Trigeminal Neuralgia: The Worst Head Pain

Anyone who has experienced toothaches, blocked sinuses, earaches, corneal scratches or migraine headaches knows that pain arising from any part of the head can be severe. Head pain is transmitted to the brain through the fifth of twelve pairs of cranial nerves at the base of the brain, named the trigeminal nerves. The worst head pain of all is the result of one or the other of these nerves misfiring, a condition known as trigeminal neuralgia, in which hundreds of episodes a day of lightning like spasms of pain on one side of the face are triggered by trivial touch or movement or by nothing identifiable. The pain is severe enough to bring sufferers to their knees and to cause more than a few to say that if it persisted they would have to commit suicide. The famous painting, The Scream, by Norwegian artist Edvard Munch, has been called a visual representation of the agony caused by trigeminal neuralgia.

Symptoms

Symptoms of trigeminal neuralgia are almost always one-sided. Spasms of facial pain are brief and explosive and described as stabbing, electrical, or like being stuck with an ice pick. Episodes can last for weeks or months, and then disappear not to return, or they may return in similar fashion, without warning, months or years later. Pain bouts may also become chronic and associated with other duller, longer lasting pain in the same area of the face. Because light touch and facial movements such as chewing and talking can trigger pain, patients avoid eating, lose weight, become depressed and socially withdrawn and look disheveled from avoiding skin care and shaving.

Why does it happen?

About 150,000 new cases of trigeminal neuralgia are diagnosed in the US every year, more often in women than men and usually over age 60. The ailment can appear in younger people, most often in conjunction with multiple sclerosis. As long ago as the eleventh century, an Arab physician, Jujani, suggested that a blood vessel in the head, near the nerve that served the face, caused spasmodic facial pain and anxiety, a prescient notion since one of the few risk factors for trigeminal neuralgia is high blood pressure. Chronic high blood pressure distorts and hardens arteries, and one of the most effective treatments of trigeminal neuralgia in modern times has been to pad the nerve, protecting it from the pounding of an overlying artery.

Bolstering Jujani’s theory, pathological examinations of trigeminal nerves have shown evidence of damage and repair to the sheaths surrounding individual nerve fibers, suggesting that pressure from a nearby artery or vein, causes intermittent, reversible damage. The association of trigeminal neuralgia with multiple sclerosis, an inflammatory disease which also causes myelin sheath damage, lends weight to the idea that stripping sensory nerve fibers of their myelin sheaths somehow causes them to be irritable and misfire. There is no specific test for trigeminal neuralgia, which can be diagnosed from the clinical history alone. However, sometimes CT and MRI scans are done to rule out tumors irritating the trigeminal nerve, or to look for evidence of demyelinating diseases like multiple sclerosis or other autoimmune problems. Spinal fluid analysis might be added a search for inflammatory or demyelinating marker proteins and cells.

Similar conditions

Other conditions may produce shooting head pains. One is glossopharyngeal neuralgia, coming from irritation of a different cranial nerve and causing pain deep in the throat and ear. It is much rarer than trigeminal neuralgia, but medical treatments are similar. Another is occipital neuralgia which comes from compression of a nerve in the upper neck, at the base of the skull. This painful condition is associated with poor posture, trauma or arthritic changes. Occipital neuralgia presents itself as stabbing or shooting pains, as well as duller aching pain and general headaches in the back and sides of the head, and at times behind the eyes. Temporomandibular joint problems (the joint that hinges the jaw to the skull) may also cause shooting pain in the side of the face, along with jaw pain and locking.

Treatments

The first effective treatment of trigeminal neuralgia was based on the concept of nerve cell irritability, with the use of drugs that treated seizures, first introduced in the mid-1900s. Though not perfect, these drugs continue to provide significant relief for the majority of trigeminal neuralgia sufferers. In addition, many attempts have been made to change the input of the trigeminal nerve to the brain physically by cutting it or injecting it with chemicals that deaden it. This treatment is called neuro-ablative surgery and the relief obtained is in direct proportion to the amount of numbness in the face caused by deliberately damaging the nerve. The more numbness, the better the pain relief. However, for some people, persistent facial numbness or unpleasant sensations are almost as intolerable as the pain, so numerous variations on such procedures have been tried. There are few controlled studies of outcome. Gamma knife surgery is the latest method. Symptoms recur within three years in 20-60% of patients, in inverse proportion to the amount of numbness produced by this deliberate nerve damage.

Microvascular decompression surgery (MVD), as mentioned above, is the most effective surgical procedure for trigeminal neuralgia. It involves opening the skull and placing a Teflon felt pad between the trigeminal nerve and the blood vessel that lies atop it, just where the nerve enters the brain stem. In experienced hands MVD produces immediate pain relief in over 90% of cases, with relief sustained for a decade or more in over 2/3 of them. Though many patients would rather try this or other surgical procedures than be dependent on anticonvulsant drugs, they must carefully weigh the risks, which are those of general anesthesia and of opening the cranial cavity, infection and damage to delicate neural structures chief among the possibilities. Procedures such as these should be done in centers where experience levels of all involved are demonstrable.

All the pains we suffer are reminders that our exquisitely complicated pain networks exist to guard the body, particularly the head and the brain within, against damage from the environment. Like every other part of the body, the pain systems can go awry, making life very difficult, but this is nature’s trade-off since life without pain is more dangerous. In conditions like trigeminal neuralgia we are left to try to understand the cause and to intervene as best we can without causing harm.

Posted by medical plaintalk

Physician turned medical writer.

Restless Legs

In 1999, Dr. William Dement, the nation’s foremost sleep researcher, lamented that 15 to 20 million Americans with Restless Leg Syndrome had fallen into a major knowledge gap in the medical care system. Doctors simply didn’t recognize the symptoms, and, more importantly, didn’t understand the serious effects of restless legs on patients’ lives. Dr. Dement wanted to educate patients and their doctors, but sleep medicine didn’t attract much public attention. Then, in 2006, the pharmaceutical industry waded into the knowledge gap, launching an advertising campaign during the Superbowl for the first drug approved for the treatment of Restless Leg Syndrome, something most of the audience had never heard of. Advertising a disorder to market a drug is not the education Dr. Dement had in mind, but at least it generates interest and curiosity, the first steps toward knowledge.

“Syndrome” means a set of symptoms. Restless Leg Syndrome (RLS) encompasses creepy, crawly sensations in the legs (but occasionally in the trunk muscles or arms), occurring mainly in the evening, getting worse on retiring for the night, and relieved by motion, particularly walking. The best estimates are that 5-15% of the population recognizes these symptoms as their own. More men than women are affected and frequency increases with age. The cause is unknown, but recent research suggests that iron metabolism in a tiny part of the brainstem is at fault.

RLS is also known as “Ekbaum’s Syndrome,” after Karl Ekbaum, who first described the problem in 1940. Jerry Seinfeld’s script writers added “Jimmy Legs” to the RLS lexicon when they had Kramer moan about a girlfriend whose nocturnal leg movements made him crazy. Kramer was actually describing not RLS but the primary sleep disorder that often accompanies it: periodic leg movement disorder (PLMD). In contrast to restless legs, Jimmy Legs often bother bedmates more than they do the afflicted sleeper, who spends much of the night bicycling away with no memory at all of the movements or of the multiple awakenings that accompany them.

More than a sleep disorder

Because restless legs cause insomnia and sleep deprivation, RLS is technically a sleep disorder. However, the sufferer’s waking world is also fraught with difficult situations that demand stillness. Theaters, airplanes, dental chairs – even operating tables- can be intolerable. The course of action taken for relief depends on the frequency and severity of the symptoms, balanced against the risks and side effects of the treatments considered.

The mildest version of RLS occurs in otherwise normal people after extreme physical exertion such as running a marathon, and it responds to time, rest and energy replenishment. At the severe end of the RLS spectrum are people whose trouble falling asleep and disrupted nighttime sleep produce severe daytime sleepiness. They need accurate diagnosis and treatment, by sleep specialists if possible. Between the mild, intermittent end of the spectrum and the severe extreme are all the rest of the RLS sufferers, including some pregnant women. These people are best served by an ongoing relationship with a doctor who understands the syndrome and the complete approach to treatment.

Diagnosis and Treatment

Treatment begins with a good history and physical exam. Restless legs are sometimes symptoms of peripheral nerve or kidney problems, and occur in the setting of diabetes. They can also reflect side effects of drugs such as antidepressants, antihistamines, and anti-nausea medicines. Even in the absence of medical problems, a check of the serum iron is on order since long clinical experience and new research implicate iron metabolism. Iron deficiency should prompt a search for a cause – usually bleeding or dietary insufficiency. Medications to reduce stomach acid, now in widespread use, can also cause iron deficiency.

Assuming there are no underlying medical problems, the next step is the elimination of stimulants from the diet – particularly in the latter half of the day. That means caffeine, cigarettes and alcohol – as well as any over the counter medicines of the types mentioned above. Developing mental alerting strategies to occupy the mind during times of boredom may help. When focused and occupied with games or puzzles the brain seems to suppress restless impulses. Increasing daily physical activity quiets the legs in over 50% of RLS patients.

When sleep suffers and normal life situations such as long automobile rides are intolerable, pharmacologic intervention is often necessary. The drugs that appear to be helpful fall into four classes: the ones that increase the neurotransmitter dopamine or act like dopamine (dopamine agonists); narcotics like codeine; the benzodiazepines like Valium, and the anticonvulsant Gabapentin. All of these are serious drugs with potential side-effects, not the least of which is a phenomenon called augmentation – the worsening of symptoms over time producing the need for more drugs. But the drugs can be true life-savers for people who are severely afflicted and in desperate need of sustained sleep and the ability to remain still.

What of the new drug touted in Superbowl ads in 2006, and a more contenders released since then? They are dopamine agonists, some of which have been around for years – FDA-approved for use in Parkinson’s disease, but also used “off-label” by doctors dealing with RLS patients. Their marketing focuses a light on the obscure world of sleep medicine, where devoted researchers who followed Dr. Dement continue to educate patients and doctors about the troubled sleep that generates many accidents and eats away at productivity and emotional resilience. That is a service to all.

Posted by medical plaintalk

Physician turned medical writer.

One-Footedness: The Key to Balance

Human beings negotiate the world on two legs, a skill mastered in toddlerhood. As children we are well balanced and swift. Then one day in mid-adulthood, we look at our children and realize that they are fast out-performing us in skills that require balance. When did balance become more difficult? Of course we cannot define that point because life sneaks up on us, nibbling away at skills we do not practice because nature works assiduously to conserve energy. Motor and mental tasks we do not practice get put to rest.

What’s involved in balance?

We maintain balance by taking in three types of sensory input and adjusting muscular activity accordingly. The three input systems are vision, messages from hair-like projections in three fluid-filled canals set at right angles to each other in the bone of the middle ear, and continuous reporting from delicately engineered receptors in our muscles and tendons that measure stretch and tension. We can learn to balance without the first two components of the balance system, but not the third. To demonstrate the importance of the input from the muscles and tendons, try getting up and walking after one foot has fallen densely asleep from pressure on the nerves which are the highways for sensory information on its way to the brain. Even if you can wiggle the foot because motor nerves are more resistant to pressure, you cannot use it without knowing where it is.

“Use it or lose it” applies to balance

Much of modern adult life involves little more than moving from one form of sitting to another, which gives the feedback systems in the eyes, ears, and muscles and tendons little exercise. Over time, balance skills deteriorate, and eventually falling happens with simply tripping or changing position or direction. Falling is the cause of many hospitalizations and, often, the injuries incurred lead to death. Living well and independently over the decades depends in no small part on maintaining the ability to walk without falling. Fortunately, balance improves with practice, and we have ample opportunity throughout the day to engage the balance systems and give them a workout.

Waking up the eyes and ears

Eyes are easy. Look around while you walk. Off to the side, up, down, straight ahead. If you are a straight-ahead looker most of the time, looking around may make you feel a bit unsteady at first. But your brain will begin to coordinate the changes with the information coming in from the ears and the muscles, so it will get easier. Once it does you can add more head movement, following your gaze. That will add more movement of the inner ear canals, which can become very accustomed to minimal movement. Young adults taking dance lessons for the first time or grandparents taking grandchildren on park equipment might be surprised to find themselves dizzy because of long unpracticed movements that involve spinning in circles or bending over. The ears are reporting unusual movements but with practice they will re-learn and stop sounding alarms. Deliberate exercises in head tilting and turning, such as the ones widely prescribed for benign positional vertigo, can speed the process.

Waken the muscle receptors by paying attention to walking

The stretch and strain receptors in the muscles are active whenever we are upright, but also lose function – even in walking, which is the most frequent and complex motor function we perform. Walking involves the subconscious coordination of over 300 muscles in a series of controlled falls that move the 200 bones of the skeleton forward or backwards in space, sometimes with the addition of upward or downward travel on stairs or ramps. Walking requires one-footed balance, with one foot bearing the body’s entire weight while the other foot swings forward. Landing the forward moving foot prevents the body from falling as it moves forward.

As the years pass, the body’s motor system tries to conserve energy by allowing you to use fewer, large muscles rather than more numerous small ones to accomplish the task of walking. Balance suffers and it is harder to adjust quickly to uneven terrain or surprises that throw you off balance. Learning to re-engage and strengthen all the smaller muscles devoted to one-legged balance re-awakens a lot of the sensory input and improves stability in all your upright activities.

Exercises for one-footedness

A good exercise for developing one-footed balance involves standing on one foot while barefoot (elevated heels throw the center of gravity forward), lifting the other knee in front of you and using a countertop for support. The gluteal muscles in the buttock on the side of the weight bearing leg will be forced to contract to keep the pelvis from dropping on the other side. The entire foot, powered by the lower leg muscles, is the stable platform that supports the rest of the body and the big toe stays in active contact with the ground. As strength and balance improve, try moving away from the support of the counter, getting the free knee up to a right angle in front of you and then swinging it down and back and a little behind you, concentrating on keeping the pelvis level and stable and the trunk upright. Adding toe lift exercises on stairs – dropping the heels a little below the stair level and pushing up from there – adds to the strength and flexibility of the ankles and to sensory input from the many intrinsic foot muscles and lower leg muscles.

Taking the exercises out for a walk

Once you get the feel of the muscle contractions necessary for one-legged balance, then try to feel the same sequences of activity while walking. Good, upright posture helps. Your head weighs 10-14 pounds when directly over your spine, but the weight doubles, triples and even quadruples in proportion to how far in front of the body it is. If you have the habit of jutting your head forward with a curve in the back of the neck, or looking at the ground while you walk, the work of balancing increases proportionately. Keep the chest lined up over the pelvis and engage the trunk muscles – the so-called core – by trying to lift the pubic bone upward with the front of the abdomen. The core muscles maintain proper pelvic tilt. Then, while walking, try to feel the one-footedness you practiced while standing next to the kitchen counter and the ankle motion you felt doing toe lifts.

The action in walking is at the hip, ankle, and foot. The role of the knee is to let the leg bend as necessary. As you shift your weight to one foot, the gluteals contract in the buttock to hold the pelvis and prevent the released side from dropping. In the brief phase before the supporting leg begins to push you forward, notice the entire sole of the foot. Its connection with the ground begins with the heel planting down and continues as the body weight rolls forward. The knee will be straightest when you push through and are about to plant the new foot.

Do not neglect the feeling coming from the toes – especially the big one as you push off and begin to move the other leg through. Toes add a significant amount of surface area, increasing the available sensory information fed into the motor system and they contribute to the push phase of the gait. Notice also how the ankle moves as the heel lifts off the ground. Notice all of it as your other leg is swinging through and really try to relate the sensations to the one footed balance exercises you have done to practice.

Notice other gaits

And while you are out walking and noticing your own one-footedness, take a look at some of the gaits you see. You will begin to learn the risk factors for falling. You’ll see people using only large muscle groups, initiating the leg swing by lifting the entire side of the body, from the shoulder down. They are already off balance. Their bodies must tip to the opposite side to allow the advancing leg to clear the ground. When the new leg lands and the weight shift begins again, the gluteals are not engaged, there is no push off from the hip and the foot, and the other side begins to lift from the shoulder. On a sunny day you will see such a walker’s shadow shift from side to side. Sometimes there are physical problems that impair walking balance, but for someone in good health, without neurological disorders like neuropathy, working to make the shadow move in a straight line pays off in a longer functional life.

Posted by medical plaintalk

Physician turned medical writer.

The Headaches that Predict Catastrophe

One of the most treacherous problems a busy emergency room physician faces is headache. “Headache” is a very common symptom, different from focal head pains attributable to sinus, eye or ear problems. While very painful and sometimes associated with nausea and vomiting, the vast majority of headaches, even if frequent and debilitating, are benign. They do not signify underlying illnesses or impending danger. But the emergency physician cannot afford to be wrong about the rare headache that predicts oncoming catastrophe and provides a chance to intervene.

Two broad categories

Catastrophic headaches fall into two broad categories. The first category includes “space-occupying lesions” such as tumors, hemorrhages, abscesses, and hydrocephalus (known commonly as “water on the brain”). The second category involves infectious and autoimmune problems that produce inflammation, triggering pain receptors in the membranes surrounding the brain and its blood vessels. Catastrophes avoided by successful interventions in both categories include death, permanent brain damage and blindness.

Tumors and abscesses

The most common fear about a bad headache is that it is caused by a brain tumor, but tumors usually produce other symptoms, involving speech, thinking, coordination or vision before they produce headache. Since the brain tissue itself has no pain receptors, tumors cause headache when they distort surrounding membranes or blood vessels, which have pain receptors. Tumor-related headaches worsen with positions and activities that normally cause the pressure in the veins in the head to rise – coughing, sneezing, lying down, straining at a bowel movement or lifting something heavy. As tumor size and pressure increase, nausea and vomiting appear. Occasionally, brain abscesses – pockets of infection surrounded by capsules -may mimic tumors. They usually come from blood infections seeding bacterial or fungal organisms into the brain.

Hemorrhages in the brain

Brain hemorrhages occupy space and increase pressure in the head. Deep small blood vessels, damaged by high blood pressure or arteriosclerosis, are usually the culprits. While these intracerebral hemorrhages can cause sudden headache, stroke-like symptoms such as paralysis, confusion, trouble speaking and loss of consciousness occur first or soon after the onset of headache.

Hemorrhages outside the brain, but inside the head

Headaches are also a symptom of epidural and subdural hematomas – collections of blood that accumulate over the surface of the brain hours to weeks after some closed head injuries (meaning no skull fracture). The history of injury, even seemingly trivial injury in an elderly patient, is crucial to correct evaluation of these headaches and there may be no other accompanying neurological symptoms. A head blow in the temple, where the skull is the thinnest is a common history. Young children and older adults are more susceptible to epidural hematomas (located between the inner skull and the the dural membrane over the brain) than those in between those age groups. Both epidural and subdural (between the dural membrane and the surface of the brain) collections of blood usually require surgical removal, sometimes as an emergency if symptoms such as change in level consciousness appear. Actor Liam Neeson’s wife Natasha Richardson did not survive an epidural hematoma incurred in a skiing related fall in 2009.

The “sentinel headache” of the aneurysm

Bleeding from brain aneurysms – weak spots at branch points of arteries – can be immediately catastrophic, even causing sudden death. But a tiny, warning leak before an aneurysm actually ruptures may cause a “sentinel headache” which allows time for life-saving surgical repair to prevent the oncoming, big rupture which typically occurs sometime in the next 10 days. A sentinel headache is sudden and severe pain involving all or part of the head, It is sometimes described like a “thunderclap.” As the little warning squirt of blood dissipates in the spinal fluid around the base of the brain, the headache dulls but a peculiar, longer-lasting pain may appear in the middle of the upper back, usually worsened with movement and probably indicating irritation from blood in the spinal fluid around the spinal cord. Diagnosis involves brain imaging with dye to study the arteries, and possibly a spinal tap to make certain bleeding has occurred. Unruptured cerebral artery aneurysms are found incidentally in 2% of autopsies so the problem is not rare.

Hydrocephalus

Hydrocephalus is a rare cause of headache, but one that should never be overlooked. The rise in pressure in the head comes from spinal fluid being trapped in the ventricles, hollow structures in the center of the brain where spinal fluid is made. Normally the spinal fluid circulates out of the ventricles via a very small channel, and bathes the surface of the brain and spinal cord before being absorbed into special veins at the top of the head. If flow is blocked, the ventricles begin to enlarge putting pressure on the surrounding brain. Most times, the onset of hydrocephalus is gradual, with headache, nausea, vomiting and balance problems gradually increasing. Unrecognized and untreated, obstructed spinal fluid flow leads to lethargy, coma and death, within 24 hours if the obstruction is sudden. Causes of obstruction include congenital anatomical abnormalities, tumors blocking the ventricular outflow tracts, scarring of these passages by inflammation from past meningitis or bleeding. Hydrocephalus most often requires surgical intervention to either remove the obstruction or to place a shunt around it, allowing cerebrospinal fluid to escape from the ventricles.

Headache from infection

Headache producing infections mainly involve the meninges, the membranes covering the brain and the spinal cord and are caused by viruses, bacteria or fungi. Viral and bacterial meningitis both cause severe headache, neck pain and rigidity and photophobia – inability to tolerate bright light. Movements of head and trunk and even eye movements are painful. Someone suffering from bacterial meningitis has a high fever, looks extremely ill and deteriorates rapidly. Identification of the infection type requires spinal fluid, obtained via spinal tap – insertion of a large needle into the spinal canal in the low back. Antibiotics are lifesaving. Viral meningitis, though painful, is less dramatic, and gets better on its own. Fungal meningitis is rare and much slower and less dramatic in its presentation than bacterial meningitis. It most often occurs in people who have impaired immune systems and requires prolonged treatment with antifungal drugs.

Non-infectious inflammatory headache: temporal arteritis

Headache from a non-infectious inflammatory condition called temporal arteritis usually presents itself in the seventh or eighth decade of life as a constant, often one-sided pain. Other symptoms that provide clues to this diagnosis are pain in the jaw muscle, especially with chewing, and tenderness of the artery just under the skin of the temple – the origin of the name for auto-immune inflammation that affects the arteries that supply the skull and brain with blood and can cause blindness and strokes. Diagnosis is confirmed when a blood test called ESR (erythrocyte sedimentation rate) is elevated and a temporal artery biopsy shows characteristic inflammatory cells in the artery wall. Treatment with steroids like prednisone, undertaken soon enough, prevents blindness and takes the headache away, but must be continued for many months.

A very useful question

One of the most useful questions an emergency room physician, or any other professional evaluating a headache complaint can ask the patient is “How worried are you about this headache?” People know themselves and have an innate sense about the nature of their symptoms. They will very often know the difference between a catastrophic headache and all the others.

Posted by medical plaintalk

Physician turned medical writer.

A Slip of Memory: Transient Global Amnesia

Search the internet for drugs that cause memory problems and you will immediately become familiar with a fascinating syndrome called transient global amnesia (TGA). A website at the top of the list of results opens with these words: My personal introduction to the incredible world of transient global amnesia (TGA) occurred six weeks after Lipitor was started during my annual astronaut physical at Johnson Space Center. TGA is not listed as an adverse effect associated with cholesterol lowering drugs, but with 12 million Americans now consuming these products, recognition of this peculiar syndrome by patients and doctors becomes important. Beyond the tightly controlled world of pre-market drug approval studies, unexpected symptoms need to be noted.

Transient global amnesia is a short–lived problem, over in less than 24 hours. It involves all aspects of new memory formation. Nothing gets recorded during the event and the sufferer fails to remember anything from the event after it is over. To appreciate the disconcerting nature of the syndrome, imagine suddenly losing your ability to put any information into your memory. In addition, you lose access to a few hours or days or even years of past memory, but not to information about yourself. You look around but cannot identify why you are where you are. Depending on how much access to the past you’ve lost, your current situation might be totally unknown to you. With great urgency you question the people around you. “What am I doing here?” How did we get here?” What’s going on?” Someone answers and for about 30 seconds you can hold on to the information. But your ability to put that information into memory has gone offline. You forget that you just asked the question. You ask again, and again, and again, with obvious anxiety. All your other mental capacities work. You can speak, read, write – even drive and problem solve. Except for your anxiety, you are the same person as always. Then the confusion ebbs. You start to encode information again. Your past gradually returns, in chronological order. Once again you are tethered to time and place, but you will not ever remember what went on while you were cut loose and for a little while you might complain of a little headache.

The first descriptions of this odd set of symptoms appeared in an obscure medical journal in 1956. More cases came to light, and by the early 1990s there were a few epidemiological studies that suggested that TGA occurs in about 10/100,000 people, or as many as 25-32/100,00 in the peak age group of 50-80. Far from being a harbinger of impending stroke or evidence of seizures, these episodes seemed to have no correlation with any problems other than a history of migraine and left no problems in their wake. They recurred in somewhere between 5 and 25% of the cases, with one patient having over a dozen recurrences. No definite cause has ever been found, though many physicians have associated them with immediately preceding, physically or emotionally strenuous events.

Very rarely, underlying brain problems like tumors involving the deep middle and frontal areas, where memory formation takes place, turn up in TGA cases, but careful examination of these cases inevitably reveals some deviation from the typical clinical symptoms, or some type of abnormality on neurological examination. TGA research studies, using sophisticated scanning and EEG techniques, suggest that there is decreased activity in areas of the brain involved in memory formation, but give no clue about the mechanisms involved.

In 1990, criteria for diagnosis of the syndrome were published (listed below), and when a diagnosis of TGA strictly adheres to these criteria, it is almost always safe to predict that there is no underlying neurologic or vascular problem. Nevertheless, when a patient appears in an emergency room with TGA symptoms, good practice still requires a CT or MRI scan and an EEG at some point to rule out the remote possibility of an underlying tumor, hemorrhage or seizure disorder.

Reports like those of Dr. Duane Graveline, the astronaut/physician author, are considered anecdotal and not of the same value as information gleaned from statistical analysis of controlled studies . The cholesterol lowering drugs are in widespread use and considered very safe by most physicians. However, there have been cases of muscle and nerve problems, as well as cases of decline in cognitive function attributable to the drugs, possibly mediated by damage to mitochondria, the power houses of all cells in the body. Since the drugs are viewed as valuable additions to the battle against heart disease and are likely to be used over long periods by increasing numbers of people, it is important to understand and catalogue their unintended consequences. Once drugs reach large populations outside medical studies, more problems emerge, sometimes beginning as odd, single cases. In the meantime Dr. Graveline has died after a progressive illness which resembled Lou Gehrig’s Disease (progressive loss of muscular strength and bulk). His memory, however, remained good.

Diagnostic Criteria for Transient Global Amnesia

A witness must be present to describe what happened.
The patient must be unable to form new memories of any kind (anterograde amnesia).
The patient must have full knowledge of his identity and an unclouded state of consciousness.
All other mental functions are normal, including speech.
There are no other neurological symptoms or signs.
There are no signs of a seizure.
There is no history of seizures within the last 2 years, or of recent head injury.
The patient is back to normal within 24 hours.

Posted by medical plaintalk

Physician turned medical writer.

The Gratitude Attitude: Five Best Days

Gratitude is an attitude that mental health professionals say promotes mental well-being. They advise practices such as noting three good things about each day and writing them down at night. Studies actually show that such habits durably improve mood. In the giving spirit of the Christmas season I would like to share with you one of our family traditions that, in retrospect, I realize promotes the gratitude attitude over the course of each year.

We began taking our boys to Colorado to learn to ski when they were very little. Economic and time constraints meant one week a year, determined by my husband’s surgical on-call schedule. That week included the turn of the year on New Year’s Eve. Children’s skiing torchlight parades, followed by dinner at a Chinese restaurant and early bedtimes gradually gave way to a movie (usually laughably bad) followed by dinner at a locals’ Italian restaurant with paper-covered tablecloths and crayons for doodling. The family expanded to include our boys’ friends – first as children and teenagers along for a vacation, then as young men who worked as ski instructors. Somewhere along the way we began the habit of discussing our five best days of the year over New Year’s Eve dinner.

The crayons and the paper table covers are very convenient – everyone begins jotting down their five best days, in order, almost as soon as the menus arrive. The entire dinner time winds up devoted to going around the table in five rounds, hearing from each person about what made each wonderful day and how they decided where to rank it in the list. The choices are life stories in snapshots, changing with growth and priorities. They are funny, poignant and surprising. We also hear from people who have been with us on past New Year’s Eves, calling, e-mailing, or texting their top five days, sometimes accompanied by pictures. They all get heard.

The reason this tradition promotes gratitude is a very practical one. If you know you are going to have to come up with your five best days of the year on New Year’s Eve you learn pretty quickly that memories are weak. You cannot cram for this test. You have to start noticing potential top five days as they happen over the course of the year. You start writing them down. Pretty soon you actually have a little journal and it contains good stuff. The good things that happen in life start to break into your awareness and compete with danger-surveillance program that runs continually in the background of your mind. All good, with none of the side effects of mood enhancing drugs (which fail in the long run anyway).

Just as a matter of historical interest, we traced this tradition back to its origins. It actually began with my husband’s surgical training at Massachusetts General Hospital. The surgical interns and residents on the general surgery service met at the end of each day and were asked to talk about the cases they helped with and to explain what they had learned. The practice helped everyone process what they had done and learn from their experiences. As a family, we were always sit-down dinner people, with candles even when there was still a high chair at the table. Like the surgery residents, we always talked about everyone’s day. Skiing days included lots of bests. Best fall, best jump, best run, best lift ride. The evolution to a summing up of bests at the end of a year was inevitable. What is a surprise is the way the practice has continued and spread from our families to others. Maybe you would like to give it a try.

Posted by medical plaintalk

Physician turned medical writer.

Human Diversity: A Mind Thing

(A version of this essay was published in Minnesota Medicine in March, 2005.)

The first snowy egret I ever saw was standing in a shallow pool, a beautiful white creature with a wispy headdress floating in a gentle breeze. When he lifted off, trailing his long black legs, I was startled by a flash of bright yellow – he looked like he was wearing a child’s rubber boots, designed to hug a bird’s clawed feet. He did a loop around the pond in flying low in a peculiar, non-aerodynamic position. His legs hung down rather than stretching out parallel to his body. He dipped closer to the water and his dangling yellow feet brushed the surface. He’s landing, I thought. But he didn’t. He repeated the maneuver four times. He’s afraid to land, I thought. But then he touched down with hardly a splash, and dipped his beak to catch a fish, then another, and another. In a moment of bird-watching epiphany, I realized that his feet were like a fisherman’s lure. He’d rounded up his lunch.

I looked around the pond. I’d just watched a bird with bright yellow feet use them to attract the curiosity of his prey. Across the water was a bird with a lower bill that expanded into a pouch to collect fish as he skimmed, openmouthed, over the water. Perched high above was another, just about to dine on a large fish he’d skewered with fearsome talons. What an impressive display of diversity! If people were this diverse, New England fishermen would grow waterproof, blubber lined hands, typists would develop extra fingers, and mothers really would have eyes in the backs of their heads.

But people are pretty much the same as far as their bodily equipment goes. Five fingers on each of two hands, ten toes divided between two feet, two eyes, upright posture, and a narrow range of physical abilities, notwithstanding the spread between ordinary and Olympian. The traits we associate with human diversity are superficial – hair color and texture, skin color, facial appearance. Almond shaped or round, eyes still see. Long, elegant skeletal frames and short squat ones all support bodies against the universal force of gravity. Skin pigmentation protects the body covering from the sun, more or less depending on the power of the sun in the areas of the world where the people originated. We are much more like each other than we are different in our biology. The birds have it all over us in the diversity contest.

After my egret experience, I packed up my binoculars, got on my bike and headed home, humbled a little by the thought that all of the talk and concern about diversity among people is overblown, maybe just another representation of man’s abiding sense of self-importance. But along the way I passed bikes and cars, houses and stores, a radio broadcast tower, and a museum. I crossed a bridge between two islands and waved to a fisherman in his boat. And I realized that each of these man made things I passed began as an idea, somewhere, some time, in somebody’s head. We might not differ much in anatomy and physiology, but no two of us have identical thoughts. The mind is the site of the real diversity among humans. The mind is plastic and ever developing. It records, collates, recalls, communicates, and combines unrelated information in new ways. Yellow feet catch the eye, but minds change the world.

Posted by medical plaintalk

Physician turned medical writer.

Why We Cry..and How We Make the Tears

“It is such a secret place, the land of tears.” The Little Prince, Antoine de Saint-Exupery

Do animals cry? Probably not. Indian gamekeepers told Charles Darwin stories of elephants that shed tears of sadness, and dog lovers have tales of canine tears, but the emotional tears of humans are unparalleled in the animal kingdom.

We are always making tears

All land dwelling animals, including people, make tears constantly. Eyes are windows on the world, and baseline tears are constant window washers. The window pane is the cornea, a thin panel of collagen, containing very few cells, no blood vessels, and more nerves per square inch than any other part of the body. These nerves signal alarm and summon an army of reflexive tears in response to a speck of dust, a cold wind, or a whiff of an onion. Reflexive tears, which are just a lot of baseline tears, wash out intruders and fill in dry patches on the cornea, keeping it clear and moist to focus light entering the eye. Emotional tears appear in humans during infancy, but not immediately. The crying that infants first do to signal their needs is much like the crying of little chimpanzees – tearless. Emotional tears come later, just like talking. Both are outward expressions of the lives of our minds, and they take a while to learn.

What tears are made of

Tears are much more than little beads of salt water running down your face. They are a three layer sandwich. The oil-containing molecules in the outer layer tighten up the surface of the watery middle layer to keep it from spilling over eyelid and sliding off the surface it protects. The oil floats on the watery middle layer and smooths its surface, optimizing the passage of light through to the eye’s interior. The third part of the sandwich, inside and closest to the eye, is the mucinous layer, kind of a gluey protein that helps tears stick to the eyeball. The mucinous proteins capture and kill biologic intruders like bacteria and viruses, and soak up some of the watery layer to help transfer nutrients, oxygen and moisture to the cornea. Both the oil and mucin slow evaporation of tears as blinking spreads them over the eye.

Evaporation and drainage

Dry spots appear on the cornea after just fifteen non-blinking seconds – easy to do while concentrating or daydreaming. Even with blinking, tears evaporate, or they drain out from the eye into the nose via two tiny lacrimal ducts on the upper and lower eyelids near the nose. If these ducts become scarred or blocked by infection, tears overflow. Six to 10% of babies are born with tear ducts not yet open, but 95% of these will open by age one without any attempt at surgical repair. Conversely, one way of treating dry eyes is to block these ducts with small plastic pellets.

Dry eyes

Too few tears, tears with abnormal composition, and decreased blinking cause dry eyes that itch, sting, burn, get red, and cause blurred vision. Dry eyes are an increasing problem in our air-conditioned, airline-traveling, contact lens-wearing, Lasiked, medicated and aging society. The list of drugs that dry eyes includes many commonly prescribed classes: decongestants, antidepressants, antihypertensives, antihistamines, beta-blockers, hormones, diuretics, ulcer medications, acne drugs, and oral contraceptives. Other causes of dry eyes are infections and immune diseases like rheumatoid arthritis and Sjogren’s syndrome as well as radiation and radioactive iodine treatment can also damage the tear producing cells. All of these conditions damage the tear producing cells – the machinery for tear production.

Remedies for dry eyes

Treatment of dry eyes is always aimed at removing offending problems and increasing lubrication. The usual tactics include artificial tears, wind protection, air humidification, cessation of unnecessary medications, and treatment of underlying diseases and infections. Excessive tearing often means allergies, or blocked lacrimal ducts leading to poor drainage of baseline tears into the nose.

The tear producing machinery

The medical conditions mentioned above shut down tear production and cause pain and swelling in the lacrimal gland, a spongy little structure tucked up under the upper outer corner of the eyelid. The lacrimal gland is the tear producing factory, aided by the oil-producing Meibomiam glands near the eyelashes, and a cluster of mucin-producing cells in the eyelid lining.

The controls for the machinery

When the lacrimal glands get a call for more tears, either reflexive or emotional, the messages come through the autonomic nervous system, which oversees the automatic functions of the body. Reflexive tears spring from messages sent from the eye and nose. Emotional tears come from messages sent by the limbic system, the deepest and oldest part of the brain, the part that conjures up feelings.

What are emotional tears?

What are emotional tears? Are they just more voluminous baseline tears? Or does emotional crying rid us of “humors of the brain,” as Hippocrates thought? In Roman times, mourners used small glass vials called lachrymators to collect their tears for burial with the one for whom they cried. In today’s laboratory, emotional tears are almost as hard to come by as research money to investigate them. Some dedicated men such as Professor William H. Frey II (Dept. of Pharmaceutics at the U. Of Minnesota) have learned enough to suggest that tears of grief rid the body of some of the products of stress, supporting the claim that crying makes people feel better. Compared to reflexive tears, emotional tears contain up to 25% more proteins, of classes related to stress. Why? No one knows – yet. Emotional tears are still a land of mystery, part of the unique expression of inner life that separates the human animal from the others.

Posted by medical plaintalk

Physician turned medical writer.

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.

Posted by medical plaintalk

Physician turned medical writer.

Polar Moods

Bipolar disorder, previously called manic-depressive disease, is a not a new diagnosis. But it is one being made with increasing frequency, particularly in children and young adults. In psychiatry diagnoses are legion, but they all fall into one of three categories: disorders of mood, thinking, or personality. Bipolar disorder is a problem in the sphere of mood, described in the 1880s by Emil Kraepelin, the German psychiatrist whose Compendium der Psychiatre was the world’s first systematic classification of mental disorders. At the time, psychiatrists recognized separate illnesses called mania and melancholia, but Kraepelin was the first to see that some patients cycled between these opposite poles of mood. Over time, the term cyclical insanity gave way to manic-depressive disease, and finally to bipolar disorder, type I or type II (the milder variety). Melancholia is now called unipolar depression and mania is no longer a diagnosis but rather a behavioral symptom in all kinds of psychiatric disorders.

Normal ups and downs in mood

Everyone has ups and downs in mood. Mood involves both subjective feelings and outward behaviors. It is clear from “normal” mood swings that both internal and external factors influence ups and downs. Many of those factors, such as sleep, stress, physical activity, diet, and abuse of alcohol, nicotine and drugs, also affect general health.

The definition of mood disorder

Normal ranges of mood vary greatly from person to person, so the psychiatric definition of “mood disorder” rests on the degree to which disrupted behavior interferes with carrying out the normal activities necessary for functioning at a given stage of life. Clearly abnormal symptoms like hallucinations, which define the thinking disorder schizophrenia, may also appear in bipolar type I. New genetic work suggests that mood and thinking disorders are not as separate as our classification systems try to make them, so it is not surprising that symptoms at times overlap.

The down side of the mood spectrum

Depression, the low side of the mood spectrum, robs a person of interest and joy in his activities. He has little energy, sleeps more than usual, or may be unable to sleep through the night, waking up anxiously at two or three AM. He may gain or lose weight. He tends to ruminate, repetitively chewing over negative thoughts. Sadness permeates his world. Of course these same symptoms can be completely appropriate responses to terrible life events that cause profound grief. A depressed mood becomes abnormal when it occurs or persists unrelated to circumstances, blocks the activities necessary for normal life, and/or includes persistent thoughts of death or suicide.

The up side

At the other end of the mood spectrum, mania, the mind speeds up. Thoughts are rapid, distractibility is high, speech is pressured, and ideas become grandiose. Sleep isn’t necessary. The manic person engages in risky behaviors and feels invincible. He undertakes grand schemes, spends money with abandon, and becomes obsessed with projects or ideas. When the exuberant moods are still under some control (hypomania), they can be very productive. The afflicted individual seems lively and charismatic, the life of the party. But when mania spirals out of control it can become life threatening. As mentioned above, mania not confined to bipolar disorder. It is a symptom that can happen in mood, thinking and personality disorders.

Bipolar: more down than up

Most patients with true bipolar disorder spend far more time on its depressive side, experiencing few manic phases. In fact, it is now felt that many cases unipolar depression, with no history at all of hypomanic or manic episodes, actually represent bipolar mood disorders, making diagnosis tricky. Correct diagnosis is important. In unipolar depression, the response to conventional antidepressant therapy takes weeks, but in bipolar depression, the same drugs can tip the patient into a manic state quickly. It is possible that the reported cases of suicide shortly after antidepressants are started may be related to this phenomenon.

The danger of wrong diagnosis

In our current medical and economic climate, the threshold for using antidepressants is very low. Frequently the drugs are prescribed by non-psychiatrists, without concurrent talk or behavioral therapy, and without adequate follow-up. So it is imperative for patients who are given antidepressants to understand that a rapid response, within days to a week, and feelings of agitation or irritability might mean that the diagnosis of unipolar depression is wrong. For bipolar patients, the drug of choice is a mood stabilizer, which calms manic states and can prevent return of depression.

Stabilizing the mood

The most effective mood stabilizer is lithium. Lithium is a simple chemical element in the same family of elements as sodium, potassium, calcium and magnesium, rather than a complicated molecule like other psychoactive drugs. Its mechanism of action remains elusive, though it is thought that it makes the neurochemical transmitter norepinephrine less available and less effective in the brain. Lithium must be monitored carefully, with urine levels performed regularly. Toxic side effects include diarrhea, tremors, thirst, weight gain, drowsiness, and impairment of kidney and thyroid function.
If lithium is ineffective or poorly tolerated, drugs normally used for treatment of seizures may work as mood stabilizers. One, valproate, is particularly effective for people who also have substance abuse problems, a not uncommon occurrence. Antidepressants may also be necessary at some point, but not without concurrent use of mood stabilizers. Bipolar disorder is a lifelong problem that requires careful monitoring, variable amounts of drug therapy, and simultaneous counseling aimed at development of cognitive skills and habits that help blunt the effects of mood swings on behavior.

Are we creating more lifelong psychiatric problems with drug treatment?

Some psychiatrists feel that the widespread use of antidepressants and other mood altering drugs to treat poor behavior or reactions to life’s inevitable problems changes brains enough to change the way true psychiatric problems now evolve. These days, we have increasing numbers of bipolar diagnoses. Compared to past decades, bipolar patients now cycle more rapidly between highs and lows. While the increasing frequency of bipolar disorder diagnosis may represent increasing labeling of behavioral problems, we also must consider the disturbing possibility that temporary alteration of brain activity with drugs is leading to long term psychological and behavioral changes. Readers who are interested in more extensive discussion might want to read Anatomy of an Epidemic: Magic Bullets, Psychiatric Drugs, and the Astonishing Rise of Mental Illness in America by Robert Whitaker, Broadway; (August 2, 2011).

Posted by medical plaintalk

Physician turned medical writer.

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