CASE REPORT  
Niger J Paed 2012; 39 (3):136 - 139  
Okposio M M  
Abhulimhen-Iyoha BI  
Idiopathic intracranial hypertension  
with altered consciousness in a  
Nigerian school girl: a case report  
and review of the literature  
DOI:http://dx.doi.org/10.4314/njp.v39i3,10  
Accepted : 4th February 2012  
Abstract Idiopathic intracranial  
hypertension (IIH) is a clinical  
condition of increased intracranial  
pressure (ICP) without an obvious  
underlying pathological brain  
lesion. It is usually characterized by  
headache, neck pain, vomiting, vis-  
ual disturbances, papilledema, cra-  
nial nerve palsy or a combination of  
these signs and symptoms. The  
diagnosis of IIH is often made in a  
patient with intact consciousness.  
We present a teenager who  
developed altered consciousness  
while being treated for suspected  
meningitis and later found to have  
IIH. This case brings to the fore the  
need for a high index of suspicion  
even in situations where features  
appear atypical to avoid the un-  
pleasant consequences of a misdi-  
agnosis.  
Okposio M M  
(
)
Department of Pediatrics, Mariere  
Memorial Central Hospital,  
Ughelli, Delta State, Nigeria  
Tel: +2348034042120  
E-mail: mattokmatok@yahoo.com  
Abhulimhen-Iyoha BI  
Department of Child Health,  
University of Benin Teaching  
Hospital,  
Keywords: Idiopathic intracranial  
hypertension, Altered conscious-  
ness, adolescent girl, Nigeria.  
Benin City, Nigeria  
Case Report  
Introduction  
O.O is a 13-year-old school girl who was referred by a  
private medical practitioner and presented at the Emer-  
gency Unit of Lily Hospital Warri, a private secondary  
health care facility in Delta State, Nigeria. She was on  
admission at the referring hospital for seven days having  
presented there with a five-day history of generalized  
throbbing headache. The headache was worse in the  
morning and aggravated by straining but was transiently  
relieved by analgesic. There was an associated neck pain  
and blurring of vision; however, there was no vomiting  
and no fever. There was also no history of seizures or  
trauma to the head and she was not on any medication  
prior to the onset of symptoms. She was placed on anti  
malaria medication on an outpatient basis, however, she  
represented to the same hospital two days later because  
of worsening symptoms. She was subsequently admitted  
after re-evaluation and treatment was commenced for  
suspected meningitis with intravenous ceftriaxone and  
analgesics. The patient’s condition, however, progres-  
sively worsened with restlessness, collapsing episodes,  
followed by deterioration in the level of consciousness  
which necessitated her referral.  
Idiopathic intracranial hypertension (IIH) is a neurologi-  
cal disorder which has undergone a series of name  
changes since it was first described as meningitis serosa  
1
by Quincke in 1893. It was last known as benign intrac-  
ranial hypertension, however, because of the potential  
visual loss that accompany the papilledema, C2orbett and  
Thompson substituted benign with idiopathic.  
Idiopathic intracranial hypertension is a clinical condi-  
tion of increased intracranial pressure (ICP) without an  
obvious underlying pathological brain lesion. It is a rare  
disorder in children and very few cases have been re-  
.
3
ported in Nigeria. It is usually characterized by head-  
ache, neck pain, vomiting, visual disturbances, papille-  
dema, cranial nerve palsy or a combination of these  
4
signs and symptoms. These clinical features can also  
be present in some other central nervous system mor-  
bidities seen in our region such as meningitis, as a result  
IIH can easily be misdiagnosed especially by non spe-  
cialist practitioners. This is more so in children where  
5
atypical features of IIH have been reported. Therefore,  
a high index of suspicion is required to accurately make  
the diagnosis of idiopathic intracranial hypertension.  
At presentation, we found an obese young adolescent  
We present the case of a 13-year-old school girl with  
idiopathic intracranial hypertension whose level of con-  
sciousness deteriorated while being treated for suspected  
meningitis.  
(
BMI 33.1), with altered consciousness,o a Glasgow  
o
coma scale of 12/15. She was afebrile (T 37 C), not  
pale, not jaundiced, pupils were dilated and sluggishly  
reactive to light. Fundoscopy revealed fundi with  
blurred disc margin and engorged retinal vessels sugges-  
1
37  
tive of bilateral papilloedema. She had bilateral ab-  
ducent nerve palsy and right sided facial nerve palsy.  
Tone and tendon reflexes were normal, but there was  
nuchal rigidity. Her pulse rate was 86 beats per minute  
and blood pressure 120/80mmHg. Further systemic ex-  
amination was largely unremarkable.  
A provisional diagnosis of raised intracranial pressure ?  
cause was made. The patient was then commenced on  
intravenous furosemide 60mg 12 hourly and intravenous  
dexamethazone 4mg 6 hourly. A computerized tomogra-  
phy (CT) scan was done and revealed a normal cranial  
vault, sella and suture lines. The brain slices revealed  
normal tissues as well as ventricular system and there  
was no areas of hyperattenuation (Figure 1,2&3). Lum-  
bar puncture was done using a 22G, 3.5-inch spinal nee-  
dle with the patient in the lateral decubitus position, re-  
laxed and legs extended after the CT scan ruled out an  
intracranial mass lesion. The cerebrospinal fluid (CSF)  
was clear and colourless and was under pressure. The  
biochemical and microbiological studies were all nor-  
mal. The complete blood count, platelet count, renal and  
liver function tests were normal. The prothrombin time  
and partial thromboplastin time were not prolonged. A  
diagnosis of Idiopathic Intracranial Hypertension was  
therefore made.  
Fig 3  
Discussion  
Idiopathic intracranial hypertension is a neurological  
disorder that is characterized by increased ICP in the  
absence of an intracranial space-occupying lesion. It is a  
diagnosis of exclusion made largely based on clinical  
parameters but radiological and la6boratory studies have  
a role in confirming the diagnosis.  
The overall annual incidence is 0.9 per 100,000 and  
there is a strong female predilection. In young adults  
7
whose body weight is 10-20% above normal, the  
incidence may be as high as 19.3% per 100,000. How-  
8
Intravenous furosemide was discontinued on the third  
day of admission after she became fully conscious and  
oral acetozolamide 500mg 12hrly was commenced. She  
made remarkable clinical improvement with resolution  
of the headache and was discharged home after 12 days  
of hospitalization. She was on two weekly follow-up  
visit during which an assessment of cranial nerves 6 and  
ever, in the paediatric age group, especially in pre-  
pubertal children, it is considered relatively rare and the  
9
incidence is equal among boys and girls. After puberty,  
predilection for female and association with obesity in-  
10  
creases, likely as a result of hormonal changes. Our  
patient was an obese young adolescent school girl with a  
BMI of 33.1 and therefore had risk factors for IIH.  
7
function were done. In addition,visual acuity and vis-  
ual field assessment were also done because of residual  
visual disturbances. By the fourth follow-up visit, the  
cranial nerves dysfunction as well as the visual obscura-  
tion had completely resolved.  
The pathogenesis of IIH is still not clear. Many postula-  
tions have been put forward based on findings of neu-  
roradiologic research and studies of cerebrospinal fluid  
hydrodynamics. These include a possible decreased  
cerebrospinal fluid absorption by the arachnoid villi, or  
an increased cerebrospinal fluid production by the chor-  
oid plexus. It is also postulated that perhaps, an  
increased water volume of the brain content diminishes  
its complian1c1e, explaining the normal or reduced ven-  
tricular size.  
Headache is the commonest symptom of IIH in both  
children and adult. It is usually worse in the morning,  
sometimes awakens the patient from sleep and increased  
with valsalva manoeuvre. The headache may be associ-  
ated with nausea and vomiting and very frequently there  
are opthalmological symptoms such as decreased or  
blurred vision, diplopia and transient visual obscura-  
Fig1  
1
2
tions.  
The diagnosis of3IIH is often made based on modified  
1
Dandy’s criteria and these include symptoms of in-  
creased intracranial pressure, no or false localizing neu-  
rological signs, normal brain imaging result, an awake  
and alert patient, normal cerebrospinal fluid finding and  
no identifiable cause of increased ICP. Our patient met  
Fig 2  
1
38  
the diagnostic criteria except that she had impaired con-  
sciousness at presentation. Although, she was conscious  
and alert when she was first seen at the referring hospi-  
tal, the subsequent alteration of consciousness makes  
this case somewhat atypical. It is important to note that  
specific paediatric diagnostic criteria have not been es-  
tablished. Interestingly, children with IIH may display a  
greater spectrum of clinical presentation than adults.  
These may include irritability, apathy, somnol4ence,  
and magnetic resonance venography (MRV) are recom-  
2
0
mended, cranial tomography scan can be used espe-  
cially in places where MRI is not available. It can ex-  
clude hydrocephalus and most mass lesions as well as  
venous sinus thro13m, 1b4osis, meningeal infiltrations and  
isodense tumours.  
Neuroimaging are mandatory not  
only to exclude potential secondary causes of elevated  
ICP but the possibility of herniation prior to a lumbar  
puncture.. The result of neuroimaging should be normal  
in IIH, however, signs of increased intracranial pressure  
may be found such as flattening of the posterior globe  
where the optic nerve inserts in 80% of patients, empty  
sella 1i3n 70% and distension of perioptic nerve sheath in  
45%. In addition, a lumbar puncture in a patient who  
has IIH should display an increased opening pressure as  
well as normal cell count, normal glucose concentration,  
normal or low protein content and the absence of infec-  
tion. In our patient neuroimaging studies as well as CSF  
biochemical and microbiological studies were all normal  
fulfilling a major diagnostic criterion. Although ma-  
nometry could not done to determine the exact CSF  
pressure because of non availability, the flow rate was  
suggestive of a CSF pressure that was very high. Robert  
et al showed in a study that CSF drops counts per given  
time can be used to estimate CSF pressure with a rea-  
1
ataxia, dizziness neck pain, stiff neck and seizure Im-  
pairment of consciousness in our patient was preceded  
by a series of collapsing episodes. This, we thought  
could have been due to a pla5teau wave; a phenomenon  
1
first described by Lundberg. It is characterized by an  
acute elevation of ICP in a situation where baseline ICP  
is already moderately elevated. A plateau wave develops  
as a result of a rapid increase in intracerebral blood vol-  
ume. These acute elevations of ICP have been observed  
in patients with brain tumour, subarachnoid haemor-  
rhage, acute h6ydrocephalus and idiopathic intracranial  
1
hypertension. and may be triggered in ambulatory pa-  
tient by postural changes. It follows a reduction in in-  
tracranial compliance. The normal process of cerebral  
autoregulation is the result of a slow rise in cerebral  
blood volume as a result of vasodilatation which occurs  
because of a reduction in cerebral perfusion pressure  
2
2
sonable degree of accuracy.  
(
CPP). However, when CPP drops below a critical level,  
the rate of vasodilatation also increases dramatically,  
resulting in a rapid increase in cerebral blood volume  
and a sudden sustained increase in ICP. This may pro-  
duce paroxysmal symptoms in patient with IIH and  
these include among others7]alteration in consciousness,  
The key to a good outcome in IIH is early recognition  
and prompt treatment. This is to forestall permanent  
optic nerve death and subsequent visual loss. Weight  
reducing measu3res should form part of the initial treat-  
2
ment regimen. Drugs that have been used with success  
1
postural and motor control  
include acetazolamide,, a sulfa-derived diuretic and car-  
bonic anhydrase inhibitor that reduces CSF production.  
The starting dose in children is 25mg/kg/day which may  
Multiple cranial neuropathies was another unusual find-  
ing in our patient. M2 ost cases of IIH presents with ab-  
2
4
be increased to 100mg/kg/day (maximum, 2g/day).  
1
ducent nerve palsy. In this case, both abducent nerves  
Furosemide, a loop diuretic, can be used in combination  
with or as an alternative to acetazolamide at a dose of  
1mg/kg/dose. Corticosteroids have also been use25d espe-  
cially in patient with rapid visual deterioration. Other  
interventions found useful in selected cases include se-  
rial lumbar puncture, although this is technically diffi-  
cult in children and their pressure lowering effect is only  
temporary. Surgery may be indicated such as Bariatric  
surgery, CSF26diversion procedures and optic nerve de-  
compression.  
and the right facial nerve were affected. Facial nerve  
palsy in IIH has b8 een described in the literature in about  
1
2
-6% of cases. Some reports suggest that increase  
pressure at the posterior fossa and9enlargement of the  
1
fallopian canal may be responsible. Other atypical fea-  
tures of IIH that have been reported include hypoglossal  
nerve palsy, hyperreflexia with positi2v0e Babinski sign,  
choreiform movement and nystagmus.  
The prior use of an antibiotics in this patient could have  
been a limitation because of the possibility of a partially  
treated meningitis; given the atypical features seen this  
patient. However, this was less likely because there is no  
significant effect of previous antimicrobial therapy on  
CSF cell count or glucose and protein a1 mong patients  
Idiopathic Intracranial Hypertention is a rare disease  
especially in children and may be easily confused with  
more common central nervous system morbidities. A  
high index of suspicion and appropriate investigations  
are therefore needed to reduce the attendant sequelae of  
a misdiagnosis.  
2
with positive or negative CSF culture. This was the  
finding of Shohet et al in a review of 115 children with  
clinical diagnoses of meningitis, of which 47 had re-  
ceived antimicrobial therapy before hospitalization. Our  
patient had normal microbiological and biochemical  
CSF results .  
Conflict of interest : None  
Funding : None  
The choice of neuroimaging studies for suspected cases  
of IIH3 is still the biggest controversy among physi-  
1
cians. Although, magnetic resonance imaging (MRI)  
1
39  
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