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Dysgeusia and amaurosis fugax: a unique presentation in spontaneous internal carotid artery dissection

      Spontaneous internal carotid artery (ICA) dissection is a significant cause of ischemic stroke in young adults
      • Debette S.
      Pathophysiology and risk factors of cervical artery dissection: what have we learnt from large hospital-based cohorts?.
      . The presenting symptoms and signs of ICA dissection (ICAD) are variable and can be due to direct local involvement of neural structures (e.g., oculosympathetic plexus causing the ipsilateral Horner syndrome) or indirect distal ischemia to brain or eye (e.g., transient ischemic attack or stroke) from thromboembolic disease
      • Downer J.
      • Nadarajah M.
      • Briggs E.
      • Wrigley P.
      • McAuliffe W.
      The location of origin of spontaneous extracranial internal carotid artery dissection is adjacent to the skull base.
      • Sturzenegger M.
      • Huber P.
      Cranial nerve palsies in spontaneous carotid artery dissection.
      . Transient monocular visual loss (TMVL) and the Horner syndrome are well-known presentations of ICAD and may occur in up to 50% of cases
      • Biousse V.
      • Touboul P.-J.
      • D'Anglejan-Chatillon J.
      • Lévy C.
      • Schaison M.
      • Bousser M.-G.
      Ophthalmologic manifestions of internal carotid artery dissection.
      . A distortion of the sense of taste (i.e., dysgeusia) in ICAD, however, is not as widely known as presenting symptoms especially among ophthalmologists. We present a case of TMVL associated with dysgeusia and describe the clinical presentation, localizing topographic anatomy, pathogenesis, and significance of dysgeusia to ophthalmologists evaluating patients with possible ICAD.
      A 55-year-old white man presented with 2 episodes of TMVL of the left eye (OS) over 2 weeks associated with a “bitter taste in his mouth.” He had been dining in a restaurant with his wife and complained to the restaurant manager about the taste of the food. He was seen at an outside clinic for eye pain and headache, which was diagnosed as “sinusitis” and treated with antibiotics. His medical history was significant for hypertension and hyperlipidemia. He had prior spine surgery for degenerative disc disease. He had no ocular history or allergies. His family history was noncontributory. His medications were amlodipine, vitamin C, CoQ10, losartan, and rosuvastatin.
      His vision was 20/20 in both eyes. There was no temporal artery nodularity or tenderness. Pupils were briskly reactive bilaterally with neither anisocoria nor relative afferent pupillary defect. Anterior segment and fundus examinations were normal. The patient had resolution of his TMVL but persistent dysgeusia.
      The patient was admitted to the stroke team at Houston Methodist Hospital. Noncontrast computed tomography (CT) of head was normal. Magnetic resonance imaging of the head was normal, including diffusion-weighted imaging. Electrocardiogram and cardiac echocardiogram were normal. Routine laboratory studies were normal, including complete blood count. Serum erythrocyte sedimentation rate was 12 mm/hr and C-reactive protein measured 0.65 mg/dL.
      Contrast CT angiogram (CTA) of the head and neck showed a dissection of the distal cervical and proximal petrous portions of the left ICA (Fig. 1A–C). The patient was started on intravenous heparin. He had initially been on corticosteroids, which were discontinued. A digital subtraction catheter arteriogram showed a small intraluminal thrombus distal to the petrous left ICAD, but no other hemodynamic insufficiency was seen in the intracranial circulation. The patient was transitioned from heparin to apixaban (Eliquis) before discharge. The patient returned for follow-up 3 months later and had a normal eye examination and complete resolution of his dysgeusia and TMVL episodes.
      Fig 1
      Fig. 1(A) Coronal computed tomography angiogram (CTA) showing dissection of the cervical and petrous portions of the left internal carotid artery (ICA). (B) Oblique CTA showing filling defects in the distal portions of the left ICA. (C) Oblique digital subtraction angiogram showing long segment narrowing and intramural irregularity in the left ICA.
      Patients with TMVL should undergo timely evaluation for embolic or nonembolic etiologies of ischemia. In older patients, evaluation and testing for giant cell arteritis is recommended (e.g., serum erythrocyte sedimentation rate and C-reactive protein, temporal artery biopsy). All patients with ischemic TMVL should undergo a full stroke work-up, including neuroimaging (e.g., acute noncontrast CT of the brain followed by magnetic resonance imaging including diffusion-weighted imaging of the brain. Vascular imaging (e.g., CT or MR angiography) of the head and neck is recommended to evaluate for vascular etiologies, including carotid dissection. In addition, echocardiogram, electrocardiogram, and a carotid Doppler are generally indicated. Older age (more than 65 years), higher blood pressure, longer duration of symptoms, and the presence of comorbidities like diabetes raise the risk of a subsequent stroke in the next 2–7 days

      Givre S., Van Stavern G.P.Amaurosis fugax (transient monocular or binocular visual loss). www.uptodate.com/contents/amaurosis-fugax-transient-monocular-or-binocular-visual-loss. Accessed

      .
      ICADs usually occur distal to the carotid bifurcation (where atherosclerotic disease is known to occur)
      • Downer J.
      • Nadarajah M.
      • Briggs E.
      • Wrigley P.
      • McAuliffe W.
      The location of origin of spontaneous extracranial internal carotid artery dissection is adjacent to the skull base.
      and are typically cervical in location. Greater than 90% of ICADs arise within 2 cm cranial to the carotid bifurcation in the cervical section (C2) of the ICA, and approximately half of these extend into the petrous portion of the ICA (C3)
      • Downer J.
      • Nadarajah M.
      • Briggs E.
      • Wrigley P.
      • McAuliffe W.
      The location of origin of spontaneous extracranial internal carotid artery dissection is adjacent to the skull base.
      • Sturzenegger M.
      • Huber P.
      Cranial nerve palsies in spontaneous carotid artery dissection.
      .
      The presenting symptoms of ICAD include direct local effects (e.g., neck pain, Horner syndrome) and indirect distal effects, which are ischemic thromboembolic or hypoperfusion in nature. The most common ischemic symptoms in ICAD are hemispheric transient ischemic attacks, hemispheric cerebral infarction, and TMVL (i.e., amaurosis fugax)
      • Sturzenegger M.
      • Huber P.
      Cranial nerve palsies in spontaneous carotid artery dissection.
      • Biousse V.
      • Touboul P.-J.
      • D'Anglejan-Chatillon J.
      • Lévy C.
      • Schaison M.
      • Bousser M.-G.
      Ophthalmologic manifestions of internal carotid artery dissection.
      ]. In one study from the ophthalmic literature, TMVL occurred in over half of patients with ICAD
      • Biousse V.
      • Touboul P.-J.
      • D'Anglejan-Chatillon J.
      • Lévy C.
      • Schaison M.
      • Bousser M.-G.
      Ophthalmologic manifestions of internal carotid artery dissection.
      .
      Expansion and extension of the ICAD can cause pseudoaneurysm formation and intramural hematoma
      • Debette S.
      • Grond-Ginsbach C.
      • Bodenant M.
      • et al.
      Differential features of carotid and vertebral artery dissections: the CADISP Study.
      . Common local direct symptoms of ICAD include Horner syndrome and neck pain
      • Sturzenegger M.
      • Huber P.
      Cranial nerve palsies in spontaneous carotid artery dissection.
      • Biousse V.
      • Touboul P.-J.
      • D'Anglejan-Chatillon J.
      • Lévy C.
      • Schaison M.
      • Bousser M.-G.
      Ophthalmologic manifestions of internal carotid artery dissection.
      • Debette S.
      • Grond-Ginsbach C.
      • Bodenant M.
      • et al.
      Differential features of carotid and vertebral artery dissections: the CADISP Study.
      . Head or facial pain (specifically in the orbital region) or ipsilateral eye pain (as seen in our case) can occur even though the ICAD is located in the neck. This referred pain results from the general visceral afferent (GVA) pain carried on the vagus being perceived by the general somatic afferent (GSA) of the trigeminal nerve nucleus in the brainstem
      • Biousse V.
      • Touboul P.-J.
      • D'Anglejan-Chatillon J.
      • Lévy C.
      • Schaison M.
      • Bousser M.-G.
      Ophthalmologic manifestions of internal carotid artery dissection.
      • Debette S.
      • Grond-Ginsbach C.
      • Bodenant M.
      • et al.
      Differential features of carotid and vertebral artery dissections: the CADISP Study.
      . This type of referred pain is similar to the false localizing left arm pain (GSA) in myocardial infarction (GVA) or the referred visceral pain of appendicitis or kidney stones (GVA) in the back or abdomen (GSA).
      ICAD can also produce lower cranial nerve palsies, specifically cranial nerves IX, X, XI, and XII
      • Sturzenegger M.
      • Huber P.
      Cranial nerve palsies in spontaneous carotid artery dissection.
      . Although cranial nerve IX is the closest to the petrous portions of the ICA, cranial nerve XII palsies seem to be the most common
      • Sturzenegger M.
      • Huber P.
      Cranial nerve palsies in spontaneous carotid artery dissection.
      . Involvement of branches of cranial nerves VII, IX, X, XI, and XII have been reported in ICAD
      • Sturzenegger M.
      • Huber P.
      Cranial nerve palsies in spontaneous carotid artery dissection.
      . The topographical localization of the dysgeusia in ICAD is thought to be due to glossopharyngeal nerve involvement or the chorda tympani, a branch of the facial nerve (VII)
      • Sturzenegger M.
      • Huber P.
      Cranial nerve palsies in spontaneous carotid artery dissection.
      .
      Ophthalmologists should be aware of the interesting combination of symptoms of TMVL and dysgeusia in ipsilateral ICAD. This is especially important in patients with TMVL who often have completely normal eye examinations. Patients with an ICAD may present to ophthalmology with symptoms and signs of direct compression (Horner syndrome) or indirect distal thromboembolic/hypoperfusion events (transient ischemic attacks/TMVL). Although the combination of Horner syndrome and TMVL is a well-known constellation for ipsilateral ICAD, the combination of TMVL and dysgeusia is also a highly suggestive symptom complex for ICAD of the petrous ICA even without the Horner syndrome.

      Disclosure

      The authors have no proprietary or commercial interest in any materials discussed in this article.

      Appendix. Supplementary materials

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        Cranial nerve palsies in spontaneous carotid artery dissection.
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