Lumbar Puncture Opening Pressure in Patients with Spinal Muscular Atrophy

Abstract

The announcement of a hydrocephalus as a possible side effect in patients with spinal muscular atrophy (SMA) receiving the drug nusinersen, promoted major concern and warrants further evaluation. In this retrospective monocentric study, we analyzed clinical data, lumbar puncture opening pressure (LOP) measurement, and ophthalmologic and neuroimaging results in 34 patients with SMA types 1 to 3 undergoing treatment with nusinersen. None of the patients reported symptoms indicative of increased intracranial pressure. In our cohort, the LOP was >20 cm H₂O in 25 patients (70.5%), and within this group ≥28 cm H₂O in 12 patients (35.3%), in two patients, it was increased prior to treatment initiation. Signs of increased intracranial pressure in ophthalmological assessments or brain imaging were only seen in one patient. We did not identify a correlation between increased LOP and SMA type, scoliosis, or age of the patients; however, it was slightly higher in patients receiving sedation. Our results raise the question whether the LOP is generally increased in SMA as part of the underlying disease, if so, what the etiology is, and whether the increased LOP needs to be treated.

Keywords

spinal muscular atrophy - intracranial pressure - nusinersen - hydrocephalus - MRI - lumbar puncture opening pressure

Contributor's Statement Page

L.L.B. collected data, drafted the initial manuscript, performed the analysis, and reviewed and revised the manuscript. A.T., C.W., and V.M. collected data, interpreted data, and reviewed and revised the manuscript. A.M.K. conceptualized and designed the study, coordinated and supervised data collection, and critically reviewed the manuscript for important intellectual content. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.

Publikationsverlauf

Eingereicht: 31. März 2020

Angenommen: 15. Oktober 2020

Artikel online veröffentlicht:
12. Februar 2021

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