S8 E2: Brain Suckers: The Story of Naegleria fowleri

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Hello and welcome to Cause of Death
one hundred seconds to midnight. I'm your

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host, Jackie Morante. This season
is called Creepy Crawley's and today I'm going

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to be talking about one of the
strangest and most dangerous among them, Nicgaleria

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faleri. This parasitic amba was suggested
to me by my friend at Unsolved Virginia.

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Anne. Thanks for the suggestion is
a good one. This animal is

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the brain eater that's made such a
huge splash pun intended in the media over

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the last couple of years. Let's
begin with ediology and pathology. Amibe are

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unicellular eukaryotic organisms. I've talked about
eukaryotic organisms before, but I'm going to

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refresh your memory. Eukaryotic cells have
a true nucleus. This nucleus holds the

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DNA of the organism. The nucleus
is contained within a membrane and separated from

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the other cellular structures. Eukaryotes include
animals, plants, fungi, and protus

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such as algae and we as humans
are eukaryotes. Amibae belonged to the domain

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Eukaryota, the kingdom Protista, the
phylum Protozoa, the class Rhizopoda, the

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order Amibida, and the family Amibidae. The structure of amiba consists of a

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cytoplasm surrounded by a cell membrane.
The outer portion of the cytoplasm, called

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the ectoplasm, is clear and gel
like, the inner portion of the cytoplasm,

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called the endoplasm, is granular and
contains organelles, including the nuclei,

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mitochondria, and vacuoles. These vacuoles
digest food and expel excess water and waste

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from the cell through the plasma membrane. Amibae uptake food by a process called

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phagocytosis. I talked about this in
a very early More Than You Ever Wanted

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to Know episode in the first season. Mammals have white blood cells called phagocytes

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that get their nutrients this way.
The process of phagocyteosis consists of an organism

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consuming their food whole through vacuoles,
then digesting it and casting off waste through

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other vacuoles. Amieba form pseudopodia or
false feet. The pseudopodia allow the organism

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to move and capture bacteria, algae, and other microorganisms in their environment.

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The vacuoles then surround the food source
and internalize it. As the food is

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digested, the vacuole dissolves. If
you're interested in this particular form of nutrient

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uptake, head to YouTube and look
up phagocytosis. It's pretty fascinating, but

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of course you have to be a
geek to really appreciate it. Amibae don't

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have respiratory organs. Respiration occurs as
dissolved oxygen in the water diffuses across the

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cell membrane. Carbon dioxide is then
diffused across the membrane back into the water.

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Amiba process water across the plasma membrane
bi osmosis, and excess water is

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then expelled through vacuoles. These organisms
reproduce by binary fission. Binary fission is

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a type of asexual reproduction typically observed
in prokaryotes and a few single celled eukaryotes,

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in this case amiba. In this
method of asexual reproduction, there is

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a separation of the parent cell into
two new daughter cells, Amiba can live

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in fresh water or saltwater environments,
wet soil, and some parasitic amiba inhabit

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animals and humans. I'll talk about
one of these later in the episode.

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This brings us to our amoeba of
choice for today's episode, Negliaria filare.

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The classification of Negliara filere differs slightly
from other amibe. It belongs to the

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domain Eukaryota, kingdom Excavada, phylum
Perclozoa, class Heterolobosia, order Schizopyrneida,

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and family Valcamphilidae. There are three
free living amibe that infect people and Canthomiba,

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Valamuthium manrolleris and Negleia phileare. This
means that these amibae are not dependent

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on a host for survival, but
can live freely in the environment. They

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are opportunistic pathogens, meaning they don't
necessarily want to make a mess of things,

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but if the opportunity arises, they'll
reacavoc enfilare is a thermophilic organism that

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prefers temperatures between eighty and one hundred
and fifteen degrees fahrenheit and can live for

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short periods of time at higher temperatures. Thermophiles love heat and generally live in

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areas with higher temperature ranges. By
contrast, cycrofiles and cryophiles love the cold

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and generally thrive in areas with lower
temperature ranges. While these organisms can exist

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in temperate areas, they usually go
into a state of dormancy until the temperature

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reaches their preferred level. These organisms
are called extreme of files. They thrive

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in extreme climates. I'll be doing
a whole season on extremo files in the

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future. They are really interesting organisms, but for now, let's get back

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to n FILOWERI. N Filowery is
typically found in warm freshwater lakes and river

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hot springs, warm water discharge from
industrial or power plants, untreated geothermal drinking

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water sources, or recreational venues such
as swimming pools, splash pads, or

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surf parks that don't have enough chlorine
or are poorly maintained. They can also

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be found in tapwater, water heaters, and soil or sediment at the bottom

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of lakes, ponds, and rivers. While this organism thrives in temperatures above

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eighty degrees fahrenheit, it can be
found in lake or river sediment at temperatures

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well below that. There is no
universal definition of a rare disease, but

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the Rare Disease Act of two thousand
and two defines it as a disease that

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affects fewer than two hundred thousand people
in the US. NFLRARI falls into this

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category in the US the United States, between twenty thirteen and twenty twenty two,

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a total of twenty nine infections were
reported in the US. Most of

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these infections happen in young boys under
fourteen years of age. There is no

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definitive reason for this, except that
boys are more likely to dive into the

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water and play in the sediment of
lakes and rivers. Infection with en philari

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most commonly occurs in the summer months
of July, August, and September,

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when the water temperatures are high and
the water levels are low. En Philari

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causes primary amobic meningo and cephalitis or
PAM. PAM occurs when the patient inhales

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fresh water from a lake or pond
where the amiba resides. Once it enters

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the nasal cavity, the amiba penetrates
the respiratory epithelium and olfactory mucosa. It

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then migrates through the cribiform plate into
the central nervous system. Its virulence depends

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on the protein NFA I, nitric
oxide production and poor forming proteins. NFA

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one mediates the attachment to target cells. Then the pseudopodia begin to move across

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the brain, ingesting tissue. As
it moves along, cysteine proteases, phospholipases,

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and phospholipolytic enzymes mediate the extensive necrosis
as an immune response, but this,

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in combination with the virulence of the
organism, lead to destruction of the

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parenchymal tissue. The olfactory bulbs,
basaler portion of the frontal cerebri and cerebellum

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tend to be the most severely affected
by PAM. Symptoms of PAM are similar

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to those of bacterial meningitis. Symptoms
begin about five days after infection and include

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fever, headache, nausea, and
or vomiting. The disease progresses to stiff

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neck, confusion, lack of attention
to people and surroundings, seizures, hallucinations,

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and coma. This disease progresses vary
rapidly, and death normally occurs within

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five to ten days of the onset
of first symptoms. The mortality rate for

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PAM is roughly ninety seven percent,
with only four people out of one hundred

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and fifty seven having survived in the
US between nineteen sixty two and twenty twenty

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two. Treatments are available for PAM, but because the disease progresses so quickly

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and infection is rare, diagnosis often
comes too late. PAM is not often

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considered as a likely diagnosis when a
patient shows up at the er. There

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are real time PCR tests for nfilari, but they aren't readily available at most

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hospitals, and sending samples to a
reference laboratory would take more time than the

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patient has. Any delay in treatment
can be a matter of life or death.

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PAM is currently treated with a combination
of amphotersin b A, zythromyacin,

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fluconazol, refampin, miltiphosine, and
Dexamethisone these drugs have been used in patients

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who have survived. Miltaphosine is the
newest of these drugs and it has been

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shown to kill Nfilari in the laboratory. However, it is an experimental drug

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that is only available for PAM infections
through the CDC. In the United States,

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Enfilari infections have occurred mostly in the
southern states such as Florida and Texas.

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However, climate change is affecting waterways
in the US and this has caused

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a shift in the geography of infections. Since twenty ten, cases of PAM

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have been confirmed in Nebraska, Iowa, Minnesota, Indiana, Maryland, and

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northern California. PAM has also been
known to infect livestock and wildlife that drink

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from water sources where en Filere exists. Let's take a break to hear from

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PAM was first identified in nineteen sixty
two in the state of Florida.

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The name en Fialari was given to
the amiba after the discovery of PAM in

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Australia in nineteen sixty five by Malcolm
Fowler, who first described this disease.

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CG. Butt named the disease caused
by the amiba primary amobic meningo cephalitis in

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nineteen sixty six. Then it was
again christened PAM by R. F.

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Carter in nineteen sixty eight in order
to differentiate this disease from another amobic invasion

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caused by Entamiba histolytica. Let's take
a left turn at Albuquerque to talk about

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antamba histalytica without going too far into
the weeds on this one. Antamba histolytica

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is a single celled parasite that causes
amibiass The classification of Antamba histalytica is domain

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Eukaryota, the kingdom Protista, the
phylum Sarcomastigophora, the class Rhizopoda, the

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order you Amymida, and the family
Antamba. Amibiasis is common in regions of

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the world where sanitation is poor.
The symptoms include loose stools, stomach pain,

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and stomach cramping. Amibic dysentery is
a more severe form, with symptoms

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that include stomach pain, bloody stools, and fever. In rare instances,

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it can spread to the liver and
cause abscesses. It's very rare that this

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disease will spread to other organs such
as the brain, and antibiotic treatment generally

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takes care of it. Only about
ten to twenty percent of people infected with

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Ntomba histolytica become ill, meaning that
most people who have this disease are asymptomatic.

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Amibiasis travels through the fecal oral route, so ingestion of fecal matter from

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those who have the disease is the
most common way to get it. It's

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a totally different disease and boasts the
mortality rate of less than one percent,

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with most patients making a full recovery
through treatment. The main difference between ntomba

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histolytica and N. Filari is that
Ehistolytica relies on a human host to complete

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its life cycle. This is an
obligate parasite that doesn't live freely in the

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environment. Okay, so that's the
long and short of eistolytica. You get

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two firs in this episode. Let's
get back to PAM. Cases of PAM

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have been reported from fifteen countries around
the world. Most are reported from Europe,

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Australia, and the US. Annually, there are between zero and eight

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cases reported worldwide. Historically, since
Faler first described the disease in nineteen sixty

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two, only three hundred or so
cases have been reported globally. Concerns about

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the epidemiology of the disease began to
mount after twenty ten, when a case

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of PAM was reported from Minnesota.
Additional cases were reported in twenty eleven and

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00:21:52.920 --> 00:21:57.880
twenty twelve in Minnesota, Indiana,
and Kansas, giving rise to the thought

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that the range of the amiba was
expanding due to climate change. A global

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study of the disease reported that between
nineteen sixty two and twenty fourteen, there

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have been two hundred and sixty cases. These included one hundred and thirty two

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cases from the US, nineteen from
Australia, seventeen from Pakistan, sixteen from

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the Czech Republic, eleven from India, nine from Mexico, nine from New

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Zealand, seven from Venezuela, five
each from Thailand and Belgium, four from

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00:22:36.839 --> 00:22:42.319
Nigeria, two from the UK,
and one each from Namimbia, Iran Costa

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Rica, New Guinea, South Africa
and Madagascar. Out of these, only

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eleven patients survived. In India,
confirmed cases of PAM occurred in patients who

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used groundwater puddles or ponds for bathing
and swimming. The existence of Nfialraarri has

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00:23:03.799 --> 00:23:07.839
been confirmed in pond water, swimming
pools and sewage canals of India, as

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00:23:07.839 --> 00:23:15.720
well as China, Hong Kong,
Xijiang, and Zambia. An uptick in

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00:23:15.799 --> 00:23:21.440
PAM cases in Asian countries has caused
concern for the CDC and WHO, and

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investigations are underway in those countries to
find out why. Recently, there have

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been occurrences of PAM in Pakistan due
to the ritual rinsing of the nose for

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00:23:32.640 --> 00:23:40.440
ablution using contaminated water. During two
thousand and eight, thirteen cases of PAM

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00:23:40.519 --> 00:23:45.519
were reported in Karachi over a seventeen
month period. None of these patients had

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any history of recreational activities that involved
swimming, but they all performed the regular

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00:23:52.759 --> 00:23:59.440
ablution practice using tap water. Between
two thousand and eight and twenty seventeen,

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00:24:00.079 --> 00:24:04.680
one hundred and five people in Pakistan
died from infection with n Fialari, the

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highest number being twenty two. In
twenty twelve. There have been no reported

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00:24:11.240 --> 00:24:18.440
cases outside of Karachi. An investigation
into the high rates of infection in Karachi

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00:24:18.839 --> 00:24:25.279
concluded that the numbers of free living
amiba in Pakistan is high due to the

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00:24:25.279 --> 00:24:37.279
current weather conditions related to global warming. It's surmised that as the Earth continues

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00:24:37.319 --> 00:24:41.079
to warm, Karachi will see higher
rates of PAM and the disease could spread

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00:24:41.079 --> 00:24:49.079
to other parts of Pakistan and very
possibly worldwide. We've already seen the effects

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00:24:49.079 --> 00:24:53.920
of global warming with the geographical shift
and infections farther North in the US,

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00:24:55.240 --> 00:25:00.880
and since N. Filari exists in
freshwater sources worldwide with the exception of Antarctica,

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there is no doubt that as water's
warm, the number of cases of

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00:25:06.319 --> 00:25:14.119
the disease could rise. Okay,
so that's all I got on Negleria fhileerre.

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It's pretty straightforward, not a whole
lot to dig into with it.

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00:25:19.799 --> 00:25:25.279
The bottom line is that global warming
could allow this disease to become far less

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rare than it is right now.
But before I get to the part of

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00:25:29.960 --> 00:25:33.359
the episode where I beg you to
visit my sponsor or sign up for my

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00:25:33.480 --> 00:25:37.000
Patreon and all that, I want
to give you some takeaways on this disease.

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00:25:38.039 --> 00:25:44.400
Neglaria fhileerre is rare, and it
can only be contracted when the amiba

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00:25:44.599 --> 00:25:49.880
are pushed through the nasal passages into
the brain. Most of the time this

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00:25:49.920 --> 00:25:56.880
would happen while swimming or diving into
freshwater lakes or ponds. This disease is

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00:25:56.039 --> 00:26:03.839
not contagious. Human to human contact
will not pass it. It also cannot

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00:26:03.839 --> 00:26:10.759
be contracted by drinking water, no
matter how sludgy it is. In the

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00:26:10.880 --> 00:26:15.319
very beginning of this episode, I
mentioned that this Amiba can live in water

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00:26:15.400 --> 00:26:19.759
heaters. Later in the episode,
I mentioned that Karachi, Pakistan, was

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00:26:19.799 --> 00:26:26.480
experiencing higher rates of PAM due to
the ritual rinsing of the nose. This

241
00:26:26.640 --> 00:26:30.680
is a thing if you're using a
netty pot to wrench your sinuses. Use

242
00:26:30.799 --> 00:26:37.200
distilled water, not tapwater. This
is a bigger deal in rural areas where

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00:26:37.240 --> 00:26:41.000
well water is used. But just
because you live in a place with treated

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00:26:41.039 --> 00:26:47.519
water, it doesn't mean that you're
not at risk. In an isolated incident

245
00:26:47.599 --> 00:26:55.039
from Arizona, two children died of
enfilary infections from playing in bathwater. Keep

246
00:26:55.119 --> 00:27:00.920
in mind that infections with enfilary are
extremely rare, so although I'm giving you

247
00:27:00.000 --> 00:27:06.359
information on the disease and a few
preventions, don't stop swimming or water skiing.

248
00:27:07.519 --> 00:27:11.400
This disease has gotten a huge amount
of press over the last five or

249
00:27:11.400 --> 00:27:15.039
so years, and much of that
attention has only raised fear, but it

250
00:27:15.119 --> 00:27:22.279
hasn't given a whole lot of useful
information. Yes, this is a serious

251
00:27:22.319 --> 00:27:26.359
disease, but only three people in
the US get it every year. That's

252
00:27:26.400 --> 00:27:33.440
not enough to keep me from heading
to the hot springs and slaking. Thanks

253
00:27:33.440 --> 00:27:37.119
again to my friend Anne for suggesting
this episode, it was a little shorter

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00:27:37.200 --> 00:27:42.079
than usual, but there really isn't
much to say about infilary. It's out

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00:27:42.119 --> 00:27:45.839
there, yes, and it could
become more prevalent as the water's warm,

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00:27:47.480 --> 00:27:51.759
But right now, despite all the
press it gets, it's not a huge

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00:27:51.799 --> 00:27:56.640
concern. As always, research is
ongoing, but the greatest chances of survival

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00:27:56.759 --> 00:28:04.079
begin with early diagnosis entry. Thanks
to all of you for listening to Cause

259
00:28:04.119 --> 00:28:08.640
of Death one hundred seconds to midnight. This show couldn't go on without all

260
00:28:08.680 --> 00:28:14.680
of you listening in. Please share
these episodes with everyone you know so they

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can get hooked on disease research too. If you have a minute, stop

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by Apple Podcasts and rate and review
the show. It really does help the

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ratings and that helps others find the
show so they can listen too. If

264
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you'd like to help the show,
please head to my Patreon page at patreon

265
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dot com slash Jackie Morante, where
you can sign up for some great perks

266
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267
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if you really don't like Patreon.
If you'd like to get in touch with

268
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me, you can send an email
to Jackie at Cause of Death one hundreds

269
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dot net, or you can leave
a message on the website at WWT Cause

270
00:29:00.720 --> 00:29:06.160
of Death one hundred sex dot net. I always love hearing from listeners.

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00:29:07.599 --> 00:29:11.759
My sponsor for this month is Trueleaf
Market. Get your grow on with Trueleaf

272
00:29:11.799 --> 00:29:18.319
Market, where you can find all
your gardening needs. I'm hoping to do

273
00:29:18.359 --> 00:29:22.920
a really short bonus episode that could
drop next week with some updates on the

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00:29:22.079 --> 00:29:29.079
novel respiratory disease in dogs, an
update on syphilis in the US, and

275
00:29:29.160 --> 00:29:33.119
a short talk about a couple of
drugs that may or may not pass clinical

276
00:29:33.200 --> 00:29:38.920
trials. One of these drugs is
donenimab, currently under pre release for Alzheimer's,

277
00:29:40.519 --> 00:29:45.279
and the other is relivrio, which
is going through clinical trials for als.

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00:29:47.640 --> 00:29:51.359
It should be interesting and it'll give
you a little inside look at the

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00:29:51.440 --> 00:29:56.680
regulations behind the release of new drugs. The next episode in the Creepy Crawley

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00:29:56.799 --> 00:30:03.079
season is going to be on toso
plasmosis. Cat people are going to be

281
00:30:03.119 --> 00:30:08.559
outsourcing their litterbox duties. Don't get
too snarky, dog people, your episode

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00:30:08.599 --> 00:30:15.480
is coming up. Until next time, Keep your head firmly above water.