Pseudotumor Cerebri in Lyme Disease: A Pediatric Case
Lyme disease can present with neurologic symptoms that are easily overlooked. In rare cases, it may mimic pseudotumor cerebri in children.
What Is Pseudotumor Cerebri?
Pseudotumor cerebri is characterized by increased intracranial pressure without an identifiable mass or structural cause.
Symptoms may include severe headaches, nausea, vomiting, and visual disturbances. In some cases, the condition can lead to optic disc swelling (papilledema) and vision loss.
Case Presentation
A 6-year-old girl presented with a 4-day history of bi-frontal, throbbing headaches accompanied by nausea and vomiting.
Examination revealed bilateral papilledema with preserved visual acuity. No additional neurologic abnormalities were identified.
Laboratory findings supported Lyme disease. The patient tested positive on enzyme-linked immunosorbent assay (ELISA) and immunoglobulin M Western blot for Borrelia burgdorferi.
Although cerebrospinal fluid PCR testing was negative for Lyme disease, the opening pressure was elevated—consistent with pseudotumor cerebri.
Clinical Implications
Pseudotumor cerebri is uncommon in pediatric practice, but this case highlights the importance of considering Lyme disease in the differential diagnosis.
Clinicians should be particularly alert in endemic regions or in patients with recent travel to such areas.
For a broader neurologic context, see Neurologic Lyme disease.
Treatment Considerations
The authors did not detail treatment in this case. However, standard management of pseudotumor cerebri may include medications such as acetazolamide, with alternatives including topiramate or furosemide.
Surgical options—such as cerebrospinal fluid diversion or optic nerve sheath fenestration—may be considered in severe cases.
Importantly, identifying Lyme disease introduces an additional treatment pathway with antibiotic therapy.
Clinical Takeaway
Lyme disease may rarely present as pseudotumor cerebri in children. Recognizing this association may expand diagnostic and treatment considerations, particularly in endemic areas.
Dr. Daniel Cameron, MD, MPH
Lyme disease clinician with over 30 years of experience and past president of ILADS.
Symptoms • Testing • Coinfections • Recovery • Pediatric • Prevention
This was my 11 year old son’s first presenting symptom, back in 2011 after influenza illness and Boyscouts camp out. Multiple spinal taps didn’t help and 5 neurosurgeons refused to shunt him, saying that his pain was due to an unknown secondary inflammatory process and he was not qualified for a shunt.
2 years after his Pseudotumor diagnosis He tested positive for Bartonella Henselae, Mycoplasma Pneumonia, Babesia Duncani and has 4 positive Lyme bands on WB. We are currently treating with combination oral antibiotics, Plasma exchanges and IVIG. Slowly his 7 year long constant head pressure and pain is dissipating and he is getting his life back!
Thank you for posting this! Way too many children are diagnosed as “Idiopathic” Intracranial Hypertension (IIH) or Pseudotumor Cerebri and have no hope of ever being cured.
My 15 year old daughter was diagnosed with pseudo tumor cerebri in Aug 2020. Doctors think cause is minocycline for acne but now that she tested positive for Lyme disease I’m not sure. Optic nerve grade 3 swelling is gone with Diamox but intracranial pressures still high and headaches persist with some flashing lights and black spots in her visual field. IgM positive but IGG for Lyme negative. Tests for Co infections for Lyme negative but question if this was a false negative? Would explain continued need for spinal taps but they really don’t relieve her headaches I have contacted Mayo Clinic for a second opinion since my daughter now had to take sodium bicarbonate for metabolic acidosis from the Diamox dose of 3000 mg per day.