The aim of this study was to describe the association between pediatric pseudotumor cerebri and low serum vitamin A levels. We retrospectively reviewed the charts of 6 children (5 boys, 1 girl; mean age 8 years) with increased intracranial pressure and low serum vitamin A levels (mean 16.0 ± 8.8 µg/dL). The etiology of the vitamin A deficiency was a restricted diet (2 children), intestinal malabsorption caused by celiac disease (2 children), and undetermined cause (2 children). Only 1 child had ocular signs of xerosis. Poor visual acuity at presentation and lower serum vitamin A levels were associated with a poor visual outcome and development of optic atrophy. In conclusion, pseudotumor cerebri in children can be associated with vitamin A deficiency even when other manifestations of xerophthalmia do not exist. Early recognition of this condition and appropriate therapy can prevent blindness.
DigreKBCorbettJJ. Idiopathic intra cranial hypertension (pseudotumo rcerebri): a reappraisal. Neurologist. 2001;7:2–67.
2.
FriedmanDIJacobsonDM. Diagnostic criteria for idiopathic intracranial hypertension. Neurology. 2002;59(10):1492–1505.
3.
BiousseVBruceBBNewmanNJ. Update on the pathophysiology and management of idiopathic intracranial hypertension. J Neurol Neurosurg Psychiatry. 2012;83(5):488–494.
4.
SoibermanUStolovitchCBalcerLJRegenbogenMConstantiniSKeslerA. Idiopathic intracranial hypertension in children: visual outcome and risk of recurrence. Childs Nerv Syst. 2011;27(11):1913–1918.
5.
DigreKWarnerJ. Is vitamin A implicated in the pathophysiology of increased intracranial pressure?Neurology. 2005;64(11):1827.
6.
WarnerJEBernsteinPSYemelyanovAAlderSCFarnsworthSTDigreKB. Vitamin A in the cerebrospinal fluid of patients with and without idiopathic intracranial hypertension. Ann Neurol. 2002;52(5):647–650.
7.
ObeidMPriceJSunL. Facial palsy and idiopathic intracranial hypertension in twins with cystic fibrosis and hypovitaminosis A. Pediatr Neurol. 2011;44(2):150–152.
8.
LewisCDTraboulsiEIRothnerADJengBH. Xerophthalmia and intracranial hypertension in an autistic child with vitamin A deficiency. J Pediatr Ophthalmol Strabismus. 2010;26:1–3.
9.
LucidiVDi CapuaMRosatiPPapadatouBCastroM. Benign intracranial hypertension in an older child with cystic fibrosis. Pediatr Neurol. 1993;9(6):494–495.
10.
PanozzoGBabighianSBonoraA. Association of xerophthalmia, flecked retina, and pseudotumor cerebri caused by hypovitaminosis A. Am J Ophthalmol. 1998;25(5):708–710.
11.
SmithJSteinemannTL. Vitamin A deficiency and the eye. Int Ophthalmol Clin. 2000;40(4):83–91.
12.
RhinnMDolléP. Retinoic acid signalling during development. Development. 2012;139(5):843–858.
13.
Gutierrez-MazariegosJTheodosiouMCampo-PaysaaFSchubertM. Vitamin A: a multifunctional tool for development. Semin Cell Dev Biol. 2011;22(6):603–610.
14.
GarcíaOP. Effect of vitamin A deficiency on the immune response in obesity. Proc Nutr Soc. 2012;71(2):290–297.
15.
WestKPJr. Vitamin A deficiency disorders in children and women. Food Nutr Bull. 2003;24(4 suppl):S78–S90.
16.
CongdonNGFriedmanDSLietmanT. Important causes of visual impairment in the world today. JAMA. 2003;290(15):2057–2060.
17.
SommerADavidsonFR.Assessment and control of vitamin A deficiency: the Annecy Accords. J Nutr. 2002;132(9 suppl):2845S–2850S.
18.
SchreckKAWilliamsKSmithAF. A comparison of eating behaviors between children with and without autism. J Autism Dev Disord. 2004;34(4):433–438.
19.
SteinemannTLChristiansenSP. Vitamin A deficiency and xerophthalmia in an autistic child. Arch Ophthalmol. 1998;116(3):392–393.
20.
BastiSSchmidtC. Vitamin a deficiency. Cornea. 2008;27(8):973.
21.
O’DonnellMTalbotJF. Vitamin A deficiency in treated cystic fibrosis: case report. Br J Ophthalmol. 1987;71(10):787–790.
22.
SembaRDMuhilalScott AL. Depressed immune response to tetanus in children with vitamin A deficiency. J Nutr. 1992;122(1):101–107.
WestKPJrLeClerqSCShresthaSR. Effects of vitamin A on growth of vitamin A-deficient children: field studies in Nepal. J Nutr. 1997;127(10):1957–1965.
25.
SommerAKatzJTarwotjoI. Increased risk of respiratory disease and diarrhea in children with preexisting mild vitamin A deficiency. Am J Clin Nutr. 1984;40(5);1090–1095.
26.
SommerATarwotjoIHussainiGSusantoD. Increased mortality in children with mild vitamin A deficiency. Lancet. 1983;2(8350):585–588.
27.
SommerATjakrasudjatmaSDjunaediEGreenWR. Vitamin A-responsive panocular xerophthalmia in a healthy adult. Arch Ophthalmol. 1978;96(9):1630–1634.
28.
TabassiASalmasiAHJalaliM. Serum and CSF vitamin A concentrations in idiopathic intracranial hypertension. Neurology. 2005;64(11):1893–1896.
29.
WarnerJEBernsteinPSYemelyanovAAlderSCFarnsworthSTDigreKB. Vitamin A in the cerebrospinal fluid of patients with and without idiopathic intracranial hypertension. Ann Neurol. 2002;52(5):647–650.
30.
CalhounMCHurtHDEatonHDRousseauJEJrHallRC Jr. Rates of formation and absorption of cerebrospinal fluid in bovine hypovitaminosis A. J Dairy Sci. 1967;50(9):1489–1494.
31.
HayesKCMcCombsHLFahertyTP. The fine structure of vitamin A deficiency. II. Arachnoid granulations and CSF pressure. Brain. 1971;94(2):213–224.
32.
MellanbyE. Skeletal changes affecting the nervous system produced in young dogs by diets deficient in vitamin A. J Physiol. 1941;99(4):467–486.
33.
HowellJMThompsonJN. Observations on the lesions in vitamin A deficient adult fowls with particular reference to changes in bone and central nervous system. Br J Exp Pathol. 1967;48(4):450–454.
34.
AbernathyRS. Bulging fontanelle as presenting sign in cystic fibrosis: vitamin A metabolism and effect on cerebrospinal fluid pressure. Am J Dis Child. 1976;130(12):1360–1362.
35.
KeatingJPFeiginRD. Increased intracranial pressure associated with probable vitamin A deficiency in cystic fibrosis. Pediatrics. 1970;46(1):41–46.
36.
KasarskisEJBassNH. Benign intracranial hypertension induced by deficiency of vitamin A during infancy. Neurology. 1982;32(11):1292–1295.
37.
BassMHFischGR. Increased intracranial pressure with bulging fontanel: a symptom of vitamin A deficiency in infants. Neurology. 1961;11:1091–1094.
