Primary central nervous system (CNS) lymphoma is an uncommon neoplasm. However, the incidence of primary CNS lymphoma has increased more than 10-fold over the past three decades, and continues to accelerate. Currently, primary CNS lymphoma represents 4 to 7 percent of all newly diagnosed primary CNS tumors. Primary CNS lymphoma may arise from different parts of the brain, with deep hemispheric periventricular white matter being the most common site of origin. The presenting symptoms in primary CNS lymphoma vary depending on the location of the mass. Involvement of the hypothalamic-pituitary axis may cause hypopituitarism, diabetes insipidus, headache, diplopia, and blurred vision.
Ji Myoung Lee, Sang Mi Park, Byung Hee Hwang, Hyun Sook Choi, Seong Su Lee, Jee Young Kim, Sung Rae Kim, Kwang Woo Lee, Ho Young Son, Sung Koo Kang, Soon Jib Yoo
J Korean Endocr Soc. 2008;23(2):142-147. Published online April 1, 2008
Idiopathic central diabetes insipidus is most likely to occur in young patients who have a clinical history of autoimmune disease. The presentation of clinical findings such as central diabetes insipidus and pituitary stalk thickening on sellar magnetic resonance imaging (MRI) in a young women would strongly suggest lymphocytic hypophysitis, which is a rare inflammatory process involving the pituitary stalk and the pituitary gland, yet this disease can sometimes regress.
Central diabetes insipidus is a heterogeneous condition that is characterized by polyuria and polydipsia, and this is due to a deficiency of arginine vasopressin. Central diabetes insipidus is rare in children and young adults, and up to 50 percent of cases are idiopathic. Genetic abnormalities in the homeobox genes have recently been shown, on sellar magnetic resonance imaging, to be associated with combined pituitary hormone deficiency with pituitary defect. We report here on a 44-year-old female who suffered from polydipsia, polyuria and primary amenorrhea since childhood. She was diagnosed with idiopathic central diabetes insipidus together with primary empty sella and combined pituitary hormone deficiency. On the genetic analysis, she was proven to have a point mutation of the PROP-1 gene, which is known as a cause of combined pituitary hormone deficiency.
Metastasis to the pituitary gland from systemic cancer is a rare condition. The breast and lung are the most common sites of primary tumor metastasis. Pituitary metastasis may present with diabetes insipidus, cranial nerve palsy and hypopituitarism, and diabetes insipidus is the most frequent symptom at presentation. We report here on a 44 year-old woman with pituitary metastasis from breast cancer, and she developed central diabetes insipidus and hypopituitarism. The clinical diagnosis was made by performing a water deprivation test, a combined pituitary test and a MRI brain scan, and the latter showed metastatic tumor in the pituitary gland with invasion of the pituitary stalk. Symptomatic relief was obtained with administration of desmopressin; the urine osmolarity was increased with this treatment. We report here on a case of pituitary metastasis from breast cancer and the patient developed central diabetes insipidus and hypopituitarism. We also include a review of the relevant literature.
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A Case of Pituitary Metastasis from Breast Cancer That Presented as Left Visual Disturbance Young Ha Kim, Beom jun Lee, Kyung Jin Lee, Jin Hee Cho Journal of Korean Neurosurgical Society.2012; 51(2): 94. CrossRef
A Case of Panhypopituitarism and Central Diabetes Insipidus Caused by Primary Central Nervous System Lymphoma Mi Sun Ahn, Soon Sun Kim, Tae Ho Kim, Seung Jin Han, Dae Jung Kim, Hugh Chul Kim, Se Hyuk Kim, Jae Ho Han, Ho Sung Kim, Yoon-Sok Chung Journal of Korean Endocrine Society.2008; 23(4): 260. CrossRef
Mi Jung Kim, Byung Wan Lee, In Kyung Jeong, Jun Goo Kang, Seong Jin Lee, Eun Gyung Hong, Hyeon Kyu Kim, Doo Man Kim, Jae Myung Yoo, Sung Hee Ihm, Moon Gi Choi, Hyung Joon Yoo
J Korean Endocr Soc. 2007;22(2):118-124. Published online April 1, 2007
Autosomal dominant familial neurohypophyseal diabetes insipidus (adFNDI) is a rare form of central diabetes insipidus (DI), and this malady is clinically characterized by polydipsia and polyuria, and it is caused by mutation in the vasopressin-neurophysin II. We identified a Korean family that suffered with adFNDI and we found a novel mutation in the NP II molecule. The index subject's DI symptoms dated to childhood, and his familial history was consistent with autosomal transmission. The diagnosis of central DI was done by performing a water deprivation test and a vasopressin challenge test. For molecular analysis, the genomic DNA was extracted and the AVP-NP II gene was amplified by polymerase chain reaction from four clinically-affected members and seven clinically-nonaffected members. Genetic analysis of AVP-NP II revealed new a heterozygous missense mutation in exon 2 of the AVP-NP II gene (+1692C > A) and this amino acid substitution (Cys105Stop) was predicted to have occurred in four clinically-affected subjects. In summary, in the present study we have described a novel mutation of the AVP-NPII gene in a Korean family suffering with adFNDI.
Dong Jin Kim, Nan Hee Kim, Ju Ri Park, Sae Jeong Yang, Hye Suk Kim, Hye Jin Yoo, Soo Yeon Park, Ohk Hyun Ryu, Kye Won Lee, Hee Young Kim, Ji A Seo, Sin Gon Kim, Kyung Mook Choi, Sei Hyun Baik, Dong Seop Choi
J Korean Endocr Soc. 2006;21(4):333-337. Published online August 1, 2006
Sheehan's syndrome occurs as a result of ischemic pituitary necrosis due to severe postpartum hemorrhage. The manifestations of this clinical syndrome are most often caused by a deficiency in the hormones produced by the anterior pituitary gland, whereas the neurohypophysis is usually preserved but can be involved in severe cases that manifest as diabetes insipidus. This is a report of Sheehan's syndrome that manifested with diabetes insipidus as presenting symptom 2 month's after delivery. The patient suffered massive bleeding, so received a blood transfusion. A combined pituitary stimulation and water deprivation test revealed deficiencies of not only anterior pituitary hormones, such as growth hormone and prolactin, but also of anti-diuretic hormone. We report this case, with a review of the literature.
Langerhans cell histiocytosis can cause central diabetes insipidus. Here, a case of Langerhans cell histiocytosis invading the pituitary stalk was experienced. The patient was 15 years old boy, with complaint of polydipsia and polyuria. A water deprivation test was carried out, and the urine osmolarity was increased from 165 to 469 mosm/kg following an injection of AVP to confirm the diagnosis of central diabetes insipidus. A pituitary function stimulation test gave a normal response. A sellar MRI was performed, which showed a thickened pituitary stalk mass (about 5.7mm), with an increased size, 6.9 mm, on a second MRI 2 month later. A tissue biopsy was performed, which showed aggregates of histiocytes and inflammatory cells, with prominent eosinophils (H&E), and also revealed strong reactivity to anti-CD1a antibody on the immunohistochemistry. After confirmative tissue diagnosis, the patient received radiotherapy (900 cGy). The thickened mass of the pituitary stalk disappeared on the MRI following the radiotherapy. The patient was managed with DDAVP nasal spray, after which the polyuric symptoms were completely relieved.
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Adult Multisystem Langerhans Cell Histiocytosis Presenting with Central Diabetes Insipidus Successfully Treated with Chemotherapy Jung-Eun Choi, Hae Ri Lee, Jung Hun Ohn, Min Kyong Moon, Juri Park, Seong Jin Lee, Moon-Gi Choi, Hyung Joon Yoo, Jung Han Kim, Eun-Gyoung Hong Endocrinology and Metabolism.2014; 29(3): 394. CrossRef
Clinical Characteristics of Langerhans Cell Histiocytosis with Hypothalamo-Pituitary Involvement Eun Shil Hong, Jung Hun Ohn, Jung Hee Kim, Yul Hwang-Bo, Jin Joo Kim, Jung Hee Kwon, Jung Won Lee, Se Youn Choi, Eun Kyung Lee, Sun Wook Cho, Chan Soo Shin, Kyong Soo Park, Hak Chul Jang, Bo Youn Cho, Hong Kyu Lee, Choong Ho Shin, Sei Won Yang, Seong Yeon Endocrinology and Metabolism.2011; 26(1): 38. CrossRef
A Case of Idiopathic Central Diabetes Insipidus together with Primary Empty Sella and Combined Pituitary Hormone Deficiency Sun Young Ahn, Kyu Hwan Bae, Myung Hwan Kim, Ji Hyun Lee, Ho Sang Shon, Eui Dal Jung Journal of Korean Endocrine Society.2007; 22(4): 272. CrossRef
Ho Jong Lee, In Myung Yang, Sun Kee Min, Jung Hyun Noh, Cheol Young Park, Seung Joon Oh, Deog Yoon Kim, Jung Taek Woo, Sung Woon Kim, Jin Woo Kim, Young Seol Kim, Young Kil Choi
J Korean Endocr Soc. 2001;16(4-5):438-446. Published online October 1, 2001
BACKGROUND Although the water restriction test(WRT) has been used as a standard test for the differential diagnosis of diabetes insipidus(DI), the measurement of plasma ADH concentration is also known to be useful method for differential diagnosis. Recent studies have shown that some patients with idiopathic central DI(CDI) were found to have a lesion on follow-up imaging studies. There have been no report in Korea on plasma ADH measurement for the differential diagnosis of DI, nor on follow-up imaging study of the idiopathic CDI. METHODS: We retrospectively reviewed the clinical and laboratory findings of 26 patients(12 men, 14 women, age 9-65 years) with CDI, including pituitary MRI or CT scan, who had been diagnosed with WRT and had undergone plasma ADH concentration measurement. RESULTS: 1) Clinical features of the patients with complete CDI did not differ from those of patients with partial CDI. 2) Maximal urine osmolality of complete CDI and partial CDI were 168+/-69mOsm/kg and 431+/-141mOsm/kg, respectively, and the percentage increase in the urinary osmolality after ADH injection was 209+/-149% and 29+/-17%, respectively. 3) Among the 26 patients, 10 patients had their plasma ADH measured. Nine patients in this group were diagnosed as CDI by WRT and plasma ADH concentration of the 9 was compatible for CDI. The plasma ADH level was also inappropriately low in one patient who had been diagnosed with primary polydipsia by WRT, the patient was diagnosed as partial CDI. 4) The findings of follow-up MRI revealed isolated thickening of the pituitary stalk in two cases of idiopathic CDI diagnosed initially with MRI. CONCLUSION: This study suggests that the measurement of plasma ADH can ensure a better differential diagnosis between partial CDI and primary polydipsia, and that the patients with idiopathic CDI should be examined regularly with MRI brain scan, including the pituitary gland.
BACKGROUND Idiopathic Central Diabetes Insipidus in children or adolescents requires a frequent follow-up regimen using serial brain MRI and CSF examinations especially if an isolated pituitary stalk thickening or loss of a hyperintense signal in the posterior pituitary lobe is observed. Although a detailed description has not been made in adults who had Idiopathic Central Diabetes Insipidus, the clinical course of a Central Diabetes Insipidus in children or in adolescents can not be applied to adults because a follow-up study is so invasive and expensive. In this report, we evaluated the clinical course of Idiopathic Central Diabetes Insipidus in adults. METHODS: The diagnosis of Idiopathic Central Diabetes Insipidus was based on the presenting clinical symptoms, the water deprivation test, biochemical studies and a brain MRI. We measured the urine specific gravity, urine and plasma osmolarity, electrolytes, and daily urine amount and we also performed an anterior pituitary evaluation. Patients had contrast-enhanced MRI and biochemical studies every 4 to 12 months. RESULTS: The patients included 8 females and 4 males. Their ages ranged from 20 to 76 years and their mean age was 45+/-17 years. Tumor markers in the CSF were not detected any of the patients. An anterior pituitary evaluation showed that four patients had hyperprolactinemia, and five patients had impaired secretory responses of Growth Hormone to an insulin induced hypoglycemia. Nine of the 12 patients had thickening of the pituitary stalk, seven had lacked the hyperintense signal of a normal neurohypophysis. The abnormalities of MRI disappeared in 3 patients by the 4th, 27th and 36th month follow up periods, respectively. The follow up duration was between 8 months and 11 years 3 months and the mean follow up duration period was 50.6+/-45.5 months. Clinical symptoms were corrected by DDAVP administration. Other symptoms were absent. CONCLUSION: In our study, of Idiopathic Central Diabetes Insipidus in adults there were no observed germinomas or other disease that were observed. Therefore this disorder may have a benign course.
Pineal germinomas are the most common intracranial germ cell tumor and occur in relatively young age, with 26% between 10 and 12, 65% between 10 and 21 and 95% before age 27. Patients with pineal germinoma present headache, lethargy, nausea, and vomiting which result from raised intracranial pressure and Parinaud's syndrome secondary to midbrain tectal compression, and rarely endocrine dysfunction such as diabetes insipidus (DI), panhypopituitarism, and precocious puberty. We experienced a case of pineal and suprasellar germinoma presenting with central diabetes insipidus and panhypopituitarism.
Sung Kil Lim, Young Duk Song, Hyun Chul Lee, Kap Bum Huh, Hyun Soo Kim, Kyung Rae Kim, Yoo Mi Lee, Yong Suk Yoon, Suk Ho Kwon, Jae Hyun Nam, Sang Hak Lee
J Korean Endocr Soc. 1997;12(4):596-601. Published online January 1, 2001
The causes of central diabetes insipidus (CDI) are numerous; some primary cases are idiopathic while most secondary cases are surgically induced or the result of tumor. The frequency of metastatic tumor as a cause of DI is 6% to 20% of cases, Variety of malignancies including breast, lung, colon, prostate cancer, and leukemia/lymphoma have been reported to metastasize to the pituitary, although most patients are asymptomatic. Clinical manifestations of pituitary metastases include anterior pituitary failure, visual disturbance, and extraocular muscle weakness. DI is the most common clinical manifestation of hypothalamic-pituitary axis (HPA) metastases. Anatomical basis far this clinical picture is that most metastases occur in the posterior lobe. We report on a 35-year-old male patient with meningeal involvement of malignant lymphoma that was thought to be associated with DI. Confirmation of primary malignancy was made by biopsy at site of cervical lymph node and tonsil, Pituitary involvement was suspected on brain MRI, and satisfactory symptornatic relief was obtained with vasopressin. Because of the progres-sion of underlying lymphorna that has shown no response to combined anticancer chemotherapy, the patient expired on 60th hospital day.
Diabetic insipidus may first present before, during or immediately after pregnancy. Pregnancy serves as a physiologic provocation test that unmasks latent diabetes insipidus. It may explain the very rare first occurrence of diabetes insipidus in the pureperium. Central diabetes insipidus occurring after delivery associated with a catastrophic delivery and postpartum hypopituitarism is more frequent entity. We recently experienced central diabetes insipidus patient, aggrevated polyuria and polydipsia after total hysterectomy due to cervical carcinoma, who had interesting past history of severe polyuria and polydipsia developed during puerperium of 1st pregnancy, 14 years ago. She have had quiet normal pregnancy and there was no definite postpartum complication of pregnancy at that time. There were no change of polyuria and polydipsia during and after second pregnancy and 2 another pregnancy terminated artificially compare to puerperium of 1st delivery. She has intact anterior pituitary gland functinally and anatomically, but the posterior pituitary hyperintense signal with pre-enhance Tl-weighted imaging was absent on MRI.
Soon Jib Yoo, Kun Ho Yoon, Moo Il Kang, Bong Yun Cha, Kwang Woo Lee, Ho Young Son, Sung Koo Kang, Myung Hee Chung, Yoon Shin, Hyuk Sang Kwon, Seung Won Jin
J Korean Endocr Soc. 1997;12(2):308-314. Published online January 1, 2001
Complications related to tuberculous menngitis (TBM) is frequently encountered in medical field during, just after treatment and long time later. Hypothalamo-pituitary dysfunctions such as diabetes incipidus, dwarfism, hypogonadism, growth failure, and hypopituitarism are one of rare complication secondary to TBM and of which obesity with hypogonadism is most commonly documented. Several pathologic mechanics like a granuloma in hypothalamus, or pituitary stalk, organization and progressive scarring of the purulent exudate in the basal cistern or progressive obliterative endarteritis that supplying the hypothalamo-hypophyseal system is well-defined in hypothalamopituitary dysfunction in neurotuberculosis. We recently experienced a diabetic patient with short stature and sexual infantilism who shows polyuria and polydipsia. Detailed endocrinological evaluation showed partial hypopituitarism and central diabetes incipidus secondary to tuberculous meningitis. Polyuria and polydipsia was improved with dDAVP and height increased 5 cm for 11 month with HGH, libido increased with oxadrolone but his extemal sexual characteristics was not changed until now. We present this case with a review of literature.