Lyme Disease Causing Normal Pressure Hydrocephalus

Hello, and welcome to another Inside Lyme Podcast. I am your host, Dr. Daniel Cameron. In this episode, I discuss a case involving a patient who gradually developed balance problems and was later diagnosed with Lyme disease with normal pressure hydrocephalus.

I first read about this case reported by Gimsing and colleagues 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, difficulty concentrating and finding words, increased irritability, muscle soreness in his arms and neck, moderate morning headaches, and increasing fatigue.

Slow Onset of Neurologic Symptoms

According to the authors, the patient had a “slow onset of progressive balance problems” along with dizziness, urinary urgency, neck soreness, and mild 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 accompanied by 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 an underlying cause can be identified.

The patient’s spinal tap revealed lymphocytic pleocytosis and increased antibody production against Lyme disease. The spinal tap confirmed Lyme disease with a positive IgG antibody and a CSF/serum ratio of 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 pleocytosis, although Lyme antibodies remained detectable. He also developed oligoclonal bands, suggesting inflammation of the central nervous system.

“An MRI scan was repeated with now almost normalized ventricle size,” the authors report. The patient was symptom-free at his 1-year follow-up.

The medical literature describes eight additional cases of secondary normal pressure hydrocephalus due to Lyme disease. The authors warn that sNPH may be mistaken for brain atrophy, neurodegenerative disease, Lyme-induced dementia, or neuropsychiatric symptoms of Lyme disease.

Although this patient did not require a ventricular shunt, this surgical treatment can sometimes improve secondary normal pressure hydrocephalus.

Conclusion

“At the one-year follow-up no symptoms remained and the ventricular system almost normalized,” the authors write.

Although the overall incidence of secondary normal pressure hydrocephalus is low, this case demonstrates the importance of early diagnosis and distinguishing secondary NPH from idiopathic NPH. Identifying the infectious cause may prevent unnecessary neurosurgical procedures.

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 misdiagnoses might Lyme disease patients receive?

Reference

1. 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.

Thanks for listening to another Inside Lyme Podcast. You can read more about these cases in the show notes and on my website at DanielCameronMD.com. Your likes, comments, reviews, and shares help spread the word about Lyme disease.

Until next time on Inside Lyme.

Please remember that the information provided is general and not intended as specific medical advice for any particular patient. If you require specific advice, please consult an experienced healthcare professional.