Complete Resolution of the Pituitary Mass Lesion and Improvement of Pituitary Function with Corticosteroid in Autoimmune Hypophysitis: A Case Report

BackgroundAutoimmune hypophysitis is the consequence of an immune-mediated inflammation of the pituitary gland, which is rare, and most frequently occurs in females during postpartum periods. It usually responds well to corticosteroid treatment with reported resolution of the pituitary mass lesion.Case ReportA 51 years male presented with a one-month history of lethargy, headache, nausea, proximal muscle weakness with intermittent flushing. He was a diabetic with metformin 500mg twice daily. No other remarkable medical history or family history of autoimmune disease. On examination, he had no neurological deficit with a normal visual field. His initial biochemical evaluation showed features of secondary hypothyroidism as evidenced by low free FT4 and suppressed TSH with normal electrolytes. The subsequent evaluation of his hormonal profile revealed panhypopituitarism. Contrast MRI of pituitary showed an enhanced homogenous mass and minimal stalk thickening with a dural tail and preserved posterior bright spot. He was managed with glucocorticoid 20 mg once daily for two weeks along with levothyroxine and testosterone replacement. After two weeks of treatment, he improved clinically. Repeat MRI imaging of the pituitary showed complete resolution of the homogenous mass.ConclusionAlthough autoimmune hypophysitis is rare in males, a careful clinical history with necessary hormonal investigations is required for the suspicion about the inflammatory pituitary disorders This current case highlights glucocorticoid as the primary modality of treatment and the need for long-term follow-up with periodic clinical assessment.     

Malignant intracranial germ cell tumor treated with chemotherapy and radiotherapy without histopathological confirmation

In two men aged 19 and 24 years, a rare malignant intracranial germ cell tumour was diagnosed in the pineal gland region and in the second patient in a suprasellar position as well. Although histological confirmation is required in general to diagnose a malignancy, obtaining it can be difficult and potentially dangerous in case of intracranial neoplasms. The clinical picture of a young patient with an intracerebral tumour localised in the midline of the brain and increased levels of the tumour markers alpha-foetoprotein and/or human chorion gonadotrophin (beta-HCG) in blood and/or CSF makes any other diagnosis highly unlikely. There is no place for radical surgery in the first-line treatment of malignant intracerebral germ cell tumours because of the sensitivity to radio- and chemotherapy. Also, the sensitivity to chemotherapy makes it possible to reduce radiation volume and dose in an effort to avoid the serious complications of craniospinal irradiation. Both patients responded well to chemotherapy based on cisplatin followed by radiotherapy.     

Suprasellar germinoma: two case reports

Suprasellar germinomas are frequent in childhood and adolescence, particularly in male sex. The clinical and neuroendocrine abnormalities depend of tumor localization: Increased intracranial pressure, visual disturbances, hypopituitarism, Parinaud syndrome. We report two cases of suprasellar germinoma in young male patients (16 and 18 years old). The first patient hrad corticotorpin insufficiency and clinical signs of hypothyro?dism and hypogonadism. The second had central hypocorticism, hypothyro?dism and hypogonadism associated with central diabetes insipidus and hyperprolactinemia. The diagnosis of germinoma was confirmed after surgery by anatomopathologic examination in the first case and by stereotaxic biopsy in the second case. Treatment by radiotherapy improves prognosis of this disease.     

Clinical presentation, treatment, and follow-up of 32 patients with a primary intracranial germinoma, registered during the previous 15 years in the Dutch Pathological-Anatomical National Automated Archive (PALGA)

OBJECTIVE: Evaluation of clinical presentation, treatment and follow-up of patients with intracranial germinoma in the Netherlands. DESIGN: Retrospective. METHOD: The case histories of 32 patients with histologically verified intracranial germinoma, registered in the period 1983-1999 in the Pathological Anatomical Nationwide Automated Archive (PALGA), were studied. Fifty of the 59 registered patients were found of whom 6 had no germinoma. Informed consent was obtained from 32 of the 44 patients with respect to studying their medical records for age, symptoms at presentation, diagnostic investigations, presence of tumour markers, treatment and follow up. RESULTS: The patient group consisted of 23 men and 9 women aged 6 to 35.6 years (mean: 17.3) and was subdivided with respect to their tumour localization. In patients with pineal localization symptoms of increased intracranial pressure and eye movement disorders were most prominent, whereas in patients with suprasellar localization endocrine disorders prevailed. Thirty-one patients were treated with radiotherapy, one with combined radiotherapy and chemotherapy and one surgically. Twenty-six patients had remained disease free after a median follow-up of 39 months (range: 0-144 months). One patient developed an intracranial embryonal carcinoma and another a testis seminoma. Two patients died because of recurrences. Two other patients died of causes not directly related to the germinoma. CONCLUSION: At the time of this study 84% of all patients treated with radiotherapy were disease-free. Although the percentage patients who had recovered after treatment (surgical and radiotherapy) was high, many patients either already had or subsequently developed neurological and endocrinological deficiencies.     

Multiple tumors in the midline of the brain with endocrine dysfunction

A patient is reported with hypothalamic-pituitary dysfunction due to multiple cerebral midline tumours of germinoma type. The symptomatology and differential diagnosis are reviewed. The necessity of stereotactic biopsy is discussed. The treatment of these tumours as proposed in recent literature is mentioned, with special reference to radiotherapy (cranial and spinal) and chemotherapy.     

急性1，2-二氯乙烷中毒脑脊液常规指标的动态监测

  目的  探讨脑脊液（cerebral spinal fluid,CSF）常规检查在急性1,2-二氯乙烷（1,2-DCE）中毒诊治中的价值。
  方法  68例急性1,2-DCE中毒患者入院后行CSF压力、常规及生化检查,并在病程中动态监测及分析CSF各项指标的变化。
  结果  急性1,2-DCE中毒患者入院时CSF外观无色透明、细胞数均正常;CSF压力增高阳性率为47.1%（32/68）,其中脑水肿组为66.7%,无脑水肿组无增高;脑水肿组CSF压力均值高于无脑水肿组（P < 0.01）;蛋白质浓度正常或轻微升高占16.2%,无脑水肿组总蛋白与白蛋白浓度高于脑水肿组（P < 0.05）;葡萄糖和氯化物大致正常;重度中毒组CSF压力均值显著高于轻度中毒组（P < 0.05）;急性1,2-DCE中毒患者入院后CSF压力持续升高,于病程（脱离接触）31~60 d最高,治疗后期患者CSF压力恢复缓慢,CSF压力的回落滞后于高颅压的临床表现及CT、MR等影像学改变。
  结论  动态监测CSF压力及各项常规指标对急性1,2-DCE中毒的临床诊治具有重大价值。
     

Sella turcica in male-to-female transsexuals

Roentgenological examination of the skull in 11 male-to-female transsexuals revealed considerable asymmetry (2.5–3 mm) of the sellar floor in three cases. There was no asymmetry exceeding 1.5 mm in 103 male controls. Asymmetry of the sella turcica suggests the presence of an intrasellar pituitary tumor. This finding in three patients with transsexualism is of interest in view of reports of endocrine disorders or hormonal secretion abnormalities in patients with atypical sexuality or gender identity problems.     

垂体柄阻断综合征的病因学分析

目的:分析垂体柄阻断综合征的相关病因,以期减少该病的发生和早期诊治。方法:将53例诊断为垂体柄阻断综合征患者作为病变组,37例相同年龄组正常人作为对照组,搜集相关孕产史及既往史。采用多变量Logistic回归分析垂体柄阻断综合征的相关病因。结果:多因素回归模型分析与垂体柄阻断综合征相关的7种病因,分别为胎位异常(OR=12.107)、新生儿窒息(OR=11.224)、孕期异常(OR=9.204)、生产环境(OR=9.161)、产次(OR=7.195)、胎龄(OR=4.439)、既往史(OR=3.763)。结论:胎位异常、新生儿窒息、孕期异常是垂体柄阻断综合征的主要危险因素。多次生产、生产环境及胎龄因素与垂体柄阻断综合征次相关。既往史与垂体柄阻断综合征无相关。     

垂体脓肿的诊断与治疗

目的 探讨垂体脓肿的临床表现及治疗方法.方法 回顾性分析6例垂体脓肿的诊治资料,并复习相关文献.结果 6例患者中,头痛5例、垂体功能低下4例、视力下降和/或颞侧偏盲4例、发热1例.MRI或cT检查5例有病灶周边环形强化.术前诊断为垂体腺瘤3例,颅咽管瘤1例,垂体脓肿2例.1例经颅手术,5例经蝶手术.术后均静脉滴注抗生素治疗3周.所有病例术后症状缓解,随访8个月至10年,1例经颅手术者2年后复发改行经蝶手术而治愈,5例经蝶手术者无复发.结论 垂体脓肿易误诊,对鞍区囊性病变应怀疑有垂体脓肿的可能.及早经蝶手术及围手术期合理使用抗生素是垂体脓肿治疗的关键.     

Nutritional status of cancer patients given different treatment modalities

The nutritional status of 91 cancer patients was assessed at the time of diagnosis and follow-up assessments were carried out at the third and sixth week after initiating different treatment modalities to study the effect of type and duration of treatment on nutritional status. Parameters assessed were anthropometry, biochemical status and clinical signs and symptoms of nutritional deficiencies. Treatment modalities studied were radiotherapy, chemotherapy, chemotherapy+radiotherapy, and combined treatment modality (surgery+radiotherapy+chemotherapy). The nutritional status of male patients was affected most by chemotherapy+radiotherapy while females were affected most with radiotherapy. Biochemical parameters showed a marginal decline in total serum protein and serum albumin concentrations. Haemoglobin concentrations declined substantially with radiotherapy and chemotherapy. The lymphocyte count decreased substantially irrespective of the treatment modality. Clinical examination revealed increased incidences of deficiency signs and symptoms in all patients during follow-up irrespective of treatment modality.     

Nutritional status of cancer patients given different treatment modalities

The nutritional status of 91 cancer patients was assessed at the time of diagnosis and follow-up assessments were carried out at the third and sixth week after initiating different treatment modalities to study the effect of type and duration of treatment on nutritional status. Parameters assessed were anthropometry, biochemical status and clinical signs and symptoms of nutritional deficiencies. Treatment modalities studied were radiotherapy, chemotherapy, chemotherapy+radiotherapy, and combined treatment modality (surgery+radiotherapy+chemotherapy). The nutritional status of male patients was affected most by chemotherapy+radiotherapy while females were affected most with radiotherapy. Biochemical parameters showed a marginal decline in total serum protein and serum albumin concentrations. Haemoglobin concentrations declined substantially with radiotherapy and chemotherapy. The lymphocyte count decreased substantially irrespective of the treatment modality. Clinical examination revealed increased incidences of deficiency signs and symptoms in all patients during follow-up irrespective of treatment modality.     

Posttraumatic hypopituitarism

A 39-year-old woman was admitted with complaints of weight gain, a decreased sense of well-being and amenorrhoea. One and a half year prior to admission she had been involved in a serious road accident and had spent several days in coma due to an epidural haematoma. She was found to have hypopituitarism with deficient somatotropic and gonadotropic axes, as well as mild hyperprolactinaemia, probably due to a pituitary stalk lesion. All patients with severe trauma to the skull are at risk of developing posttraumatic hypopituitarism, so that pituitary function testing should be performed routinely, certainly in the presence of symptoms.     

Cystic abnormalities of the pancreas: inflammatory pseudocyst or neoplasm

A 79-year-old woman presented with complaints of upper abdominal pain, nausea and vomiting since a few days. Laboratory tests showed no abnormalities except for some indications of an inflammation. Based on the medical history, physical examination and findings from radiological examination, initially the diagnosis was 'chronic pancreatitis with formation ofa pseudocyst caused by alcohol abuse'. After one week the patient developed cholestatic liver function disorders with elevated serum pancreatic enzymes. A CT scan of the abdomen showed a dilated gallbladder and progression of the cystic lesion in the pancreatic head with compression of the distal common bile duct. An endoscopic retrograde cholangiopancreatography was performed and the findings fitted a diagnosis of an intraductal papillary mucinous neoplasm. Differentiation between an inflammatory or neoplastic origin of cystic lesions in the pancreas can be difficult. There is a risk ofmisdiagnosing a cystic neoplasm for a pseudocyst. This may lead to delays in making the correct diagnosis.     

The importance of a complete diagnostic workup in patients with nontraumatic (partial) paraplegia

In two women, aged 86 and 56 years, respectively, who suffered from back pain and loss of strength, and in a 55-year-old man who lost sensation and strength in his left leg, spinal-cord compression in connection with vertebral destruction was seen on radiological examination. When spinal-cord compression is the result of a local malignant tumour, the therapy often entails emergency radiotherapy. In the first two patients, histological examination revealed a solitary plasmocytoma and curative high-dose radiotherapy was applied. The third patient also had a lung tumour and received low-dose palliative radiotherapy to the vertebrae, as a metastasis was suspected. Later, however, histopathologic examination of the vertebral lesion revealed osteomyelitis due to Listeria monocytogenes and the lung tumour was diagnosed as a pT2N0M0 broncho-alveolar carcinoma which was surgically removed. When a patient is referred with a nontraumatic spinal-cord injury, it is important to complete the radiological and histological examinations before starting emergency radiotherapy in order to prevent an inadequate or even incorrect treatment.     

Cerebrospinal fluid rhinorrhoea in pituitary tumours

Three cases of CSF rhinorrhoea due to pituitary tumours are reported and the literature reviewed. The treatment of choice appears to be trans-sphenoidal exploration of the pituitary fossa with insertion of a free muscle graft followed by radiotherapy. The probability of the tumour being a prolactin-secreting adenoma is discussed.     

孕早中期唐氏综合症720例产前诊断数据分析

[目的]探讨孕早中期孕妇产前筛查唐氏综合征和其他严重先天缺陷的方法及临床应用。[方法]应用全自动时间分辨荧光分析法对720例孕早中期孕妇进行血清标志物(PAPP-A/freeβ-HCG或者AFP/freeβ-HCG/μE3)联合筛查,使用Multical软件筛查高风险孕妇,通过羊水或脐带血细胞培养获得染色体核型以明确诊断。[结果]筛查随访的396例孕妇中,筛查出唐氏综合征及18-三体高风险者2例,NTD高风险者1例,单项指标异常者5例,明确诊断唐氏综合征1例、18-三体1例、臂间倒位1例、缺失1例、重型地中海贫血1例,无脑畸形1例。[结论联合筛查血清标志物的效率高于单项筛查,血清标志物单项筛查阳性者也应建议行产前诊断。唐氏综合征筛查是预测不良妊娠结局的有效指标,联合产前诊断对防止先天缺陷儿出生有重要的临床意义。     

Meningitis caused by Toscana virus during a summer stay in Italy

A 44-year-old woman suffered fever, headache and meningism during a summer stay in a region of central Italy where sand-fly fever is endemic. A few days after returning to the Netherlands, she appeared mentally and physically slow but had no fever. Because of the possibility of viral meningitis an examination of the cerebrospinal fluid (CSF) was carried out. This revealed a cell count of 1074/3 cells. Toscana virus antibodies of the IgG and IgM subclass were detected in two respective serum samples and in the CSF. This led to the diagnosis 'Toscana virus meningo-encephalitis'. Toscana virus is classified amongst the sandfly fever virus group. These viruses are transmitted by sand flies (Phlebotomus species) which reside in humid areas around the Mediterranean. Toscana virus is the main cause of viral meningoencephalitis in some areas of central Italy and possibly in southern Spain. The patient recovered within a few days without antiviral therapy. Toscana virus meningo-encephalitis should be included in the differential diagnosis of holidaymakers with neurological symptoms returning from the Mediterranean.     

病毒性脑炎患儿神经元特异性烯醇化酶的水平变化及临床意义

目的:探讨病毒性脑炎患儿脑脊液(CSF)及血清中神经元特异性烯醇化酶(NSE)水平的变化及临床意义。方法:以28例病毒性脑炎患儿作为观察组,选择同期住院怀疑中枢神经系统疾病但脑脊液正常、最后确诊为上呼吸道感染的患儿作为对照组,两组患儿均于入院后24~48 h行腰椎穿刺取脑脊液,同时抽取静脉血,用酶连免疫吸附法(ELISA)检测脑脊液和血清中的NSE浓度。结果:病毒性脑炎患儿脑脊液和血清中NSE水平分别为(23.16±5.93)μg/L和(17.80±4.27)μg/L,对照组分别为(8.96±1.03)μg/L和(7.78±0.94)μg/L,两组有显著性差异(P均<0.01),且血清中NSE浓度与脑脊液中NSE浓度呈正相关(r=0.84,P<0.01)。结论:病毒性脑炎患儿脑脊液和血清中NSE浓度明显升高,脑脊液和血清中NSE可作为判断病毒性脑炎患儿脑组织损伤的客观指标之一。     

Intrathecal cytokine responses in Trypanosoma brucei rhodesiense sleeping sickness patients

Intrathecal cytokine levels and blood-cerebrospinal fluid (CSF) barrier function were studied in 91 Trypanosoma brucei rhodesiense-infected patients. The CSF concentration of the cellular immune activation marker neopterin and the cytokines IL-6 and IL-10 were increased over control and post-treatment levels in all patients, with maximal levels observed in late-stage (meningoencephalitic) individuals. Analysis of CSF/serum concentration quotients indicated that IL-10 and neopterin were derived from central nervous system synthesis in at least 25% of the patients. Blood-CSF barrier dysfunction occurred in 64% of late-stage patients but not in early-stage patients. While the high level of neopterin observed in the late-stage patient CSF is indicative of widespread cellular activation, the increased levels of IL-6 and IL-10 suggest that counter-inflammatory cellular responses may be important in the regulation of neuropathogenesis in late-stage human African trypanosomiasis.