Can Lyme Disease Cause Normal Pressure Hydrocephalus?
Balance problems, urinary symptoms, and cognitive decline
Normal pressure hydrocephalus can mimic neurodegenerative disease
Rare Lyme disease cases may improve with treatment
Hello, and welcome to another Inside Lyme Podcast. I am your host Dr. Daniel Cameron. In this episode, I’ll be discussing a case involving a patient who slowly began having difficulties with his balance and was later diagnosed with Lyme disease and secondary normal pressure hydrocephalus.
Normal pressure hydrocephalus is particularly important because some cases may be reversible when an underlying cause is identified and treated.
A case report published in BMC Neurology describes a patient with Lyme disease and secondary normal pressure hydrocephalus (NPH), a condition characterized by gait impairment, cognitive symptoms, and urinary dysfunction. Recognition of this rare complication may help avoid delayed diagnosis and unnecessary procedures.
Can Lyme Disease Mimic Normal Pressure Hydrocephalus?
A 67-year-old man developed multiple symptoms over a 3- to 4-month period. He complained of balance problems, diffuse dizziness, urinary urge-incontinence, problems concentrating and with finding words, increased irritability, sore muscles of the arms and around the neck, a moderate morning headache, and an increasing feeling of exhaustion.
According to the authors, the patient had a “slow onset of progressive balance problems, also presented unspecified dizziness, urge feeling, neck soreness and discrete cognitive complaints.”
An MRI scan revealed an enlarged ventricular system compatible with normal pressure hydrocephalus (NPH).
“NPH was first recognized in 1965, as a syndrome of hydrocephalus with normal CSF opening pressure and with cognitive decline, urinary incontinence and gait disturbance, potentially reversible by neurosurgical procedures,” the authors explain.
The term secondary NPH was introduced in 2000 for cases in which the cause can be identified.
Because normal pressure hydrocephalus may resemble dementia or other neurologic disorders, identifying potentially reversible causes is important.
Gait disturbance is often considered one of the hallmark features of normal pressure hydrocephalus and may be among the earliest symptoms recognized by patients and physicians.
The patient’s spinal tap revealed “lymphocytic pleocytosis and an increased antibody production to Lyme disease.” The spinal tap was positive for Lyme disease based on positive IgG for Lyme disease with a CSF/serum-ratio = 11.7.
Lyme Disease and Secondary Normal Pressure Hydrocephalus
The man was diagnosed with Lyme disease and secondary normal pressure hydrocephalus. He was treated with a 10-day course of doxycycline.
A repeat spinal tap showed a marked reduction in his pleocytosis but persistent antibody to Lyme disease. He also developed oligoclonal bands, which suggest inflammation of the CNS due to an infection or disease.
“An MRI scan was repeated with now almost normalized ventricle size,” the authors write. The patient was reportedly symptom-free at his 1-year follow-up.
The literature describes 8 other cases of secondary normal pressure hydrocephalus due to Lyme disease. “sNPH might be mistaken for atrophic, neurodegenerative brain, Lyme induced dementia, or neuropsychiatric symptoms of Lyme disease,” the authors warn.
Because secondary normal pressure hydrocephalus can resemble neurodegenerative disorders, delayed recognition may lead to unnecessary diagnostic procedures or treatment delays.
Although this patient did not receive a ventricular shunt, it is known to improve secondary normal pressure hydrocephalus.
Conclusion
“At the one-year follow-up no symptoms remained and the ventricular system almost normalized,” the authors write.
The authors recognize that the overall incidence of secondary normal pressure hydrocephalus is low. Nevertheless, this case demonstrates “the importance of early diagnosis and distinction from [idiopathic] NPH, as the cheap and minimally invasive procedure of LBP can shorten the symptom duration and completely prevent an unnecessary surgical intervention.”
Frequently Asked Questions
Can Lyme disease cause normal pressure hydrocephalus?
Rare case reports describe secondary normal pressure hydrocephalus associated with Lyme disease. In reported cases, treatment of the underlying infection has been associated with improvement in symptoms and imaging findings.
What are the symptoms of normal pressure hydrocephalus?
Common symptoms include gait disturbance, balance problems, urinary urgency or incontinence, and cognitive decline.
Can normal pressure hydrocephalus mimic dementia?
Yes. Normal pressure hydrocephalus can present with cognitive symptoms that resemble dementia, making diagnosis challenging.
How is normal pressure hydrocephalus diagnosed?
Diagnosis typically involves clinical evaluation, brain imaging, and cerebrospinal fluid testing.
Clinical Takeaway
This case highlights a rare association between Lyme disease and secondary normal pressure hydrocephalus. The patient’s balance problems, cognitive symptoms, urinary complaints, MRI findings, and cerebrospinal fluid abnormalities improved following treatment.
Although uncommon, Lyme disease should be considered among the potentially reversible causes of secondary normal pressure hydrocephalus.
Related Articles
Neurologic Lyme disease
Brain fog and Lyme disease
Neuropsychiatric Lyme disease
Persistent Lyme disease symptoms
References
- Gimsing LN, Hejl AM. Normal pressure hydrocephalus secondary to Lyme disease, a case report and review of seven reported cases. BMC Neurol. 2020;20:347.
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
In 2012 I experienced a very similar set of s/s …. also treated as Lyme though blot did not confirm.
Symptoms are still persisting….
I believe this phenomenon is a little talked about but extremely common presentation. A complete nightmare for those suffering through it completely alone.
Thank you for this pod and discussion….invaluable !!
Dear Dr. Cameron,
Please help me to understand something I’m not clear about early lyme disease. On your site, you noted that early lyme can go missed in its early stages because some don’t get a rash or any symptoms early and the lyme is just dormant until months or years later. But, even without symptoms, would everyone with early lyme show positive blood test signs if they had been tested in the first month after a bite? In other words, it would still show up on the blood in the early stages somewhere, right? I’m talking about the IGM early reflex tests, such as the ELISA, Western Blot IGM, borellia burgdorfi PCR, borellia spp PCR.
Thank you.
The tests may never be positive even in early disease even in patients with a clear tick bite and a classic Bull’s eye rash.