Normal pressure hydrocephalus secondary to Lyme disease, a case report and review of seven reported cases

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Normal pressure hydrocephalus secondary to Lyme disease, a case report and review of seven reported cases Louise Nørreslet Gimsing1*

and Anne-Mette Hejl2

Abstract Background: Infection with tick borne Borrelia Burgdorferi (Lyme disease) can without treatment rarely develop into a chronic phase. Secondary Normal Pressure Hydrocephalus (sNPH) based on chronic infection with Borrelia Burgdorferi (Bb) is an even rarer entity, that with the right treatment is potentially curable. Case presentation: A 67-year-old male with 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 NPH. After further liquor dynamic procedures, cerebrospinal fluid (CSF) was analysed with the surprising results of lymphocytic pleocytosis, and signs of increased antibody production. Microbiology revealed chronic neuroborreliosis and the patient was treated with antibiotics accordingly. At the oneyear follow-up no symptoms remained and the ventricular system almost normalized. Conclusions: We describe the 7th published case of sNPH secondary to chronic Borreliosis in a previous healthy adult. Existing published literature has been reviewed and previous cases showed similarly nearly full clinical recovery. Primary/idiopathic NPH (iNPH) is treated with the surgical intervention of ventriculoperitoneal shunt and can be mistaken for a sNPH. The awareness of rare causes of sNPH like chronic Borreliosis is important as it is easily treated non surgically. Keywords: Secondary normal pressure hydrocephalus, Chronic Borreliosis, Normal pressure hydrocephalus, Lyme disease

Background The chronic phases of infection with the spirochete Borrelia Burgdorferi (Bb), European Lyme disease is characterized by involving several organ systems. Involvement of the nervous system, neuroborreliosis, can develop in untreated individuals usually within 2–6 weeks [1], and typically includes signs of meningeal irritation comprising of nuchal tenderness, fatigue, nausea and the two cardinal symptoms: painful meningoradiculitis and peripheral motor deficits (the clinical part of Bannwarth’s triad [1]). * Correspondence: [email protected] 1 Department of Specialized Neurorehabilitation, Hvidovre Hospital, Kettegårds Allé 30, 2650 Hvidovre, Denmark Full list of author information is available at the end of the article

The rare condition of chronic neuroborreliosis (duration > 6 months) can evolve to a variety of different sub conditions, normal pressure hydrocephalus (NPH) being one of them. In this article we present a rare adult case of NPH in a prior healthy individual, who turned out to have developed the clinical and radiological syndrome secondary to chronic neuroborreliosis. Full recovery was achieved after antibiotic (AB) treatment.

Case presentation A 67-year-old male, healthy and with no prior admissions, was seen in autumn of 2009 by his General

© The Author(s). 2020 Open Ac