Intracranial hypotension mimicking chronic progressive external ophthalmoplegia

Orbit. 2018 Oct;37(5):371-374. doi: 10.1080/01676830.2017.1423346. Epub 2018 Jan 4.


Intracranial hypotension (ICH) is characterized by low cerebrospinal fluid pressure, postural headaches, and diffuse pachymeningeal enhancement on magnetic resonance imaging (MRI). A variety of ophthalmoparetic manifestations have been reported in the context of the ICH. The authors describe an unusual case of a 64-year-old woman who presented with rapid onset of headaches, bilateral upper-lid ptosis, and blurring of vision within 4 days after sustaining a trivial head injury. She was noted to have bilateral symmetrical ophthalmoplegia and ptosis-simulating chronic progressive external ophthalmoplegia. MRI revealed characteristic features of ICH. Subsequent autologous epidural patch therapy led to resolution of the headache and imaging findings; however, her ptosis and motility disorder persisted. Despite existing therapeutic measures for ICH, irreversible cranial nerve damage may ensue due to significant cerebral decent or ischemic injury.

Keywords: Blepharoptosis; chronic progressive external ophthalmoplegia; epidural patching; intracranial hypotension; upper-lid ptosis.

Publication types

  • Case Reports

MeSH terms

  • Blepharoptosis / diagnosis*
  • Blepharoptosis / etiology
  • Craniocerebral Trauma / complications
  • Diagnosis, Differential
  • Female
  • Headache / diagnosis*
  • Headache / etiology
  • Humans
  • Intracranial Hypotension / diagnostic imaging*
  • Intracranial Hypotension / etiology
  • Magnetic Resonance Imaging*
  • Middle Aged
  • Ophthalmoplegia, Chronic Progressive External / diagnostic imaging*
  • Ophthalmoplegia, Chronic Progressive External / etiology
  • Vision Disorders / diagnosis*
  • Vision Disorders / etiology