Edit concept Create issue ticket

Lateral Medullary Syndrome

Wallenbergs Syndrome

Lateral medullary syndrome occurs as a result of either vertebral or cerebellar artery occlusion. Ischemia of brain tissue and the tracts passing through the medulla manifest with various symptoms, most commonly ataxia, nystagmus, voice changes, dysphagia and sensory deficits. The diagnosis is made clinically and with the help of magnetic resonance imaging. Treatment includes symptomatic measures and anticoagulant drugs.


Presentation

The clinical presentation of patients with this syndrome may significantly vary and depends on which site and vessel is affected. Symptoms may appear abruptly, which is the case in the majority of patients, but they may gradually occur as well. Patients in whom trigeminal nerve nuclei and pathways are damaged, ipsilateral facial pain or loss of pain and temperature sensation may be present and if the spinothalamic tract is affected, contralateral sensory loss is observed [7]. Vestibular nuclei involvement may present with nystagmus, diplopia, blurred vision, nausea, vomiting and vertigo. Dysphagia, dysphonia, dysarthria, hoarseness and in rare cases, stridor are symptoms that are reported in patients in whom nucleus ambiguus is affected [8[ [9]. Cerebellar involvement includes symptoms such as gait abnormalities, weakness and ataxia. Horner's syndrome and headaches are also common findings in these patients, while hiccups may be reported as well [9].

Paroxysmal Sneeze
  • Paroxysmal sneezing can be an initial manifestation of lateral medullary ischemia and may not necessarily indicate an underlying vertebral artery dissection as the cause.[ncbi.nlm.nih.gov]
  • sneezes Results Paroxysmal sneezing at beginning stage of LMS can be a symptom of early ischemia in the medullary sneezing center.[prezi.com]
Diplopia
  • This is a case of a 61-year-old woman who developed progressive right hemisensory deficit, left facial sensory deficit, vertigo, staggering to the left, left ptosis, vertical diplopia, and ataxia of the left upper extremity.[ncbi.nlm.nih.gov]
  • Nystagmus, diplopia, and hiccups were also evident. A left lateral medullary syndrome in the vascular distribution of the posterior inferior cerebellar artery was diagnosed.[ncbi.nlm.nih.gov]
  • Eleven patients had ocular symptoms (diplopia or blurred vision). Horner's syndrome was found in 91%, ipsilateral ataxia in 85%, and contralateral hypalgesia in 85%. Nystagmus (61%) and facial weakness (42%) were less frequent.[ncbi.nlm.nih.gov]
  • This syndrome is characterised by: vestibulocerebellar symptoms: vertigo, falling towards the side of lesion, diplopia, and multidirectional nystagmus ( inferior cerebellar peduncle and vestibular nucleus ) 1-3 autonomic dysfunction: ipsilateral Horner's[radiopaedia.org]
  • […] features Structure affected Ipsilateral Dysphagia Gag reflex Hoarseness , dysphonia Nucleus ambiguus motoric nucleus Ataxia , dysmetria , dysdiadochokinesia Inferior cerebellar peduncle Horner syndrome Uncontrollable hiccups Sympathetic fibers Nystagmus , diplopia[amboss.com]
Blepharoptosis
  • Blepharoptosis : Once life-threatening conditions have been ruled out and the patient is stable, visually symptomatic ptosis can be managed surgically. The surgical approach varies on surgeon preference.[eyewiki.aao.org]
Facial Pain
  • He also described the other cases reported by Golby et al ., and Warren et al . [5] Facial pain may be a feature in some cases of LMS.[neurologyindia.com]
  • Symptoms such as facial pain may be seen in patients with trigeminal nuclei ischemia, since it is responsible for sensory innervation of the face, while vestibular nuclei are responsible for coordination.[symptoma.com]
  • Risk factors: atherosclerosis Chief complaints: Vertigo Difficulty walking Facial pain Difficulty swallowing Difficulty speaking History Of Present Illness: Onset of symptoms will be sudden (given the pathophysiology of this condition) Nature of vertigo[stepwards.com]
  • Side Manifestation Comments Ipsilateral Sensory loss – face – pain and temperature CN 5 nucleus Ipsilateral Facial pain CN 5 nucleus Ipsilateral Ataxia – arm and leg Restiform body, cerebellum Ipsilateral Gait ataxia Restiform body, cerebellum Ipsilateral[strokecenter.org]
  • Clinical symptoms include difficulty swallowing, slurred speech, facial pain, vertigo, Horner syndrome, and possibly palatal myoclonus Otolaryngological features include dysphonia, dysphagia and nasal regurgitation related to ipsilateral vocal fold paralysis[medicine.uiowa.edu]
Nystagmus
  • The disturbance of visual perception associated with nystagmus is a rare phenomenon.[ncbi.nlm.nih.gov]
  • Torsional nystagmus was recorded in 3 patients with the lateral medullary syndrome. Magnetic search coil oculography demonstrated slow phases of nystagmus of increasing, decreasing, and constant velocity.[ncbi.nlm.nih.gov]
  • Audiovestibular test battery revealed bilateral normal hearing, bilateral gaze nystagmus, rebound nystagmus beating toward the right side, loss of visual suppression with augmentation of caloric nystagmus in light on the left side, and delayed vestibular[ncbi.nlm.nih.gov]
  • Seesaw and hemi-seesaw nystagmus are rare in lateral medullary syndrome. We describe a patient with lateral medullary syndrome who, in addition to other more typical ocular manifestations, demonstrated hemi-seesaw nystagmus (HSSN).[ncbi.nlm.nih.gov]
  • In most patients smooth pursuit, optokinetic nystagmus and visual suppression of the vestibulo-ocular reflex were still impaired when this spontaneous drift was minimal or absent.[ncbi.nlm.nih.gov]
Vertigo
  • Gait ataxia (88%), vertigo/dizziness (91%), nausea/vomiting (73%), dysphagia (61%), hoarseness (55%), Horner sign (73%), and facial (85%) and hemibody (94%) sensory changes were frequent clinical findings.[ncbi.nlm.nih.gov]
  • A 39-year-old male was admitted to our hospital due to vertigo, dysarthria and numbness of the left face and the right limbs.[ncbi.nlm.nih.gov]
  • This is a case of a 61-year-old woman who developed progressive right hemisensory deficit, left facial sensory deficit, vertigo, staggering to the left, left ptosis, vertical diplopia, and ataxia of the left upper extremity.[ncbi.nlm.nih.gov]
  • A 41-year-old man was admitted to the neurology department with the complaints of fever, headache, vertigo, tinnitus and ataxia.[ncbi.nlm.nih.gov]
  • Such patients have acute vertigo with weakness or deviation of the jaw.[angelfire.com]
Ataxia
  • The triad of Horner's syndrome, ipsilateral ataxia, and contralateral hypalgesia will clinically identify patients with lateral medullary infarction.[ncbi.nlm.nih.gov]
  • Gait ataxia (88%), vertigo/dizziness (91%), nausea/vomiting (73%), dysphagia (61%), hoarseness (55%), Horner sign (73%), and facial (85%) and hemibody (94%) sensory changes were frequent clinical findings.[ncbi.nlm.nih.gov]
  • The damage to the cerebellum or the inferior cerebellar peduncle can cause ataxia .[en.wikipedia.org]
  • […] resulting from a cerebrovascular accident involving part of the medulla oblongata with consequent loss of pain and temperature sensation in the orofacial region, loss of taste, and palatal palsy and loss of gag reflex, together with Horner's syndrome and ataxia[ncbi.nlm.nih.gov]
  • This is a case of a 61-year-old woman who developed progressive right hemisensory deficit, left facial sensory deficit, vertigo, staggering to the left, left ptosis, vertical diplopia, and ataxia of the left upper extremity.[ncbi.nlm.nih.gov]
Dysarthria
  • A 39-year-old male was admitted to our hospital due to vertigo, dysarthria and numbness of the left face and the right limbs.[ncbi.nlm.nih.gov]
  • A 67-year-old man was admitted to our hospital for dizziness, dysarthria, and dysphagia. He was diagnosed with lateral medullary syndrome based on the neurological examination and brain magnetic resonance imaging. Horner syndrome was absent.[ncbi.nlm.nih.gov]
  • Initially anxiety and migraine attack were impressed in the emergency room, but dizziness accompanied with dysarthria and dysphagia was noted later.[ncbi.nlm.nih.gov]
  • Speech therapy may be often necessary, as patients may present with severe dysarthria that may persist for a prolonged period of time.[symptoma.com]
  • According to a researcher Wallenberg syndrome can also occur after a chiropractic treatment (chiropractic manipulation Caplan, 1986) Horner's syndrome Nystagmus Brainstem infarctation (see arrow) hoarseness (dysphonia) difficulty to articulate (dysarthria[braininjury-explanation.com]
Dizziness
  • The patient complained severe dizziness with hyperventilation while watching television. Initially anxiety and migraine attack were impressed in the emergency room, but dizziness accompanied with dysarthria and dysphagia was noted later.[ncbi.nlm.nih.gov]
  • A 67-year-old man was admitted to our hospital for dizziness, dysarthria, and dysphagia. He was diagnosed with lateral medullary syndrome based on the neurological examination and brain magnetic resonance imaging. Horner syndrome was absent.[ncbi.nlm.nih.gov]
  • Gait ataxia (88%), vertigo/dizziness (91%), nausea/vomiting (73%), dysphagia (61%), hoarseness (55%), Horner sign (73%), and facial (85%) and hemibody (94%) sensory changes were frequent clinical findings.[ncbi.nlm.nih.gov]
  • […] and medullary reticular zone Most common vascular lesion resulting in lateral medullary syndrome Proximal or middle portion of intracranial vertebral artery Most common presentation of lateral medullary syndrome Pain or dysesthesia in ipsilateral face Dizziness[quizlet.com]
  • He presented with headache, dizziness, nausea and vomiting, blurred vision, and a tendency to fall to the right.[pediatricneurologybriefs.com]

Workup

The initial diagnosis of lateral medullary syndrome can be made based on findings encountered during physical examination, but a definite diagnosis is made through imaging techniques such as CT, MRI and MR angiography [10]. CT scan, as an initial diagnostic method, can provide clues about the location of the infarction, but MRI is still the gold standard in the diagnosis of this syndrome. MRI of the endocranium can provide more specific images in comparison to CT and can detect medullary infarction with greater sensitivity and specificity. More importantly, it can determine its exact location, and thus identify the artery that was damaged. MR angiography is a method that wherein contrast is used to visualize vessels supplying the medulla. It provides an optimal view of the affected arteries. Stenosis of the vessel lumen, presence of intramural hematomas and dissection of the vessel may all be identified with this diagnostic method [10] [11].

Additional tests that can be made during the diagnostic protocol include evaluation of blood pressure, levels of cholesterol and triglycerides and a complete blood count. Hypertension is a significant risk factor in these patients, and should be diagnosed and properly treated if present. Platelet count may be raised, which is why antiplatelet therapy is one of the main treatment strategies in these patients.

Treatment

Treating patients with lateral medullary syndrome includes symptomatic treatment as well as antiplatelet medications. The scope of symptomatic therapy depends on the associated findings. Hypertension should be corrected with angiotensin-converting enzyme inhibitors (ACEs), angiotensin-receptor blockers, diuretics, beta blockers or calcium antagonists, while pain is managed with non-steroidal anti-inflammatory drugs (NSAIDs). In patients with poor general condition, nasogastric tube placement for adequate nutrition, orotracheal intubation and assisted ventilation may be necessary.

The use of antiplatelet therapy is still not standardized in patients with lateral medullary syndrome, but their role in the treatment of cervical artery dissection and other cerebrovascular events seems to be beneficial [12]. Aspirin belongs to the group of NSAIDs, but one of its main properties is prevention of platelet aggregation, thus promoting anticoagulatory mechanisms in the cerebral vessels, while clopidogrel is an antiplatelet agent that reduces the rate of platelet formation. However, these agents should be used with caution, because in the case of arterial dissection, several complications may occur, including subarachnoid hemorrhage, formation of pseudoaneurysms and dislocation of thrombi that may occlude other vessels. Surgery is rarely indicated as a therapeutic strategy, as the occluded vessels lie deep in the endocranium and are not accessible without extensive tissue damage. But in patients who are not responding to therapy, it may be performed.

Speech therapy may be often necessary, as patients may present with severe dysarthria that may persist for a prolonged period of time. Repetitive transcranial magnetic stimulation (rTMS) has been used in management of dysphagia with promising results, but further studies are necessary prior to its introduction in clinical practice [13].

Prognosis

The prognosis depends on the location and severity of occlusion. Patients may recover without any sequelae, but neurological deficits may persist for a prolonged period of time and can become permanent in cases of severe ischemia. Certain studies have established mortality rates of about 11% in the acute phase of illness, while the long-term prognosis is generally good, with only a few patients experiencing recurrences and sequelae [6].

Etiology

The principal cause of lateral medullary syndrome is occlusion of either vertebral or posterior inferior cerebellar artery (PICA). The vertebral artery is a much more common site of occlusion. Pathogenic mechanisms include arteriosclerosis, formation of blood clots, embolism, or in some cases, dissection of the arterial vessel. In rare cases, occlusion of the basilar or extracranial segments of the vertebral artery is observed [3].

Epidemiology

This condition seems to affect male patients more commonly than women, typically in late adulthood. Various studies have confirmed this finding, and several risk factors have been established. Hypertension and preexisting arteriosclerosis have been identified as the most important risk factors in these patients [4], while other factors, such as coronary heart disease, diabetes mellitus, cocaine abuse, trauma, infectious encephalitis and brain tumors have all been linked to the development of this syndrome.

Sex distribution
Age distribution

Pathophysiology

The pathogenesis of lateral medullary syndrome starts with blood vessel stenosis and obstruction, most commonly on the grounds of preexisting hypertension and atherosclerosis [5]. Vessels that are affected supply the structures in the brain stem, including the medulla oblongata, pons, cerebellum and various cranial nuclei. Once the blood vessel is occluded, oxygen and nutrients cannot reach the target tissue, which leads to ischemia and development of neurological deficits. Several cranial nerves and tracts can be damaged, primarily the nuclei of the vestibular and trigeminal nerves, while the spinothalamic tract, nucleus ambiguus and other sites may be damaged. Symptoms such as facial pain may be seen in patients with trigeminal nuclei ischemia, since it is responsible for sensory innervation of the face, while vestibular nuclei are responsible for coordination. The inferior cerebellar peduncule can also be affected by ischemia, which is involved in maintaining balance and gait. The spinothalamic tract carries signals responsible for pain and temperature contralaterally, while nucleus ambiguus is a network of motor neurons that innervate various structures in the oropharynx, including the pharyngeal and soft palatal muscles and the larynx, giving typical symptoms of hoarseness, dysphonia and dysphagia.

One of the most common clinical findings in patients - Horner's syndrome, is presumed to occur due to the damage of the descending sympathetic fibers that originate in the hypothalamus.

Prevention

For cerebrovascular insults, including lateral medullary syndrome, factors such as hypertension, smoking, alcohol consumption, poor dietary habits and physical inactivity are all presumed to increase the risk for its development. Significant steps in terms of prevention can be made, including regular blood pressure control, which sometimes necessitates pharmacologic therapy, while cessation of smoking and reduction in daily alcohol intake is recommended. Changes in dietary habits, as well as regular exercise are proven to have beneficial effects on the cardiovascular system.

Summary

Lateral medullary syndrome is a form of cerebral infarction that occurs in the brain stem and primarily due to occlusion of vertebral and cerebellar arteries although arterial dissection may also be a cause. It was initially described more than 100 years ago as Wallenberg syndrome after the German physician who was the first to document the typical neurological features associated with the condition [1]. It is caused by vessel occlusion due to atherosclerosis, clot formation, or embolization of vertebral or posterior inferior cerebellar artery (PICA), while basilar and external parts of the vertebral artery can also be sites of occlusion. Ischemia leads to impaired nutrient and oxygen delivery to the tissues with the development of symptoms which may appear either abruptly or insidiously. This syndrome more commonly develops in male adults, usually in late adulthood. Hypertension is presumed to be the main risk factor. Clinical presentation depends on the areas which are affected. These may include nucleus ambiguus, cerebellum, vestibular or trigeminal nuclei, spinothalamic tract, and other parts of the brain stem. Common symptoms include ataxia, weakness, gait disturbances, vertigo, dysphonia, dysarthria, hoarseness, diplopia and nystagmus, while dysphagia and aspiration are seen as well [2]. Horner's syndrome, comprised of ptosis, anhydrosis and miosis is commonly encountered in these patients and should immediately suggest cerebellar infarction in the differential diagnosis. Lateral medullary syndrome is diagnosed on clinical grounds, while magnetic resonance imaging (MRI) is used to confirm medullary infarction. Magnetic resonance angiography may identify the exact blood vessel that is damaged, and treatment is aimed at alleviating symptoms together with antiplatelet therapy, usually with aspirin or clopidogrel. In severe cases, nasogastric tube insertion and intubation may be necessary because of poor general condition. Surgical treatment is reserved for patients who do not respond to therapy.

Patient Information

Lateral medullary syndrome (also known as Wallenberg syndrome, after the physician who initially described the neurological features of the disease) is a form of stroke that occurs due to inadequate oxygen and nutrient supply of various parts of the brain because of arterial occlusion. The most important risk factor is presumed to be hypertension, in which atherosclerosis of blood vessels predisposes individuals to this syndrome. Arteries that are involved include the vertebral artery, through which blood travels from the neck into the brainstem, and the posterior inferior cerebellar artery (PICA), which supplies portions of the little brain. When obstruction of blood flow through the affected blood vessel occurs, oxygen delivery is impaired, leading to onset of various symptoms. Patients may present with balance disturbances, difficulties in swallowing, facial pain, voice changes, vision problems and loss of pain sensation. Symptoms may appear either abruptly or slowly, and depend on which site of the brain is affected. The physician can make a presumptive diagnosis during physical examination, while a definite diagnosis is made through imaging of the head. Magnetic resonance imaging (MRI) is the diagnostic technique of choice because it provides a clear view of the brain and is able to identify the part of the brain that is affected. Magnetic resonance angiography is used as it is a superior method to identify which vessel is damaged and is performed by injecting a contrast into the bloodstream, which aids in depicting which vessels are affected. Treatment includes symptomatic therapy, which may range from pain management to placement of feeding tubes and assisted ventilation, as well as anticoagulant therapy, which aims at reducing formation of blood clots that may further occlude the arterial vessels. Because this disorder is linked to hypertension, significant preventive measures can be performed, including regular blood pressure therapy, proper dietary habits, regular exercise and avoiding cigarette smoking and alcohol consumption. The prognosis of this syndrome is good, but significant neurological deficits may persist for a prolonged period of time, and even fatal outcomes are reported, which is why a timely diagnosis and immediate treatment is very important.

References

Article

  1. Wallenberg A. Akute BulbäraVektion (Embolie der Arteria cerebelli post inf sinistra). Archives fur Psychiatry. 1895;27: 504–540.
  2. Kim H, Chung CS, Lee KH, Robbins J. Aspiration subsequent to a pure medullary infarction: lesion site, clinical variables and outcome. Arch Neurol. 2000;57:478-483.
  3. Quast M, Liebegott G. Pathogenesis of Wallenberg's syndrome [Article in German]. Beitr Pathol. 1975;154(3):308-323.
  4. Kim JS. Pure lateral medullary infarction: clinical-radiological correlation of 130 acute, consecutive patients. Brain. 2003;126(8):1864-1872.
  5. Shetty SR, Anusha R, Thomas PS, Babu SG. Wallenberg’s syndrome. J Neurosci Rural Pract. 2012;3(1):100-102.
  6. Norrving B, Cronqvist S. Lateral medullary infarction: Prognosis in an unselected series. Neurology. 1991;41:244-248.
  7. Sacco RL, Freddo L, et al. Wallenberg's lateral medullary syndrome. Clinical-magnetic resonance imaging correlations. Arch Neurol. 1993;50:609–614.
  8. Vaidyanathan S, Capper R, Chadha D. Stridor: an unusual presentation of lateral medullary syndrome. J Laryngol Otol. 2007;121:e9.
  9. Park MH, Kim BJ, Koh SB, et al. Lesional location of lateral medullary infarction presenting hiccups. J Neurol Neurosurg Psychiatry. 2005;76:95–98.
  10. Kim JS, Lee JH, Choi CG. Patterns of lateral medullary infarction: vascular lesion-magnatic resonance imaging correlation of 34 cases. Stroke. 1998;29(3):645-652.
  11. García-García J, Ayo-Martín Ó, Segura T. Lateral Medullary Syndrome and Ipsilateral Hemiplegia (Opalski Syndrome) Due to Left Vertebral Artery Dissection. Arch Neurol. 2009;66(12):1574-1575.
  12. Kim YK, Schulman S. Cervical artery dissection: pathology, epidemiology and management. Thromb Res. 2009;123(6):810-821.
  13. Khedr EM, Abo-Elfetoh N. Therapeutic role of rTms on recovery of dysphagia in patients with lateral medullary syndrome and brainstem infarction. J Neurol Neurosurg Psychiatry 2010;81(5):495-499.

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!
Last updated: 2018-06-21 20:55