Chronic Fatigue Syndrome Gets Renamed

Imagine the way you felt the last time you had the flu. You were flattened, devoid of all energy. Staying upright to get dressed was more than you could handle. You slept – and slept – and slept – and still experienced none of the normal refreshment that a good night’s sleep provides. A fog descended on your mind and fuzzed up memory, destroyed drive and made your head ache. You could not concentrate on simple mental tasks like reading. Though you were doing nothing physical, your muscles ached. Then it all went away and you forgot about it.

But now imagine that it didn’t go away. The same misery persists and dramatically alters your life. You cannot work. You move from bed to couch and back to bed. You go to doctor after doctor and they find nothing wrong. Routine blood tests, X-ray and scan results are normal. Someone prescribes an antidepressant, confirming the suspicions of family, friends, and some doctors that your debilitating physical symptoms are “all in your head.” Eventually, you find your way to a doctor who makes a diagnosis. You have CFS which stands for chronic fatigue syndrome, and which, as of early 2015, has been renamed system exertion intolerance disease or, in our acronym-laden age, CFS/SEID.

A long history, with different names

CFS/SEID has probably been around for more than 200 years, making its appearance in the medical literature as “neurasthenia,” a term applied to patients who were lacking in physical, emotional and cognitive energy without any discernible disease to account for their malaise, without any improvement over time and without any progression that brought them to a worsened state. They were mostly ladies, whose frail constitutions prevented them from exerting themselves and who mysteriously took to their beds for weeks at a time.

The Yuppie flu

British doctors in the 1950s christened the symptom complex myalgic (painful muscles) encephalitis (inflammation of the brain), even though there was no evidence for inflammation to account for the headaches, difficulty concentrating and memory problems patients experienced. In the US in the 1980s, the syndrome was dubbed the Yuppie Flu because it seemed to follow viral infections like infectious mononucleosis and occurred in cities where young urban professionals (“yuppies”) congregated. When reported from other settings as well, the name was changed to chronic fatigue syndrome.

No apparent cause, but a real illness

Because no single infectious, hormonal or immunologic cause for CFS emerged from many attempts to identify its cause, because it was impossible to measure the subjective complaints constituting the syndrome, and because some improvements occurred when antidepressants were prescribed, CFS was, for decades, viewed as a psychological disorder. But this view became more and more untenable as it became clear that the illness hit people who had no history of depression or inability to cope with life. Many CFS patients continued to be very productive, learning how to manage their lives within the limitations of their fatigue and mental fog. Laura Hillenbrand, author of Seabiscuit and Unbroken is one outstanding example. Though no cause has yet been identified for the illness, the name change from chronic fatigue syndrome to systemic exertion intolerance disease signals that the illness is one rooted not in psychology but in an, as yet, unidentified physical cause.

Epidemiology and diagnostic criteria

It is estimated that there are about 1 million patients with CFS/ SEID in the US at any given time. There is no evidence that its incidence is increasing, but it is quite possible that some cases are hidden on among the legions of people who have been diagnosed only with depression. CFS/SEID is more common in women than in men. Sometimes it follows directly upon an acute flu-like illness, but at other times appears out of nowhere. The diagnostic criteria at this time include 6 months of unexplained, life-altering fatigue and orthostatic intolerance, which means the inability to stand for more than very short periods. Four of eight other symptoms are also required and these include disturbances in memory and concentration, persistent sore throat, tender lymph nodes, muscle pain, joint pain, headache, disturbed sleep patterns, and malaise following even minimal exertion. Additional symptoms may include increased sensitivity to tastes, odors, temperature and noise.

A relapsing illness

A small minority of CFS/SEID patients get completely better and never suffer a relapse. The majority suffer relapses for prolonged periods of time, perhaps the rest of their lives. Relapses are triggered by infections, surgery, temperature extremes and stressful events. Another minority are severely affected from the beginning of their illness and require support in the activities of daily living for the rest of their lives. Deterioration, though, is unusual and suggests the diagnosis of CFS is wrong and further attempts to find the correct diagnosis are indicated.

Problems in mitochondrial energy production?

While there is no identifiable single cause for CFS/SEID, poor energy production seems to be at the root of the many symptoms in this illness, which has focused some researchers’ attention on mitochondria – the powerhouses of all cells in the body. Mitochondria must continuously recycle the molecules they use to produce energy and there is some indication that this process is impaired in people with CFS/SEID. Perhaps this is why experience has taught many CFS/SEID patients to pace their lives, always allowing significant time for recovery from exertion.

Boosting energy production

In addition to pacing life to allow recovery time, lifestyle alterations that seem to help CFS/SEID patients minimize relapses also happen to be useful in maximizing mitochondrial function. These include avoidance of drugs and environmental toxins, avoidance of processed foods with high carbohydrate and sugar concentrations, addition of whole foods containing plenty of antioxidants and high quality protein, correction of hormonal problems, especially of the thyroid gland, and decreasing chronic inflammation associated with obesity and allergies. Gradual and graded programs of exercise, outdoors with some sun exposure help prevent the loss of muscle associated with inactivity and improve Vitamin D levels, with positive effects on immune function. Continued research will most likely show that CFS/SEID has many causes, all of which result in impaired mitochondrial function.

Lyme Disease: A Whodunit Tale

Some medical advances begin with old-fashioned detective work. Lyme disease, which was unknown in this country prior to 1975 is a good example.  That fall, two mothers from Old Lyme, Connecticut convinced the state Department of Public Health and Yale University to explore a mysterious outbreak of cases of inflammatory arthritis among the town’s children, because they were unsatisfied with the explanations they had been given for the cause. The investigation that winter centered on thirty-nine children and twelve adults from Old Lyme, all of whom had developed painful swelling of one or more joints between June and September.

Clues in clinical histories

Although blood tests and physical exams of the affected people had not previously revealed any known cause for the painful, swollen joints, investigators noted that there were striking similarities in the patients’ histories. Especially notable was a peculiar spreading rash that appeared about a month prior to the development of the arthritis and resembled an archer’s bull’s eye target. The affected people also lived close to one another, all in heavily wooded areas. The researchers concluded that the area where the cases clustered and the time of year in which they occurred were both crucial clues to the mystery. They believed that the illness could be an unknown type of infection but would have to await the next disease “season” for confirmation of this theory.

More clues in old European medical literature

In the meantime, investigators began combing through European medical literature, where they discovered similar descriptions of rashes going back to 1909. Over time, the Europeans had named the skin lesion erythema migrans and associated it with an illness that was similar to the one being reported in Connecticut, although without the arthritis. Some European reports mentioned tick bites in conjunction with the rashes, as well as successful treatment with antibiotics. Back in Connecticut, the next summer produced thirty more cases of what was by then being called “Lyme arthritis,” which investigators now believed was some kind of infection transmitted during outdoor activity.

Figuring out the tick relationship

The next pieces of evidence came from field studies of ticks. The distribution of a particular type of tick called Ixodes scapularis (variously known as the blacklegged tick, deer tick, or bear tick) near Old Lyme matched the distribution of local arthritis cases. Tick autopsies conducted in New York on Shelter Island, another hot spot for this mystery arthritis, showed that most of the ticks carried a spiral-shaped bacterium called Borrelia burgdorferi. Blood tests on affected individuals for antibodies to this organism tied it to the clinical cases of arthritis. Over the next two decades, the explosion of the deer population carrying the tick made the disease more common and widely known.As knowledge about and experience with the new disease accumulated, Lyme arthritis was renamed Lyme disease.

Early  Lyme disease symptoms

Lyme disease symptoms include an early stage of fatigue, muscle and joint pains, swollen glands, and headaches and fever that begin days to weeks after the infected tick bite. These symptoms represent the immune system’s response to the bacterial invasion. If a bull’s eye rash at the site of a former tick bite is present, diagnosis is easy. If not, diagnosis depends on a clear history of a tick bite and on the development of antibodies to the organism, which usually occurs later than the first few weeks of the illness.

Later symptoms

Left untreated, some, but not all infected patients develop symptoms within the next few weeks to months after the infected tick bite. Symptoms include arthritis, nerve pains, facial nerve paralysis, heart palpitations, shortness of breath, and chest pains. An even less common late phase that can occur up to two years after an infected tick bite might include migrating joint pains, muscle aches, abnormal muscle movements, weakness, heart arrhythmias, and cognitive complaints such as memory problems. These symptoms are not well understood and may represent a combination of the body’s ongoing fight against persistent bacteria and an autoimmune response that they trigger.

Treatment

Treatment of Lyme disease with oral antibiotics, either doxycycline or amoxicillin, is usually curative. If an infected tick is attached for more than thirty-six hours (the least amount of time it takes for transmission of the infection) and was encountered in an area where more than 20 percent of the deer tick population carries Borrelia burgdorferi, most patients are given a prophylactic one-time dose of doxycycline. Otherwise, treatment with antibiotics for two to four weeks begins as soon as the diagnosis of Lyme disease is made. The earlier the treatment, the faster the disease responds and symptoms subside. Late-phase treatment of neurological, cardiac, or arthritic symptoms may require intravenous delivery of antibiotics over longer periods. Although rare cases of persistent symptoms after treatment exist, no study has yet shown enough benefit from very long-term antibiotic use to justify the potential adverse effects of such a treatment.

Prevention of tick bites

Prevention of Lyme disease is the best way to deal with the illness, and there are things you can do to protect yourself. In the states where most cases occur (the New England states and New York, New Jersey, Maryland, Virginia, Wisconsin, and Minnesota), be aware that ticks tend to cling to high grasses and shrubbery in areas where deer roam. By hiking in the center of paths, away from tall grasses and shrubs, and wearing protective clothing, such as long sleeves and pants, you can reduce the chances of a tick bite. Shirt tails should be kept tucked in at the waist, sleeves should be kept closed at the wrists, and pants cuffs should be kept tucked into socks at the ankles. Additionally, spraying with insect repellent containing 20 to 30 percent DEET can help.

Self-examination is very important after potential tick exposure

The type of tick that transmits Lyme disease is Ixodes scapularis. It may be either a six-legged, immature tick nymph the size of a poppy seed or the slightly larger, eight-legged mature tick. Both forms excrete an anesthetic in their saliva that prevents you from feeling their bite, so close examination of your body is necessary after potential exposure. Bathe within two hours of coming inside and do a full body exam, with the aid of a mirror, paying close attention to areas covered with hair. Inspect all gear, clothing, and pets for ticks, and tumble clothing in a dryer at high heat to kill any hidden ticks.

Tick removal

Should you find an attached tick on your body, to remove it place the tip of a clean, fine-tipped tweezer as close to the skin as possible and pull gently, in a straight line. Dispose of all ticks in a toilet or drown them in alcohol and then seal them in a plastic bag for disposal. Clean bites with alcohol or iodine. Because the transmission of an infection from a tick to a human requires thirty-six to forty-eight hours of attachment, ridding yourself of ticks in the first twenty-four hours is effective prevention. Longer attachments that occur in high-risk parts of the country merit a single dose of doxycycline within seventy-two hours of a bite. Otherwise, be alert for symptoms or a rash, and seek treatment as soon as possible if they occur.  (See blow for a link to an interesting tick removal tool.*)

Research continues

The detective work surrounding the unraveling of the Lyme disease mystery continues today in the laboratory. Now researchers tend to focus on the rare people who, despite receiving adequate antibiotic treatment after contracting Lyme disease, experience persistent, unexplained, or recurring symptoms. These people remain almost as much of a mystery to researchers today as the initial thirty-nine children and twelve adult with arthritis were to researchers in the mid-1970s.

 

*Tick removal tool

https://www.thegrommet.com/tickease?utm_campaign=20180626&utm_content=49931&utm_medium=email&utm_source=CC&trk_msg=77TUPK4NDPL4R992MUGHP52NOS&trk_contact=4ACPOO38FT83AKKO084SUBGRPC&trk_sid=ICRD996NV2C3PQ9D216CFKVDLG

 

Sleep Debt: The Hidden Costs

Everyone has a sleep bank. Each night your accounts get credited with 7-8 hours of the physical and mental benefits of sleep and each day the accounts pay out those benefits in the form of emotional, intellectual and physical energy. Just like in any bank account, withdrawals can’t exceed deposits without incurring debt. Sleep debt, though, is easy to ignore because physical activity keeps alertness high. As long as you move around instead of reading or watching TV, you won’t nod off and you can keep thinking that 5 or 6 hours of sleep a night meets your needs. But covering the debt with activity is like keeping a bank balance out of the red by borrowing money and paying interest. Sleep debt exacts a toll on the body that goes beyond depressed mood, irritability and lack of ability to concentrate and learn, not to mention the potential for causing motor vehicle accidents.

The biological clock

As sleep debt mounts, the body’s biologic clock goes awry. This clock, located deep in the brain, controls circadian rhythms – regular ups and downs in behavior, body temperature, appetite, hormone production, alerting mechanisms, and the urge to sleep. When the clock malfunctions chronically, the results show up in the form of weight gain, high blood pressure, diabetes and diminished immunity to infection.

Setting the clock

Regular periods of darkness are required to set the brain’s internal clock to keep the body in synch with the 24-hour day set by the sun. Sleep researchers have shown that, when living in a research setting where there are no external clues about time of day or night, subjects’ internal clocks actually work on a 25-hour cycle. Normal peaks of sleepiness and alertness work themselves into the wrong time of the  24-hour day and night outside the sleep lab, producing weeks of daytime sleepiness and nighttime insomnia in the research subjects. Over time, the peaks cycle back into synchrony with day and night producing several weeks of normal daytime alertness and nighttime sleepiness.

Laboratory settings may exaggerate these patterns, but most people know that during some weeks they simply perform better during the day and sleep better at night  than during other weeks, indicating that in the modern, artificially lit world, the 24-hour day is more like a 24-25 hour day as far as the body’s natural rhythms are concerned. This clock drift is very sometimes very evident. Cyclical insomnia and daytime sleepiness are in common in blind people, in people at very high latitudes where the summer sun circles the sky for almost 24 hours, and in shift workers who are up all night in brightly lit environments. These problems, while distressing, respond to maintaining regular sleeping schedules and closing out all light during sleep periods, which resets the clock.

Why the clock matters

The internal clock is easily disrupted by one or two day episodes of sleep deprivation that people experience for reasons as varied as extra work loads, exams, brief periods of emotional upheaval, or any of the other myriad problems that keep people awake, but studies have repeatedly demonstrated that a few days of “catching up” on sleep restore the body to normal rhythms, contributing to a widely held impression that sleep deprivation, while responsible for serious accidents, doesn’t cause real health problems.
However, bigger problems do come from disturbing circadian rhythms more chronically. In recent years research attention has shifted from short term sleep deprivation to the chronic, partial sleep deprivation that is so common in our modern society, where nodding off during monotonous and sedentary activities like reading or watching TV are almost expected. Many people think they need no more than 5-7 hours of sleep at night, but while a few truly short sleepers exist, most people require around 8 hours of sleep each night to achieve maximal alertness throughout the day. Chronically shortchanging sleep by even an hour a day changes the timing and levels of multiple hormones, causing other metabolic changes and weakening the immune system.

Lack of sleep wreaks havoc on hormones

One of the first hormonal changes produced by chronic short sleep involves cortisol, the stress hormone produced by the adrenal gland. Normally cortisol levels decline during late evening hours, but without enough sleep, production continues unabated, Cortisol then begins to contribute to immune stress and to insulin resistance, which leads to diabetes and fat deposition. A second contributor to insulin resistance is a change in growth hormone secretion from one large burst during sleep to two, smaller bursts before and after sleep. A third change comes from failure of the pituitary gland to produce its normal night-time rise in thyroid stimulating hormone, the stimulus for the thyroid gland to produce more thyroid hormone. All of these changes are consistent with the fact that as little as one week of 4 hour sleeping nights can convert healthy young people to a pre-diabetic state. Observational studies do show higher rates of diabetes in chronically sleep-deprived women.

Lack of sleep and obesity

If these hormone changes are not enough to convince a short sleeper to turn out the lights earlier, studies on the appetite influencing hormones leptin and ghrelin, produced by fat tissue and the stomach respectively, might help. Leptin, which signals when to stop eating, diminishes markedly after 6 days of four- hour sleeping nights, despite no change in caloric intake. Ghrelin, which stimulates appetite, particularly for high carbohydrate foods, goes up when sleep is short.

Sleep debt is all around you

    All of these hormonal factors are significant in society where people lead overscheduled lives in stimulating, loud and bright environments without regard to natural day and night. We do not need sleep studies to tell us that we are in an age of significant sleep debt – just count the number of people, including children, asleep on planes and buses, over books and newspapers, and on couches in front of TVs. If you fall asleep regularly under these circumstances, you are in chronic sleep debt. Given the increase in obesity and diabetes over the last few decades, sleep is another potential therapeutic avenue – a fruitful and inexpensive area of health over which we have considerable control.

Managing the sleep budget: factors under your control

Environmental
1. Take the television out of the bedroom.
2.Darken the room completely, or wear a comfortable, opaque eye mask.
3. If noise is a problem were soft ear plugs.
4. Keep the temperature low at night and invest in a comfortable mattress that does not move.

Behavioral
1. Keep the biologic clock in sync with the sun by getting outside regularly.
2. Get regular exercise like walking, but avoid exercise in the last 3-4 hours before bedtime.
3. Keep naps short – 45 minutes or so – and confined to early afternoon hours.
4. Avoid heavy meals and alcohol in the last 4 hours before sleep.
5. Aim for the same bedtime every night, well before midnight, and develop a quiet bedtime ritual

Internal factors
1. Empty your bladder right before getting in bed.
2. Seek medical treatment for heartburn if causes frequent awakening. Ditto for urination.
3. Evaluation for sleep apnea is a must for someone who snores and suffers from daytime sleepiness.
4. Treatment of arthritis with exercise, physical therapy and medications, if necessary.
5. Try to get weight down to normal: sleep apnea, heartburn, and arthritis pain all benefit

Fatigue: Gentle Messenger…and Tyrant

As Supreme Court Justice Potter Stewart famously said, when confronted with a decision about what constituted pornography, the definition is hard, but “I know what it is when I see it.” An all-encompassing definition of fatigue is similarly difficult, but everyone knows what fatigue feels like. The profound lassitude that signals an oncoming flu is a gluey, mesmerizing state of mind and body that renders one incapable of remembering ever feeling good, of imagining ever feeling energetic again, or of conceiving of a desire to participate in any physical, social or mental activity beyond crawling beneath the bedcovers.  

The perception of energy failure

 Where there is life, there is fatigue. All plants and animals run on energy produced in little chemical factories (mitochondria) in every cell. The ultimate source of biologic energy is the sun’s nuclear energy, converted to usable form by plants and transferred to animals as food. The more complex the living thing, the more obvious the need for periods of rest and recovery to replenish energy. When the demand energy use outpaces the time needed for recovery, or when normal function is derailed by illness, drugs or toxins, fatigue is the name we give to what we feel, mentally and physically. To the research scientist, fatigue is a by-product of numerous little proteins (cytokines) produced by the immune system to protect us from outside invaders and internal disorders like cancer. How these proteins create the feeling of fatigue is a mystery, but there is admirable logic in a system that commandeers a patient’s energy, drive and ambition and sends him packing off to bed while an internal battle rages.  

Voluntary fatigue

Less admirable is our ability to override the biology that produces tiredness, and to become passive, cranky and sleep-deprived. In fact, most complaints of fatigue reflect the deliberate choice to ignore the symptom and would and yield to simple lifestyle changes – if one were willing and able to sleep more, lose weight, eat regular, well-balanced meals, exercise enough, manage time wisely, avoid smoking, excess alcohol, and junk food, and engage in satisfying work. In our culture these are tall orders, and a background level of fatigue is often accepted as normal. 

Evaluation of fatigue 

New, unexpected and persistent tiredness, however, may signal underlying illness or environmental stress and warrants a serious evaluation, with clear communication about exactly what fatigue means to the patient. First, a description of the patient’s normal “background energy” is important. Some people are full of energy from the day they are born. Others are inveterate couch potatoes, happy to sit and watch life go by. The feeling of fatigue that prompts one to see a doctor is, by definition, different from the patient’s normal state, but the doctor sees only a snapshot in time. Patients and families should never be shy about volunteering information about what life used to be like. 

Defining the symptom

Next, the language used by patients to describe fatigue needs to be clear. “I’m tired” sometimes means “I’m weak,” and “I’m weak” sometimes means “I’m tired,” but in the jargon of medicine, weakness means loss of muscle strength. Provided that they exert full effort, tired people can generate normal muscle power upon request, but people with strokes or nerve and muscle diseases cannot. Separating weakness from fatigue is the doctor’s first job – otherwise he may head off on the wrong diagnostic road. Description of the activities affected by tiredness and/or weakness, and characterization of changes fatigue brings to daily life are crucial to the process of diagnosis.   

Finding the source

Once a doctor understands the way fatigue affects life for a patient, he moves on to a “review of systems” – a top to bottom list of questions ranging over all the body’s organs, looking for clues to the presence of heart, kidney or liver disease, diabetes, cancer, sleep apnea, restless leg syndrome, insomnia, degenerative neurologic diseases like Parkinson’s, autoimmune illnesses like lupus or MS, chronic infections, eating disorders and problems of the thyroid, adrenal and pituitary glands. A good doctor will then delve into the lifestyle and life events surrounding the appearance of fatigue. Tiredness is a complex, high level symptom that may also originate in the mind – it is one of the cardinal symptoms of depression. 

Is it the drugs

Next comes a careful inventory of all medicines in use, prescription and non-prescription. New fatigue symptoms may parallel the addition of new drugs (even antibiotics can cause fatigue). An inventory of potential toxins and hazards in the environment may turn up a faulty furnace producing carbon monoxide or exposure to toxins such as volatile hydrocarbons that can damage the part of the brain called the cerebellum – a major player in energy balance. 

Following the clues

 Following a good, inquisitive medical history, a complete physical exam (the kind that requires undressing) may turn up other clues that suggest the need for more than “routine” tests. Fatigue is messenger bringing information about conditions ranging from minor to mortal. When not readily explained, fatigue warrants the best of our medical tools to ferret out the source of trouble. The first step though, is still a careful history and physical examination. Without these, advanced medical technological evaluation of fatigue is little better than a fishing expedition sent to sea with no information about where the fish hang out. 

                                                    The Chronic Fatigue Syndrome

Definition:

Profound, life-altering fatigue lasting more than 6 months.

May follow a viral infection, but no test abnormalities persist along with the fatigue.

Physical and mental activities both worsen symptoms.

Variety of accompanying symptoms: weakness, muscle and skeletal aches and pains, impaired memory, lack of drive, poor sleep.

Diagnosis:

No specific tests, other than exclusion of other illnesses that produce these symptoms, among others. CFS is a “diagnosis of exclusion.”

Conditions to be excluded:

Chronic infections, mononucleosis, autoimmune disorders (lupus, M.S.), hypothyroidism, low adrenal function, sleep apnea, cancer (particularly pancreatic), obesity, eating disorders, drug and alcohol abuse, major psychiatric disturbances: schizophrenia, depression. 

The Master Gland and its Tumors

“When you hear hoof beats think of horses before zebras.”
Adage familiar to most doctors, reminding them that most symptoms come from common problems. Author unknown.

Pituitary gland tumors are common, often found as unsuspected abnormalities in brain scans and in autopsies, and counted as the most common “brain tumors” removed by neurosurgeons. Technically though, the most common pituitary tumors – the horses – are not brain tumors, but gland tumors called adenomas, usually benign and eminently treatable. While more dangerous tumors arising from nearby parts of the brain or skull may closely mimic pituitary adenomas, they are rare – the zebras of pituitary problems. This column is about the horses and for simplicity will refer to pituitary adenomas as pituitary tumors.

What and where is the pituitary gland?

    The pituitary gland hangs like a little globe from the base of the brain. The back half of the gland is neural tissue, connected to the deepest regions of the brain above. The front half is glandular tissue, which, like other glands, makes hormones, secretes them directly into the blood, sends them out to perform many functions in other parts of the body and is prone to adenoma formation as life goes on.

Types of pituitary tumors

    Pituitary tumors which are “non-functioning,” i.e. producing no hormones, may never cause symptoms. Larger tumors or those that produce hormones typically come to attention in midlife, more often in women than men because female reproductive cycles is exquisitely sensitive to hormonal variations. Small, unsuspected tumors turn up in about 10% of MRI scans of the head done for unrelated reasons like sinus disease or head trauma, and in 20-25% of autopsies.

     The factors which determine whether or not a pituitary tumor produces symptoms and requires treatment include its size, its ability to produce hormones of its own, and the degree to which it compresses and damages normal pituitary gland tissue and other surrounding structures. Damage to the normal parts of the gland that diminishes production of pituitary hormones is a condition called pituitary insufficiency. Production of a hormone by a pituitary tumor is called pituitary hypersecretion, which causes predictable signs and symptoms related to the effects of hormonal overdose on the given hormone’s target organs and tissues. Pressure on nearby brain structures by a large pituitary tumor is a phenomenon called tumor mass effect.

Pituitary insufficiency

    Some pituitary hormones such as thyroid stimulating hormone (TSH) prompt other glands to produce their hormones. Other pituitary hormones work directly on many body tissues. Growth hormone, for instance, affects all tissues in the body, controlling growth in early life and many aspects of tissue repair later. Still others control menstrual function, ovulation, and production of sperm, testosterone and breast milk. The rear half of the pituitary gland, which arises from the brain, makes one hormone that helps concentrate urine and another, called oxytocin which stimulates uterine contraction during labor, and has recently been suspected to play a role in some moods and behaviors.

When insufficiency becomes failure

    If a pituitary tumor compresses the normal parts of the gland, causing it to fail, wide-ranging symptoms such as fatigue, headache, weakness, abnormal menstrual cycles, decreased libido, decreasing muscle mass and body hair, weight loss or weight gain and mood alterations may appear long in advance of a correct diagnosis. These are all symptoms which might easily be passed off as lifestyle problems, nutritional deficiencies and aging.

Pituitary Hypersecretion 

    If pituitary tumors are functional, i.e. producing hormones,  symptoms come from excessive hormonal effects on the body. For instance, growth hormone (GH) and the adrenal gland stimulating hormone ACTH are the most common tumor-produced hormones. In someone still growing, too much growth hormone produces a giant – someone whose proportions are normal, but who far exceeds the normal range of sizes. Think Andre the Giant. Once growth ceases, overabundant growth hormone still causes overgrowth in certain bones and tissues, especially the jaw, hands and feet, the nose, heart and tongue, and the heel pads. This condition is called acromegaly.

    ACTH overproduction produces Cushing’s disease, named after Harvey Cushing, the father of neurosurgery in the US, one of the first to try surgically removing a pituitary tumor. Weight increases around the trunk and in the face and neck; muscles and tendons weaken and atrophy. Bones lose calcium. Eyes bulge. Skin bruises and thins. Blood pressure goes up.

Tumor Mass Effect 

    A large tumor in the pituitary may compress not only the normal gland around it but also the surrounding structures in the brain and skull. The gland sits right below the junction of the optic nerves carrying visual information from the eyes to the brain, and in between the bones where the nerves that control eye movements enter the orbits. In addition, the large veins which drain blood from the brain travel beside the pituitary on their way out of the skull. The optic nerves fibers that carry vision from the sides of the visual field are most vulnerable to pressure, which impairs sight on both sides of the patient’s field of vision. Pressure on the nerves to the eye muscles causes double vision. Headache, eye pain, or eye redness comes from mass effect on the large veins coursing beside an enlarged pituitary. Very large tumors may affect the deep brain structures above, resulting in a host of emotional symptoms or seizures.

Diagnosis 

    Symptoms which suggest pituitary gland insufficiency, hypersecretion or tumor mass effect warrant hormonal testing, an ophthalmologist’s examination of the visual fields, and imaging studies of the base of the brain. Tumors found incidentally on scans done for other reasons should prompt a good medical history and physical examination, and possibly some hormonal testing to evaluate the functional status of the tumor.

Treatment 

    Surgical removal of the pituitary tumor is the treatment for functional tumors or those that damage surrounding structures. After surgery, patients might require either temporary or permanent supplementation with pituitary hormones. On rare occasion, abrupt pituitary failure called pituitary apoplexy is the result of a pituitary tumor bleeding. this is a medical emergency, requiring  emergency surgery with meticulous attention to fluid balance and blood pressure because of failure of the hormones that modulate those functions. Non-functioning, small pituitary tumors call for regular follow-up imaging to monitor the tumor size – and should not carry the fearsome designation of brain tumor. They are horses, not zebras.

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