Neuroscience & Medicine, 2013, 4, 215-216
Published Online December 2013 (
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Idiopathic Intracranial Hypertension in Sickle Cell
Disease: A Paediatric Case Report
Bharath Gowda*, Sanjay Sahi
Department of Pa ediatrics, Queen Mary’s Hospital, Sidcup, UK.
Email: *
Received August 6th, 2013; revised September 8th, 2013; accepted October 5th, 2013
Copyright © 2013 Bharath Gowda, Sanjay Sahi. This is an open access article distributed under the Creative Commons Attribution
License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
In this article, we present the case of a 12-year-old girl with sickle cell disease (SCD), who presented with the severe
headache. She had bilateral 6th cranial nerve palsy and papilloedema. The common sickle cell-related vascular causes
of headache were ruled out by neuro-imaging. She then had a lumbar puncture and was diagnosed with idiopathic in-
tracranial hypertension (IIH). This case demonstrates that IIH can affect younger children with SCD and should form a
part of differential diagnosis when investigating causes of headache in SCD.
Keywords: Sickle Cell Disease; Idiopathic Intracranial Hypertension; Headach e in Children
1. Introduction
The common causes of headache in healthy children are
tension headaches or migraine [1]. Children with sickle
cell disease (SCD) frequently complain of headaches. In
one study, the overall prevalence of frequent headache
was 32.4% in subjects with SCD which were similar to
control subjects. However, younger children with SCD
were reported to have headaches more frequently than
controls [2].
The cause of headache in SCD is multi-factorial just as
in general population but SCD-specific factors such as
bone infarction, severe anaemia, or frequent opioid medi-
cation use may contribute. However, the headache could
be a manifestation of seri ous cerebro-vascular disease [2].
2. Case Report
We report a child with SCD-SC type who presented with
headache and was diagnosed with idiopathic intracranial
hypertens ion (IIH).
A 12-year-old girl with SCD-SC presented to the
emergency department with eight days history of head-
ache, vomiting, dizziness and diplopia. She was previ-
ously well. She never had a sickle cell crisis and routine
trans-cranial Doppler study done on an annual basis had
been normal. Her medications were folic acid and pro-
phylactic penicillin.
Examination revealed no signs of meningism or en-
cephalitis. Her weight was 72.7 Kgs and height 173.4
cms with a body mass index of 24.4 (96th centile for age).
She had bilateral sixth cranial nerve palsy and papillae-
She underwent extensive radiological evaluation in-
cluding MRI, MRV and MRA for possible SCD-related
cerebrovascular disease. All the tests were normal. A
bilateral enlarged blind spot was noted on visual field
She underwent a lumbar puncture. The cerebrospinal
fluid (CSF) opening pressure was elevated at 60 cms
H2O. The CSF studies were normal. She was diagnosed
with idiopathic intracranial hypertension (IIH) and com-
menced on oral acetazolamide therapy.
A month later, her headache and eye signs had re-
solved, but she still had persistent enlarged blind spot
and papilloedema. She underwent second lumbar punc-
ture and th e opening CSF pressure was 33 cms H2O. The
acetazolamide therapy was continued and after a further
month, her visual field normalised and papilloedema
resolved. After three months of therapy, acetazolamide
was weaned over a fur ther six weeks and stopp ed with no
recurrence of headache or eye signs.
3. Discussion
IIH is classified as a headache syndrome characterised by
*Corresponding a uthor.
Idiopathic Intracranial Hypertension in Sickle Cell Disease: A Paediatric Case Report
1) ra ise d CSF pressure; 2) normal composition of CSF; 3)
normal neurological examination (with the exception of
papilloedema and sixth cranial nerve palsy); and 4) nor-
mal consciousness [Dandy criteria] [3].
The modified Dandy criteria include an additional cri-
terion of normal CT/MRI brain without evidence of throm-
bosis [4 ].
The 90th percentile of opening CSF pressure for age
group 10 - 14 years is 28 cms of H2O [5]. Our patient’s
opening CSF pressure was significantly elevated at 60
cms H2O.
There are very few reported cases of IIH in a patient
with SCD in medical literature. One case of recurrent IIH
in a woman with SCD during pregnancy was reported in
1985 [6]. However, there are only two reports of IIH in
SCD in childhood involving five children in total [7,8].
None of these five children had any evidence of cerebro-
vascular disease. Three of them were 15 - 16 years old
with high body mass index. The other two cases were
under 10 years of age and had normal body mass index.
All reported cases responded well to lumbar puncture
and acetazolamide which is the standard initial manage-
ment of IIH [9].
Our patient was a 12-year-old with high body mass
index who responded well to the standard management
IIH has been reported in several other types of anae-
mia but our case is amongst the only few reported in
children with SCD.
4. Conclusions
When investigating cases of SCD which present with
headache, IIH should be part of the differential diagnosis,
particularly in teenagers with high body mass index.
However, IIH can affect much younger children with
normal body mass index, too.
IIH can be caused by an alteration of one or more of
the four determinants of cerebrospinal fluid (CSF) pres-
sure: 1) intrasagittal sinus pressure; 2) resistance of ara-
chnoid villi to the egress of CSF; 3) rate of produ ction of
CSF, and 4) compliance of the CSF space [10].
The effect of sickle cell disease on one or more of the
above remains to be determined.
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