Etiological Spectrum with Diagnosis and Prognosis of Central Diabetes Insipidus needs Long Term Followup:A Single Centre Experience

Introduction:Central Diabetes insipidus(CDI)is a rare disorder caused by vasopressin deficiency characterized by the excretion of copious volumes of unconcentrated urine.Objective:To assess the etiological,clinical,biochemical and radiological spectrum of Central DI in our institute and long term follow up of these cases.Material and Methods:32 patients with Central DI admitted in Department of Endocrinology,Guwahati Medical College,Assam in the last 2.5 years were included.Detailed clinical assessment,biochemical evaluation and MRI(Magnetic Resonance imaging)brain were done in all the patients.Central DI without any identifiable cause was considered Idiopathic and those with structural lesion in hypothalamic pituitary region were considered organic.Result:Idiopathic CDI was present in 12(37.5%)patients and 20(62.5%)patients had organic CDI with acute onset of presentation.12(60%)patients with organic CDI present with neurological symptoms but 8(40%)patients had no neurological symptoms even with organic cause.Pituitary dysfunction was common in organic CDI as compared to idiopathic CDI.Paediatric patients commonly present with organic cause for CDI with low cortisol most common hormonal deficit.One patient of idiopathic CDI with normal stalk thickness at baseline presented with clinical and radiological features of LCH(Langerhans cell histiocytosis)on follow up.Conclusion:Organic CDI more likely to have acute onset of presentation than idiopathic CDI and even in absence of neurological features.Paediatric patients commonly have organic cause for CDI.We propose the paramount importance of long-term clinical follow-up and reassessment of endocrine function in patients with CDI for definitive diagnosis of autoimmune and inflammatory causes of idiopathic CDI and timely treatment of pituitary hypofunction.

Caution on diagnosis of idiopathetic central diabetes insipidus

Idiopathetic central diabetes insipidus （CDI） is a heterogeneous hypothalamus-pituitary disease due to the absence or deficiency of arginine vasopressin （AVP）.

Diabetes insipidus with impaired vision caused by germinoma and perioptic meningeal seeding:A case report

BACKGROUND Germinoma is a type of germ cell tumor that most frequently arises in the midline axis of the brain.Impaired vision is a clinical manifestation of germinnoma.Although rare,intracranial germinoma seeding to the perioptic arachnoid space is one cause of visual acuity decrease.CASE SUMMARY An 11yearold girl who presented with polyuria and polydipsia and subsequently developed diminution of vision.Imaging showed bilateral heterogeneous enhancement of the optic nerve sheaths and atrophy of the optic nerve,and transsphenoidal biopsy revealed a germinoma.The patient experienced poor visual recovery following chemotherapy and radiotherapy.Germinomas are rare and they are mostly identified in children and adolescents.The manifestations include diabetes insipidus,pituitary dysfunction,visual complaints,etc.The mechanisms that lead to visual loss include intracranial hypertension,compression of optic chiasma,and tumor invasion.A literature review was performed to summarize the cases with a tumor infiltrating the optic nerve.Most of the reported patients were adolescents and presented with anterior pituitary hormone deficiency.Enhancement of optic nerve sheaths and optic disc pallor could be identified in most of the cases.The purpose of this report is to provide awareness that in cases where a germinoma is associated with visual loss,though rare,perioptic meningeal seeding should be taken into consideration.CONCLUSION The case report suggests that children with diabetes insipidus need a complete differential diagnosis.

Clinical features of patients with pituitary stalk thickening: a review of 159 cases from one medical center

Background:Pituitary stalk thickening (PST) used as an imaging diagnosis plays a major role in diagnosis and treatment of the hypothalamus and pituitary disease at an early stage.Up until now,several lines of systematic investigations have been conducted among adolescents,and the results indicated that PST is possibly one of the principal early manifestations of sellar region tumor.To characterize the major clinical features of PST and to identify the most effective treatments we conducted this study.Methods:This was a retrospective review of patients with PST evaluated at Huashan Hospital over an eight-year period between 2007 and 2014.One hundred fifty nine patients who were diagnosed PST were included.The data including demographics,clinical presentations,imaging examinations,laboratory results and surgery were thoroughly reviewed and carefully analyzed.According to their clinical presentations and examinations,relevant treatments were adopted and different prognosis were observed.Results:Of the 159 patients,57 were males and 102 were females (median age was 29 and 43.4％ were under the age of 21 years).And of the patients in the age group between 30 and 35 years,84％ were females.'Polydipsia and polyuria' was the most common clinical manifestation observed in 90 cases (＞50％).49.5％ of the patients were diagnosed to have at least one anterior pituitary dysfunction.The sellar region MRI (SR-MRI) data showed that patients who had different clinical presentations or pituitary function showed different SR-MRI appearance.Over 60％ patients who performed followup MRI showed shrunken PST.Fourteen cases who received radiotherapy appeared to be improved as shown by MRI.Conclusions:PST is most commonly seen in the teenagers and the women of reproductive age.Distinct etiology may be identified based on the clinical manifestations,age and SR-MRI,which would help to make the final decision for treatments.Radiotherapy and immuno-suppressed therapy are effective in alleviating clinical symptoms and shrinking the stalk thickening.