Pseudotumor Cerebri and Sickle Cell Disease

Abstract

Neurology| June 01 2004 Pseudotumor Cerebri and Sickle Cell Disease AAP Grand Rounds (2004) 11 (6): 64. https://doi.org/10.1542/gr.11-6-64 Views Icon Views Article contents Figures & tables Video Audio Supplementary Data Peer Review Share Icon Share Twitter LinkedIn Tools Icon Tools Get Permissions Cite Icon Cite Search Site Citation Pseudotumor Cerebri and Sickle Cell Disease. AAP Grand Rounds June 2004; 11 (6): 64. https://doi.org/10.1542/gr.11-6-64 Download citation file: Ris (Zotero) Reference Manager EasyBib Bookends Mendeley Papers EndNote RefWorks BibTex toolbar search toolbar search search input Search input auto suggest filter your search All PublicationsAll JournalsAAP Grand RoundsPediatricsHospital PediatricsPediatrics In ReviewNeoReviewsAAP NewsAll AAP Sites Search Advanced Search Topics: pseudotumor cerebri, sickle cell anemia Source: Henry M, Driscoll MC, Miller M, et al. Pseudotumor cerebri in children with sickle cell disease: a case series. Pediatrics. 2004;113:e265–e269. Three girls with sickle cell disease (SCD) ages 9, 8, and 16 years who presented with headache were diagnosed with pseudotumor cerebri (PC) at the Children’s National Medical Center, Washington, DC. These cases are the first reported of PC with pediatric SCD. Patient 1 had SCD-hemoglobin SC (Hb SC) and patients 2 and 3 had SCD-hemoglobin SS (Hb SS). Hemoglobin levels in patients 1, 2, and 3 were 9.3, 8.4, and 6.6 g/dL, respectively. Ophthalmologic examination revealed bilateral papilledema and normal visual acuity, except for an enlarged blind-spot in patient 1. Brain magnetic resonance (MR) imaging, including MR angiography and MR venography, were normal with no signs of hydrocephalus, stroke, or venous thrombosis. Lumbar puncture showed elevated opening pressures of 44.5, 29, and 36 cm H2O, respectively (normal ≤20cm H2O). The cerebrospinal fluid (CSF) protein, glucose, and cell counts were normal. Oral acetazolamide treatment was started at 8–15 mg/kg/d and was titrated up to 20/mg/kg/d. Treatment was discontinued after 3 months in patient 2 without relapse, patient 1 continued on a maintenance dose of 8 mg/kg/d at 10-month follow-up, and patient 3 was maintained on 15 mg/kg/d at 9-month follow-up. Patient 1 was obese, with a body mass index of 26.6 kg/m2 (95th percentile for age), and required prolonged treatment with acetazolamide and 2 therapeutic lumbar punctures for relief of symptoms of PC. Hydroxyurea, given to patients with SCD to diminish erythrocyte sickling, was discontinued in patient 1 because of continued severe headache (hydroxyurea also increases red blood cell mass and blood viscosity, perpetuating the mechanisms responsible for PC). The diagnostic criteria for PC, also termed Idiopathic Intracranial Hypertension (IIH) (see also AAP Grand Rounds, 2001;6:45), include: headache, papilledema, no or only false neurological localizing signs (eg, VIth nerve palsy), normal brain imaging, an awake and alert patient, no identifiable cause of increased intracranial pressure (ICP)1 (defined as CSF pressures of >25cm H2O), and normal CSF findings. Increased ICP caused by venous sinus thrombosis, sleep apnea, or choroid plexus papilloma is termed “secondary pseudotumor syndrome.” Theories advanced in the pathogenesis of PC include: 1) excess CSF production; 2) increase in cerebral blood volume or brain water content; 3) disturbed CSF absorption secondary to increased sagittal sinus pressure; and 4) endocrinological dysfunction related to obesity and female preponderance. Among secondary causes, the association of increased ICP with dural venous sinus thrombosis has long been recognized as “otitic hydrocephalus” secondary to otitis and mastoiditis.2 MR venography should exclude sinus thrombosis in atypical patients with suspected PC. Other secondary causes for PC are those associated with medications including vitamin A, antibiotics (eg, tetracycline), sulfa drugs, growth hormone, oral contraceptives, corticosteroid withdrawal, and lithium. Systemic diseases associated with PC include systemic lupus erythematosus, malignancies, Addison’s disease, thyroid disease, uremia, and various anemias,... You do not currently have access to this content.