Granulomatous Amebic Encephalitis

Granulomatous Amebic Encephalitis

Article
Brain & Nerve
Diseases & Conditions
+2
Contributed byMaulik P. Purohit MD MPHOct 09, 2018

What are the other Names for this Condition? (Also known as/Synonyms)

  • Granulomatous Amoebic Encephalitis
  • Granulomatous Cerebral Amebiasis
  • Granulomatous Cerebral Amoebiasis

What is Granulomatous Amebic Encephalitis? (Definition/Background Information)

  • Granulomatous Amebic Encephalitis is a life-threatening infection of the brain caused by the free-living amoebae Acanthamoeba spp., Balamuthia mandrillaris and Sappinia pedata
  • Symptoms include mental status changes, loss of coordination, fever, muscular weakness or partial paralysis affecting one side of the body, double vision, sensitivity to light and other neurologic problems
  • The diagnosis is difficult and is often made at advanced stages. Tests useful in the diagnosis include brain scans, biopsies, or spinal taps and in disseminated disease, biopsy of the involved sites and testing by the laboratory experts
  • Early diagnosis is important for the prognosis. No single drug is effective; hence multiple antibiotics are needed for successful treatment. A combination of surgical and medical interventions involving multiple specialty experts is required to prevent death and morbidity in survivors

(Source: Granulomatous Amebic Encephalitis; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

Who gets Granulomatous Amebic Encephalitis? (Age and Sex Distribution)

  • Granulomatous Amebic Encephalitis is a rare disorder. The presentation of symptoms may occur at any age
  • Both males and females may be affected
  • Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Granulomatous Amebic Encephalitis? (Predisposing Factors)

Individuals with compromised immunity are at an increased risk for Granulomatous Amebic Encephalitis, although healthy people may also develop the infection. The conditions that may lead to reduced immunity may include:

  • HIV infection and AIDS
  • Uncontrolled diabetes
  • Lupus
  • Liver cirrhosis
  • Cancer
  • Being on steroid medication
  • Excessive use of antibiotics
  • Being on immune-suppressing drugs due to organ transplantation 
  • Disorders with abnormal quantity or function of white blood cells
  • Clotting/coagulation disorders

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Granulomatous Amebic Encephalitis? (Etiology)

  • Granulomatous Amebic Encephalitis is usually caused by the free-living amoebae Acanthamoeba spp.
  • It is also less commonly caused by the pathogens Balamuthia mandrillaris and Sappinia pedata
  • The ameoba may be found in soil and water. These pathogens may gain entry through broken skin, nasal passages, or the eye
  • When the amoebae enter the body through the nasal passage or broken skin, they enter the bloodstream and spread to the central nervous system, resulting in Granulomatous Amebic Encephalitis

What are the Signs and Symptoms of Granulomatous Amebic Encephalitis?

The signs and symptoms of Granulomatous Amebic Encephalitis may include:

  • Fever
  • Muscle weakness
  • Altered mental status 
  • Loss of coordination
  • Partial paralysis affecting one side of the body 
  • Double vision
  • Sensitivity to light

How is Granulomatous Amebic Encephalitis Diagnosed?

Granulomatous Amebic Encephalitis is diagnosed on the basis of the following information:

  • Complete physical examination
  • Thorough medical history evaluation
  • Assessment of signs and symptoms
  • Laboratory tests, which may include cerebrospinal fluid collection using a spinal tap and testing it for the presence of pathogen(s)
  • Imaging studies, including brain scans
  • Biopsy of sites that are involved such as skin lesions

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Granulomatous Amebic Encephalitis?

The complications of Granulomatous Amebic Encephalitis may include:

  • Progression to disseminated infection
  • Secondary bacterial infections
  • Progressive worsening of neurological symptoms

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Granulomatous Amebic Encephalitis Treated?

The treatment for Granulomatous Amebic Encephalitis may include both medication and surgery. Skin lesions may be excised surgically, and a combination of anitbiotics may be prescribed to target the pathogen.

How can Granulomatous Amebic Encephalitis be Prevented?

  • At the present time, no methods or guidelines are available for the prevention of Granulomatous Amebic Encephalitis
  • An early diagnosis of the infection may lead to better outcomes, and therefore, regular medical screening at periodic intervals with tests and physical examinations are recommended, particularly in individuals with compromised immune system

What is the Prognosis of Granulomatous Amebic Encephalitis? (Outcomes/Resolutions)

  • The prognosis of Granulomatous Amebic Encephalitis is poor, since most cases get diagnosed at an advanced stage. In many cases, the affected individuals succumb to the infection
  • If the infection is diagnosed at an early stage, recovery may be possible by instituting prompt and adequate treatment measures

Additional and Relevant Useful Information for Granulomatous Amebic Encephalitis:

  • Acanthamoeba species are commonly found in lakes, swimming pools, tap water, and heating and air conditioning units

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/

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Maulik P. Purohit MD MPH picture
Approved by

Maulik P. Purohit MD MPH

Assistant Medical Director, Medical Editorial Board, DoveMed Team

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