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Abstract Details

Dural Arteriovenous Fistula Presenting as an Unusual Cause of Tongue Atrophy
Cerebrovascular Disease and Interventional Neurology
P14 - Poster Session 14 (8:00 AM-9:00 AM)
4-002

To present a case and resolution of hypoglossal nerve palsy caused by a dural arteriovenous (AV) fistula.

Hypoglossal nerve palsies are uncommon but can be associated with a variety of systemic peripheral nervous system pathologies, including motor neuron disease (MND). However, anatomical causes must also be considered. The hypoglossal nuclei are located adjacent to the medial medulla. Following its exit through the hypoglossal canal, the nerve courses through the base of the skull, enters the carotid space and joins the ansa hypoglossi (C1) nerve fibers. The nerves then traverse the suprahyoid neck before supplying the musculature of the tongue itself. Due to the association with the ansa hypoglossi fibers, lesions proximal to the carotid space do not interfere with strap muscle functions of the neck.
A 73 year old man was referred to the ALS Clinic for evaluation of suspected bulbar-onset MND. He presented with a five-month history of worsening "tongue twitching" associated with dysarthria and dysphagia. Prior history was significant for head trauma after falling from a tree. Physical examination revealed pulsatile tinnitus in the right ear, flaccid dysarthria, and mild right facial asymmetry. Tongue examination was notable for prominent right hemiatrophy, fasciculations, and deviation of the tongue to the right. Remainder of the sensorimotor exam was unremarkable. MRI imaging was significant for flow voids surrounding the lower brain stem at spinomedullary level. Given his prior trauma, there was concern for a compressive vascular lesion. Cerebral angiography revealed a dural arteriovenous malformation involving the skull base. On examination one year following the procedure, he had only residual fasciculations, improved tongue bulk and strength, as well as normal speech and swallowing.
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A detailed understanding of clinical neuroanatomy, combined with attentive neurologic examination can aid in diagnosing cranial neuropathies that may be mistaken for more ominous pathologies, including MND.
Authors/Disclosures
Bhageeradh Mulpur, MD (University of Miami/Jackson Memorial Hospital - Vascular Neurology)
PRESENTER
Dr. Mulpur has nothing to disclose.
Erik P. Pioro, MD, DPhil, FÂé¶¹´«Ã½Ó³»­ (University of British Columbia) Dr. Pioro has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Avanir Pharmaceutical, Inc.. Dr. Pioro has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Amylyx Pharmaceuticals. Dr. Pioro has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Argenx. Dr. Pioro has received personal compensation in the range of $500-$4,999 for serving as a Consultant for MT Pharma America, Inc.. Dr. Pioro has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for NeuroTherapia, Inc.. Dr. Pioro has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for MT Pharma America, Inc..