Correspondence

Case 1-1998: A Boy with a Seizure

N Engl J Med 1998; 338:1549-1550May 21, 1998

Article

To the Editor:

In his discussion of an 11-year-old boy with a seizure (Jan. 8 issue),1 Riviello states that in pseudotumor cerebri, pleocytosis is typically present on lumbar puncture. Our experience is that the lumbar puncture is normal. Pseudotumor cerebri is usually associated with a normal cerebrospinal fluid cell count and normal protein content.2-5

Alejandro Awad, M.D.
Ricardo Gutierrez Children's Hospital of Buenos Aires, Buenos Aires, Argentina

5 References

1. 1

   Case Records of the Massachusetts General Hospital (Case 1-1998).N Engl J Med 1998;338:112-9.
   

2. 2

   Pseudotumor cerebri. In: Nelson WE, ed. Nelson textbook of pediatrics. 15th ed. Philadelphia: W.B. Saunders, 1996:1735-6.
   

3. 3

   Weisberg LA, Chutorian AM. Pseudotumor cerebri of childhood. Am J Dis Child 1977;131:1243-1248
   Web of Science | Medline

4. 4

   Oski FA, ed. Principles and practice of pediatrics. Philadelphia: J.B. Lippincott, 1990:869.
   

5. 5

   Baker RS, Baumann RJ, Buncic JR. Idiopathic intracranial hyperten-sion (pseudotumor cerebri) in pediatric patients. Pediatr Neurol 1989;5:5-11
   CrossRef | Web of Science | Medline

Author/Editor Response

Dr. Riviello replies:

To the Editor: Dr. Awad correctly states that the results of lumbar puncture are usually unremarkable in childhood pseudotumor cerebri. However, the discussion at that point in the Case Record involved central nervous system manifestations of Lyme disease. Either pleocytosis or protein elevation occurs with aseptic meningitis, meningoencephalitis, and radiculitis in Lyme disease. What do we know about instances in which both diseases occur?

We have cared for a nine-year-old girl with childhood pseudotumor cerebri and Lyme disease, confirmed by titers, in whom diplopia and conjunctivitis developed without rash or arthritis and who had mild pleocytosis of the cerebrospinal fluid (7 white cells per cubic millimeter). Raucher et al.1 reported on two girls, one 8 years old and the other 10, with protein values of 52 mg per deciliter and 39 mg per deciliter, respectively, both without pleocytosis. Jacobson and Frens2 reported on an eight-year-old girl with 97 white cells per cubic millimeter (69 percent lymphocytes and 30 percent monocytes). In a review of the neurologic manifestations of Lyme disease in 96 children in North America, 6 had papilledema with elevated cerebrospinal fluid pressures, 25 had mild protein elevations (range, 32 to 58 mg per deciliter), and 15 had cerebrospinal fluid pleocytosis (range, 6 to 106 white cells per cubic millimeter) without clinical signs of meningitis.3 The lumbar punctures of the six patients with childhood pseudotumor cerebri showed protein elevation in four and pleocytosis in two.

Although it is not always the case, an abnormal cerebrospinal fluid examination in patients with childhood pseudotumor cerebri may suggest the presence of Lyme disease, especially in an endemic area, in the absence of another explanation.

James J. Riviello, Jr., M.D.
Children's Hospital, Boston, MA 02115

3 References

1. 1

   Raucher HS, Kaufman DM, Goldfarb J, Jacobson RI, Roseman B, Wolff RR. Pseudotumor cerebri and Lyme disease: a new association. J Pediatr 1985;107:931-933
   CrossRef | Web of Science | Medline

2. 2

   Jacobson DM, Frens DB. Pseudotumor cerebri syndrome associated with Lyme disease. Am J Ophthalmol 1989;107:81-82
   Web of Science | Medline

3. 3

   Belman AL, Iyer M, Coyle PK, Dattwyler R. Neurologic manifestations in children with North American Lyme disease. Neurology 1993;43:2609-2614
   Web of Science | Medline