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July 21, 2020; 95 (3) Article

Insights into the natural history of spontaneous intracranial hypotension from infusion testing

Levin Häni, Christian Fung, Christopher Marvin Jesse, Christian Thomas Ulrich, Timo Miesbach, Debora Rosalba Cipriani, Tomas Dobrocky, View ORCID ProfileWerner Josef Z'Graggen, Andreas Raabe, Eike Imo Piechowiak, Jürgen Beck
First published June 10, 2020, DOI: https://doi.org/10.1212/WNL.0000000000009812
Levin Häni
From the Department of Neurosurgery (L.H., C.M.J., C.T.U., W.J.Z., A.R., J.B.), Institute of Diagnostic and Interventional Neuroradiology (T.D., E.I.P.), and Department of Neurology (W.J.Z.), Inselspital, Bern University Hospital, Switzerland; and Department of Neurosurgery (C.F., T.M., D.R.C., J.B.), Medical Center, University of Freiburg, Germany.
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Christian Fung
From the Department of Neurosurgery (L.H., C.M.J., C.T.U., W.J.Z., A.R., J.B.), Institute of Diagnostic and Interventional Neuroradiology (T.D., E.I.P.), and Department of Neurology (W.J.Z.), Inselspital, Bern University Hospital, Switzerland; and Department of Neurosurgery (C.F., T.M., D.R.C., J.B.), Medical Center, University of Freiburg, Germany.
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Christopher Marvin Jesse
From the Department of Neurosurgery (L.H., C.M.J., C.T.U., W.J.Z., A.R., J.B.), Institute of Diagnostic and Interventional Neuroradiology (T.D., E.I.P.), and Department of Neurology (W.J.Z.), Inselspital, Bern University Hospital, Switzerland; and Department of Neurosurgery (C.F., T.M., D.R.C., J.B.), Medical Center, University of Freiburg, Germany.
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Christian Thomas Ulrich
From the Department of Neurosurgery (L.H., C.M.J., C.T.U., W.J.Z., A.R., J.B.), Institute of Diagnostic and Interventional Neuroradiology (T.D., E.I.P.), and Department of Neurology (W.J.Z.), Inselspital, Bern University Hospital, Switzerland; and Department of Neurosurgery (C.F., T.M., D.R.C., J.B.), Medical Center, University of Freiburg, Germany.
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Timo Miesbach
From the Department of Neurosurgery (L.H., C.M.J., C.T.U., W.J.Z., A.R., J.B.), Institute of Diagnostic and Interventional Neuroradiology (T.D., E.I.P.), and Department of Neurology (W.J.Z.), Inselspital, Bern University Hospital, Switzerland; and Department of Neurosurgery (C.F., T.M., D.R.C., J.B.), Medical Center, University of Freiburg, Germany.
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Debora Rosalba Cipriani
From the Department of Neurosurgery (L.H., C.M.J., C.T.U., W.J.Z., A.R., J.B.), Institute of Diagnostic and Interventional Neuroradiology (T.D., E.I.P.), and Department of Neurology (W.J.Z.), Inselspital, Bern University Hospital, Switzerland; and Department of Neurosurgery (C.F., T.M., D.R.C., J.B.), Medical Center, University of Freiburg, Germany.
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Tomas Dobrocky
From the Department of Neurosurgery (L.H., C.M.J., C.T.U., W.J.Z., A.R., J.B.), Institute of Diagnostic and Interventional Neuroradiology (T.D., E.I.P.), and Department of Neurology (W.J.Z.), Inselspital, Bern University Hospital, Switzerland; and Department of Neurosurgery (C.F., T.M., D.R.C., J.B.), Medical Center, University of Freiburg, Germany.
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Werner Josef Z'Graggen
From the Department of Neurosurgery (L.H., C.M.J., C.T.U., W.J.Z., A.R., J.B.), Institute of Diagnostic and Interventional Neuroradiology (T.D., E.I.P.), and Department of Neurology (W.J.Z.), Inselspital, Bern University Hospital, Switzerland; and Department of Neurosurgery (C.F., T.M., D.R.C., J.B.), Medical Center, University of Freiburg, Germany.
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  • ORCID record for Werner Josef Z'Graggen
Andreas Raabe
From the Department of Neurosurgery (L.H., C.M.J., C.T.U., W.J.Z., A.R., J.B.), Institute of Diagnostic and Interventional Neuroradiology (T.D., E.I.P.), and Department of Neurology (W.J.Z.), Inselspital, Bern University Hospital, Switzerland; and Department of Neurosurgery (C.F., T.M., D.R.C., J.B.), Medical Center, University of Freiburg, Germany.
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Eike Imo Piechowiak
From the Department of Neurosurgery (L.H., C.M.J., C.T.U., W.J.Z., A.R., J.B.), Institute of Diagnostic and Interventional Neuroradiology (T.D., E.I.P.), and Department of Neurology (W.J.Z.), Inselspital, Bern University Hospital, Switzerland; and Department of Neurosurgery (C.F., T.M., D.R.C., J.B.), Medical Center, University of Freiburg, Germany.
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Jürgen Beck
From the Department of Neurosurgery (L.H., C.M.J., C.T.U., W.J.Z., A.R., J.B.), Institute of Diagnostic and Interventional Neuroradiology (T.D., E.I.P.), and Department of Neurology (W.J.Z.), Inselspital, Bern University Hospital, Switzerland; and Department of Neurosurgery (C.F., T.M., D.R.C., J.B.), Medical Center, University of Freiburg, Germany.
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Insights into the natural history of spontaneous intracranial hypotension from infusion testing
Levin Häni, Christian Fung, Christopher Marvin Jesse, Christian Thomas Ulrich, Timo Miesbach, Debora Rosalba Cipriani, Tomas Dobrocky, Werner Josef Z'Graggen, Andreas Raabe, Eike Imo Piechowiak, Jürgen Beck
Neurology Jul 2020, 95 (3) e247-e255; DOI: 10.1212/WNL.0000000000009812

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Abstract

Objective To assess the pathophysiologic changes in patients with spontaneous intracranial hypotension (SIH) based on measures of CSF dynamics, and on the duration of symptoms, in a retrospective case-controlled study.

Methods We included consecutive patients investigated for SIH at our department from January 2012 to February 2018. CSF leak was considered confirmed if extrathecal contrast spillage was seen on imaging (CT or MRI) after intrathecal contrast application, or dural breach was detected by direct intraoperative visualization. We divided patients with a confirmed CSF leak into 3 groups depending on the symptom duration, as follows: ≤10, 11–52, and >52 weeks. Clinical characteristics and measures of CSF fluid dynamics obtained by computerized lumbar infusion testing were analyzed over time and compared with a reference population.

Results Among the 137 patients included, 69 had a confirmed CSF leak. Whereas 93.1% with <10 weeks of symptoms displayed typical orthostatic headache, only 62.5% with >10 weeks of symptoms did (p = 0.004). Analysis of infusion tests revealed differences between groups with different symptom duration for CSF outflow resistance (p < 0.001), lumbar baseline pressure (p = 0.013), lumbar plateau pressure (p < 0.001), baseline pressure amplitude (p = 0.021), plateau pressure amplitude (p = 0.001), pressure–volume index (p = 0.001), elastance (p < 0.001), and CSF production rate (p = 0.001). Compared to the reference population, only patients with acute symptoms showed a significantly altered CSF dynamics profile.

Conclusion A CSF leak dramatically alters CSF dynamics acutely, but the pattern changes over time. There is an association between the clinical presentation and changes in CSF dynamics.

Glossary

ICHD=
International Classification of Headache Disorders;
Pss=
sagittal sinus pressure;
PVI=
pressure–volume index;
RCSF=
resistance to CSF outflow;
SIH=
spontaneous intracranial hypotension

Footnotes

  • Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

  • CME Course: NPub.org/cmelist

  • Received May 26, 2019.
  • Accepted in final form December 19, 2019.
  • © 2020 American Academy of Neurology
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