A onset of fever with painful myalgia, a

A
Historical Account of Epidemic Typhus

The four postulates of the Germ Theory of Disease, which
states that diseases are caused by microorganisms, that had been laid down by
Robert Koch in the late 19th century were a remarkable advancement
in the field of Microbiology, and provided an understanding of the cause, and
possible cure of various diseases.

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Known by various synonyms such as camp fever, jail fever,
famine fever, putrid fever, louse-borne typhus, etc., the infamous bacterial
disease typhus has been responsible for various epidemics in history. As the
names suggest, these epidemics tend to follow conditions when social
organisation is disrupted, and public health and personal hygiene is compromised.
Epidemic typhus is caused by the bacterium Rickettsia prowazekii, and
transmitted by Pediculus humanus humanus, the body louse, as discovered
by Charles Nicolle in 1909.

Manifestations:

Epidemic typhus is a life-threatening, acute exanthematic
feverish disease. Once in the victim’s blood stream Rickettsia
prowazeki begins to multiply. About twelve days after infection, the
clinical symptoms of the disease begin. It is primarily characterised by the
abrupt onset of fever with painful myalgia, a severe headache, malaise and a
rash. Non-specific symptoms sometimes include a cough, abdominal pain, nausea
and diarrhoea. The rash, which is a characteristic of typhus fever, appears on
the abdomen a few days after the onset of the symptoms, and radiates centrifugally
to the extremities. Gangrene and necrosis of the extremities, leading to the
need for amputation, might also be observed. The term typhus comes from the Greek word “tuphos”, meaning “smoke” or
“stupor”, evoking the delirious state that infected individuals may develop. Neurologic
symptoms include confusion and drowsiness. Coma, seizures and focal neurologic
signs may develop in a minority of patients. In self-resolving cases, R.
prowazekii can persist for life in humans, and under stressful conditions
recrudescence may occur as a milder form of Brill–Zinsser disease. R.
prowazekii bacteraemia occurs in Brill–Zinsser disease so it can initiate
an outbreak of epidemic typhus when body lice are present on the infected
individuals.

History:

The first written recordings of typhus came in 1489 during
the Spanish army’s siege of Granada. An epidemic of louse-borne typhus had struck
the Spanish army, and within a month had killed 17,000 of the original 25,000
soldiers. Of the total war causalities, only 3,000 Spaniards had died in actual
combat. The remnants of the Spanish army fled, and in so doing, introduced
typhus to many other parts of Europe.

In 1546, Girolamo Fracastoro,
a Florentine physician, described the pestilential disease in his
famous treatise on viruses and contagion, De Contagione et Contagiosis
Morbis.

In 1759 authorities estimated nearly 25% of English
prisoners lost their lives to this disease, thus earning the name gaol or jail
fever. Huddling together of prisoners in dark, cramped jail cells, coupled with
unhygienic conditions such as failure to bathe and change one’s clothes daily
catalysed the proliferation of Rickettsia infected body lice, and the inevitable spread of typhus.

Typhus had also afflicted the soldiers of Napoleon’s Grand
Army in Vilnius in 1812 after their retirement from Russia. It is estimated that
fewer than 100,000 French soldiers lost their lives to Russian soldiers, while
as many as 300,000 French soldiers perished from typhus.

Nicolle’s
findings in 1909 put an end to the mystery behind the occurrence of the
disease. However, research and control techniques had not advanced rapidly
enough to prevent major outbreaks of typhus on the Eastern Front during the
First World War. Austria’s declaration of war on Serbia (Yugoslavia) following the
Archduke Ferdinand’s assassination was immediately followed by an all-out
invasion of Serbian soil by Austrian forces. Over-crowding, a dearth of
sanitary facilities and hospitals, and the general panic caused by the Austrian
invasion prepped the stage for the proliferation of infected body lice.

In November 1914, typhus made its first appearance
among refugees and prisoners, and it then spread rapidly among the troops,
leaving the entire
country helpless amidst one of the worst epidemics in history. It killed
200,000 Serbian soldiers on the front, almost one-quarter of their entire armed
forces, and almost 30,000 of the 60,000 Austrian prisoners captured in the
counterattack. The mortality rate reached an epidemic peak of approximately 60
to 70%. This dramatic situation dissuaded the German-Austrian commandment from
invading Serbia in an attempt to prevent the spread of typhus within their
borders.

Drastic measures were taken, such as the quarantine of
people with the first clinical signs of the disease, but attempts were also
made to apply standards of hygiene among the troops to prevent body lice
infestations. On the Western Front, although body lice were also endemic among
the troops, there was no outbreak of typhus. The situation lacked the R.
prowazekii bacteraemia to trigger a typhus epidemic, as had happened on the
Eastern Front.

On the Russian front, throughout the last two years of the
conflict and during the Bolshevik revolution, approximately 2.5 million deaths
were recorded. Soldiers
and refugees imported typhus and propagated it across the country. It was
during the hard winter of 1917– 1918 that the biggest outbreak of typhus in
modern history began in a Russia that was already devastated by famine and war.

As World War II raged, typhus reappeared in war-torn areas
and in Jewish ghettos, where cramped, harsh conditions were a perfect breeding
ground for lice. Thousands of prisoners held in appalling conditions in Nazi concentration
camps such as Auschwitz, Theresienstadt, and Bergen-Belsen also died of typhus
during World War II, including Anne Frank at the age of 15 and
her sister Margot. The Nazis employed Dr. Rudolf Weigl to produce a typhus
vaccine. Weigl created a technique that involved raising millions of infected
lice in a laboratory and harvesting their guts to get the materials for a
vaccine. He had allowed infected lice to feed on volunteers, allowing R. prowazekii to propagate in the louse gut, and then use
phenol-buffered suspensions of the typhus engorged intestine as vaccines.

In 1938, Herald Cox, a
bacteriologist working for U.S Public Health Service, cultivated the bacteria
in the yolk sacs of chick embryos, and by doing so, laid down the foundation
for advanced typhus vaccine development. Although vaccines created from louse
feces, or killed, typhus-infected vectors were created prior to the work of Cox,
their methods were labour intensive, and ineffective for large scale
production. Cox made the first rickettsial vaccine using formalin-killed
suspension of yolk derived R. rickettsiae, and through the chick embryo
culture system was able to produce large enough quantities of the antigen for
commercial production.

 

Today, the diagnosis of typhus is
based on the patient’s clinical history, physical exam, and tests based on identification
of the bacterial genus and species by PCR testing of a skin biopsy from
skin rash or lesions, or blood samples. Immunohistological staining can
identify the bacteria within infected tissue. Typhus can also be diagnosed,
usually late or after the disease has been treated, with antibiotics, when
significant titers of anti-rickettsial antibodies are detected by immunological
techniques. Mortality is low in children under fifteen years of age, but
usually ranges from ten to hundred percent in adults, increasing with age. With
good supportive care and early judicious use of antibiotics such as the
tetracyclines, quinilones, chloramphenicol, and paraaminobenzoic acid, the risk
of a fatal issue is greatly reduced.

Conclusion:

Epidemic typhus is an
unpredictable disease, and may resurface at times of both, man-made and natural
catastrophe. Not only had each of the mentioned occurrences caused great
devastation and loss of human life, but had also, potentially, affected the
course of several wars, and had played a key role in shaping history. The knowledge
of seemingly harmless vectors causing such destruction horrifying when one
thinks of the potential use of body lice as bio-weapons. Epidemic typhus is
still characterised as a “disease under surveillance” by the World Health
Organisation. Thus, the eradication of poverty, famine, and warfare are
not only political ideals, but medical and historical ideals as well, and the
prevention of future outbreaks lies in the control of the proliferation and
spread of bacteria infected lice.