Primary amebic meningoencephalitis (PAM) is a rare entity. Usual presenting features are fever, headache and seizures with meningeal signs and this disease carries high mortality rate. We present a case report of PAM presenting as status epilepticus

Bharti, V

Gargi, G

Jaret, P

Panda, P

Raina, R

Sharma, A

English

A Sharma

R Raina

P Jaret

V Bharti

G Gargi

P Panda

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Primary Amoebic (Naegleria fowleri) Meningoencephalitis Presenting as Status Epilepticus

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Case Report:
Primary Amoebic (Naegleria fowleri) Meningoencephalitis Presenting as Status Epilepticus
A Sharma, Professor, 
R Raina, Associate professor,
P Jaret, Assistant professor,
V Bharti, Senior resident,
G Gargi, Junior resident,
P Panda, Junior resident,
Dept. of Medicine, Indira Gandhi Medical College, Shimla, INDIA.
Address for Correspondence:
Dr. Prashant Panda,
Junior Resident,
Department of Medicine,
Indira Gandhi Medical College,
Shimla, INDIA. 
E-mail: prashantpanda85@gmail.com
Citation:  Sharma  A, Raina R, Jaret P,  Bharti  V,  Gargi  G,  Panda P.  Primary Amoebic  (Naegleria fowleri)  Meningoencephalitis 
Presenting as Status Epilepticus. Online J Health Allied Scs. 2011;10(1):22
URL: http://www.ojhas.org/issue37/2011-1-22.htm
Open Access Archives: http://cogprints.org/view/subjects/OJHAS.html and http://openmed.nic.in/view/subjects/ojhas.html
Submitted: Jan 22, 2011; Accepted: March 31, 2011; Published: April 15, 2011
Abstract:
Primary  amebic  meningoencephalitis  (PAM) is  a  rare  entity. 
Usual presenting features are fever, headache and seizures with 
meningeal signs and this disease carries high mortality rate. We 
present a case report of PAM presenting as status epilepticus.
Key Words:  Primary amebic meningoencephalitis;  Naegleria  
fowleri; Status epilepticus
Introduction:
N. fowleri is the only species of Naegleria genus that is patho-
genic to humans. It is ubiquitous in natural  fresh water lakes 
and ponds  (especially  warm water).  It  causes extremely  rare 
and sporadic  CNS infections.  Typically,  N.  fowleri produces 
primary amebic meningoencephalitis  (PAM), which is clinic-
ally indistinguishable from acute bacterial meningitis.
Case Report:
A 21 years female residing at district Bilaspur of Himachal Pra-
desh, presented with fever, headache and vomiting for 1 week 
and seizures for 1 day. Fever was high grade (up to 103°F), in-
termittent,  associated with chills and rigors. Headache was dif-
fuse dull  aching to start with,  present all  over  the head, and 
later on it increased in severity. She also had vomiting 7-8 epis-
odes per day,  mainly consisting of undigested food particles, 
non bilious, not containing any blood, mucus. There was also 
history  of  7-8  episodes  of  generalised  tonic  clonic  seizures 
without regaining consciousness. General physical examination 
was unremarkable.  Neurological examination revealed uncon-
scious state with neck rigidity and positive Kernig’s sign. The 
rest of the systemic examination was normal. Complete haemo-
gram, LFT’s , RFT’s,  and blood culture were normal. CSF ex-
amination revealed proteins 55 mg%, sugar  30 mg%, cytology 
7 WBCs, mostly lymphocytes. Wet mount specimen of CSF re-
vealed motile  flagellate forms of  Naegleria fowleri. CSF was 
repeated  twice  which  revealed  similar  form  on  microscopy. 
MRI brain was done which was normal. Retrospectively history 
revealed that she had taken a bath at a local pond. On the day 1, 
patient was initially started empirically on ceftriaxone, vanco-
mycin,  acyclovir  and  steroid  on  the  lines  of  acute  bacterial 
meningitis  and  acute  viral  encephalitis  and  also  patient  was 
dilantinised for status epilepticus. On day 2 after CSF report, 
patient was started on injection amphotericin. Despite this, pa-
tient deteriorated and she expired on 5th day of admission.
Discussion:
Primary amebic meningoencephalitis  is  caused by free living 
amebas of genera  Naegleria fowleri, Acanthamoeba and Bal-
amuthia  mandrillaris  formerly known as leptomyxid  amoeba. 
These  are free  living,  amephizoic  and opportunistic  protozoa 
that are ubiquitous in nature. These amebas are found in soil, 
water, air samples from all over world. Man gets infected from 
inhalation of trophozoites or cysts on exposure to polluted wa-
ter in ponds, swimming pools and manmade lakes.  Nageleria  
fowleri is a thermophilic amoeba that grows well in tropical and 
subtropical climates and raised temperatures during hot summer 
months or warm water from power plants facilitates its growth. 
Human infection involves brain, lung, skin and eyes and has in-
creased significantly since last 10 years. The portal of entry to 
CNS is through olfactory neuroepithelium. CNS infection oc-
curs in two forms:
a)  Primary  amebic  meningoencephalitis,  (PAME)  caused  by 
Naegleria fowleri
b) Granulomatous amebic encephalitis, (GAE) caused by spe-
cies of Acanthamoeba and Balamuthia mandrillaris.
Incubation  period  for  N.  fowleri is  3  to  7 days  and that  for 
acanthamoeba  is  unknown  but  perhaps  more  than  10  days. 
PAME produced by N. fowleri is characterized by acute fulmin-
ant  meningoencephalitis  with  severe  headache,  high  grade 
fever,  nausea,  vomiting,  altered  consciousness  with  signs  of 
meningeal irritation. Cranial nerve palsies, seizures, confusion, 
agitation, and coma may follow and death occurs within a week 
if not treated early. Most patients die, usually by the seventh 
day.2-5 
1
This work is licensed under a
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No Derivative Works 2.5 India License
Online Journal of Health and Allied Sciences
Peer Reviewed, Open Access, Free Online Journal
Published Quarterly :  Mangalore, South India : ISSN 0972-5997
Volume 10, Issue 1; Jan-Mar 2011
Recognition of primary amoebic meningoencephalitis depends 
on clinical suspicion based on patient history. A spinal tap usu-
ally  reveals  an  elevated  white  blood  cell  count  (>100 
cells/mm3) with a high percentage of polymorphonuclear leuk-
ocytes (>70%), a depressed glucose level (<50 mg/dL), and an 
elevated protein level (>60 mg/dL), mimicking those of bacteri-
al meningitis. Occasionally, amebae can be observed on Gram-
stained smears.  In  CSF,  both  amoeboid  and  flagellate  forms 
have been reported. If primary amoebic meningoencephalitis is 
suspected, a fresh-centrifuged specimen of CSF should be in-
spected  by wet-mount  preparation  and  fixation  and  staining. 
Special culture media are required for culture of amoeba. Sero-
logical tests performed are indirect Immunofluorescence assay 
(IIA),  dot  immunobinding  assay  (DIBA)  and  enzyme  linked 
immunotransfer blot technique (EITB). Neuroimaging findings 
nonspecific brain edema suggestive of meningitis, favouring N. 
fowleri, and  punctuate  enhancing  lesions  due  to 
acanthamoeba.6-8
A few patients have been successfully treated using a combina-
tion  of  miconazole,  oral  rifampin  and high dose  of  injection 
amphotericin.1,9 The risk of infection from water containing N. 
fowleri is  unknown  but  probably  small,  since  thousands  of 
people swim in lakes, ponds and hot springs known to contain 
these organisms, and yet cases of PAM are extremely rare. This 
case is  peculiar  because  we found  flagellate form in CSF in 
contrast  to  amoeboid  form reported  in  other  case  reports  al-
though both are known to cause PAM. PAM is known to cause 
seizures,  but presentation as status epilepticus is rare.  To the 
nest of our knowledge, no case of PAM has been reported from 
the  Sub-Himalayan  region.  PAM should  be  suspected in  pa-
tients with acute meningitis who do not respond to conventional 
treatment and who have fulminant course.
References:
1. Brown RL. Successful treatment of primary amebic 
meningoencephalitis  (PAM).  Arch  Intern  Med 
1991;151:1201-1202  
2. Jain R, Prabhakar S, Modi M, Bhatia R, Sehgal R. 
Naegleria  meningitis:  a rare survival.  Neurol India 
2002;50:470-472.
3. Martinez AJ. Free living amoebas: Infection of the 
central  nervous  system.  Mt  Sinai  J  Med 
1993;60:271-278.
4. Shamsizadeh A, Zaker N, Maraghi S, Momen A. A 
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5. Tungikar SL , Kulkarni AG, Deshpande AD, Gosavi 
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6. Rodriguez  R,  Mendez  O,  Molina  O,  Luzardo  G. 
CNS infection by free living amebasc Report of 3 
Venezuelan  cases.  J  Rev Neurol  1998;26:1005-
1008.
7. Garcia  TJ,  Sotolongo  GF,  Cepero  NF,  Ibarra  SE, 
Garcia  OJ.  Primary  amebic  meningoencephalitis. 
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8. Jain R, Prabhakar S, Modi M, Bhatia R, Sehgal R. 
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9. Singh SN, Patwari AK, Dutta R, Taneja N, Anand 
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2


A Primary Amebic Meningoencephalitis.

CNS infection by free living amebasc Report of 3 Venezuelan cases.

Free living amoebas: Infection of the central nervous system.

Naegleria meningitis: a rare survival.

Naegleria meningitis: a rare Survival. Neurol

Naegleria meningitis. Ind Pediatr

Primary Amebic Meningoencephalitis.

Primary amebic meningoencephalitis. Report of suspicious case and review of the medical literature. Rev Cubana Med Trop. Sep-Dec

Successful treatment of primary amebic meningoencephalitis (PAM).

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Primary Amoebic (Naegleria fowleri) Meningoencephalitis Presenting as Status Epilepticus

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