Case Records of the Historical Grand Rounds:
Case 6: Walter Payton

Walter Payton (1953-1999) playing for the Chicago Bears

One of the greatest players in the history of American football, Walter Payton was known for his exceptional skill and toughness, missing only a single game during his record-setting thirteen seasons in the NFL. He was also known as a very kind and generous man off the field, earning him the nickname “Sweetness” among his fans and teammates. Twelve years after his retirement, he held a press conference announcing that he was suffering from a rare disease and would need a liver transplant to survive. His doctors commented that the condition had caused Walter to lose a lot of weight, and “wasn’t related to alcohol, steroids, hepatitis, or immune deficiency.” It also went unnoticed for years before he presented with weight loss, fatigue, and most likely pruritus. As the disease progressed he became increasingly incapacitated and needed stents placed in his common bile duct.

In his last few months, Payton become a strong advocate for organ transplantation and began several campaigns to raise awareness and encourage people to register as donors. While awaiting an organ at the Mayo Clinic, Walter was further diagnosed with a malignancy that is a known complication of his condition. After he passed away later that year, the issue of organ donation became a major headline nationwide, and there was a surge in the number of people trying to register as donors. A foundation created during his lifetime continues to fight for this cause today. What condition did he have and what was the malignancy he died from?

Answer (highlight to see):
Primary sclerosing cholangitis, complicated by cholangiocarcinoma.

Case Records of the Historical Grand Rounds:
Case 5: Ernest Hemingway

Ernest Hemingway, 1899-1961

In 1960, after a 40 year career as an inspired author and war reporter, Ernest Hemingway arrived at the Mayo Clinic seeking treatment for his severe depression. Treatments were limited at the time, though his case was so severe that his doctors decided to try several rounds of electric shock therapy. Unfortunately their attempts did more harm than good, and only served to worsen his already declining memory, ending his writing career for good. They also happened to discover that Hemingway had a whole host of other medical problems that demanded attention. For many years he suffered from severe arthritis that was particularly advanced for a man of his age. He also had a long history of diabetes and had clear signs of cirrhosis. At the time his doctors thought years of alcoholism were likely responsible, though no unifying diagnosis was made. During his two month stay at the Mayo Clinic, he was also diagnosed with a heart condition that today would likely be labeled "congestive heart failure with preserved ejection fraction".

In 1961, Hemingway returned for another round of electric shock therapy which was again ineffective. Later that year he committed suicide as a result of his longstanding depression, chronic pain, and declining mental capabilities. While none of his individual problems are particularly rare, medical records released 30 years later reveal a single unifying diagnosis made shortly before his death. It is something surprisingly common among those of Northern European heritage, and may even have contributed to his depression and cognitive decline. What is it? Also, which characteristic type of arthritis may he have had?

Answer:
Hemochromatosis (diabetes, cirrhosis, restrictive cardiomyopathy, iron deposits in the brain). These patients are particularly prone to pseudogout.

Case Records of the Historical Grand Rounds:
Case 4: Eleanor Roosevelt

 

Eleanor Roosevelt (1884-1962) serving as UN ambassador

Undoubtedly one of the most prominent and influential women in American history, Eleanor Roosevelt became known as the "First Lady of the World" for her human rights work and extensive social activism. She also helped form the foundation of FDR's career, and was instrumental in his reentry into politics following his paralysis from poliomyelitis. The disease that ultimately lead to her death is somewhat complicated and controversial, so for the sake of objectivity I will present it from the perspective of a physician seeing her during a hospital stay in July, 1962. All details are public record of course.

A 78 year-old woman with a history of aplastic anemia (diagnosed in 1960) presents with a chief complaint of fever. For the past four days she has recorded fevers up to 104F along with chills and night sweats. The fever has been persistent and began two days after a routine blood transfusion. She has noticed low-grade fevers after transfusions in the past but nothing this severe. She also endorses a non-productive cough that has been worsening over the past 2-3 weeks, as well as weakness, fatigue, and easy bruisability that have been worsening over the past four months. Review of systems is otherwise negative. She has been receiving regular blood transfusions for the past year to treat chronic macrocytic anemia and thrombocytopenia. She was started on 20mg prednisone three months ago to help with her increased symptom burden and "stimulate the growth of red blood cells and bone marrow." Her most recent hemoglobin was 7.9 and a bone marrow biopsy from last year showed hypocellularity with 5% myeloblasts. She has no known sick contacts or recent travel, but has been to the middle-east and sub-Saharan Africa several times over the past decade.

On exam the patient is febrile (104.5F) and appeared fatigued with scattered ecchymoses and conjunctival pallor. Examination of the heart, lung, and abdomen revealed no abnormal findings. There was no photophobia or neck stiffness. Laboratory findings included pancytopenia and an ESR of 128. Chest x-ray done shortly after admission showed scarred lungs but no signs of acute infection. Blood cultures were drawn and the patient was placed on empiric penicillin and streptomycin, though they were discontinued on day 4 as cultures showed no growth. Repeat bone marrow aspiration showed no changes from her previous study. Six days after admission the patient's fever went down to 99F and an extensive conversation was held regarding goals of care. The patient said she would never want to die in a hospital and expressed an interest in returning home. She was subsequently discharged with a diagnosis of "Fever of Unknown Origin".

While there is a wide differential for Eleanor's acute presentation, which single diagnosis explains her entire medical history? What test would you have sent (in 1962) to confirm it?

Answer (highlight to see): 
Diagnosis: Miliary tuberculosis with bone marrow infiltration 
Test: acid fast smear and culture from a bone marrow biopsy

Rheumatology (2nd Edition)

1. Nobel laureate Dorothy Hodgkin was one of the great British scientists of the 20th century. Her discovery of protein crystallography gave her the tools to describe the structure of many key biomolecules for the first time, including insulin and vitamin B12. The picture below illustrates a classic physical finding of her rheumatic disease, the extent of which is rarely seen in our age of disease-modifying drugs.

Dorothy Hodgkin, 1910-1994

2. Once again we visit the medical records of King George the VI. As mentioned in a previous post, one of the ways he dealt with his famous speech impediment was chronic cigarette smoking. This eventually lead to severe COPD, lung cancer, and a total left lung resection. In his last years he also suffered from episodes of pain, rash, and signs of decreased perfusion in his feet. During one episode in 1949, the arterial occlusion was so severe that his doctors severed his lumbar sympathetic nerves to avoid having to amputate his leg. The procedure was a relative success, and the king retained the use of his limbs until his death from coronary artery disease in 1953. Apart from atherosclerosis, what possible disease was the king suffering from?

3. In 1665, Rembrandt painted a portrait of fellow Dutch artist Gerard de Lairesse. Little is known about the medical history of this man, but the painting shows a very unique physical finding. What are two rheumatic diseases that could lead to this deformity, and what did he most likely have?

Gerard de Lairesse, 1641-1711

4. The annals of ancient medical history do not offer a wealth of rheumatology cases. However, one passage from Hippocrates gives a very clear description of a specific autoimmune disease. It wasn't until 1937 that the modern medical community fully described the condition.

"There were other fevers also, which I shall describe in due course. Many had aphthae and sores in the mouth. Fluxes about the genitals were copious; sores, tumours external and internal; the swellings which appear in the groin. Watery inflammations of the eyes, chronic and painful. Growths on the eyelids, external and internal, in many cases destroying the sight, which are called "figs". There were also often growths on other sores, particularly in the genitals. Many carbuncles in the summer, and other affections called "rot". Large pustules. Many had large tetters (herpetes)."

Hippocrates refusing the gifts of artaxerxes, 1792. by Anne Louis Girodet

5. In the later years of his acting career, Sir Laurence Olivier's poor health forced him to take smaller roles in "undistinguished films" due to his extreme weakness and fatigue. In 1974, he spent over three months in the hospital due to proximal muscle weakness and pain. After he recovered, he resumed his acting career, and a still from his 1976 movie Marathon Man shows the remnants of a subtle skin finding that may point to his diagnosis. What is it?

Take a close look at the hands.

6. Opera singer Maria Callas also had her career interrupted by this autoimmune disease. It began in 1958 during a performance for the Italian President. She was forced to leave during intermission because of weakness in her vocal chords and a sudden inability to control fluctuations in her voice. She was initially diagnosed with acute tracheitis, but as this became a chronic problem her personal doctor also noted her chronic generalized hypotonia, somnolence, and red discolorations around the base of her neck. She began symptomatic cortisone injections, but many critics commented on her gradual vocal decline and unsteadiness. She ultimately succumbed to cardiovascular complications of the disease (or possibly treatment) at the age of 53.

One of her most celebrated arias, from the opera Norma

7. One more exercise in physical diagnosis:

Michael Jackson, 1958-2009

 Answers: (highlight to see)
1. Rheumatoid Arthritis
2. Thromboangiitis Obliterans (Buerger's Disease)
3. Relapsing polychondritis, granulomatosis with polyangiitis. Most likely congenital syphilis.
4. Behcet's disease
5. Dermatomyositis - Gottron papules
6. Dermatomyositis
7. Malar rash (Discoid lupus in this case)

Case Records of the Historical Grand Rounds:
Case 3: Georgiana Cavendish, Duchess of Devonshire

Georgiana Cavendish, 1757-1806

Today we will discuss a condition that received prominent mention in our anatomy lectures, despite there being almost no reported cases of it in over 50 years. Georgiana, Duchess of Devonshire became a commanding presence in the realm of politics, fashion, and high society at a time when women were seldom known to do so. At the age of 17 she married the most powerful Duke in England and immediately became a social celebrity. This was all up until the Duke grew tired of their marriage and took her best friend as his mistress, sending Georgiana into a downward spiral that included a string of scandalous affairs, crippling debts, and a whole host of other social embarrassments. This is all addressed quite decently in the movie The Duchess (2008).

These hardships were made worse by an eye problem that struck her at the age of 35. Georgiana often complained of migraine-like headaches and vague eye pain, but one night she went to bed with a headache that developed into excruciating right eye pain and swelling. She also complained of nausea, vomiting, and photosensitivity. Apparently her screams were so loud  her children had to be transferred to a different estate. Three or four days into the episode she lost the ability to move the right eye and was permanently confined to a dark room. It is unclear whether or not she had a fever though there were definite chills and diaphoresis, which worsened with the development of a corneal ulceration. The left eye was also mildly swollen. The most esteemed physicians of the day were called, including the "Senior Surgeon-Extraordinary to the King", who recommended attaching leeches to the eye and partial strangulation to flush the eye of excess blood. These efforts were not terribly successful. Her mother Lady Spencer described her appearance: (read carefully...)

"The inflammation has been so great that the eye, the eyelids and the adjacent parts were swelled to the size of your hand doubled, and projecting forward from the face. Every attempt was made to lower this inflammation so as to prevent any ulceration, but this has been in vain. A small ulcer has formed on top of the cornea and has burst, and as far as that reaches the injury is not to be recovered. If the inflammation should increase, the ulcer form again, and again burst, it would destroy the whole substance of the eye, which would then sink. The eyelids are still much swelled and scarred with the leeches, and the little opening between them is always filled with a thick white matter... The eye itself, to those who see it (for I cannot) is still more horrible."

Lady Spencer to Ms. Sarah Trimmer, August 4th, 1796

Over the course of several weeks the swelling and headaches subsided, but the Duchess could still only make out shapes with the affected eye and needed to be kept in a dark room for months. She became increasingly frail over the next few years and had recurrent bouts of fever and headache. Charles Darwin's grandfather, Erasmus Darwin, even tried treating her with electric shock therapy to help with her vision, unfortunately without much success. Some wrote that she managed to re-enter society with great courage and a new-found confidence, though she never fully regained her vision or former appearance. She died of complications from a liver abcess at the age of 48. 

So what pathological event do you think was the most likely cause of the Duchess's eye problem?

The Duchess famously used her allure for political gain, trading kisses for votes at election rallies

Answer: (highlight to see)
Cavernous sinus thrombosis (orbital cellulitis is a less likely cause of exophthalmos and chemosis)

Case Records of the Historical Grand Rounds:
Case 2: Miles Davis

Regarded as one the most influential musicians of the 20th century, Miles Davis epitomized the style, individuality, and improvisation commonly associated with jazz music. The son of a wealthy dentist, he was sent to study music at Julliard Academy in the 1940s, but left after growing tired of their emphasis on Classical style. He went on to have a successful but turbulent career that was interrupted on several occasions by his numerous medical conditions. In addition to suffering from poorly controlled type II diabetes and several bouts of alcohol and drug abuse, he began having crippling joint pain in his late twenties that forced him to temporarily retire from public performance. By the 1960s he was playing in almost "constant pain" and frequently self-medicated with alcohol and narcotic drugs. His later years were also marked by severe back pain, episodes of abdominal pain leading to a cholecystectomy, and a hip replacement for a "collapsed segment of bone". He died in 1991 after a hospitalization for a stroke lead to a rapidly-progressing episode of pneumonia. Throughout his life he rarely spoke about his illness and only mentions it briefly in his autobiography. Which congenital condition ties all of his symptoms together?

Miles Dewey Davis III (1926-1991)

 

Answer (highlight to see): Sickle-Cell Anemia (Vaso-occlusive crises, avascular necrosis, gallstones, stroke, pneumococcal bacteremia)

Images in Historical Medicine: Edith Piaf

 

The famous French singer suffered from severe Rheumatoid Arthritis in her later years. Note the MCP, PIP, and wrist deformity with spared DIP joints.

Case Records of the Historical Grand Rounds: 
Case 1: A Fever of Unknown Origin

In 1935, a renowned physician named E.H. Derrick was sent to the Australian countryside to investigate a mysterious illness plaguing workers in the meat-packing industry. The patients were all men who were otherwise healthy, and the onset of the illness was similar to that of a common viral illness: fever, malaise, headache, anorexia, and muscle pains. However, over time the fever became more intense, and rose dramatically once every day or two, putting the patients into a stuporous state. In most patients the fevers lasted two to three weeks, then subsided with only supportive care. Other unusual features noted by the doctor included a slow pulse rate, severe headaches with photophobia, and weakness that lasted for months after resolution of the other symptoms. All blood cultures and agglutination tests were negative, as were separate tests for various forms of typhoid fever. Doctors also noticed that a few of the patients who recovered had mysterious episodes of culture-negative endocarditis years later. The medical community referred to the illness as "the query" for two years before the immunologist and future Nobel laureate Sir Frank Burnet discovered the etiology of the disease. What was it?

Australian pathologist Sir Edward Derrick (1898-1976)

 





















Answer: (highlight to see)
 Q fever (Q stands for Query), Pathogen is Coxiella Burnetii, named for the two doctors who discovered it.