Lateral medullary syndrome: Difference between revisions

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*[[Ageusia]] or [[loss of taste]] on one side of the [[tongue]]
*[[Ageusia]] or [[loss of taste]] on one side of the [[tongue]]
*[[Ataxia]] or [[incoordination]]
*[[Ataxia]] or [[incoordination]]<ref name="pmid2389292">{{cite journal |vauthors=Ferbert A, Brückmann H, Drummen R |title=Clinical features of proven basilar artery occlusion |journal=Stroke |volume=21 |issue=8 |pages=1135–42 |date=August 1990 |pmid=2389292 |doi=10.1161/01.str.21.8.1135 |url=}}</ref>
*[[Diplopia]] or [[double vision]]
*[[Diplopia]] or [[double vision]]
*Oscillopsia
*Oscillopsia
Line 98: Line 98:
*[[Dysphonia]] or [[hoarseness]]
*[[Dysphonia]] or [[hoarseness]]
*[[Dysarthria]] or [[slurred speech]]
*[[Dysarthria]] or [[slurred speech]]
*Ipsilateral sensory  deficits (pain and temperature sensation) affecting the face and cranial nerves
*Ipsilateral sensory  deficits (pain and temperature sensation) affecting the face and cranial nerves<ref name="pmid19269682">{{cite journal |vauthors=Kim YK, Schulman S |title=Cervical artery dissection: pathology, epidemiology and management |journal=Thromb. Res. |volume=123 |issue=6 |pages=810–21 |date=April 2009 |pmid=19269682 |doi=10.1016/j.thromres.2009.01.013 |url=}}</ref>
**absence of pain on the ipsilateral side of the face, as well as an absent [[corneal reflex]] (Damage to the spinal [[trigeminal nucleus]])
**absence of pain on the ipsilateral side of the face, as well as an absent [[corneal reflex]] (Damage to the spinal [[trigeminal nucleus]])
*[[Ataxia]]
*[[Horner's syndrome]]
*[[Horner's syndrome]]
*Ipsilateral vocal fold paralysis
*Ipsilateral vocal fold paralysis

Revision as of 23:02, 4 August 2020

Lateral medullary syndrome
The three major arteries of the cerebellum: the SCA, AICA, and PICA. (Posterior inferior cerebellar artery is PICA.)
ICD-10 G46.3
DiseasesDB 10449
MeSH D014854

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Wallenberg's syndrome; posterior inferior cerebellar artery syndrome (PICA)

Overview

The lateral medullary syndrome is one of the most common clinical syndromes of brain stem caused by the decreased blood supply to the lateral medulla. It is also commonly known as Wallenberg's syndrome or posterior inferior cerebellar artery syndrome (PICA). The most common cause is thromboembolic occlusion of vertebral arteries. It was described in 1895. The lateral medullary syndrome is basically a manifestation of the vaso-occlusive disease of intracranial vertebral arteries (ICVA) such as vertebral artery or posterior inferior cerebellar artery. The various pathophysiologic mechanisms involved can include; atherosclerosis, athero-embolic phenomenon (heart, aorta, or vertebral arteries), dissection and increased vascular tortuosity, vascular insufficiency, Virchow’s triad play an important role in understanding the pathogenesis of Wallenberg's syndrome.

Historical Perspective

  • Gaspard Vieusseux, in 1808, was the first person to describe Wallenberg's syndrome.[1]
  • This syndrome was later on further elaborated by Adolf Wallenberg, in 1895.[2]
  • Thomas William was the first person to document extensive anatomy and physiology of brain stem, the cerebellum, and the ventricles in the 17th century. He performed necropsies and extensive dissections on his patient's brains.
  • Joseph Jules Dejerine (1849–1917) and his wife Dejerine-Klumpke demonstrated extensive visual illustrations of the various brain stem and cerebellar lesions.
  • Charles Foix (1882–1927) was the first person to write an extensive case series on posterior cerebral arteries occlusion related syndromes and lateral medullary syndrome.
  • Vertebral Basal Insufficiency (VBI) was first introduced by clinicians at the Mayo Clinic, Bob Siekert and Clark Millikan in 1970s.[3]

Pathophysiology

The lateral medullary syndrome is basically a manifestation of the vaso-occlusive disease of intracranial vertebral arteries (ICVA) such as vertebral artery or posterior inferior cerebellar artery. The various pathophysiologic mechanisms involved can include;[4][5][6]

  • Atherosclerosis,
  • Athero-embolic phenomenon (heart, aorta, or vertebral arteries)
  • Dissection and increased vascular tortuosity
  • Vascular insufficiency
  • Virchow’s triad play an important role in understanding the pathogenesis of Wallenberg's syndrome
    • An abnormality of the intima and vascular wall
    • An abnormality of blood flow, and
    • An abnormality of blood coagulability

Involvement of various structures in lateral medulla along with respective manifestation or clinical signs include;

  • Nucleus ambiguous: dysphagia, dysphonia, and dysarthria, laryngeal, pharyngeal and palatal paralysis
  • Trigeminal nucleus: ipsilateral facial and corneal anesthesia
  • Spinothalamic tract: loss of pain and temperature sensation to the opposite side of the body
  • Cerebellum: ataxia
  • Hypothalamic fibers: sympathetic nervous system abnormal c/w Horners syndrome
  • Deiters' nucleus and other vestibular nuclei: nystagmus and vertigo
  • Central tegmental tract: palatal myoclonus

Causes

Risk Factors

Natural History, Complications and Prognosis

  • The natural history, complications, and prognosis of Lateral medullary syndrome depends upon the size and location of the infarct/hemorrhagic area of the medulla.
  • Some people may experience a gradual improvement in their symptoms with complete resolution of the symptoms within the week to months while others may worsen or show no improvement despite the treatment.
  • Overall, the prognosis is good and most of the patients are able to return back to a normal baseline. Ataxia is seen as the most common sequelae.
  • The most common complications seen are;
    • Aspiration pneumonia
    • Deep vein thrombosis
    • Pulmonary embolism
    • Myocardial infarction

Diagnosis

History and Physical Examination

  • Rostral lesions present as marked dysphagia and dysphonia (nucleus ambiguous)
  • Caudal lesion present as vertigo, ataxia, nausea/vomiting, and Horner syndrome

Ipsilateral (same side of lesion):

Contralateral (opposite side of lesion):

  • Contralateral sensory deficits (pain and temperature sensation) affecting the trunk and extremities
  • No or minimal hemiparesis

Evaluation:

Differential Diagnosis:

  • Hemorrhagic stroke
  • Multiple sclerosis
  • Acute labyrinthitis
  • Acute neuromyelitis optic
  • Chronic pain syndrome
  • Lacunar stroke
  • Middle cerebral artery stroke
  • Migraine headache
  • Posterior reversible encephalopathy syndrome
  • Subarachnoid hemorrhage
  • Subdural hematoma
  • Systemic lupus erythematosus
  • Vertebrobasilar stroke

Diagnostic Tests:

  • MRI is the best diagnostic test to establish the diagnosis of Wallenberg's syndrome resulting from an infarct.
  • CTA and MRA can also be done to determine the vascular occlusion sites and to rule out dissection.
  • An EKG should be done to rule out any underlying thromboembolic phenomenon such as afib.

Localization of the Lesion

Dysfunction Effects
lateral spinothalamic tract contralateral deficits in pain and temperature sensation from body
spinal trigeminal nucleus ipsilateral loss of pain and temperature sensation from face
nucleus ambiguus (which affects vagus X and glossopharyngeal nerves IX) dysphagia, hoarseness, diminished gag reflex
vestibular system vertigo, diplopia, nystagmus, vomiting
descending sympathetic fibers ipsilateral Horner's syndrome
central tegmental tract palatal myoclonus

Treatment

  • An interprofessional approach, aiming at a rapid response and coordinated team effort, involving neurologist, neurology specialty nurse, and the pharmacist has shown improved outcomes.
  • Treatment of Wallenberg's syndrome, like other stroke management, is aimed to achieve 3 goals
    • Reducing the size of infarction
    • Preventing any medical complication
    • Improving patient outcome and prognosis
  • Management includes:
    • IV Thrombolytics
      • Tissue plasminogen activator (TPA) within 3-4.5 hours
    • Endovascular revascularization
      • For larger intracranial vessels
    • Carotid endarterectomy
      • For larger extracranial vessels
    • Antithrombotics has a controversial role in the setting of an acute stroke but have shown improved outcomes when combined with aspirin
      • Oral anticoagulants and antiplatelet agents should be considered upon discharge for secondary prevention of stroke
    • High dose statins
    • Close ICU monitoring for first 24 hrs after giving TPA
      • Blood pressure monitoring, allow permissive hypertension and lower the BP only if,
        • BP > 220/120 mmHg
        • Patient receives IV TPA
      • Normal saline is preferred for IV fluids and hypotonic fluids should be avoided to prevent cerebral edema
    • Speech therapy to assess the risk of aspiration. A feeding tube or PEG tube may be considered for patients with severe dysphagia.
    • Low dose heparin or low molecular weight heparin (LWMH) for DVT prophylaxis
    • Physical therapy and Occupational therapy

References

  1. Ogawa K, Suzuki Y, Oishi M, Kamei S (May 2015). "Clinical study of 46 patients with lateral medullary infarction". J Stroke Cerebrovasc Dis. 24 (5): 1065–74. doi:10.1016/j.jstrokecerebrovasdis.2015.01.006. PMID 25817616.
  2. Lui F, Tadi P, Anilkumar AC. PMID 29262144. Missing or empty |title= (help)
  3. Amarenco P, Hauw JJ (1989). "[Anatomy of the cerebellar arteries]". Rev. Neurol. (Paris) (in French). 145 (4): 267–76. PMID 2535662.
  4. Hong YH, Zhou LX, Yao M, Zhu YC, Cui LY, Ni J, Peng B (2018). "Lesion Topography and Its Correlation With Etiology in Medullary Infarction: Analysis From a Multi-Center Stroke Study in China". Front Neurol. 9: 813. doi:10.3389/fneur.2018.00813. PMC 6170644. PMID 30319537.
  5. Kim JS, Caplan LR (2016). "Clinical Stroke Syndromes". Front Neurol Neurosci. 40: 72–92. doi:10.1159/000448303. PMID 27960164.
  6. Caplan LR (January 2015). "Lacunar infarction and small vessel disease: pathology and pathophysiology". J Stroke. 17 (1): 2–6. doi:10.5853/jos.2015.17.1.2. PMC 4325635. PMID 25692102.
  7. Inamasu J, Nakae S, Kato Y, Hirose Y (2018). "Clinical Characteristics of Cerebellar Infarction Due to Arterial Dissection". Asian J Neurosurg. 13 (4): 995–1000. doi:10.4103/ajns.AJNS_373_16. PMC 6208259. PMID 30459855.
  8. Park MG, Choi JH, Yang TI, Oh SJ, Baik SK, Park KP (August 2014). "Spontaneous isolated posterior inferior cerebellar artery dissection: rare but underdiagnosed cause of ischemic stroke". J Stroke Cerebrovasc Dis. 23 (7): 1865–70. doi:10.1016/j.jstrokecerebrovasdis.2014.02.023. PMID 24809669.
  9. Saleem F, M Das J. PMID 31869134. Missing or empty |title= (help)
  10. Ferbert A, Brückmann H, Drummen R (August 1990). "Clinical features of proven basilar artery occlusion". Stroke. 21 (8): 1135–42. doi:10.1161/01.str.21.8.1135. PMID 2389292.
  11. Kim YK, Schulman S (April 2009). "Cervical artery dissection: pathology, epidemiology and management". Thromb. Res. 123 (6): 810–21. doi:10.1016/j.thromres.2009.01.013. PMID 19269682.

External links

Template:Diseases of the nervous system Template:Lesions of the spinal cord and brainstem

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