Can Lyme Disease Cause Pseudotumor Cerebri?
Pseudotumor cerebri may rarely occur with Lyme disease.
Symptoms can include headaches, nausea, and papilledema.
Pediatric Lyme neuroborreliosis may mimic other neurologic disorders.
Individuals with pseudotumor cerebri develop increased intracranial pressure — pressure around the brain — without evidence of a brain tumor or another obvious structural cause.
Symptoms may include severe headaches, nausea, vomiting, papilledema, and vision changes. In some cases, untreated disease can lead to vision loss. [2]
Pediatric Lyme Disease Presenting as Pseudotumor Cerebri
Ahmad from the Department of Pediatric Neurology at the State University of New York in Buffalo described the case of a 6-year-old girl who presented with:
- Bi-frontal throbbing headaches
- Nausea and vomiting
- Bilateral papilledema
Her symptoms had been present for four days.
Visual acuity remained normal, and no other neurologic abnormalities were identified on examination.
Laboratory Findings Supporting Lyme Disease
The child tested positive by enzyme-linked immunosorbent assay (ELISA) for polyvalent antibodies to Borrelia burgdorferi and also had a positive IgM Western blot.
Although spinal fluid PCR testing for Lyme disease was negative, the lumbar puncture revealed elevated opening pressure consistent with pseudotumor cerebri.
“Pseudotumor cerebri is an uncommon condition in pediatric practices,” wrote Ahmad.
The authors advised clinicians to consider Lyme disease in the differential diagnosis of pseudotumor cerebri, particularly in Lyme-endemic areas.
What Is Papilledema?
Papilledema refers to swelling of the optic disc caused by increased intracranial pressure.
Patients may experience:
- Headaches
- Blurred vision
- Transient visual obscurations
- Nausea and vomiting
- Double vision
Recognition of papilledema is important because prolonged elevation of intracranial pressure can threaten vision.
Treatment Considerations
The authors did not describe the girl’s Lyme disease treatment in detail.
However, Thurtell and Wall from the Department of Ophthalmology and Visual Sciences at the University of Iowa reviewed common treatment approaches for pseudotumor cerebri.
According to the authors:
“Acetazolamide is the mainstay of treatment, but other medications, such as topiramate and furosemide, can be considered when acetazolamide is poorly tolerated or insufficient.”
They also described surgical options including:
- Cerebrospinal fluid diversion procedures
- Ventriculo-peritoneal shunting
- Lumbo-peritoneal shunting
- Optic nerve sheath fenestration
The presence of Lyme disease may introduce additional treatment considerations directed at the underlying infection and associated neuroinflammation.
Lyme Neuroborreliosis and Intracranial Pressure
Lyme neuroborreliosis can affect the nervous system in many ways, including meningitis, cranial neuropathies, cognitive dysfunction, radiculopathy, and rarely elevated intracranial pressure syndromes.
Pediatric patients may present differently than adults, making diagnosis challenging.
Clinical Perspective
Pseudotumor cerebri is uncommon in children, but this case highlights that Lyme disease may occasionally appear as an underlying cause in endemic regions.
Clinicians evaluating unexplained headaches, papilledema, or elevated intracranial pressure may need to consider infectious causes including Lyme disease.
FAQ: Lyme Disease and Pseudotumor Cerebri
Can Lyme disease cause pseudotumor cerebri?
Rarely, Lyme neuroborreliosis has been associated with elevated intracranial pressure syndromes including pseudotumor cerebri.
What symptoms occur with pseudotumor cerebri?
Symptoms may include headaches, nausea, vomiting, papilledema, blurred vision, and visual disturbances.
What is papilledema?
Papilledema is swelling of the optic nerve caused by increased intracranial pressure.
Related Articles:
References:
- Din F, et al. Lyme in the coconut: Paediatric neuroborreliosis. J Paediatr Child Health. 2016;52(11):1042.
- Thurtell MJ, Wall M. Idiopathic intracranial hypertension (pseudotumor cerebri): recognition, treatment, and ongoing management. Curr Treat Options Neurol. 2013;15(1):1-12.
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.