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Pseudotumor cerebri following oral betamethasone for common cold
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     Department of Pediatrics, Kanchi Kamakoti CHILDS Trust Hospital (KKCTH), 12-A, Nageswara Road, Nungambakkam. Chennai - 600 034, India

    An 86-day-old female infant presented with the complaints of bulging fontanelle for 10 days, incessant cry and poor feeding for 1 week. The infant's mother had taken her on the 15th day after birth to a family physician for cold and noisy breathing. She was advised to give betamethasone oral drops 0.5ml (0.25 mg) bid for 3 days. In view of persisting noisy breathing, the mother continued to administer the same. As there was no symptomatic improvement, betamethasone drops was stopped abruptly after administering for 45 days. A few days later, the baby developed swelling over the fontanelle. At the time of hospitalization, she weighed 4.5 kg, was irritable and had cushingoid features with bulging anterior fontanelle. Her pulses and blood pressure were normal and there was no cranial nerve palsy or focal neurological deficit. Other systems were clinically normal. Her hemogram, ultrasonogram of abdomen, liver and renal function tests were normal. The CT scan of brain and Cerebro Spinal fluid (CSF) analysis was normal. After 48 hours of hospitalization, the infant developed fever with respiratory distress and rapidly progressed to septic shock. Her blood culture grew E.coli . She required fluids, inotropes, antibiotics and ventilator support. Intravenous hydrocortisone was added. The infant recovered from sepsis and bulging anterior fontanelle, regressed back to normal within 7 days of hospitalization. Oral prednisolone in tapering doses was continued and withdrawn gradually over a period of 4 weeks. The infant is on follow-up for 8 months now and is doing well.

    Pseudotumor cerebri is characterized by increased intracranial pressure with normal CSF study, normal ventricular size, anatomy and position in the absence of any intracranial mass or lesion. One of the causes of pseudotumor cerebri is long-term steroid therapy and sudden withdrawal of steroids.[1] It is not commonly seen in children and is rare in early infancy.[2] The causes of pseudotumor cerebri include drugs, infections, connective tissue disorders, hematological and metabolic disorders.[3] Clinical features like headache, diplopia, pulsatile tinnitus are common in adults. In infants, it manifests as bulging anterior fontanelle, irritability, incessant crying and vomiting due to increased intracranial pressure. However, there is usually no deterioration in the level of consciousness.[4] The pathogenesis of pseudotumor cerebri is due to increased blood volume that in turn increases intracranial pressure, increased CSF production and decreased CSF absorption.[5] Diagnostic criteria as per International Headache Society's Clinical Criteria are a) CSF pressure above 200 mm H2O, (b) CSF Study should reveal normal white cell count with normal or low protein content, (c) normal neurological examination except for papilloedema and 6th Cranial nerve palsy, (d) no mass lesion or ventriculomegaly on neuroimaging and (e) no clinical or neuroimaging evidence of venous sinus thrombosis.[6] Treatment other than repeated lumbar punctures[7] is usually with acetazolamide, digoxin and prednisolone. Pseudotumor cerebri has been reported in infants as young as 4 months.[2] Even topical application of steroid for a long period and its sudden withdrawal can result in pseudotumor cerebri.[8] Abuse of betamethasone drops as a remedy for running nose has been reported in India.[9] However, pseudotumor cerebri was not reported as a feature in those cases.

    This infant had increased intracranial tension as evidenced by bulging anterior fontanelle and irritability. Her CT scan brain showed normal ventricles without any mass or evidence of venous sinus thrombosis. Although her CSF pressure was not measured, she had jetting of CSF at the beginning of the lumbar puncture and the CSF analysis was normal. She had developed pseudotumor cerebri following prolonged use and sudden withdrawal of oral betamethasone which was prescribed for common cold.

    References

    1. Cardinale A, Rosati C, Giani I, Pezzati M. Pseudotumor cerebri due to cessation of corticosteroids- A case report. Minerva Pediatr 1991; 43: 457-460.

    2. Maria Carmen B Wilson. Pseudotumor cerebri: Pediatric perspective. Available from URL:www.emedicine.com. (Accessed February 2004).

    3. Robert H.A. Haslam. Pseudotumor cerebri. In Behrman, Kliegman, Jensen, eds. Nelson textbook of Pediatrics. 17th edn. Philadelphia; W.B. Saunders, 2004; 2048-2049.

    4. Tamer S.K, Tamer U, Warey P. Infantile Pseudotumor cerebri related to viral illness. Indian J Pediatr 1996; 63: 645-649.

    5. Lawton NF. Benign Intracranial Hypertension. In Weatherall DJ, Ledingham JGG, Warrell DA, eds. Oxford Textbook of Medicine 3rd edn. Oxford, New York, Tokyo; Oxford University Press, 1996; 4040-4043.

    6. Ishaq Abu-Arafen. Headache and migraine. In Neil McIntosh, Peter J Helms, Rosalind L Smyth, eds. Forfar and Arneil's Textbook of Pediatrics. 6th edn. Newyork, Philadelphia, Toronto; Churchill Livingstone, 2003; 951-957.

    7. Cody CM. Benign intracranial hypertension. Am Fam Physician 1992; 45: 1671-1678.

    8. Roussounis SH. Benign Intracranial Hypertension after withdrawal of topical steroids in an Infant. Br Med J 1976; 2: 504.

    9. Ravichetan, Ramesh S, Ramana AV, Indirabai K. Abuse of betamethasone drops. Indian Pediatr 1991; 28: 1083-1084.(Balasubramanian S, Shivba)