Acanthamoeba Infections

Key Features

Essentials of Diagnosis

• Acute meningoencephalitis or chronic granulomatous encephalitis after contact with warm fresh water

• Keratitis, particularly in contact lens users

General Considerations

• Free-living amebas of the genus Acanthamoeba, Naegleria, Balamuthia, and Sappinia

• Found in soil and in fresh, brackish water

Granulomatous amebic encephalitis

• Caused by Acanthamoeba species, Balamuthia mandrillaris, and Sappinia

• More chronic than primary amebic meningoencephalitis (see Amebic Meningoencephalitis, Primary)

• Neurologic disease

• May be preceded by skin lesions, including ulcers and nodules

• Develops slowly after an uncertain incubation period

Keratitis

• Painful, sight-threatening corneal infection

• Associated with corneal trauma, most commonly after use of contact lenses and contaminated saline
solution

Clinical Findings

Symptoms and Signs

Granulomatous amebic encephalitis

• Headache

• Meningismus

• Nausea, vomiting

• Lethargy

• Low-grade fevers
• Focal neurologic findings, mental status abnormalities

Keratitis

• Progresses slowly, with waxing and waning clinical findings over months

• Severe eye pain

• Photophobia

• Tearing

• Blurred vision

Differential Diagnosis

• Many cases of Acanthamoeba keratitis are misdiagnosed as viral keratitis

Diagnosis

Laboratory Tests

Granulomatous amebic encephalitis

• Cerebrospinal fluid

• Shows lymphocytic pleocytosis with elevated protein levels

• Amebas not typically seen

• Diagnosis can be made by biopsy of skin or brain lesions

• Lumbar puncture is dangerous due to increased intracranial pressure

Keratitis

• Lack of response to antibacterial, antifungal, and antiviral topical treatments and potential use of
contaminated contact lens solution are suggestive of the diagnosis

• Ocular examination shows corneal ring infiltrates, but these can also be caused by other pathogens

• Diagnosis can be made by examination or culture of corneal scrapings

• Available diagnostic techniques include

• Examination of a wet preparation for cysts and motile trophozoites

• Examination of stained specimens

• Evaluation with immunofluorescent reagents, culture of organisms, and polymerase chain reaction
Imaging Studies

• CT and MRI show single or multiple nonspecific lesions in patients with encephalitis

Treatment

Medications

Granulomatous amebic encephalitis

• Some patients have been treated successfully with various combinations of

• Flucytosine

• Pentamidine

• Fluconazole or itraconazole

• Sulfadiazine

• Trimethoprim-sulfamethoxazole

• Azithromycin

• However, no treatment has been proved effective

Keratitis

• Can be cured with local therapy

• Topical propamidine isethionate (0.1%), chlorhexidine digluconate (0.02%), polyhexamethylene

biguanide, neomycin-polymyxin B-gramicidin, miconazole, and combinations of these agents have been
used successfully

• Oral itraconazole or ketoconazole can be added for deep keratitis

• Drug resistance has been reported

• Use of corticosteroid therapy is controversial

Therapeutic Procedures

• Debridement and penetrating keratoplasty have been performed in addition to medical therapy

• Corneal grafting can be done after the amebic infection has been eradicated

Outcome

Prognosis
• With early treatment, many patients can expect cure and a good visual result

• Untreated encephalitis can lead to death in weeks to months

• Untreated keratitis can progress slowly over months and can lead to blindness

When to Refer

• All patients with keratitis should be referred to an ophthalmologist

When to Admit

• All cases of encephalitis

Prevention

• Prevention of keratitis requires immersion of contact lenses in disinfectant solutions or heat

sterilization

• Lenses should not be cleaned in homemade saline solutions

• Lenses should not be worn while swimming

References

Bravo FG et al. Balamuthia mandrillaris infection of the skin and central nervous system: an emerging
disease of concern to many specialties in medicine. Curr Opin Infect Dis. 2011 Apr;24(2):1127. [PMID:
21192259]

Grate I. Primary amebic meningoencephalitis: a silent killer. CJEM. 2006 Sep;8(5):3659. [PMID:
17338852]

Schuster FL et al. Under the radar: Balamuthia amebic encephalitis. Clin Infect Dis. 2009 Apr
1;48(7):87987. [PMID: 19236272]

Visvesvara GS. Amebic meningoencephalitides and keratitis: challenges in diagnosis and treatment. Curr
Opin Infect Dis. 2010 Dec;23(6):5904. [PMID: 20802332]