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Published on: July 11, 2025
Unlocking the 'locked-in syndrome'.
Mano Shanmuganathan1, Charles P Warlow, Rustam Al-Shahi Salman
1Department of Neurosurgery, Western General Hospital, Edinburgh, UK. manoshanmuganathan@nhs.net
A man with locked-in syndrome was diagnosed with a petrous apex dural arteriovenous fistula causing brainstem venous hypertension. Surgical clipping resulted in full neurological recovery, highlighting this rare condition.
Area of Science:
- Neurology
- Neurosurgery
- Vascular Imaging
Background:
- Brainstem stroke is a critical diagnosis with significant morbidity.
- Locked-in syndrome presents as severe immobility with preserved consciousness.
- Petrous apex dural arteriovenous fistulas are rare vascular malformations.
Observation:
- A 48-year-old male presented with symptoms mimicking a brainstem stroke, leading to a clinical diagnosis of locked-in syndrome.
- Initial investigations were insufficient to identify the underlying cause of the patient's neurological deficits.
Findings:
- Advanced imaging revealed a petrous apex dural arteriovenous fistula as the cause of the patient's condition.
- The fistula induced profound venous hypertension within the brainstem, explaining the neurological symptoms.
Implications:
- This case underscores the importance of considering rare vascular etiologies in patients presenting with locked-in syndrome.
- Surgical intervention, specifically clipping of the fistula, can lead to complete neurological recovery.
- Accurate diagnosis and timely management of petrous apex dural arteriovenous fistulas are crucial for favorable patient outcomes.
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