Coenurosis In Humans
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Coenurosis is a
parasitic Parasitism is a close relationship between species, where one organism, the parasite, lives (at least some of the time) on or inside another organism, the host, causing it some harm, and is adapted structurally to this way of life. The ent ...
infection An infection is the invasion of tissue (biology), tissues by pathogens, their multiplication, and the reaction of host (biology), host tissues to the infectious agent and the toxins they produce. An infectious disease, also known as a transmis ...
that results when humans ingest the eggs of dog
tapeworm Eucestoda, commonly referred to as tapeworms, is the larger of the two subclasses of flatworms in the class Cestoda (the other subclass being Cestodaria). Larvae have six posterior hooks on the scolex (head), in contrast to the ten-hooked Ce ...
species '' Taenia multiceps'', '' T. serialis, T. brauni,'' or '' T. glomerata.'' It is important to distinguish that there is a very significant difference between intestinal human
tapeworm infection Eucestoda, commonly referred to as tapeworms, is the larger of the two subclasses of flatworms in the class Cestoda (the other subclass being Cestodaria). Larvae have six posterior hooks on the scolex (head), in contrast to the ten-hooked Ce ...
and human coenurosis. Humans are the definitive hosts for some tapeworm species, the most common being '' T. saginata'' and '' T. solium'' (beef and pork tapeworms). This means that these species can develop into full grown, reproductively capable adult worms within the human body. People infected with these species have a tapeworm infection. In contrast, the four species that cause human coenurosis can only grow into mature, reproductively capable worms inside their definitive hosts,
canids Canidae (; from Latin, ''canis'', "dog") is a biological family of caniform carnivorans, constituting a clade. A member of this family is also called a canid (). The family includes three subfamilies: the Caninae, and the extinct Borophaginae ...
such as dogs, wolves, foxes and coyotes. Humans who ingest eggs from any of these four species of ''Taenia'' become intermediate hosts, or places where the eggs can mature into larvae but not into adult worms. When humans ingest these eggs, the eggs develop into tapeworm larvae that group within cysts known as coenuri, which can be seen in the
central nervous system The central nervous system (CNS) is the part of the nervous system consisting primarily of the brain, spinal cord and retina. The CNS is so named because the brain integrates the received information and coordinates and influences the activity o ...
,
muscle Muscle is a soft tissue, one of the four basic types of animal tissue. There are three types of muscle tissue in vertebrates: skeletal muscle, cardiac muscle, and smooth muscle. Muscle tissue gives skeletal muscles the ability to muscle contra ...
s, and
subcutaneous tissues The subcutaneous tissue (), also called the hypodermis, hypoderm (), subcutis, or superficial fascia, is the lowermost layer of the integumentary system in vertebrates. The types of cells found in the layer are fibroblasts, adipose cells, and m ...
of infected humans. People with coenurosis do not develop a tapeworm infection because the larvae of coenurosis-causing parasites cannot develop into worms inside of humans.


Symptoms and signs

In humans, this parasitic infection causes a variety of symptoms, depending on where the cyst occurs. The tapeworm larvae group together to form fluid filled cysts in various body tissues. These cysts start out small, but as the larvae grow, the cyst can reach the size of an egg. The cysts of ''T. multiceps'' are usually between 2 and 6 cm in diameter and are most commonly found in the CNS and can contain anywhere from a few to over a hundred worm larvae within them. ''T. serialis'' and ''T. glomerata'' cysts present in the CNS, muscles, or subcutaneous tissue, and ''T. brauni'' cysts occupy these same areas but occur in the eye more frequently than the other three species. When the cyst occurs in the brain, as it often does, the infected individual may experience neurological symptoms of headaches, seizures, vomiting,
ataxia Ataxia (from Greek α- negative prefix+ -τάξις rder= "lack of order") is a neurological sign consisting of lack of voluntary coordination of muscle movements that can include gait abnormality, speech changes, and abnormalities in e ...
, paralysis affecting one side of the body (
hemiplegia Hemiparesis, also called unilateral paresis, is the weakness of one entire side of the body ('' hemi-'' means "half"). Hemiplegia, in its most severe form, is the complete paralysis of one entire side of the body. Either hemiparesis or hemiplegia ...
), paralysis involving one limb (
monoplegia Monoplegia is paralysis of a single Limb (anatomy), limb, usually an arm. Common symptoms associated with monoplegic patients are weakness, numbness, and pain in the affected limb. Monoplegia is a type of paralysis that falls under hemiplegia. W ...
), and loss of ability to coordinate muscles and muscle movements. Many of these symptoms are due to the buildup of inter-cranial pressure from the growing cyst or from the cyst pressing on other parts of brain. When the cyst occurs in the spinal cord, it can cause severe pain and inflammation, and loss of feeling in some nerves (
arachnoiditis Arachnoiditis is an inflammatory condition of the arachnoid mater or 'arachnoid', one of the membranes known as meninges that surround and protect the central nervous system. The outermost layer of the meninges is the dura mater (Latin for hard ...
). When the cyst occurs in the eyes, it causes decreased vision and headaches. In the muscular and subcutaneous tissues, the cyst causes disfiguring nodules that can protrude out of the body. These nodules can be painful, uncomfortable, and can cause loss of muscle function.


Transmission

The definitive hosts for these ''Taenia'' species are canids. The adult tapeworms live in the intestines of animals like dogs, foxes, and coyotes. Intermediate hosts such as rabbits, goats, sheep, horses, cattle and sometimes humans get the disease by inadvertently ingesting tapeworm eggs (gravid proglottids) that have been passed in the feces of an infected canid. This can happen from ingesting food, water or soil that has been contaminated by dog feces. The disease cannot be transmitted from one intermediate host to another, but it is still not a good idea to eat meat that presents with cystic nodules from coenurosis.


Reservoir

Wild carnivores/ canids like dogs, coyotes, foxes and wolves.


Incubation period

Cysts can present in humans anywhere from a few months to a few years after ingestion. Once the cyst develops, symptoms associated with the cyst develop rapidly.


Morphology

The following are pictures of coenurosis cysts, some which have been surgically removed from humans and others that have been removed from animals after death.


Life cycle

Eggs and gravid proglottids are shed in feces into the environment by infected definitive hosts (canids). Many animals may serve as intermediate hosts, including rodents, rabbits, cattle, sheep, goats and humans. These intermediate hosts ingest the eggs. Once ingested, the eggs hatch in the intestine, releasing oncospheres. These oncospheres circulate in the blood until they lodge in suitable organs (skeletal muscle, eyes, brain, muscles or subcutaneous tissue). After about three months or so, the oncospheres develop into cystic like larvae balls called coenuri. The cycle perpetuates when a canid (definitive host) ingests the tissue of an infected intermediate host. When this happens, the maturing larvae reside in the small intestine of the definitive host until they mature into adult worms and begin producing eggs (which will eventually be passed through feces into the environment once again).


Diagnosis

Since coenurosis is very rare in humans, there are not many ways to diagnose the disease. The most common method for diagnosis involves using CT or
MRI Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to generate pictures of the anatomy and the physiological processes inside the body. MRI scanners use strong magnetic fields, magnetic field gradients, and rad ...
scans to visualize the cysts in the body. Some other clinical findings that can be used for diagnosis include
papilledema Papilledema or papilloedema is optic disc swelling that is caused by increased intracranial pressure due to any cause. The swelling is usually bilateral and can occur over a period of hours to weeks. Unilateral presentation is extremely rare. In ...
s, hypoglycorrhacia, and high
intracranial pressure Intracranial pressure (ICP) is the pressure exerted by fluids such as cerebrospinal fluid (CSF) inside the skull and on the brain tissue. ICP is measured in millimeters of mercury ( mmHg) and at rest, is normally 7–15 mmHg for a supine adu ...
caused by obstruction of the ventricles. Some serological and microscopic tests can confirm the presence of ''Taenia'' larvae once surgery has taken place and a portion of the cyst can be removed to undergo examination and biopsy. Because of the lack of specificity in diagnostic technique, coenurosis can be misdiagnosed as neurocysticercosis or echinococcosis, other parasitic diseases affecting nervous system tissue. An important consideration in diagnosing coenurosis properly is learning about the infected person’s exposure history. If the person presenting symptoms lives in an area with poor sanitation, high wild dog population, or known endemic tapeworm, his chance of having coenurosis is much higher. Also, this disease is seen more often in children than adults because children spend time outside in the mud and generally are more likely than adults to come into contact with canid feces.


Prevention

This disease has no vaccination. Preventative measures can be taken at community and individual levels. Communities and governments can make sure their water supply remains sanitary and free of dog feces and avoid fertilizing fields with dog manure. Communities can control wild dog populations, thus preventing infection of the definitive host. Individuals should wash all fruits and vegetables thoroughly before eating and make sure their dogs are not infected with tapeworm.


Management

The most common and widely recognized treatment for this disease is surgical removal of the cysts. The disease is more complicated and severe when the oncosphere cysts form in the
central nervous system The central nervous system (CNS) is the part of the nervous system consisting primarily of the brain, spinal cord and retina. The CNS is so named because the brain integrates the received information and coordinates and influences the activity o ...
tissue since this makes operating more difficult than when the disease presents in the muscles or subcutaneous tissues. Alternative methods for treating coenurosis include administering
anthelmintic Anthelmintics or antihelminthics are a group of antiparasitic drugs that expel parasitic worms (helminths) and other internal parasites from the body by either stunning or killing them without causing significant damage to the host. They may also ...
s and
glucocorticoid Glucocorticoids (or, less commonly, glucocorticosteroids) are a class of corticosteroids, which are a class of steroid hormones. Glucocorticoids are corticosteroids that bind to the glucocorticoid receptor that is present in almost every vertebra ...
s. 
Praziquantel Praziquantel, sold under the brandname Biltricide among others, is a medication used to treat a number of types of parasitic worm infections in mammals, birds, amphibians, reptiles, and fish. In humans specifically, it is used to treat schist ...
,
Niclosamide Niclosamide, sold under the brand name Niclocide among others, is an anthelmintic medication used to treat tapeworm infestations, including diphyllobothriasis, hymenolepiasis, and taeniasis. It is not effective against other worms such as ...
, and
Albendazole Albendazole is a broad-spectrum antihelmintic and antiprotozoal agent of the benzimidazole type. It is used for the treatment of a variety of intestinal parasite infections, including ascariasis, pinworm infection, hookworm infection, trichuri ...
are the anthelminthics that are commonly used to treat coenurosis. Praziquantel works by causing contraction of the parasite by altering the permeability of the
cell membrane The cell membrane (also known as the plasma membrane or cytoplasmic membrane, and historically referred to as the plasmalemma) is a biological membrane that separates and protects the interior of a cell from the outside environment (the extr ...
. This causes paralysis of the parasite from loss of its intracellular calcium. The drug also causes
vacuolization Vacuolization is the formation of vacuoles or vacuole-like structures, within or adjacent to cells. Perinuclear vacuolization of epidermal keratinocytes is most likely inconsequential when not observed in combination with other pathologic fin ...
and disintegration of the parasite. Niclosamide works by uncoupling
oxidative phosphorylation Oxidative phosphorylation(UK , US : or electron transport-linked phosphorylation or terminal oxidation, is the metabolic pathway in which Cell (biology), cells use enzymes to Redox, oxidize nutrients, thereby releasing chemical energy in order ...
and inhibiting glucose uptake in the parasite. Albendazole works by binding to β- tubulin sites and inhibiting their polymerization into
microtubule Microtubules are polymers of tubulin that form part of the cytoskeleton and provide structure and shape to eukaryotic cells. Microtubules can be as long as 50 micrometres, as wide as 23 to 27 nanometer, nm and have an inner diameter bet ...
s. This decreases the absorption ability of the parasite, leading to decreased glucose uptake and glycogen stores. The lack of glucose prevents the parasite from making enough ATP leading to the parasite’s death. 
Dexamethasone Dexamethasone is a fluorinated glucocorticoid medication used to treat rheumatic problems, a number of skin diseases, severe allergies, asthma, chronic obstructive pulmonary disease (COPD), croup, brain swelling, eye pain following eye su ...
is the glucocorticoid that is normally administered for coenurosis. It inhibits pro-inflammatory signals and promotes anti-inflammatory signals in the body. Dexamethasone also causes decreased
vasodilation Vasodilation, also known as vasorelaxation, is the widening of blood vessels. It results from relaxation of smooth muscle cells within the vessel walls, in particular in the large veins, large arteries, and smaller arterioles. Blood vessel wa ...
in capillaries and inhibits apoptosis in
neutrophil Neutrophils are a type of phagocytic white blood cell and part of innate immunity. More specifically, they form the most abundant type of granulocytes and make up 40% to 70% of all white blood cells in humans. Their functions vary in differe ...
s. Glucocorticoids can be used to help subdue the inflammatory symptoms of the disease.


Prevalence

This disease is very rare in humans and only about 100 cases have ever been recorded. It is a more common problem in sheep and cattle and can be problematic for farmers in endemic regions of the world. Most human cases occur in developing countries such as India and Sub Saharan Africa where the dog population is not controlled or treated for tapeworm, and in areas lacking proper sanitation. ''T. multiceps'' has been reported in regions all over the world (both human and animal infections) and is the most common coenurosis causing species. ''T. serialis'' has been seen in North America, Europe and Africa, and ''T. brauni'' and ''T. glomerata'' have only been seen in Africa. This disease still occurs, even in developed countries. Some of the most recent reported cases occurred in France, Italy, Israel, Canada and the United States. Some experts suspect under reporting of this disease, especially in impoverished and developing countries where reporting technologies are more difficult to obtain. The global prevalence might be much higher than present data suggest.


History

Because this disease so rarely occurs in humans, it took a long time for it to become recognized within the population, and species differentiation among the four different types is still somewhat difficult. Many cases of coenurosis probably existed years before it was recognized or discovered, but the first cases to be diagnosed for each of the four '' Taenia spp.'' were as follows: * ''T. multiceps'': In 1913, a man in Paris presented symptoms of CNS nerve degeneration. He had convulsions and trouble speaking/ understanding speech. During his autopsy, two coenuri were found in his brain. * ''T. glomerate'': In 1919, the first diagnosis was made in Nigeria. It is thought to be responsible for coenurosis in parts of Africa, but this is still unclear. * '' T. serialis'': In 1933, a French woman was proven to have coenurosis when the cyst that had been growing under her skin was extracted from her subcutaneous tissue and fed to a dog, which later developed tapeworm infection due to T. serialis. * ''T. brauni'': In 1956, the first coenurosis case due to ''T. brauni'' was diagnosed in Africa. is endemic only in Africa.


Cases

Human cases have been recorded in Uganda, Kenya, Ghana, South Africa, Rwanda, Nigeria, Italy, Israel, Mexico, Canada and the United States, and animal cases have been found in many other countries as well. In 1983, a 4-year-old girl in the USA was admitted to the hospital with progressive, generalized muscle weakness, inability to walk, rash, abdominal pain and deteriorating neurological ability. When the doctors did a CT scan, they saw fluid filled lumps in her brain and decided to operate. While operating, coenuri were found and the patient was immediately given chemotherapy with praziquantel. The coenurosis had already done too much damage to the CNS and the girl did not survive. The most recent North American case took place in 1994 in Los Angeles, CA, when a 39-year-old man presented an enlarging mass on his back. When doctors tried to operate a large intramuscular capsule was found. The surgery was terminated, and a fine needle aspiration test revealed muscular coenurosis. The man was treated with praziquantel. The drug successfully killed the larvae and his infection never returned. The most recent case on record to date took place in Israel in 2006. A 4-year-old girl had ''T. multiceps'' in her subcutaneous tissue. She received proper treatment and made a full recovery. In all of these recent cases, the infected individuals had been exposed to wild dogs in regions where canid tapeworm is considered endemic, and probably ingested the parasite accidentally through contact with contaminated food or water.


See also

* Coenurosis in other animals * Canine vector-borne disease


References


External links


ParaSites2005: Coenurosis
stanford.edu

Division of Parasitic Diseases, Centers for Disease Control & Prevention {{Helminthiases Zoonotic parasitic diseases