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Intracranial hypotension mimicking chronic progressive external ophthalmoplegia

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ABSTRACT 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… Click to show full abstract

ABSTRACT 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: chronic progressive; progressive external; intracranial hypotension; ophthalmoplegia; external ophthalmoplegia

Journal Title: Orbit
Year Published: 2018

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