Context:

Published data demonstrates that hypopituitarism is common following traumatic brain injury (TBI). Hormone deficiencies are transient in many but the natural history of the acute changes following TBI has not been documented. In addition, it is not clear whether there are any early parameters which accurately predict the development of permanent hypopituitarism.

Objectives:

1. To describe the natural history of plasma cortisol (PC) changes and sodium balance following TBI2. To identify whether acute hypocortisolaemia or cranial diabetes insipidus (CDI) predict mortality3. To identify whether acute pituitary dysfunction predicts the development of chronic anterior hypopituitarism

Design:

Each TBI patient underwent sequential measurement of PC, plasma sodium, urine osmolality and fluid balance following TBI. All other anterior pituitary hormones were measured on day 10 following TBI. The results from 15 surgical comparisons defined a PC < 300nmol/L as inappropriately low for an acutely ill patient. CDI was diagnosed according to the Seckl and Dunger criteria (1). Surviving TBI patients underwent dynamic anterior pituitary testing at least 6 months following TBI.

Setting:

The patients were recruited from the Irish National Neurosurgery Centre.

Patients:

100 sequential TBI patients were recruited. 15 patients admitted to ITU following major surgery were recruited as comparisons.Main Outcome Measures:PC in TBI patients was compared with that of comparisons. Mortality rate was compared between TBI patients with and without acute hypocortisolaemia. Results of follow-up dynamic pituitary testing were compared between those with and without acute hypocortisolaemia.

Results:

78% of TBI patients developed inappropriately low PC following TBI. Low PC and CDI were predictive of mortality. 39% of patients who had follow-up testing had at least one pituitary hormone deficit, all of whom had had previous acute hypocortisolaemia or CDI.

Conclusions:

Acute hypocortisolaemia and CDI are predictive of mortality and long term pituitary deficits in TBI.

Granulomatosis with polyangiitis (GPA) is a small vessel vasculitis that can affect several organs, most commonly the respiratory tract and kidneys. Pituitary involvement is exceptionally rare. Most case reports of GPA of the pituitary gland have been described in middle-aged women who have concomitant ears, nose and throat involvement. The most frequent manifestation is diabetes insipidus due to a preponderance of posterior pituitary infiltration. The majority of cases sustain permanent damage to the pituitary gland even with remission of the underlying granulomatous disease. Here, the authors describe a case of pituitary GPA involving both the anterior and posterior pituitary glands with permanent residual pituitary insufficiency.

Autoimmune hypophysitis (AH) is commonly believed to be a rare chronic inflammatory condition of the pituitary gland. In clinical practice, however, the disease is often seen indeed. It typically presents with hypopituitarism and pituitary mass found by MRI. We report here unusual presentations of two females with AH followed by empty sella syndrome. The two females, aged at 64 and 57-years-old, presented with anterior pituitary dysfunction, diplopia and diabetes insipidus. By MRI the two patients shared the common characteristics with diffuse homogenous contrast enhancement of the gland and increased stalk thickness. After a long period treatment with glucocorticoids, empty sella was eventually detected by MRI.

Most of the clinical data on the safety profile of desmopressin (DDAVP), which is an effective treatment for both polyuric conditions and bleeding disorders, originate from studies on the tailoring of drug treatment, whereas few reports exist describing severe side effects secondary to drug-drug interaction. We herein describe a case of severe hyponatremia complicated by seizure and coma due to the intake of non-steroidal anti-inflammatory drugs (NSAIDs) in a patient on DDAVP replacement therapy for central diabetes insipidus (DI). A 50-yr-old Caucasian man, with congenital central DI, developed an episode of generalized tonic-clonic seizure, resulting in coma immediately after being admitted to the Emergency Unit for weakness and emesis. Based on his medical history and clinical findings, water intoxication secondary to ketoprofen intake (200 mg/day for the last 3 days) concomitant with DDAVP replacement therapy (Minirin® 60 mcg 4 tablets a day) was hypothesized as being the cause of the severe euvolemic hypotonic hyponatremia (natremia 113 mEq/l, plasma osmolality 238 mOsm/Kg). After standard emergency procedures, appropriate gradual restoration of serum sodium levels to the normal range was achieved in 72 hours. Hydratation was maintained according to water excretion and desmopressin therapy was re-introduced. We discuss this case report in the context of the published literature. The present report first highlights the potentially life-threatening side effects associated with over-the-counter NSAIDs during DDAVP replacement therapy for central DI. Risks and benefits of co-treatment should be carefully considered and therapeutic alternatives to NSAIDs should be recommended to patients with central DI in order to improve DDAVP safety.

Objective To summarize our experience in the management of adipsic central diabetes insipidus(ADI) accompanied with intracranial calcification. Method The clinical data of one ADI patient accompanied with intracranial calcification who was treated in our hospital since December 2011 were retrospectively summarized.Results The 24-hour urine volume was 800 ml. She didn't feel thirsty even with increased plasma sodium concentration(153 mmol/L) and blood osmotic pressure(333 mmol/L) . Combined water deprivation and vasopressin test revealed the diagnosis of central diabetes insipidus. The high intensity signal(on T1-weighted magnatic resonance imaging) in the posterior lobe of pituitary gland was found. Computed tomography showed calcifications in the bilateral basal ganglia.Serum cytomegalovirus IgG was positive. She was treated with desmopressin and asked for regular water intake regardless of the adipsia. The plasma sodium concentration was still below 150 mmol/L during the 4-month follow-up. Conclusions Routine adipsia evaluation and combined water deprivation and vasopressin test are critical for the diagnosis and treatment of ADI. Past insidious intracranial cytomegalovirus infection may explain the cause of ADI and calcification.

Context:

Nephrogenic diabetes insipidus (NDI) is caused by partial or complete renal resistance to the effects of antidiuretic hormone. Acquired NDI can be caused by electrolyte imbalances (e.g., hypercalcemia), renal/extra-renal diseases (e.g., chronic pyelonephritis) and drugs (e.g., lithium toxicity). Syphilis has never been reported to cause NDI.

Objective:

To report the case of a 56-year-old man with NDI secondary to syphilis.Case:The 56-year-old patient presented with polyuria and polydipsia since more than 40 days. His urine specific gravity was 1.002. He had no history of chronic kidney disease or contact with toxicants. He had normal blood glucose levels. A water-deprivation test and vasopressin administration indicated NDI. His rapid plasma reagin (RPR) titer was 1:128. The serum Treponema pallidum-particle agglutination test was positive. He reported engaging in unprotected, extramarital sex 6 months before polydipsia onset, and thereafter, developing a skin lesion on the external genitalia and arthralgia, both of which resolved spontaneously. Examination of renal biopsy specimens showed abundant plasmocytic and lymphocytic infiltration of the interstitium and low and flat tubular epithelial cells, indicating renal tubular injury. Silver staining revealed T. pallidum-like organisms. Immunohistochemical analysis with T. pallidum-specific antibody confirmed the presence of treponemes.Intervention:He received 2.4 million units benzathine penicillin i.m., once a week for 3 weeks.

Results:

His urine output gradually reduced; he recovered 1 month later. His urine specific gravity was 1.026, and syphilis RPR titer was 1:8.

Conclusion:

Syphilis can cause NDI. The manifestations of syphilis and causes of acquired NDI are diverse.

A 10-year-old boy with acute onset cranial diabetes insipidus and multiple autoimmune disorders had evolving panhypopituitarism, thought to be due to autoimmune hypophysitis. Over 18 months, a dramatic clinical course with progressive hypopituitarism and development of type 1 diabetes mellitus was evident. Serial brain imaging showed changes suggestive of germinoma.

BACKGROUND:

Wolfram syndrome is a rare hereditary or sporadic neurodegenerative disorder also known as DIDMOAD. The classically described presentation is of insulin-dependent diabetes, followed by optic atrophy, central diabetes insipidus, and sensory neural deafness. Also included are less well-described presentations of Wolframs syndrome. We here present three cases of atypical presentation of this syndrome. CASE 1: A 15-year-old boy with insulin-dependent diabetes was presented for evaluation of depressive symptoms associated with suicidal tendency. Neuropsychiatric manifestations are described with Wolframs syndrome, and wolframin gene, in recessive inheritance, is associated with psychiatric illnesses without other manifestations of Wolframs syndrome. CASE 2: A 17-year-old diabetic boy on insulin with good control of blood sugar presented for evaluation of delayed puberty. Central hypogonadism and other anterior pituitary hormone dysfunctions are the less publicized hormone dysfunctions in Wolframs syndrome. CASE 3: A 23-year-old female who was on insulin for diabetes for the past 14 years, got admitted for evaluation of sudden loss of vision. This patient had developed a vitreous hemorrhage and, on evaluation, was found to have optic atrophy, sensory neural hearing loss, and diabetes insipidus, and presented differently from the gradual loss of vision described in Wolframs syndrome.

CONCLUSION:

Wolframs syndrome being a multisystem degenerative disorder can have myriad other manifestations than the classically described features. Neuropsychiatric manifestations, depression with suicidal risk, central hypogonadism, and secondary adrenal insufficiency are among the less well-described manifestations of this syndrome.

Pituitary hypophysitis (PH) is characterized by pituitary infiltration of lymphocytes, macrophages, and plasma cells that could lead to loss of pituitary function. Hypophysitis may be autoimmune or secondary to systemic diseases or infections. Based on the histopathological findings PH is classified into lymphocytic, granulomatous, xanthomatous, mixed forms (lymphogranulomatous, xanthogranulomatous), necrotizing and Immunoglobulin- G4 (IgG4) plasmacytic types.To report a case of lymphocytic hypophysitis (LH).A 15-year-old girl presented with history of headache, amenorrhea, and history of polyuria for past 4 months. Initial evaluation had suppressed follicular stimulating hormone (<0.01 mIU/ml), high prolactin levels (110.85 ng/ml) and diabetes insipidus (DI). Magnetic resonance imaging of sella was suggestive of pituitary macroadenoma with partial compression over optic chiasma. Patient underwent surgical decompression. Yellowish firm tissue was evacuated and xanthochromic fluid was aspirated. Histopathology was suggestive of LH. She resumed her cycles postoperatively after 4 months, prolactin levels normalized, however, she continues to have DI and is on desmopressin spray. This case has been presented here for its rare presentation in an adolescent girl because it is mostly seen in young females and postpartum period and its unique presentation as an expanding pituitary mass with optic chiasma compression.Definitive diagnosis of LH is based on histopathological evaluation. Therapeutic approach should be based on the grade of suspicion and clinical manifestations of LH.

: Langerhans cell histiocytosis is part of a larger group of syndromes described as histiocytoses. The disease may involve single or multiple systems including skin and nervous system. Here we report an adult case where Langerhans cell histiocytosis presented with diabetes insipidus and cutaneous ulcers.