Fascinomas- Fascinating Medical Mysteries

by Clifton K Meador

  Kaiser sat at the desk in the small exam room and faced the couple sitting off to his right. He introduced the nurse.

  Then, in a most serious monotone, Kaiser began his talk. “I have thought long and hard about your problems with the abscesses on your left arm. I must be completely honest with you. It will be no help at all if we continue this false guise of pretending you have some obscure disease. I am certain of one thing. I am certain that you are injecting or rubbing something into your left arm to cause the abscesses. You are right handed. The abscesses are only on the left arm. From the mixture of bacteria, you are either rubbing saliva or dirt or feces into the left arm.”

  Kaiser, the nurse witness, and both Henderson’s sat in silence for several minutes. Kaiser, in telling this case story, said his heart was pounding, not knowing what to expect. After a few more minutes, both Henderson’s rose and walked out of the exam room, saying nothing… nothing.

  Dr. Kaiser never saw the Henderson’s again. He did hear months later from their local family doctor that the abscesses had cleared from Henderson’s arm.

  There is an old dictum about self induced injuries or infections. It says, “The doctor who makes the diagnosis of self infliction will never be the doctor who treats it.” That is so true. Whenever the relationship drops to that level of deception and lack of trust, there can be no healing doctor-patient relationship.

  *Case shared by:

  Allen Kaiser, M.D.

  Chief of Staff

  Vanderbilt Health System

  Professor of Medicine, Vanderbilt University

  Chapter Thirty Three

  A Case of the Blues *

  Jake Deacon rode motorcycles with his Harley Club every weekend. Even though he spent his weekdays as a truck driver, to him there was nothing like the feeling of traveling those same open roads on his motorcycle. He loved to ride.

  In the late spring of 1984, Deacon was cruising a Tennessee back road between Memphis and Jackson when his motorcycle spun out on a curve. His body slid more than 30 yards across the gritty asphalt. His leathers offered little protection.

  The skin over the right side of his body was severely abraded. The femur and tibia of his right leg were badly fractured, exposing the bones of his thigh and lower leg.

  He was airlifted to the University Hospital in Memphis and admitted to the Intensive Care Unit.

  After multiple orthopedic procedures, skin grafts and a three-month stay in a rehab unit, Deacon was finally discharged to his home near Nashville. It had been a long and painful process, but his injuries were healing. He was grateful to be alive and looked forward to getting back to his life.

  Back home, Deacon’s health didn’t continue to improve as he hoped. He remained in constant pain — not just where he was injured, but throughout his body. He spiraled into depression and suffered from a profound lack of energy.

  Driving a truck was no longer possible, so he quit and applied to Social Security for disability. This led to an extensive psychiatric evaluation, which labeled him as “malingering and seeking money for his alleged but unproven disability.” His claim was denied.

  Deacon began seeing different doctors, hoping someone could diagnose and cure what he knew to be a very real physical debility. Among the many who saw him, there were diverse opinions about the cause for his continued troubles. Some believed he had severe depression, but trials on antidepressants failed to give any relief. Others believed he had chronic fatigue syndrome, while still others thought he had some obscure malignancy yet to surface. This latter diagnosis stemmed from the fact that lymph nodes in his neck and under his arm were tender. But a biopsy showed no abnormality of the nodes.

  In fact, all tests revealed normal values. There was no evidence for liver, heart, kidney, gastrointestinal or neurological diseases. Repeated measurements also remained normal.

  Deacon continued to describe himself as “just feeling sick.” He said he woke in the morning feeling somewhat better than when he went to bed. As the day wore on, he felt sicker and sicker. He suspected he often had a low-grade fever, but an elevated temperature was never documented. He also reported having an itchy rash from time to time, but no doctor ever saw it.

  His major complaint was aching in all his major joints. Deacon consulted a rheumatologist who ordered tests for known joint or soft tissue diseases. All were normal. A biopsy of his calf muscle revealed no inflammation of his small blood vessels, ruling out a number of types of vasculitis.

  It had now been two years since Deacon’s motorcycle accident. He remained unemployed, undiagnosed and chronically ill.

  He was referred to see Dr. Paul Barnett, a general internist known for his diagnostic abilities in patients with obscure and undefined symptoms. Dr. Barnett repeated most of the tests Deacon had been given, and results were still normal. Over the course of several weeks, the doctor explored the habits and psychological aspects of Deacon’s life and found no stressors to account for the chronic complaints. Deacon had a solid marriage and two healthy children. Although he was unemployed, his wife made enough money to support the family. Again, a dead end for any explanation of Deacon’s complaints.

  One day, Deacon came in to see Barnett and sat directly across the desk from the doctor. Barnett squinted at Deacon. “What’s that white streak on your chin?” he asked.

  Deacon rubbed his chin, smiled and said, “Oh that‘s just some blue cheese dressing. I love the stuff. Eat a salad with it almost every day.”

  Barnett leaned forward in his chair, considering a new possibility. He asked Deacon to tell him, in detail, exactly how much and how often he ate blue cheese. The answer was, literally, “Every day.”

  “Let’s try a long shot,” the doctor said. “Hold off the blue cheese and let’s see what happens. Don’t eat any — not one little bit.”

  Well, this was by far the weirdest “prescription” Deacon had gotten from all the many doctors he’d seen. But he was willing to try anything to feel better and agreed to stay away from his favorite food.

  Deacon came back a week early for his next appointment. He was so excited, he couldn’t wait to see Dr. Barnett. “I’m completely well,” he blurted out as soon as the good doctor walked in the room. “Wake up normal. Ready to go. No more aching anywhere. Tender lumps in my neck? Gone. Sleep through the night. I’m even applying for my truck driving job. It’s a miracle, Doc.”

  Since his last visit with Deacon, Barnett had done some research to confirm what he suspected when he heard about his patient’s blue cheese habit. Sure enough, he found that the smelly, moldy cheese contains either Penicillin roqueforti or Penicillin glaucum. Both molds release a form of penicillin. Dr. Barnett knew that constant penicillin in the bloodstream can produce serum sickness from a chronic immune response. Deacon had a case of serum sickness.

  Serum sickness is caused by the immune system responding to something in the blood that it “sees” as abnormal. Immune complexes form and cause inflammation in the joints, lymph nodes and sometimes small blood vessels. Antitoxin sera of various sorts can also cause the reaction. Antibiotics are known causes, penicillin most often listed.

  The symptoms can be partially relieved by corticosteroids or non-steroidal anti-inflammatory agents. All symptoms usually go away within a week of stopping the offending drug or agent. The symptoms return if the drug is taken again.

  An extensive search of the medical literature fails to find even one other case of serum sickness caused by blue cheese. Most people consume blue cheese intermittently, but Jake Deacon’s daily consumption created a sustained blood level of penicillin, thus triggering the immune response and serum sickness. This is believed to be the only case of serum sickness from blue cheese.

  “I was cured within a week after I stopped eating blue cheese,” Deacon said. “Who ever thought that all this time of being sick would be caused by eating a salad I thought was good for me?”

  *Case shared by

  Paul Barnett, M.D.

  Associat
e Clinical Professor of Medicine

  Department of Medicine

  Vanderbilt University School of Medicine”.

  Chapter Thirty Four

  Open and Shut Case *

  Editor Note: This highly technical case illustrates the extraordinary diagnostic power when knowledge in physiology and technology are combined. The solution to this case required both.

  Chronic pain in Lester Littler’s fingers and knees was the first sign something wasn’t right. The 62-year-old mechanic and farmer also noticed slight swelling and redness at his joints, but he wasn’t particularly alarmed.

  He happened to mention this to his family doctor, Dr. Moore, who wasn’t alarmed either. He thought Littler might have mild rheumatoid or osteoarthritis and prescribed Motrin.

  The Motrin provided some relief at first, but over a three-month period the joint pains increased. Littler returned to see his doctor. Now thinking the joint pain might be gout, Dr. Moore tested blood uric acid level. He also tested for rheumatoid arthritis. Both came back normal.

  At this visit, Dr. Moore noticed something else: Littler’s fingernails appeared unnaturally curved and rounded. It looked like a classic case of “clubbing,” which can be caused by a number of diseases, some of them dire. The doctor ordered x-rays for both chest and bones.

  The chest x-ray revealed a nodule the size of a golf ball in Littler’s left lung. The bone x-rays revealed inflammation and elevation of the covering of the bones, the so-called periosteal layer on the surface of the bones. This condition is called “hypertrophic osteoarthropathy.” It is a rare manifestation of lung cancer, as is the clubbing of the fingernails.

  Littler underwent surgery in which lung cancer was found and his entire left lung removed. There was no evidence for the cancer spreading.

  Immediately after surgery, Littler’s knees and hands started to feel normal again. The pain and inflammation he had experienced disappeared completely over about two weeks; his fingernails soon returned to normal.

  The clinical story up to this point was relatively simple: Lester Littler had a rare manifestation of lung cancer, the lung cancer was removed and the symptoms of painful joints and clubbing disappeared.

  But then things began to get complicated.

  Littler recovered nicely from the operation and, after two weeks, was back doing mild chores on the farm. About six weeks after the operation, however, he started feeling short of breath on his daily routine activities. This worsened until walking fast or going up stairs carrying loads made him uncomfortably short of breath.

  He went back to Dr. Moore whose exam could find no reason for Littler’s distress. Yet he had a respiratory rate well above normal, the normal rate of breathing being 10 to 12 breaths per minute. Littler was breathing 18 times a minute just sitting quietly in the exam room.

  The doctor did pulmonary function tests which revealed only the expected loss of lung volume due to the removal of his left lung. But this did not explain his rapid breathing. A CT angiogram of the arteries to the lung showed no evidence for an embolus (clot in the pulmonary artery), and his blood count was normal, ruling out anemia as an explanation for the shortness of breath.

  The Moore had no answers, but noted that Littler’s oxygen saturation was 88%, whereas normal is 95%. He decided to begin Littler on 2 liters of nasal oxygen at home, to see if his breathing improved.

  Over the next several weeks, the shortness of breath worsened. Littler was now breathing over 30 breaths a minute. Unable to get from his bed to the bathroom without unbearable shortness of breath, he went to the local ER on a Sunday afternoon.

  The ER physician, lacking any explanation, called Dr. John Newman at Vanderbilt Medical Center for a pulmonary consultation. In the local ER, Littler’s oxygen saturation was only 75%. This did not rise even when the nasal oxygen was raised to 10 liters per minute, the maximum deliverable via the nasal catheter. Littler was saying over and over that he would blow his brains out if he did not get relief soon. He was having terrible headaches and mild chest pains on top of the extreme shortness of breath.

  Dr. Newman quickly reviewed the other findings. The chest x-ray showed the expected fluid in the left chest where the cancerous lung had been removed. The right lung was normal, with no pneumonia or sign of abnormal arteries or veins.

  Newman then suggested the local ER doctor give Littler an oxygen mask which would deliver pure oxygen at a 100% level. When the ER doctor reported back that Littler’s saturation was only 78% while breathing 100% pure oxygen, Dr. Newman knew the answer to their puzzle.

  Recalling basic respiratory physiology, he knew a low oxygen saturation in the face of breathing 100% oxygen meant only one thing: the presence of an arterio-venous shunt. The low oxygen containing venous blood was going directly into the arteries without passing the oxygen sacs in the lung, a so-called “right to left shunt” of blood. In other words, the blood was getting through the chest without being exposed to oxygen in the lung alveoli and then passing into the body with very low oxygen levels. In effect, the body was barely staying alive on nearly pure venous blood.

  Dr. Newman was sure there must be a shunt bypassing the lung, but where was it? Littler was transferred to the Intensive Care Unit at Vanderbilt Medical Center. When he arrived, he was breathing 30 breaths a minute, sitting up straight in bed. He had constant chest pain. An echocardiogram (EKG) at the bedside showed no hole in the heart, and even with injection of saline with small bubbles as tracers, there was no visible shunt. The small bubbles passed directly into the arteries of the lungs as they do normally. No shunt was found by this method.

  Now the case really gets complicated. All evidence says there must be a shunt from right to left from veins to arteries somewhere in the chest. Otherwise the 100 percent oxygen would have increased the oxygen level in Littler’s blood.

  Still convinced there had to be a shunt despite failure to show it with the echocardiogram, Dr. Newman insisted on a full cardiac catheterization – the absolute gold standard for looking for a shunt. At catheterization, as suspected, there it was: an open hole between the two atria of the heart known as the foramen ovale, a vestige from fetal life when the foramen ovale is open. The case is solved. The surgical closure of the foramen ovale will cure the problem.

  Here we need a short digression. Before birth, the infant has no need for oxygen from the lungs, because oxygen comes from its mother’s blood in the placenta. So the blood from the lungs is bypassed through a hole in baby’s heart. That hole is the foramen ovale. At birth, the hole in the heart miraculously closes, and venous blood then flows into the arteries of the baby’s lungs where it receives the oxygen breathed in by the infant. Littler’s foramen ovale, closed since birth, was forced open from the rotation and twisting of his remaining right lung into the space of the removed left lung. This allowed the venous blood to flow into the arterial side, thus shunting the blood away from the oxygen in the lungs.

  But why did the echocardiogram fail to reveal the shunt? The answer: The saline tracer was injected into an arm vein. Blood that traversed the foramen ovale was streaming up from the leg and abdominal veins via the inferior vena cava. Blood from the arm veins passes directly into the artery to the lung failing to reveal the open foramen ovale.

  Littler was taken to the OR by the thoracic surgeons and the open foramen ovale was confirmed and sutured closed. The arterial oxygen level returned to normal within a few minutes of the closure.

  Knowledge of basic physiology permitted an accurate solution to this case: Breathing pure oxygen raises the oxygen content of blood unless there is an arterio-venous shunt. But the sequence of events were most uncommon, there being only five reported cases of a foramen ovale opening after lung resection.

  As for Littler, he returned to his farm and never had another problem with his lung.

  *Case shared by:

  John Newman, M.D.

  Elsa S. Hanigan Professor of Pulmonary Medicine

  Division of Pulmonary and Critical
Care Medicine

  Department of Medicine

  Vanderbilt University School of Medicine

  Chapter Thirty Five

  Itch in a College Student *

  Fred Orville was winding down his last week in college. The last few weeks had been filled with all night parties and “serious drinking,” as he liked to say.

  For over a month, Fred noticed a rash begin to appear on his legs and arms. Sometimes it looked like hives but then it changed into small raised plaques. The itching became intolerable so he went to the Student Health Center where he was given calomel lotion and some Benadryl to take by mouth. They told him he was allergic to something he had touched.

  The drugs gave him no relief and the itching increased. So did the area of the rash. He now had lesions inside his mouth and over his entire body. He had to get some relief. The Student Health nurse got him an appointment with the Dermatology Clinic the next day.

  Dr. Michael Jenkins met Fred in his exam room, took one look at the skin lesions and said, “You’ve got lichen planus. That’s for sure. Of course I will biopsy to be certain.”

  Jenkins did not tell Fred that sometimes the lichen planus lesions can predispose to the development of skin cancer (squamous cell type.)

  Fred kept saying, “I‘ve got to get some relief. Can you do something to stop this itching?”

  Dr. Jenkins told Fred he would start him on oral cortisone-like steroids and see him in a week with the biopsy report. Jenkins took a small biopsy on Fred’s arm.

  By the second day of steroids, Fred’s itching was much less and he was free of the itch by the fourth day. The biopsy confirmed the diagnosis of lichen planus and showed no evidence for skin cancer.

  Dr. Jenkins was glad to hear that the itching had been relieved. He explained to Fred that most cases of lichen planus are “idiopathic”, meaning the cause is unknown. He went on to name a long list of substances known to cause the disease. The list included antimalarial drugs, penicillamine, thiazide diuretics, beta blockers, non-steroidal anti-inflammatory agents, quinidine, angiotensin-converting enzyme inhibitors, or gold containing compounds. The disease also has been linked to hepatitis C viral infections.