Secondary normal pressure hydrocephalus due to Lyme disease

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. I will discuss lyme disease and normal pressure hydrocephalus.

I first read about this case by Gimsing et al. in the journal BMC Neurology. [1]

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 muscles 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.

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 with 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.

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.”

This podcast addresses the following questions:

1. What is normal pressure hydrocephalus (NPH)?
2. What are the symptoms of NPH?
3. What is the difference between idiopathic NPH and secondary NPH?
4. How do you diagnose NPH?
5. Have you seen Lyme patients with NPH?
6. What is the treatment for NPH?
7. What are the misdiagnoses that Lyme disease patients might receive?

1. Gimsing, L.N., Hejl, AM. Normal pressure hydrocephalus secondary to Lyme disease, a case report and review of seven reported cases. BMC Neurol 20, 347 (2020).

Thanks for listening to another Inside Lyme Podcast. You can read more about these cases in my show notes and on my website @DanielCameronMD.com. As always, it is your likes, comments, reviews, and shares that help spread the word about Lyme disease. Until next time on Inside Lyme.

Please remember that the advice given is general and not intended as specific advice as to any particular patient. If you require specific advice, then please seek that advice from an experienced professional.