia con diagnóstico presuntivo de meningoencefalitis, y por estudios complementarios se llega al diagnóstico de apoplejía hipofisaria. Ambos casos llevaron tratamiento quirúrgico, con evolución favorable. Conclusiones: la apoplejía hipofisaria es una enfermedad que se presenta con poca frecuencia, por lo tanto, es importante la sospecha diagnóstica para una temprana identificación y tratamiento de la enfermedad(AU)

Background: pituitary apoplexy is an acute clinical syndrome, potentially fatal, that occurs as a complication of a pituitary adenoma. It presents a diagnostic complexity, and it can mimic numerous intracranial processes. Objective: to carry out the bibliographic review of the subject, and to describe two clinical cases given the infrequency of presentation of the disease and its diagnostic complexity. Development: case 1, male patient, 52 years old with pathological history of arterial hypertension and non-functioning pituitary macroadenoma. During follow-up by Neurosurgery, hyperprolactinemia was detected, so treatment with bromocriptine was started with a progressive dose, which triggered undefined acute symptoms. Three months later he presented new acute symptoms, which was diagnosed as pituitary apoplexy. Case 2, male patient, 49 years old, with a history of arterial hypertension under irregular treatment. He was admitted in the intermediate therapy service with a presumptive diagnosis of meningoencephalitis, and by complementary studies the diagnosis of pituitary apoplexy was reached. Both cases led to surgical treatment, with favorable evolution. Conclusions: pituitary apoplexy is a disease that occurs infrequently, therefore, diagnostic suspicion is important for early identification and treatment of the disease(AU)

Apoplejía hipofisaria, presentación de dos casos clínicos y revisión del tema / Pituitary apoplexy, presentation of two clinical cases and review of the subject

Antecedentes: la apoplejía hipofisaria es un síndrome clínico agudo, potencialmente fatal, que acontece como complicación de un adenoma hipofisario; presenta una complejidad diagnóstica, y puede imitar numerosos procesos intracraneales. Objetivo: realizar la revisión bibliográfica del tema, y describir dos casos clínicos, dada la poca frecuencia de presentación de la enfermedad y su complejidad diagnóstica. Desarrollo: caso 1, paciente masculino, de 52 años con antecedentes patológicos de hipertensión arterial y macroadenoma hipofisario no funcionante. Durante su seguimiento por Neurocirugía se detectó hiperprolactinemia, por lo que se inició tratamiento con bromocriptina a dosis progresiva, lo cual desencadenó cuadro agudo no definido. Tres meses después presentó nuevo cuadro agudo, que se diagnosticó como apoplejía hipofisaria; caso 2, paciente masculino, de 49 años, con antecedente de hipertensión arterial con tratamiento irregular. Fue ingresado en servicio de terapia intermedia con diagnóstico presuntivo de meningoencefalitis, y por estudios complementarios se llega al diagnóstico de apoplejía hipofisaria. Ambos casos llevaron tratamiento quirúrgico, con evolución favorable. Conclusiones: la apoplejía hipofisaria es una enfermedad que se presenta con poca frecuencia, por lo tanto, es importante la sospecha diagnóstica para una temprana identificación y tratamiento de la enfermedad(AU)

Background: pituitary apoplexy is an acute clinical syndrome, potentially fatal, that occurs as a complication of a pituitary adenoma. It presents a diagnostic complexity, and it can mimic numerous intracranial processes. Objective: to carry out the bibliographic review of the subject, and to describe two clinical cases given the infrequency of presentation of the disease and its diagnostic complexity. Development: case 1, male patient, 52 years old with pathological history of arterial hypertension and non-functioning pituitary macroadenoma. During follow-up by Neurosurgery, hyperprolactinemia was detected, so treatment with bromocriptine was started with a progressive dose, which triggered undefined acute symptoms. Three months later he presented new acute symptoms, which was diagnosed as pituitary apoplexy. Case 2, male patient, 49 years old, with a history of arterial hypertension under irregular treatment. He was admitted in the intermediate therapy service with a presumptive diagnosis of meningoencephalitis, and by complementary studies the diagnosis of pituitary apoplexy was reached. Both cases led to surgical treatment, with favorable evolution. Conclusions: pituitary apoplexy is a disease that occurs infrequently, therefore, diagnostic suspicion is important for early identification and treatment of the disease(AU)

Panhipopituitarismo secundario a macroadenoma hipofisario no funcionante en la adolescencia

Objetivo: Presentar el caso poco frecuente de una adolescente con un macroadenoma hipofisario no funcionante que le produjo un hipopituitarismo. Se hace una revisión de la literatura. Caso Clínico: Adolescente femenina de 16 años 2 meses de edad quien presenta poco progreso en talla y ausencia de caracteres sexuales secundarios; refiere concomitantemente cefaleas ocasionales. Al examen físico presenta talla y peso por debajo del pc 3, velocidad de crecimiento 2,8 cm/año, cubitus valgus, paladar ojival, cuarto metacarpiano corto, no tiromegalia. Genitales, mamas, vello axilar y púbico Tanner I. Se hace diagnóstico de talla baja patológica y retraso puberal y se indican exámenes de paraclínicos. Los resultados de laboratorio muestran un déficit de hormona de crecimiento (GH) y un hipocortisolismo, con función tiroidea conservada. Edad ósea de 11 años para una edad cronológica de 15 años. Cariotipo 46,XX. Rx lateral de cráneo muestra silla turca amplia y excavada. Us pélvico: útero en anteversion, central, con longitud de 20 mm; no se visualizan línea endometrial ni ovarios. La RMN muestra el piso de la región selar deformado, excavado, con imagen compatible con LOE de contornos más o menos definidos con señal intermedia baja y dishomogénea en T1, con realce hipertenso dishomogéneo tras la administración del medio de contraste, de aproximadamente 2,9 x 1,36 cm. de diámetro, con compromiso de los elementos supraselares, ejerciendo efecto compresivo sobre el infundíbulo el cual deforma el quiasma óptico. Se hace diagnóstico de Panhipopituitarismo secundario a Macroadenoma Hipofisario no Secretante, con Déficit de GH, Hipogonadismo Hipogonadotropo e Insuficiencia Suprarrenal Secundaria (Déficit de ACTH). Se indica tratamiento con hidrocortisona por vía oral. Se refiere para resolución quirúrgica por vía transesfenoidal ya que en nuestro centro no se realiza. Se desconoce el reporte histológico del tumor y la evolución de la paciente. Conclusiones: Los adenomas hipofisarios representan menos del 2-3% de todos los tumores intracraneales y sus manifestaciones clínicas dependen de la suma de un efecto masa, que causa alteraciones neurológicas, y la afectación de la secreción hormonal, ya sea por exceso o por defecto. Es importante realizar un diagnóstico temprano, así como un tratamiento efectivo y seguimiento a largo plazo.

Objective: To present the uncommon case of a teenager with a non-functioning pituitary macroadenoma that resulted in a hypopituitarism. A literature review is done. Case Report: Female adolescent of 16 years 2 months of age who presents little progress in stature and absence of secondary sexual characters; concomitantly refers occasional headaches. Physical examination: height and weight below the 3 pc, growth velocity 2.8 cm/year, cubitus valgus, ojival palate, short fourth metacarpal, no thyroid enlargement. Genitals, breasts, axillary and pubic hair, Tanner I. The diagnosis of pathological short stature and delayed puberty is done and paraclinical examinations are indicated. Laboratory results show a deficit of growth hormone (GH) and a hypocortisolism with preserved thyroid function. Bone age of 11 years for a chronological age of 15 years. Karyotype 46, XX. Rx of the skull shows a wide and excavated sella turcica. Pelvic Us: central uterus, in anteversion, 20 mm in length and the endometrium and ovaries were no visible. NMR shows the floor of the sellar region warped, carved, with a image compatible with tumour of defined contours, with low-intermediate and dishomogenea signal on T1, with hypertensive and heterogeneou enhancement after administration of contrast medium, approximately 2.9 x 1.36 cm. in diameter, with involvement of the supraselares elements exerting compressive effect on the infundibulum, which deforms the optic chiasm. The diagnosis of panhypopituitarism secondary to pituitary non functioning macroadenoma is done, with GH deficiency, hypogonadotropic hypogonadism and secondary adrenal insufficiency (ACTH deficiency). Treatment with oral hydrocortisone is indicated. The patient is referred for resolution by transsphenoidal surgery. The histological report of the tumor and the evolution of the patient are unknown. Conclusions: Pituitary adenomas account for less than 2-3% of all intracranial tumours, and their clinical manifestations depend on the sum of a mass effect, which causes neurological disorders, and the disruption of hormonal secretion, either up or down. It is important to make an early diagnosis, an effective treatment and long-term monitoring.