Wallenberg–Zakharchenko syndrome in vascular neurology emergency care: A review

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Abstract

Wallenberg–Zakharchenko syndrome associated with lateral medullary infarction has been known to neurologists since the end of the 19th century. However, to this day, its diagnosis is challenging due to the polymorphic, atypical, and rapidly changing clinical manifestations. Timely verification of the syndrome provides essential information regarding its etiology and also prevents serious complications. The paper presents clinical and anatomical correlates of lateral medullary infarction, its etiology, features of the clinical presentation, complications, and prognosis. In conclusion, a diagnostic algorithm that can be used in everyday practice is given.

About the authors

Aleksey A. Kulesh

Vagner Perm State Medical University; City Clinical Hospital №4

Author for correspondence.
Email: aleksey.kulesh@gmail.com
ORCID iD: 0000-0001-6061-8118

D. Sci. (Med.)

Russian Federation, Perm; Perm

Dmitry A. Demin

Federal Center for Cardiovascular Surgery

Email: aleksey.kulesh@gmail.com
ORCID iD: 0000-0003-2670-4172

Cand. Sci. (Med.)

Russian Federation, Astrakhan

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2. Fig. 1. Anatomy and blood supply of the medulla oblongata.

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3. Fig. 2. LMI due to vertebral artery dissection. A 41-year-old female patient. History of Wolff-Parkinson-White syndrome, radiofrequency catheter ablation, and sinus venosus atrial septal defect. On January 24, 2024, after a long car trip, the patient developed dizziness, pain in the occipital region, nasal voice, numbness of the face on the right, decreased sensitivity, and a burning sensation in the left extremities. She had been bothered by neck pain for a month, and had undergone dental treatment 5 days before admission. She was admitted 2 hours after the onset of the disease. On admission, Horner's syndrome was detected on the right (a), horizontal-torsional nystagmus directed to the left, a positive OLD test on the right, mild dysarthria and dysphagia, decreased pain and temperature sensitivity (to a greater extent) in the left half of the body, and severe truncal ataxia. CT of the brain was performed, revealing pronounced concomitant deviation of the eyes to the right - RadOLD (d). CTA revealed occlusion of segments III and IV of the right vertebral artery with a "peanut shell" pattern (hypotensive lumen and accumulation of contrast by the wall of the horizontal segment of the artery); b, as well as medial displacement of the right vocal cord, indicating its paresis (c). Dysphagia regressed after 3 days. On the 5th day, MRI of the brain was performed, allowing visualization of right-sided dorsolateral medullary infarction (d) and signs of intramural hematoma of the wall of segment III of the left vertebral artery as a sign of dissection (e).

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4. Fig. 3. Clinical example of typical LMI. A 61-year-old patient. In 2021, he suffered a myocardial infarction and takes clopidogrel. On February 25, 2024, his wife noticed that the patient had slurred speech, and soon unsteadiness when walking, nausea, and vomiting developed. He was admitted 6 hours after the onset of the disease with complaints of dizziness. On admission, Horner's syndrome was detected on the left (a, lower image), horizontal-torsional nystagmus directed to the right, a positive OLD test on the left (a), mild dysarthria and dysphagia, decreased pain and temperature sensitivity (to a greater extent) in the right half of the body, and severe truncal ataxia. CT scan of the brain was performed, visualizing the developing area of ​​decreased density in the lateral sections of the medulla oblongata on the left (c2), calcifications in the projection of segment IV of the left vertebral artery (c1), and pronounced concomitant deviation of the eyes to the left (b). CTA revealed pronounced stenosis of segment IV of the left vertebral artery (d), as well as medial displacement of the left vocal cord, indicating its paresis (e). The next day, dysphagia progressed to severe, a nasogastric tube was inserted. After 2 weeks, according to bronchoscopy with swallowing assessment, grade 1 dysphagia, paresis of the upper third of the larynx and left vocal cord persisted. A month later, MRI (T2-weighted image) of the brain was performed, visualizing an area of ​​cystic changes in the lateral sections of the medulla oblongata on the left (c3).

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5. Fig. 4. Dynamic anisocoria in Horner's syndrome in a patient with LMI. Using Frenzel video glasses, the phenomenon of delayed pupil dilation was demonstrated during a sharp transition from bright light to complete darkness in a patient with LMI (a, DWI MRI) due to chronic occlusion of segment IV of the right vertebral artery (c – CTA, occlusion is indicated by an asterisk).

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6. Fig. 5. Clinical example of Opalski syndrome. A 51-year-old patient. He has a long-term history of arterial hypertension and type 2 diabetes mellitus; in 2018, he suffered from myocardial infarction. On the evening of December 2, 2022, he felt dizzy and unsteady when walking. The next day, weakness in the left leg appeared. He was admitted a day after the onset of the first symptoms. The neurological status on admission was intense horizontal nystagmus directed to the right, a positive OLD test on the left, Horner's syndrome on the left (a, upper image), mild left-sided hemiparesis (a, lower image), dysarthria, and hiccups. CT of the brain was performed, calcifications were visualized in the projection of segment IV of the left vertebral artery, as well as pronounced concomitant deviation of the eyes to the left (b). CTA revealed marked stenosis of segment IV of the right vertebral artery (c), as well as medial displacement of the left vocal cord, indicating its paresis (d). MRI performed the following day allowed visualization of infarction of the dorsolateral sections of the medulla oblongata on the left, extending below the pyramidal decussation (e). Contrast administration revealed its intense concentric accumulation in the wall of segment IV of the left vertebral artery, indicating unstable substenotic atheroma (f).

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7. Fig. 6. Algorithm for early diagnosis of LMI.

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