Friday, February 26, 2010

Addison's disease presenting with idiopathic intracranial hypertension in a 24-year-old woman: a case report

Dushyant Sharma email, Rohini Mukherjee email, Peter Moore email and Daniel J Cuthbertson email

Journal of Medical Case Reports 2010, 4:60doi:10.1186/1752-1947-4-60

19 February 2010

Abstract (provisional)

Idiopathic intracranial hypertension can rarely be associated with an underlying endocrine disorder such as Cushing's syndrome, hyperthyroidism, or with administration of thyroxine or growth hormone. Though cases of idiopathic intracranial hypertension associated with Addison's disease in children have been reported, there is only one documented case report of this association in adults. We describe a case of an acute adrenal insufficiency precipitated by idiopathic intracranial hypertension in a Caucasian female.

Case presentation

A 24-year-old Caucasian woman was acutely unwell with a background of several months of generalised fatigue and intermittent headaches. She had unremarkable neurological and systemic examination with a normal computerised tomography and magnetic resonance imaging of the brain. Normal cerebrospinal fluid but increased opening pressure at lumbar puncture suggested intracranial hypertension. A flat short synacthen test and raised level of adrenocorticotrophic hormone were consistent with primary adrenal failure.


Addison's disease can remain unrecognised until precipitated by acute stress. This case suggests that idiopathic intracranial hypertension can rarely be associated with Addison's disease and present as an acute illness. Idiopathic intracranial hypertension is possibly related to an increase in the levels of arginine vasopressin peptide in serum and cerebrospinal fluid secondary to a glucocorticoid deficient state.

The complete article is available as a provisional PDF. The fully formatted PDF and HTML versions are in production.

Tuesday, February 23, 2010

Removing Tumors Through the Nose

Wednesday, February 17, 2010 3:58 PM EST


A brain tumor caused one woman's body to grow uncontrollably.

"It took me from being one person to being a completely different person," Erin Kelley (Cushing’s Help message board member)  said.

Each year, 10 to 15-million Americans are diagnosed with Cushing's Syndrome. It occurs when the body is exposed to high levels of the hormone cortisol, often caused by a benign tumor deep inside the brain.

Now, doctors have been saying that a new kind of tool can make removing these tumors an easier process on the patient.

A year ago, Kelley felt like her body was out of control.

"I gained 120 pounds, went from being an extremely athletic person to not being able to do much at all without getting really sick," Kelley said.

Kelley was diagnosed with Cushing's Syndrome. A tumor on her pituitary gland was throwing off her body's hormonal balance.

Traditionally, removing pituitary tumors means making an incision underneath the lip and going through the nasal cavity to reach the tumor.

Dr. Richard Chole, otolaryngologist at the Washington University School of Medicine in St. Louis, Missouri, invented a new instrument that allows him to do the same surgery with a less-invasive approach through the nose.

"It allows us to do it safely through the nose without any incisions," Chole said.

The blades of his tool expand the sinuses, exposing the pituitary tumor and clearing a path for it to be pulled out through the nose. The device eliminates the need for incisions, avoids any possible nerve damage in the mouth, and there's no swelling or eating issues afterwards.

"This way is more direct. The exposure is just different, and it's proving to be a very successful way of doing it," Chole said.

"I am 900 percent better than I was before," Kelley said.

Less than six months after surgery, Kelley's feeling more like herself every day and has dropped nearly half of her weight. Now that her tumor's history, she's taking her life back.

People who are obese and have type two diabetes and high blood pressure have an increased risk of developing Cushing's Syndrome.

Doctors said the new technology may be useful for other kinds of brain tumors as well.

Medical Breakthroughs Research Summary

Topic:       Removing Tumors Through The Nose
Report:      MB #3104

Cushing's Disease: Cushing's Syndrome is a hormonal disorder caused by prolonged exposure to high levels of cortisol. The condition most often affects adults aged 20 to 50, and people who are obese and have type 2 diabetes along with poorly controlled glucose and high blood pressure are at higher risk of developing the condition.

According to the National Institutes of Health, many people develop Cushing's Syndrome because they take glucocorticosteroids like prednisone.

However, pituitary adenomas or tumors that arise in the brain's pituitary gland account for 70 percent of Cushing's cases besides those linked to use of glucocorticosteroids. The benign tumors cause the pituitary gland to secrete extra ACTH, a hormone that stimulates the adrenal glands to release cortisol.

The adenomas are five times more common in women than in men.

Treatment of Cushing's Syndrome depends on the cause and may involve surgery, radiation, chemotherapy or the use of cortisol-inhibiting drugs.

Surgery: According to the National Institutes of Health, the cure rate from surgery to remove tumors associated with Cushing's disease is more than 80 percent when performed by a surgeon with extensive experience; and if the first surgery fails, it can be repeated, often with good results.

One type of surgery to remove pituitary tumors involves making an incision underneath the lip to access the sinuses.

"The incision is uncomfortable," Chole said. "It takes time to heal. Some of the nerves are affected by it, and that takes some time to recover."

The surgery also carries risk of damage to tooth roots.

Another approach is endoscopic surgery through the nose. Chole uses a specially designed tool to remove the tumors. The instrument is transferred through an endoscope carrier, and two blades are expanded once inside the operating area.

"The instrument spreads in a certain way to expose the area of the pituitary gland," Chole explained.

"The complexity of the instrument is that it will open and adjust in several different ways, depending on exactly what exposure is needed and the exact anatomy inside that particular person."

Chole also in addition to eliminating the need for incisions, this surgical approach avoids the problem of nerve damage in the mouth and reduces postsurgical complications like swelling and trouble eating.

For More Information, Please Contact:
Judy Martin
Public Affairs
Washington University School Of Medicine, St. Louis



Wednesday, February 17, 2010

Influence of hydrocortisone dosage scheme on health-related quality of life in patients with adrenal insufficiency

Authors: Bleicken, Benjamin; Hahner, Stefanie1; Loeffler, Melanie1; Ventz, Manfred2; Decker, Oliver3; Allolio, Bruno1; Quinkler, Marcus2

Source: Clinical Endocrinology, Volume 72, Number 3, March 2010 , pp. 297-304(8)

Publisher: Blackwell Publishing



Summary Context

Recent studies suggest that current glucocorticoid replacement therapies fail to completely restore well-being in patients with adrenal insufficiency (AI).



The objective of this study was to investigate health-related quality of life (QoL) in patients with AI depending on dose and frequency of daily intake of hydrocortisone (HC).


Design and patients

In a cross-sectional study, primary and secondary AI patients were contacted and asked to complete three validated self-assessment questionnaires [Short Form-36 (SF-36), Giessen Complaint List (GBB-24), Hospital Anxiety and Depression Scale (HADS)]. HC doses were corrected for body surface area. Results were compared with sex- and age-matched controls drawn from the questionnaire-specific reference cohort.



Completed questionnaire sets were available from 334 patients on HC (primary AI n = 194; secondary AI n = 140). Patients on higher doses of HC (>30 mg/day) showed significantly impaired subjective health status in two of eight SF-36 dimensions, and three of five GBB-24 scales compared with those on lower HC doses. No significant differences in QoL were found between lower HC doses (15-30 mg/day) or between primary or secondary AI. Patients on HC with thrice daily intake showed significantly impaired QoL in one of eight SF-36 dimensions (15-20 mg/day, 20-25 mg/day), in one of five GBB-24 scales (15-20 mg/day), as well as higher anxiety scores.



Health-related QoL was impaired in patients with primary and secondary AI. HC doses above 30 mg/day were associated with a worse health status. Thrice daily intake of HC was not superior to twice daily intake. Our data support the perception that current replacement strategies are still insufficient to fully restore well-being and daily performance.


Document Type: Research article

DOI: 10.1111/j.1365-2265.2009.03596.x

Affiliations: 1: Endocrinology & Diabetes Unit, Department of Medicine I, University Hospital, University of Wuerzburg, Wuerzburg, Germany 2: Clinical Endocrinology, Charité Campus Mitte, Charité University Medicine Berlin, Berlin, Germany 3: Department of Psychotherapy and Psychosomatic Medicine, University Hospital Leipzig, Leipzig, Germany



Thursday, February 11, 2010

A Cushing’s Patient: Fine is waived, but why issue a ticket at all?

Annie Buckley: ‘There should be compensation for the time wasted and anxiety the

Cushing’s Syndrome sufferer calls for ‘discretion and common sense’ for disabled motorists

February 11, 2010

ANNIE Buckley gets a lot of parking tickets, despite her “Blue Badge” free parking pass.

But should disabled drivers whose mental health can sometimes be affected by their condition always be made to pay up?

Ms Buckley suffers from a rare hormonal condition known as Cushing’s Syndrome.

Patients suffer from bouts of exhaustion and are susceptible to psychological disturbances that can make it difficult, Ms Buckley says, for her to understand parking rules and signs.

Ms Buckley, who lives in Doynton Street, Highgate, is entitled to park on single yellow lines and in residents’ parking bays.

Two weeks ago she was fined for straddling a residents’ bay and a yellow line while parking in Quadrant Grove, Queen’s Crescent.

She contested the ticket, but Camden Councilhas said it was satisfied the £120 charge was correct and threatened her with a court hearing if she did not pay immediately.

Ms Buckley said: “I felt like they were attacking me. They should show some discretion and common sense.

“I have a Blue Badge. If I can park in both yellow lines and parking bays, why shouldn’t they let me park in both?

“I am fine driving with my condition. But it can mean that I do not process information properly all the time.

“If I’m reading a book, for example, I tend to miss out sentences, or not read some parts.

“There are so many signs on the street. When you get out and look at them, it’s like reading a book – and sometimes when I look at them I can’t easily line things up. It means I get a lot of tickets.

“They say you can’t [park] at this time and that time. I get exhausted very quickly and I can’t wait around for hours to find a space.”

She added: “All my time is being eaten up contesting parking tickets – I have had four tickets this month.

“I think there should be some sort of compensation for the time wasted and the anxiety these fines cause.”

Following a complaint from the New Journal, the council has admitted it was wrong and agreed to waive the fine.

Ms Buckley said she would get back to work on her course. She is studying theatre design at university and has recently returned from Athens where she saw the famous Theatre of Dionysus.

She used to work as a tour guide on open top buses driving round famous sites in central London, but had to stop work after becoming unusually tired.

For around 10 years doctors had no idea what was wrong with her – until she was diagnosed 15 years ago.

She said: “I thought I was being a hypochondriac for a long time. But Cushing’s is quite rare.

“I can have quite horrible moments, but I don’t want to complain.”


Wednesday, February 10, 2010

If only it were this easy…

An optometrist writes at

I was concerned that the patient might have a problem in her brain. And after diving a little deeper into her symptoms and medical history, I suspected pituitary adenoma. My suspicions proved correct...she was quickly treated for a pituitary adenoma, or brain tumor.

Wow, this guy must be good. So many of us test for years and years, going from doctor to another, spending countless thousands of dollars, when all we needed was to get our eyes checked.

I’d like to read more about these types of situations than the ones that I do see each and every day.

Sunday, February 7, 2010

Environmental Issues and Cushing’s

We’ve had quite a bit of discussion on this topic on the Cushing’s Help message boards lately.  A few samples:

We live in a part of Ontario known as "the Chemical Valley". We are surrounded by Dow Chemical, Imperial Oil, Dupont, British Petroleum, Shell Oil and about 12 other chemical plants.

There has been many people complaining about the high rate of cancer in our area and the government was forced to do a health study in our area but as of yet they haven't figured out how to do the testing. My guess is they don't want us to know how sick we really are.

We are part of the Goiter Belt which I think extends to PA. There are very few people here who do not have thyroid problems.

My 2 brothers and 2 sisters are suffering the same as I am and so are all our children! Both my parents died in their 50's from untreated hypothyroid disease. Probably had adrenal/pituitary damage too when I think about their symptoms.

I see hypothyroid people everywhere I look and have since started checking for the hump and cushing signs.

Holy endocrine system Batman, I think we are all suffering at the hands of the Big Oil Companies. My husband works for British Petroleum!!!!

I hate to even think about it. Growing up in Buffalo - erie county new york, which is nestled between lake ontario & lake erie, I don't believe the water is safe to drink. There are several epa areas of concern around lake ontario & lake erie. AOC's (areas of concern) are highly polluted areas. Specificlly erie canal & buffalo river are awful. I found out some years ago that a playground that I frequented as a child was a landfill for hazardous chemicals. Now I have a pituitary tumor, coincidence? Probably not

I live near Green Bay WI, which is part of Lake Michigan. I believe our drinking water comes from the Bay. The water is polluted from the papermills (PCPs). I also did play on a heavily fertilized and treated lawn from a chemical company for at least 5 years when I was little. I had a thyroid nodule removed, hypothyroidism, and I am still in the testing phase to see if I have a pituitary tumor. My father also has hopothyroid, and seems to have kind of a hump. He has had cancer as well.

I remember the nuclear accident in the 80's. It was really scary. I remember them saying something like it was worse than what they reported.

This is one of my future quests, I live in a town on 10,000 people and there are many cases of brain and pituitary tumors, I hear it all the time, I know of at least 3 definite pituitary cushing's cases in my small town. My future goal when I am feeling better is to put my story in the paper, have people call me if they or someone they know has a funtioning pituitary tumor, also brain tumors and brain cancer has some large numbers too. The state sent me a letter I had to fill out when I first found out about my tumor, it was manditory, if I did not fill it out they where going to have my doctor fill it out so I did. So somewhere someone is keeping track of brain tumors in my town. I want to find out the numbers, if it is as bad as I think it is I am going to calll CDC to find out why. I also want to start a support group. But I need to feel better first because this is going to be a big undertaking.

There are many more postings on this topic.

From Wennersten: There’s something in the water

Scientists now tell us there is something in our waters that we least expected.

That “something” is a class of chemicals called endocrine disruptors, and Dr. Vicki Blazer, a fisheries biologist at the United States Geological Survey, thinks the chemicals are responsible for the high concentrations of intersex fish found in the Potomac, and other rivers in the mid-Atlantic.

The chemicals also prove a threat to human health, but a bit of explanation, first.

Our body’s endocrine system is a complex network of glands and hormones that regulate growth, development, and the operation of various organs. The endocrine glands (for example the thyroid, adrenal, pancreas, testes, ovaries and pituitary glands) release hormones that act as chemical messengers and regulate many life functions.

Endocrine disrupters are chemicals that interfere with this system, by either acting like a hormone, or blocking a hormone’s function. They can be natural, but many are man-made such as PCBs, dioxin, DDT and other pesticides, pharmaceuticals and plasticizers. They are found in many products, including plastic bottles, metal food cans, detergents, flame retardants, food, toys, cosmetics and pesticides. They enter the environment and are now commonly found in our streams, rivers, bays and oceans, where scientists are observing problems.

Then Great Lakes Area of Concerns shows a map of problem areas

Forty-three AOCs have been identified: 26 located entirely within the United States; 12 located wholly within Canada; and five that are shared by both countries. Two Canadian AOCs have been delisted and one U.S. AOC has been delisted leaving 30 AOCs remaining on the U.S. side of the border.

RAPs are being developed for each of these AOCs to address impairments to any one of 14 beneficial uses (e.g., restrictions on fish and wildlife consumption, dredging activities, or drinking water consumption) associated with these areas.  USEPA has assigned RAP Liaisons for AOCs.  Sediments have been identified as serious problems in many AOCs. AOC Principles and Guidelines have been finalized for formally delisting these areas as beneficial uses are restored.

What do YOU think?  Are you in one of these areas? Check out this post on the Cushing’s Help message boards  and be heard!

Thursday, February 4, 2010

Use of the Desmopressin Test in the Differential Diagnosis of Pseudo-Cushing State from Cushing's Disease

Giacomo Tirabassi, Emanuela Faloia*, Roberta Papa, Giorgio Furlani, Marco Boscaro,  and Giorgio Arnaldi

Division of Endocrinology (G.T., E.F., G.F., M.B., G.A.), Polytechnic University of Marche, 60020 Ancona, Italy; and Scientific-Technological Area, INRCA (Italian National Institute on Aging) (R.P.), 60124 Ancona, Italy

* To whom correspondence should be addressed. E-mail:

Context: The desmopressin (DDAVP) test has been proposed to discriminate Cushing's disease (CD) from pseudo-Cushing states (PC); however, current information on its value is scarce and contradictory.

Objective: The aim of the study was to assess the ability of the DDAVP test in distinguishing between these conditions, with emphasis on subjects with mild hypercortisolism.

Design and Setting: We conducted a retrospective/prospective study at the Division of Endocrinology, Polytechnic University of Marche, Ancona, Italy.

Patients: The study included 52 subjects with CD, 28 with PC, and 31 control subjects (CT).

Intervention(s): We performed the DDAVP test and standard diagnostic procedures for the diagnosis of Cushing's syndrome.

Main Outcome Measure(s): The diagnosis/exclusion of CD was measured.

Results: Interpretation of the DDAVP test based on percentage and absolute increment of cortisol and ACTH did not afford acceptable values of both sensitivity (SE) and specificity (SP). CD diagnosis based on simultaneous positivity for basal serum cortisol greater than 331 nmol/liter and absolute ACTH increment greater than 4 pmol/liter and its exclusion in subjects negative for one or both measures yielded an SE of 90.3% and an SP of 91.5%. The approach was also highly effective in distinguishing PC from: 1) CD with moderate values of urinary free cortisol (SE, 86.9%; SP, 92.8%); 2) CD with moderate values of serum cortisol after dexamethasone suppression (SE, 86.6%; SP, 92.8%); and 3) CD with moderate values of midnight serum cortisol (SE, 100%; SP, 92.8%).

Conclusions: Interpretation of the DDAVP test through a combination of parameters allowed effective discrimination of CD from PC, even in subjects with mild hypercortisolism.


Wednesday, February 3, 2010

Delayed Remission after Transsphenoidal Surgery in Patients with Cushing's Disease

Elena Valassi, Beverly M. K. Biller, Brooke Swearingen, Francesca Pecori Giraldi, Marco Losa, Pietro Mortini, Douglas Hayden, Francesco Cavagnini,  and Anne Klibanski*

Neuroendocrine Unit (E.V., B.M.K.B., A.K.) and Department of Neurosurgery (B.S.), Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts 02114; Chair of Endocrinology (E.V., F.P.G., F.C.), Universita' di Milano, Ospedale San Luca, Istituto Auxologico Italiano, Istituto di Ricovero e Cura a Carattere Scientifico, 20149 Milan, Italy; Department of Neurosurgery (M.L., P.M.), Universita' Vita-Salute, Istituto Scientifico San Raffaele, 20132 Milan, Italy; and Biostatistics Center (D.H.), Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts 02114

* To whom correspondence should be addressed. E-mail:

Background: Transsphenoidal surgery (TSS) is the treatment of choice for Cushing's disease (CD). Postoperative hypercortisolemia mandates further therapy.

Objective: The aim of the study was to characterize patients without immediate postoperative remission who have a delayed decrease to normal or low cortisol levels without further therapy.

Design and Setting: A retrospective case series was conducted at three tertiary care centers.

Patients and Intervention: We reviewed the records of 620 patients (512 females, 108 males; mean age, 38 ± 13 yr) who underwent transsphenoidal pituitary surgery for CD between 1982 and 2007.

Results: Outcomes were classified into the following three groups based upon the postoperative pattern of cortisol testing: group IC (immediate control) included 437 of the 620 patients (70.5%) with hypocortisolism and/or cortisol normalization throughout the postoperative follow-up; group NC (no control) included 148 of 620 patients (23.9%) with persistent hypercortisolism; and group DC (delayed control) included 35 of 620 patients (5.6%) who had early elevated or normal UFC levels and developed a delayed and persistent cortisol decrease after an average of 38 ± 50 postoperative days. The total rate of recurrence was 13% at a median follow-up time of 66 months after TSS; the cumulative rate of recurrence at 4.5 yr was significantly higher in group DC vs. group IC (43 vs. 14%; P = 0.02).

Conclusions: Hormonal assessment in the immediate postoperative period after TSS for CD may be misleading because delayed remission can occur in a subset of patients. Expectant management and retesting may spare some patients from unnecessary further treatment. Optimal timing to determine the need for further therapy after TSS remains to be determined.


Would you be interested in a Cushing's iPhone/iPod app?

I've made a mockup you can play with at

It has the podcasts, upcoming events, latest bios, newest doctors, Cushings Tweets, blog and news/abstracts.

It's all made - I just have to pay the site to make this live.

I don't know if it would be worth it, though - it would be free to users but cost $199 to upload.

What do you think?

Monday, February 1, 2010

Cushing's syndrome due to ACTH-independent macronodular adrenal hyperplasia

Author: Andre Lacroix, MD
Section Editor: Lynnette K Nieman, MD
Deputy Editor: Kathryn A Martin, MD


Among the causes of Cushing's syndrome are three rare types of nodular adrenocortical diseases that are usually bilateral:

  • Corticotropin (ACTH)-dependent bilateral macronodular hyperplasia secondary to long-term adrenal stimulation in patients with Cushing's disease or ectopic ACTH syndrome
  • ACTH-independent micronodular adrenal hyperplasia and its most frequent variant primary pigmented nodular adrenocortical disease (PPNAD), which may be sporadic or familial (as part of the Carney complex)
  • ACTH-independent bilateral macronodular adrenal hyperplasia (AIMAH)

ACTH-independent bilateral macronodular adrenal hyperplasia will be reviewed here. Other causes of Cushing's syndrome, and primary pigmented nodular adrenocortical disease are discussed separately. (See "Causes and pathophysiology of Cushing's syndrome" and "Cushing's syndrome due to primary pigmented nodular adrenocortical disease".)


ACTH-independent macronodular adrenal hyperplasia (AIMAH) has been described by various terms, including massive macronodular adrenocortical disease (MMAD), autonomous macronodular adrenal hyperplasia (AMAH), ACTH-independent massive bilateral adrenal disease (AIMBAD), and "giant" or "huge" macronodular adrenal disease. AIMAH results in hypercortisolism through a number of mechanisms that are described below. (See 'Pathogenesis' below.)

AIMAH is associated with adrenal glands weighing from 24 to 500 g that contain multiple nonpigmented nodules greater than 5 mm in diameter [1-3]. The nodules appear to be typical benign adrenal nodules, but the internodular cortex may be either hyperplastic or atrophic.

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Cushing's syndrome in pregnancy

Author: Lynnette K Nieman, MD   
Section Editors: Andre Lacroix, MD; Charles J Lockwood, MD
Deputy Editor: Kathryn A Martin, MD



The hypercortisolemia in Cushing's syndrome is usually due to a corticotropin (ACTH)-producing pituitary tumor (Cushing's disease), ectopic ACTH secretion by a nonpituitary tumor, or cortisol secretion by an adrenal adenoma or carcinoma. There are also very rare tumors that secrete corticotropin-releasing hormone (CRH) ectopically, and occasional cases are caused by cortisol secretion by ACTH-independent macronodular or micronodular hyperplasia of the adrenal cortex. (See "Establishing the cause of Cushing's syndrome".)

This topic will review issues related to Cushing's syndrome in pregnancy. The clinical manifestations, diagnosis, and treatment of Cushing's syndrome in nonpregnant patients are discussed separately. (See "Clinical manifestations of Cushing's syndrome" and "Establishing the diagnosis of Cushing's syndrome".) and (see "Overview of the treatment of Cushing's syndrome".


Cushing's syndrome is associated with a high prevalence (up to 75 percent) of ovulatory disturbances induced by cortisol excess [1,2]. As a result, women with untreated Cushing's syndrome rarely become pregnant [2].

The frequency of ACTH-independent cases is increased in pregnant as compared to non-pregnant individuals. Of the approximately 136 reported cases, approximately 60 percent had ACTH-independent Cushing's syndrome (48 percent adenoma and 10 percent carcinoma); most of the rest had Cushing's disease or bilateral adrenal hyperplasia [1-4]. Four pregnant women with the ectopic ACTH syndrome have been reported [1,3,5,6].

The reason for this unusual pattern is not known. Pure cortisol-secreting adrenal adenomas may interfere with reproductive function less than the mixed cortisol and androgen excess produced by adrenal carcinomas or ACTH-dependent adrenal hyperplasia. In addition, most patients with the ectopic ACTH syndrome are men [3].

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