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Neurocrit Care. 2017 Feb;26(1):103-108. doi: 10.1007/s12028-016-0320-4.

Reversible cerebral vasoconstriction syndrome and posterior reversible encephalopathy syndrome associated with intracranial hypotension.

Author information

1
Department of Neurology, University Hospital, Campus Grosshadern, Marchioninistrasse 15, 81377, Munich, Germany. katharina.feil@med.uni-muenchen.de.
2
German Center for Vertigo and Balance Disorders, University Hospital, Campus Grosshadern, Marchioninistrasse 15, 81377, Munich, Germany. katharina.feil@med.uni-muenchen.de.
3
Department of Neuroradiology, University Hospital, Munich, Germany.
4
Department of Neurology, University Hospital, Campus Grosshadern, Marchioninistrasse 15, 81377, Munich, Germany.
5
Institute of Clinical Neuroimmunology, University Hospital, Munich, Germany.
6
German Center for Vertigo and Balance Disorders, University Hospital, Campus Grosshadern, Marchioninistrasse 15, 81377, Munich, Germany.

Abstract

BACKGROUND:

Reversible cerebral vasoconstriction syndrome (RCVS) and posterior reversible encephalopathy syndrome (PRES) are both rare disorders. The pathophysiology of both diseases is not yet fully understood.

METHODS:

We report the unique case of a 19-year-old comatose woman who was brought to the ER after a series of generalized tonic-clonic seizures 6 days post peridural anesthesia for cesarean section. Vital signs and initial laboratory testing including urine analysis and drug screening were unremarkable. Initial cranial CT scan showed an acute small subdural hematoma (17 mm length × 6 mm width × 30 mm height), cerebral edema with slit ventricles, and slight cerebellar tonsillar herniation as signs of intracranial hypotension. CT angiography depicted narrowing of the proximal intracranial vessels consistent with RCVS. MR imaging was also suggestive of both intracranial hypotension and RCVS and showed, in addition, vasogenic edema consistent with PRES. An extensive CSF leakage involving T1 to L2/L3 was confirmed by spinal MRI.

RESULTS:

The patient underwent conservative therapy for intracranial hypotension (e.g., head-down position) as well as epidural blood patch, which led to regression of the clinical symptoms within a few days. Follow-up MRI showed complete resolution of all radiological changes.

CONCLUSIONS:

In summary, our patient developed clinical and neuroradiological signs of intracranial hypotension and a combination of PRES and RCVS associated with a CSF leakage caused by peridural anesthesia; by treating the intracranial hypotension, the other syndromes resolved. From a clinical point of view, it is important to look for CSF leakage as a treatable possible cause of PRES and/or RCVS triggered by intracranial hypotension as in our patient postpartum. Moreover, it is vital to obtain a good history as, in cases of suspected CSF leakage with classic postural headache, a recent spinal/cranial procedure is typically present.

KEYWORDS:

Epidural blood patch; Epileptic seizures; Epileptic status; Neurological intensive medicine; Postdural puncture headache; Posterior reversible encephalopathy syndrome; Reversible cerebral vasoconstriction syndrome

PMID:
27848124
DOI:
10.1007/s12028-016-0320-4
[Indexed for MEDLINE]

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