Figure 1: One of Muzaffar al-Din Shah's last photographs.
The rivalry between the shah's French and English physicians had been going on for a long time, dating back to Dr. Joseph Désiree Tholozan's death a decade earlier. Although Schneider's overbearing manners played a large role in alienating Iranians of all classes including the shah, at the urging of the French president he was appointed Hakim-bashi. Over time, his improved attitude together with his substantial clinical abilities caused him to rise in the monarch's esteem. However, all of Schneider's gains came to an abrupt end when he fell ill and had to curtail his responsibilities to the shah in May. As a result, Dr. Lindley was named Hakim-bashi, to the chagrin of the French. It was particularly troubling to the French because their influence was at a low ebb during the Constitutional Revolution, a critical period in Iran's history. The multinational consultation was seen as a way for the French to regain "influence over the weak spirit of the King." However, this would soon be no longer a matter of concern. Although the prognosis presented to the prime minister appeared upbeat and predicted "a significant survival time," the French knew that the shah "would not survive the winter," and the crown prince would arrive in Tehran with his own well-established French Hakim-bashi, Dr. Coppin.
Muzaffar al-Din Shah's primary illness had been a hereditary form of gout, a condition whose symptoms he had struggled with throughout his life. Gout is a disease that is caused by an excess of uric acid in the body or hyperuricaemia. Uric acid is a byproduct of the body's normal metabolism; however, higher levels of this chemical can be part of the inherited makeup of some families such as the Qajars. The excess uric acid in the blood may deposit in the joints, eventually forming needle-like crystals that lead to the excruciating arthritic pain that characterizes acute gouty attacks. The image of Muzaffar al-Din Shah as a bedridden monarch probably emerged as a result of his inability to move during these frequent flare-ups. Uric acid may also collect under the skin (as tophi) or in the urinary tract as kidney stones, which also plagued Muzaffar al-Din. Kidney stones manifest themselves by sudden excruciating pain that starts in the small of the back, under the ribs, or in the lower abdomen, and which at times radiates to the groin. Muzaffar al-Din's attacks lasted for minutes or hours, followed by periods of relief which probably included blood in the urine as well as nausea and vomiting. Repeated trauma as a result of a lifetime of kidney stone formation, which Muzaffar al-Din Shah experienced, could lead to renal scarring and other damage which could conceivably bring about kidney failure. However, it was the overproduction of urate secondary to the monarch's gout that was most responsible for the acute progressive deterioration in his renal function. Indeed, in a separate evaluation, Muzaffar al-Din Shah was diagnosed with "Bright's Disease" by Dr. Schneider, his French physician.
Figure 2: Portrait of Muzaffar al-Din Shah and Premier Abd al-Majid Mirza, Ayn al-Dawleh
Muzaffar al-Din's progressive renal failure had consequences for other bodily systems, which might explain some of his behavior. One undocumented repercussion of Muzaffar al-Din's stones and progressive renal failure was a probable anemic condition which explained his perpetual fatigue. This anemia could have been brought about by both microscopic blood loss from his kidneys (hematuria) due to the trauma of stone passage and from the progressive decrease in erythropoetin production due to renal failure and subsequent congestive heart failure. Erythropoetin is a chemical substance produced by the kidneys that serves as the primary stimulant for red blood cell production by the bone marrow. A decreased erythropoetin production inevitably leads to anemia in a patient, a condition which usually brings lassitude, easy fatigability, and a lack of motivation, all of which characterized Muzaffar al-Din's behavior, particularly the last years of his life. Though hypertension is a common complication of renal disease, the most severe outcome of Muzaffar al-Din's renal failure was the damaging effect it exercised on his heart, namely congestive heart failure (CHF). This condition is a malfunction of the contractile properties of the heart leading to lower than normal cardiac output. The physical manifestation of this condition is a widespread swelling in the body due to the buildup of excess fluid in the tissues (edema and acities), including some flooding of the lungs which would have made it difficult at times for Muzaffar al-Din Shah to breath or to exert himself.
One of the unexpected complications or outgrowths of the shah's advanced heart failure was its potential to lead to the destruction of brain cells due to low oxygenation (hypoxic encephalopathy), with resultant irritability, loss of attention span, and restlessness, which also characterized Muzaffar al-Din's lack of interest in the affairs of state. Making matters worse, cardiac disease is a major risk factor for stroke, ranking third after age and hypertension. Considering the report's neurological findings, which cited the Shah's left-sided paralysis, his speech problems, his problems ambulating (lumbering gait), exaggerated reflexes, and "+ Babinski" there is significant evidence pointing to a history of severe stroke.
Even the best physicians can sometimes make errors in recording information, and historians should always rely on other resources to corroborate their findings. In this case, a photo of Muzaffar al-Din Shah from the last months of his life does show the seated monarch with a drooping eyelid and face, together with a limp arm (held by the other hand), and a leaning body (see Fig. 1) consistent with a partial paralysis. The portrait of Muzaffar al-Din Shah and Premier 'Abd al-Majid Mirza 'Ayn al-Dawla also clearly illustrates the severity of the shah's acities as indicated by his protuberant abdomen (Fig. 2).
Finally it should be acknowledged that written reports and pictures cannot replace an actual clinical evaluation. However, while admitting that medical and historical evaluations might have flaws associated with the examining physician's skills and the fund of medical knowledge available at the time, it is nevertheless incumbent upon diplomatic historians to make use of available materials and evaluate events accordingly.
Copy of the Real [consultation] on October 1st 1906 (Fig. 3)
Which was handed to His Highness the Sadr-i Azam
Figure 3: Copy of the Real [consultation] on October 1st 1906 Which was handed to His Highness the Sadr-i Azam enclosed in: AMAE), Correspondance Politique et Commerciale (Nouvelle Serie) 1897-1918 (CP), Perse, Francais au Service de Perse: médecins dossier personelles 1896-1905 Volume #57, Dossier 6A. Dépeche (direction politique n. 72) Le Ministre de France á Tehran á Son Excellence Le Ministre Des Affaires Étrangeres á Paris. Téhéran, 30th October, 1906.
The undersigned physicians, remitted in consultation on the first of October 1906, after having carefully examined His Imperial Majesty the Shah, and having read the different documents and analysis concerning his august health for the past eleven years are of opinion:
Renal: They are beset by chronic nephritis, of interstitial predominance, secondary to hereditary gout and the old and aggravated effects of trauma which resulted from the constant passage of the detritus of uric acid and even the accidental passage of voluminous calculi.
Cardiac: It [the heart] presents with a considerable hypertrophy and dilatation resulting from the backup due to the renal illness. There is a sclerosed artery with arterial hypertension which presents much resistance. The edema, which started 15 days ago, currently occupies the two lower extremities, the scrotum and the peritoneum (acities). At the bilateral bases of the lungs we detect several rales without crepitus.
A complete left hemiplegia (including the face) with speech impediments that surfaced 5 months ago. Currently we can ascertain: the force has nearly totally returned in the members. There still exists a little paresis of the face. A lumbering gait [is observed]. There is no sensory deficiency. The knee reflex is exaggerated. There is a positive Babinski sign. All these symptoms prove the organic nature of the lesion.
The state of health of His Majesty is serious, but because of His Majesty's resistance, it can be hoped that with a treatment and an appropriate diet, an amelioration and a significant survival [time] can be obtained.
Tehran the 1st October 1906
Signed by: The Doctors Schneider, Wishard, Sadovsky, Scott, George, Regling.
Have approved the diagnosis and prognosis: Doctors Hakim ol-Molk, Nazem ol-Atteba, Bagher Khan, Moadeb od-Dowleh.
Have refused to take part in or to sign the composition of this transcript: Doctors Alam ed-Dowleh and Dr. Lindley.
They contented themselves to sign the benign and consoling consultation that was voluntarily inaccurate destined for His Majesty the Shah.
AMAE, Correspondance Politique et Commerciale (Nouvelle Serie) 1897-1918 (CP), Perse, Francais au Service de Perse: médecins dossier personelles 1896-1905 Volume #57, Dossier 6A. Dépeche (direction politique n. 60) Le Ministre de France á Tehran á Son Excellence Le Ministre Des Affaires Étrangeres á Paris. Téhéran, 16th August 1906.
AMAE, Correspondance Politique et Commerciale (Nouvelle Serie) 1897-1918 (CP), Perse, Francais au Service de Perse: médecins dossier personelles 1896-1905 Volume 57, Dossier 6A. Dépeche (direction politique n. 762) Le Médecin 1re Classe Schneider á Monsieur Le Ministre Des Affaires Étrangeres á Paris. Téhéran, 15th July, 1906.
AMAE, Correspondance Politique et Commerciale (Nouvelle Serie) 1897-1918 (CP), Perse, Francais au Service de Perse: médecins dossier personelles 1896-1905 Volume 57, Dossier 6A. Dépeche (direction politique n. 72) Le Ministre de France á Tehran á Son Excellence Le Ministre Des Affaires Étrangeres á Paris. Téhéran, 30th October, 1906.
Other causes of hyperuricaemia include high intake of foods containing purine (an amino acid), which is abundant in certain meats, seafood, dried peas, and beans. Because of the foods in which purine is found, this disease has been frequently associated with a "rich" diet common to the more aristocratic strata of society. Other causes of hyperuricaemia include obesity, high alcohol intake, certain analgesic and blood-pressure medications, as well as longstanding kidney disease which can lead to an accumulation of uric acid in the system.
Lee Goldman et. al., Cecil Textbook of Medicine Twenty-first Ed. (Philadelphia, 2000), 599.
Named after the nineteenth century English physician, Richard Bright, Bright's is an obsolete eponym for a disease of the kidneys (acute or chronic). It generally refers to nonsuppurative inflammatory or degenerative kidney diseases characterized by proteinuria and hematuria and sometimes by oedema, hypertension, and nitrogen retention. For Dr. Schneider's diagnosis of Bright's disease in Muzaffar al-Din see AMAE, Correspondance Politique et Commerciale (Nouvelle Serie) 1897-1918 (CP), Perse, Francais au Service de Perse: médecins dossier personelles 1896-1905 Volume 57, Dossier 6A. Dépeche (direction politique n. 762) Le Médecin 1re Classe Schneider á Monsieur Le Ministre Des Affaires Étrangeres á Paris. Téhéran, 15th July, 1906.
The most common regulatory defect in causing kidney failure is the body's inability to get rid of sodium. In the case of the shah, sodium retention also led to increased fluid retention, overloading his heart and leading to cardiac output malfunction.
In its worst state, this condition could progress to stupor or coma.
Cardiac disease is a major risk factor for stroke, ranking third after age and hypertension. See: M. Pullicino, et. al. "Stroke in patients with heart failure and reduced left ventricular ejection fraction," Neurology 54 (2000): 288-94.
A positive Babinski sign is characterized by an upturned big toe and the fanning of the other toes. A positive Babinski sign is always abnormal in adults (though present in children) and it usually indicates a lesion in the upper nervous system. In this case a combination of a Babinski sign together with hyperreflexia (also abnormal) and the paresis and hemiplegia all point to a lacunar stroke. Lacunar strokes are small subcortical infarctions of the deep penetrating arteries.
"The Sick Man of Persia: The Shah's Serious Illness," The Illustrated London News, December 29th 1906, 984.
While in modern times nephropathy cased by analgesic agents (phenacetin and aspirin) are important cause of chronic interstitial nephropathy and by extension chronic renal failure. However, historically and in Muzaffar al-Din Shah's case metabolic causes caused by his grout induced hyperuricemia and uric acid stones were probably the responsible agents.
Hyperuricemia, or the supersaturation of plasma and extracellular fluids with uric acid, is the hallmark of gout. Under the right conditions, excess uric acid can become crystallized and bring about some of the more dramatic clinical aspects of the disease such as acute gouty arthritis and renal acid (kidney) stones (calculi).
Rales are often described as a wet crackling sound whereas crepitus is a dryer crinkly, crackling, or grating sound of the lungs. These nuances can be appreciated by auscultation.
A complete hemiplegia is total paralysis of the arm, leg, and trunk on the same side of the body.