Wallenberg Syndrome

Article Author:
Forshing Lui
Article Author (Archived):
Prasanna Tadi
Article Editor:
Arayamparambil Anilkumar
Updated:
6/4/2019 7:05:58 PM
PubMed Link:
Wallenberg Syndrome

Introduction

Wallenberg syndrome is also known as lateral medullary syndrome or the posterior inferior cerebellar artery syndrome. Wallenberg described the first case in 1895. This neurological disorder is associated with a variety of symptoms that occur as a result of damage to the lateral segment of the medulla posterior to the inferior olivary nucleus. It is the most typical posterior circulation ischemic stroke syndrome in clinical practice. [1]

Etiology

Wallenberg syndrome is caused most commonly by atherothrombotic occlusion of the vertebral artery, followed most frequently by the posterior inferior cerebellar artery, and least often, the medullary arteries. Hypertension is the commonest risk factor followed by smoking and diabetes. Cerebral embolism is a less frequent cause of the infarction. The other important cause to remember is vertebral artery dissection which may have risk factors including neck manipulation or injury, Marfan syndrome, Ehlers Danlos syndrome, and fibromuscular dysplasia. Vertebral artery dissection is the commonest cause of Wallenberg syndrome in younger patients.[2][3]

Epidemiology

Wallenberg syndrome is the most prevalent posterior ischemic stroke syndrome. There are nearly 800,000 patients who suffer from an acute stroke each year in the United States. Of these, 83% are ischemic strokes. Twenty percent of the ischemic strokes occur in the posterior circulation. If clinicians assume that about half of these suffer from Wallenberg syndrome, it can be estimated that there are more than 60,000 new cases of Wallenberg syndrome each year in the United States. There is a predominance of men in their sixth decade. Large artery atherothrombotic causes account for about 75% of the cases followed by cardioembolism in 17% and vertebral dissection in 8%.

Pathophysiology

The primary pathology of Wallenberg syndrome is occlusion of the posterior inferior cerebellar artery (PICA) or one of its branches[4]. The syndrome can also be due to occlusion of the vertebral artery, or the inferior, middle, or superior medullary vessels. Anatomically the infarcted area in Wallenberg syndrome is supplied by the posterior inferior cerebellar artery (PICA). It turns out occlusion of the PICA accounts for only a small number of cases. The majority (80%) are caused by occlusion of the vertebral artery which gives rise to the PICA and the anterior spinal artery before it joins with the opposite vertebral artery to form the basilar artery. The commonest mechanism of occlusion of the vertebral artery or PICA is atherothrombosis.[5]

History and Physical

A typical patient with Wallenberg syndrome is an elderly patient with vascular risk factors. Like any acute stroke syndrome, the onset is acute. The most common symptoms of onset are dizziness with vertigo, loss of balance with gait instability, hoarseness of voice and difficulty swallowing. The symptoms often progressed over several hours to sometimes a couple of days. [5]

Usually, there is no weakness associated with this syndrome and so this condition is often misdiagnosed or missed. A careful neurological examination is key to the diagnosis.  A complete Wallenberg syndrome is not common, yet partial syndromes are good enough for the diagnosis most of the time. The important points in clinical diagnosis are a combination of crossed hemiparesis or hemianesthesia to indicate a brainstem lesion and the involvement of structures in the posterolateral medulla to localize where in the brainstem.

Different combinations of the following deficits may all be found in Wallenberg syndrome:

On the side of lesion:

  • Vertigo with nystagmus (inferior vestibular nucleus and pathways). The nystagmus is typically central beating to the direction of gaze. Nausea and vomiting, and sometimes hiccup are associated with vertigo. Hiccup can often be intractable.
  • Dysphonia, dysarthria, and dysphagia (different nuclei and fibers of the IX and X nerves), often with ipsilateral loss of gag reflex
  • Horner syndrome (sympathetic fibers)
  • Ipsilateral ataxia with a tendency to fall to the ipsilateral side (inferior cerebellar hemisphere, spinocerebellar fibers, and inferior cerebellar peduncle)
  • Pain and numbness with impaired facial sensation on the face (descending trigeminal tract)
  • Impaired taste sensation (involvement of nucleus tractus solitarius)

On the contralateral side:

  • Impaired pain and temperature sensation in the arms and legs (spinothalamic tract)
  • It is important to note that there is no or only minimal weakness of the contralateral side (corticospinal fibers are ventral in location)

It is clinically interesting to note that more rostral lesions tend to be more ventrally located. These patients present with more dysphagia and dysphonia due to the involvement of the nucleus ambiguus. More caudal lesions involve more dorsolateral structures. These patients present with vertigo, ataxia, nausea/vomiting, and Horner syndrome.

Evaluation

The clinical differential diagnoses include:

  1. Other causes of vertigo especially peripheral vertigo such as acute labyrinthitis: the patient may be younger without many vascular risk factors. The nystagmus is peripheral and unidirectional or rotary. There may be associated tinnitus without other brainstem signs. Head thrust test will be positive which if present, is most helpful in differentiating a central cause such as Wallenberg syndrome from a peripheral cause.[6][7]
  2. Hemorrhagic stroke: much less common and headache is much more prominent.
  3. Acute demyelination in multiple sclerosis: patients are generally much younger, more likely female and known history of demyelinating disease
  4. Acute relapse/attack of neuromyelitis optica with the involvement of the area postrema. Patients are most likely young adult female and the signs may suggest more than one central nervous system (CNS) lesion.

The diagnosis is usually made or suspected from a clinical exam and history of presentation. MRI with diffusion-weighted imaging is the best diagnostic test to confirm the infarct in the inferior cerebellar area or lateral medulla.[8] Up to 30% of patients with nondisabling stroke do not have a lesion on DWI-MRI brain. These patients are DWI-negative stroke patients and secondary prevention should be started to prevent future strokes. [9]

A CT angiogram or MR angiogram is very helpful in identifying the site of vascular occlusion and rule out uncommon causes such as vertebral artery dissection[10].

An ECG is helpful in excluding any underlying atrial fibrillation or unexpected acute coronary syndrome.

Checking the serum electrolytes is important.

Patient with dysphagia or dysarthria needs to be assessed by the speech pathologist because any food or medicine can be given orally.

Treatment / Management

Similar to the management of any acute ischemic stroke, remember "TIME IS BRAIN." Rapid evaluation is essential to an orderly approach(algorithm) developed within each hospital or stroke center. Management in certified stroke centers has shown to improve overall patient outcome. Treatment aims at reducing the size of infarction and preventing any medical complication with the final target of improving patient outcome and prognosis. [5]

The management steps include:

  1. Intravenous (IV) thrombolysis with IV TPA within 3 or 4.5 hours of onset of the ischemic stroke with slightly different exclusion criteria. Overall IV thrombolysis, whether within 3 or 4.5 hours, improve functional stroke outcome by 30%. There have been studies showing that the window for posterior circulation strokes may be longer than 4.5 hours.[11]
  2. Endovascular revascularization: the newer devices have been shown to improve outcome with the number needed to treat to be as low as 3. These are indicated mainly for large vessel intracranial occlusion which carries very poor prognosis without revascularization.
  3. General medical therapy: Patient is best monitored in the intensive care unit (ICU) for 24 hours after IV thrombolysis. Otherwise, it will be best to manage the patients in dedicated stroke beds or units.
  • IV fluid: avoid hypotonic solution to reduce risks of cerebral edema. Normal saline generally is the best.
  • Blood pressure management: cerebral autoregulation is impaired in the infarcted areas of the brain. Blood pressure very often comes down gradually without any drug treatment. In general, BP does not need to be lowered unless the patient receives IV thrombolysis or when it is over 220/120.
  • Speech therapy assessment: very important to prevent aspiration.
  • Deep vein thrombosis prophylaxis: with sequential pressure devices and low dose heparin or low molecular weight heparin.
  • Blood sugar: best to keep the patient normoglycemic.
  • Fever: the source of fever needs to be identified and treated. A simple antipyretic with acetaminophen is helpful.
  • Antithrombotics: numerous more recent clinical trials failed to show any benefit or anticoagulants in acute stroke even in atrial fibrillation. Antithrombotic therapy with aspirin does improve the outcome.
  • Early physical therapy and occupational therapy with a good plan for rehabilitation.

Secondary stroke prevention will be decided soon. This will again include a multimodality approach:

  1. Carotid endarterectomy for significant large vessel extracranial stenosis
  2. Oral anticoagulation for cardioembolic strokes
  3. Antiplatelets such as aspirin, clopidogrel, or Aggrenox (ASA/Dipyridamole) for other forms of stroke
  4. Statin
  5. Smoking cessation
  6. Good control of diabetes
  7. Good blood pressure control
  8. Healthy diet and lifestyle with regular exercises

This multimodal approach can reduce the risk of subsequent stroke by 80%.

Prognosis

Overall Wallenberg syndrome has a better functional outcome than most other stroke syndromes. Most patients can return to satisfactory activities of daily living. The commonest sequel is gait instability.

Pearls and Other Issues

Hypertension is the commonest risk factor. Atherothrombosis of the vertebral artery is the most frequent underlying vascular cause. Vertebral artery dissection needs to be considered in younger patients especially with a history of trauma, neck manipulation, or underlying collagen disorders such as Marfan syndrome. Head thrust (head impulse) test in the emergency department can differentiate peripheral vertigo due to acute labyrinthitis from central cause due to Wallenberg syndrome.[12] The outlook for patients with Wallenberg syndrome depends on the size of the infarct, but in general, most patients have a better outcome compared with other ischemic stroke syndromes with the exception of some lacunar syndromes. Gait instability or ataxia are the most typical sequelae. Sometime hiccup can be intractable.

Enhancing Healthcare Team Outcomes

Treatment of Wallenberg Syndrome requires a rapid response and coordinated team approach involving clinicians, nurses, and pharmacists to provide the patient with the best possible outcome. [Level V]


References

[1] Ogawa K,Suzuki Y,Oishi M,Kamei S, Clinical study of 46 patients with lateral medullary infarction. Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association. 2015 May     [PubMed PMID: 25817616]
[2] Inamasu J,Nakae S,Kato Y,Hirose Y, Clinical Characteristics of Cerebellar Infarction Due to Arterial Dissection. Asian journal of neurosurgery. 2018 Oct-Dec     [PubMed PMID: 30459855]
[3] Park MG,Choi JH,Yang TI,Oh SJ,Baik SK,Park KP, Spontaneous isolated posterior inferior cerebellar artery dissection: rare but underdiagnosed cause of ischemic stroke. Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association. 2014 Aug     [PubMed PMID: 24809669]
[4] Hong YH,Zhou LX,Yao M,Zhu YC,Cui LY,Ni J,Peng B, Lesion Topography and Its Correlation With Etiology in Medullary Infarction: Analysis From a Multi-Center Stroke Study in China. Frontiers in neurology. 2018;     [PubMed PMID: 30319537]
[5] Kim JS,Caplan LR, Clinical Stroke Syndromes. Frontiers of neurology and neuroscience. 2016     [PubMed PMID: 27960164]
[6] Saber Tehrani AS,DeSanto JR,Kattah JC, Neuroimaging "HINTS" of the Lateral Medullary Syndrome. Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society. 2017 Dec     [PubMed PMID: 28471903]
[7] Chen K,Schneider AL,Llinas RH,Marsh EB, Keep it simple: vascular risk factors and focal exam findings correctly identify posterior circulation ischemia in "dizzy" patients. BMC emergency medicine. 2016 Sep 13     [PubMed PMID: 27619651]
[8] De Cocker LJ,Lövblad KO,Hendrikse J, MRI of Cerebellar Infarction. European neurology. 2017     [PubMed PMID: 28095387]
[9] Makin SD,Doubal FN,Dennis MS,Wardlaw JM, Clinically Confirmed Stroke With Negative Diffusion-Weighted Imaging Magnetic Resonance Imaging: Longitudinal Study of Clinical Outcomes, Stroke Recurrence, and Systematic Review. Stroke. 2015 Nov;     [PubMed PMID: 26419965]
[10] Spectrum of the posterior inferior cerebellar artery territory infarcts. Clinical-diffusion-weighted imaging correlates., Kumral E,Kisabay A,Ataç C,Calli C,Yunten N,, Cerebrovascular diseases (Basel, Switzerland), 2005     [PubMed PMID: 16205055]
[11] Salerno A,Cotter BV,Winters ME, The Use of Tissue Plasminogen Activator in the Treatment of Wallenberg Syndrome Caused by Vertebral Artery Dissection. The Journal of emergency medicine. 2017 May     [PubMed PMID: 28258876]
[12] Guler A,Karbek Akarca F,Eraslan C,Tarhan C,Bilgen C,Kirazli T,Celebisoy N, Clinical and video head impulse test in the diagnosis of posterior circulation stroke presenting as acute vestibular syndrome in the emergency department. Journal of vestibular research : equilibrium & orientation. 2017     [PubMed PMID: 29081427]