International Journal of Clinical Case Reports 2018, Vol.8, No.1, 1-4
3
Elevated intracranial pressure with normal cerebrospinal fluid (CSF) composition
A neuroimaging study that shows no etiology for intracranial hypertension
No other apparent cause of intracranial hypertension
3 Pathogenesis
The pathogenesis of PC is unknown. There may be association between PC and venous sinus stenosis. Many
studies at present suggests that apparent venous sinus narrowing is not the primary cause in most cases but is
secondary to elevated intracranial pressure. Other theories include increased cerebrospinal fluid outflow resistance;
obesity-related raised abdominal and intracranial venous pressure; altered sodium and water retention mechanisms;
and abnormalities of vitamin A metabolism.
ATRA at high doses increases the production of cerebrospinal fluid and delays CSF absorption at arachnoid villi
due to alteration of the lipid constituents of the arachnoid villi. There is a progressive age-related decline in the
RAR expression in the central nervous system explaining the decreased risk of PC in the adult patient of APML as
compared to the pediatric age group (Visani et al., 1996).
4 Conclusions
As all-transretinoic acid can cause pseudotumour cerebri, careful consideration should be given to the patients of
acute promyelocytic leukemia if they complain of headache, blurring of vision and altered visual acuity.
Pseudotumor cerebri is suspected then regular follow-up visits with serial examinations including visual acuity,
visual field testing and a fundus examination are required until they stabilize, to prevent sequelae of raised
intracranial pressure such as optic atrophy and vision loss. As in most patients, symptoms worsen slowly and with
treatment, gradual improvement and/or stabilization occurs, but they may not have necessarily complete recovery;
many patients have persistent papilledema, elevated intracranial pressure as documented on lumbar puncture, and
residual visual field deficits.
Authors’ contributions
There was no conflict of interest.
Acknowledgments
Financial support and sponsorship- Nil
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