Pseudotumor Cerebri in Children With Sickle Cell Disease: A Case Series

Abstract

Headache is a frequent symptom in sickle cell disease (SCD) that usually is attributable to anemia or cerebrovascular disease. We report 3 pediatric patients with SCD (1 patient with SCD-SC and 2 patients with SCD-SS) who presented with headache and were diagnosed with pseudotumor cerebri (PC). All 3 patients had elevated opening pressures during a lumbar puncture with normal cerebrospinal fluid studies. Magnetic resonance imaging revealed no evidence of hydrocephalus or arteriopathy in all 3 cases. Magnetic resonance venograms performed in 2 of the patients at diagnosis revealed no evidence of cerebral sinus thrombosis. Each patient received a thorough ophthalmologic examination. A diagnostic funduscopic examination revealed bilateral papilledema without signs of retinopathy in all 3 patients. There were no clinically significant changes in visual acuity or abnormalities of color vision in any patient. Goldmann or Humphrey visual-field assessment was abnormal only in patient 1, who demonstrated bilaterally enlarged blind spots at diagnosis and later developed reduced sensitivity in the inferomedial quadrant of the left eye in an arcuate pattern (which later resolved). The diagnosis of PC was made in all 3 patients, and acetazolamide treatment was started. Two of the patients’ symptoms resolved completely with medical treatment, whereas the third patient’s symptoms improved. None of these patients had permanent visual-field deficits as a result of their syndrome. PC has been reported in several other types of anemia including SCD-SC, but these cases are the first reported in conjunction with pediatric SCD. Early recognition of the signs and symptoms of PC in patients with SCD who present with headache can expedite proper diagnosis and treatment and prevent long-term ophthalmologic sequelae.

Hypoxic adaptation during development: relation to pattern of neurological presentation and cognitive disability

“…Headache, common in SCD (Palermo, Platt‐Houston, Kiska & Berman, 2005) is also seen at altitude (Jaillard, Mazetti & Kala, 1997), apparently in association with oxyhaemoglobin desaturation despite relative polycythaemia (Arregui, Leon‐Velarde, Cabrera, Paredes, Vizcarra & Umeres, 1994). Pseudotumour cerebri has been reported in SCD (Henry, Driscoll, Miller, Chang & Minniti, 2004) as well as OSA in adults (Wolin & Brannon, 1995).…”

Headache and Migraine in Children with Sickle Cell Disease Are Associated with Lower Hemoglobin and Higher Pain Event Rates But Not Silent Cerebral Infarction

“…The most common neurological complaint, headache, occurs in one-quarter to one-third of children with SCD, which is higher than in the general pediatric population. 1–4 There are multiple potential causes of headache in SCD, including tension and migraine headaches, as well as causes more specifically related to SCD: infection, bony infarction, 5, 6 idiopathic intracranial hypertension, 7, 8 severe anemia, 9 and pain medication-overuse or withdrawal. 10, 11 Headache can be a significant contributor to the pain and disability of SCD.…”

Unusual causes of papilledema: Two illustrative cases

“…[43] reported anemia in 15 of 96 patients who presented with clinical intracranial hypertension. Many types of anemia have been associated with intracranial hypertension, including hemolytic anemia,[49] aplastic anemia,[19253033] sickle cell anemia,[16] iron-deficiency anemia,[82141] anemia secondary to paroxysmal nocturnal hemoglobinuria,[1] and pernicious anemia. [38] In addition to optic disc edema, other ocular findings in patients with anemia include cotton wool spots, nerve fiber layer/preretinal hemorrhages, vitreous hemorrhages, and central retinal vein occlusion.…”