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granulomatous amebic encephalitis
ICD-10 Codes
Description
What is Granulomatous Amebic Encephalitis (GAE)?
Granulomatous amebic encephalitis (GAE) is a rare and life-threatening central nervous system (CNS) infection caused by free-living amoebae, specifically the species Acanthamoeba spp., Balamuthia mandrillaris, and Sappinia pedata. This disease has a subacute to chronic onset and primarily affects immunocompromised individuals.
Causes of GAE
GAE is caused by the invasion of free-living amoebae into the CNS, leading to inflammation and damage to brain tissue. The exact mechanism of infection is not fully understood, but it is believed that the amoebae enter the body through contaminated water or soil, and then migrate to the CNS.
Symptoms of GAE
The symptoms of GAE can be highly variable and may include:
- Mental status changes
- Loss of coordination
- Fever
- Muscular weakness or partial paralysis affecting one side of the body (hemiparesis)
- Double vision (diplopia)
- Headache
- Seizures
- Coma
Treatment and Prognosis
GAE is a life-threatening disease, and treatment options are limited. The standard treatment involves surgical debridement of cutaneous lesions, as well as the use of antifungal and antibacterial medications to control secondary infections. However, the prognosis for patients with GAE is generally poor, with mortality rates ranging from 50% to 100%.
References
- [1] October 15, 2024 - Granulomatous amoebic encephalitis (GAE) is a rare, often fatal, subacute-to-chronic meningoencephalitis caused by either of two species of free-living ameba, Acanthamoeba spp. or Balamuthia mandrillaris.
- [2] March 10, 2023 - Amebic Brain Infection: Granulomatous Amebic Encephalitis - Learn about the causes, symptoms, diagnosis & treatment from the Merck Manuals - Medical Consumer Version.
- [3] It is an opportunist protist, which is known to cause rare yet fatal infection of the central nervous system (CNS), granulomatous amebic encephalitis (GAE) in humans. GAE cases are increasingly been reported among immunocompromised patients, with few cases in immunocompetent hosts.
- [4] Amebic encephalitis (granulomatous amebic encephalitis, GAE) an extremely rare disease occurring in immunocompromised patients. Presentation and early imaging
Additional Characteristics
- coma
- fever
- headache
- seizures
- immunocompromised patients
- Balamuthia mandrillaris
- Granulomatous amebic encephalitis (GAE)
- free-living amoebae
- Acanthamoeba spp.
- Sappinia pedata
- central nervous system (CNS) infection
- inflammation and damage to brain tissue
- contaminated water or soil
- mental status changes
- loss of coordination
- muscular weakness or partial paralysis affecting one side of the body (hemiparesis)
- double vision (diplopia)
- surgical debridement of cutaneous lesions
- antifungal and antibacterial medications
- mortality rates ranging from 50% to 100%
- opportunist protist
- granulomatous amebic encephalitis (GAE) in humans
- am ebic encephalitis (granulomatous amebic encephalitis, GAE)
- extremely rare disease
- presentation and early imaging
Signs and Symptoms
Common Signs and Symptoms of Granulomatous Amebic Encephalitis (GAE)
Granulomatous amebic encephalitis (GAE) is a rare and often fatal brain infection caused by free-living amoebae. The symptoms can be subtle at first, but as the infection progresses, they can become more severe.
Early Symptoms:
- Fever [1]
- Headache [4][5][14]
- Vomiting [3][7]
- Tiredness or lethargy [7]
- Nausea [3][9]
Progressive Symptoms:
- Mental status change, such as confusion or altered mental state [4][12][14]
- Seizures [4][5][14]
- Skin sores or lesions (in some cases) [12][14]
- Blurred vision [14]
- Muscle weakness or partial paralysis affecting one side of the body [6]
Other Possible Symptoms:
- Sensitivity to light [7]
- Behavioral changes [5][7]
- Weight loss [7]
It's essential to note that these symptoms can be similar to those of other conditions, and a proper diagnosis by a medical professional is crucial for
Additional Symptoms
- Seizures
- Tiredness or lethargy
- Mental status change (confusion or altered mental state)
- Skin sores or lesions
- Muscle weakness or partial paralysis affecting one side of the body
- Behavioral changes
- weight loss
- nausea
- blurred vision
- vomiting
- fever
- light sensitivity
- headache
Diagnostic Tests
Diagnostic Tests for Granulomatous Amebic Encephalitis
Granulomatous amebic encephalitis (GAE) is a life-threatening infection caused by free-living amoebae, and its diagnosis can be challenging. However, several diagnostic tests can help confirm the presence of GAE.
- Brain Scans: CT or MRI scans with contrast can show abnormalities in the brain, such as lesions or swelling, which may indicate GAE [8][9].
- Biopsies: Biopsy of skin lesions or brain tissue can establish the diagnosis by detecting the presence of amoebae [4][5][7].
- Spinal Taps: Spinal tap (lumbar puncture) can be used to collect cerebrospinal fluid, which can be tested for the presence of amoebae or other signs of infection [5][6].
- Cerebrospinal Fluid Analysis: Analysis of cerebrospinal fluid can help exclude other possible causes of symptoms and may provide evidence of GAE [9].
It's worth noting that serologic tests are of uncertain value in diagnosing GAE, as antibodies to the amoebae can be present in healthy individuals [7]. Therefore, a combination of clinical evaluation, imaging studies, and laboratory tests is often necessary to confirm the diagnosis.
References:
[4] Context 4 [5] Context 5 [6] Context 6 [7] Context 7 [8] Context 8 [9] Context 9
Additional Diagnostic Tests
- Cerebrospinal Fluid Analysis
- Biopsies
- Brain Scans
- Spinal Taps
Treatment
Treatment Options for Granulomatous Amebic Encephalitis
Granulomatous amebic encephalitis (GAE) is a rare but serious condition caused by infection with certain types of amoeba. Treatment typically involves a combination of medications, as there is no single cure.
- Miltefosine: This medication has shown in vitro evidence of amebicidal or amebistatic activity and has been used to treat GAE (1).
- Pentamidine: Another medication that exhibits in vitro evidence of amebicidal or amebistatic activity, pentamidine is often used in combination with other drugs to treat GAE (1).
- Azithromycin: This antibiotic has also shown in vitro evidence of amebicidal or amebistatic activity and may be used as part of a treatment regimen for GAE (1).
- Nitroxoline: In some cases, nitroxoline has been successfully used to treat Balamuthia mandrillaris granulomatous amebic encephalitis (2).
- Voriconazole: This antifungal medication was used in combination with miltefosine to treat a patient with Acanthamoeba infection (3).
Treatment Regimens
While there is no single ideal treatment regimen for GAE, a combination of medications is typically used. Treatment may also involve surgical debridement of cutaneous lesions (8). The therapy often empirically associates a large diversity of drugs, all exhibiting a high toxicity (6).
Key Takeaways
- A combination of medications is typically used to treat granulomatous amebic encephalitis.
- Miltefosine, pentamidine, and azithromycin have shown in vitro evidence of amebicidal or amebistatic activity.
- Nitroxoline has been successfully used to treat Balamuthia mandrillaris granulomatous amebic encephalitis.
- Treatment regimens may involve surgical debridement of cutaneous lesions.
References:
(1) Context result 1 (2) Context result 2 (3) Context result 3 (6) Context result 6 (8) Context result 8
Recommended Medications
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Differential Diagnosis
Differential Diagnosis of Granulomatous Amebic Encephalitis
Granulomatous amebic encephalitis (GAE) is a rare and often fatal meningoencephalitis caused by free-living amoebas. When diagnosing GAE, it's essential to consider other conditions that may present with similar symptoms. Here are some of the differential diagnoses for GAE:
- Sarcoidosis: A condition characterized by the formation of granulomas in various organs, including the brain (Maehara, 2022) [4].
- Amoebic encephalitis: Another type of amoebic infection that can cause similar symptoms to GAE.
- Fungal granuloma: A fungal infection that can cause granulomatous inflammation in the brain.
- Toxoplasmosis: An infection caused by the Toxoplasma gondii parasite, which can affect the brain and cause similar symptoms to GAE.
- Septic emboli, hemorrhagic infarcts, abscesses, and neoplasms: Other conditions that can cause inflammation or damage to the brain tissue (Seshagiri, 2023) [5].
- Infective encephalitis: A type of viral or bacterial infection that can cause inflammation in the brain.
- Tumors (primary or secondary): Cancerous growths in the brain that can cause similar symptoms to GAE.
- Infarction: Damage to the brain tissue due to lack of blood supply.
These differential diagnoses are essential to consider when diagnosing granulomatous amebic encephalitis, as they may present with similar symptoms and require different treatment approaches.
References:
[4] Maehara, T. (2022). Clinically, sarcoidosis was ranked high among the differential diagnoses because of the presence of skin lesions showing granulomatous inflammation, confirmed by biopsy.
[5] Seshagiri, DV. (2023). Differential diagnosis included amoebic encephalitis, fungal granuloma, toxoplasmosis, septic emboli, hemorrhagic infarcts, abscesses, and neoplasms.
Note: The above information is based on the search results provided in the context section.
Additional Differential Diagnoses
- Tumors
- Neoplasms
- Infarction
- Abscesses
- Fungal granuloma
- Septic emboli
- Hemorrhagic infarcts
- Infective encephalitis
- encephalitis
- sarcoidosis
- toxoplasmosis
Additional Information
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- A parasitic protozoa infectious disease that results in infection of the brain caused by Acanthamoeba or Balamuthia mandrillaris. The symptoms include headaches, altered mental status, and focal neurologic deficit, which progresses over several weeks to death.
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