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Wallenberg Syndrome (Lateral Medullary Syndrome)
Eponym: Named after German neurologist Adolf Wallenberg, who described it in 1895 and provided the precise anatomical description in 1901.
Also called: Posterior Inferior Cerebellar Artery (PICA) syndrome, Vertebral Artery syndrome, Lateral Medullary Syndrome (LMS).
Anatomy of the Lateral Medulla
The lateral medulla contains a wedge-shaped region supplied by the PICA (or directly by the vertebral artery). The structures packed into this small area explain the rich clinical syndrome:
| Structure | Function |
|---|
| Spinal nucleus & tract of CN V (trigeminal) | Ipsilateral facial pain/temperature |
| Spinothalamic tract | Contralateral body pain/temperature |
| Nucleus ambiguus (CN IX, X, XI) | Swallowing, phonation, palatal elevation |
| Descending sympathetic fibers | Ipsilateral Horner's syndrome |
| Vestibular nuclei | Vertigo, nystagmus, nausea/vomiting |
| Inferior cerebellar peduncle | Ipsilateral cerebellar signs |
| Nucleus tractus solitarius | Taste, autonomic regulation |
The pyramidal tract, tongue (CN XII), and dorsal columns are spared, as they lie in the medial medulla.
Etiology
Most commonly caused by ischemic infarction of the dorsolateral medulla. In a series of 130 consecutive patients with pure lateral medullary infarctions:
- Large vessel atherosclerosis (vertebral or PICA stenosis/occlusion): ~50%
- Vertebral artery dissection: ~15% - especially in younger patients, often preceded by neck pain or recent neck manipulation
- Small vessel disease: ~13%
- Cardioembolism: ~5%
Less common causes include cocaine abuse, medullary tumors (usually metastases), demyelination (MS), abscess, vascular malformation hemorrhage, radionecrosis, and neck trauma.
Classic Clinical Features
The hallmark is a crossed sensory deficit - ipsilateral face, contralateral body.
1. Sensory
- Ipsilateral facial hypalgesia and thermoanesthesia - loss of pain and temperature on the same side face (spinal nucleus/tract of CN V)
- Contralateral body hypalgesia and thermoanesthesia - loss of pain and temperature in the trunk and limbs on the opposite side (spinothalamic tract)
- Vibration, proprioception, and touch are preserved (medial lemniscus is in medial medulla)
2. Motor/Bulbar
- Dysphagia - difficulty swallowing
- Dysarthria/dysphonia/hoarseness - weakness of palate, pharynx, vocal cord
- Ipsilateral palatal, pharyngeal, and vocal cord paralysis (nucleus ambiguus involvement - CN IX, X)
- No limb weakness (pyramids are spared)
3. Sympathetic
- Ipsilateral Horner's syndrome - ptosis, miosis, anhidrosis (descending hypothalamospinal sympathetic fibers)
4. Vestibular/Cerebellar
- Vertigo, nausea, vomiting (vestibular nuclei)
- Nystagmus (horizontal, torsional, or mixed)
- Ipsilateral cerebellar ataxia - limb ataxia, gait ataxia, falling to the side of the lesion (inferior cerebellar peduncle)
- Lateropulsion - strong tendency to fall/veer toward the side of the lesion
5. Ocular
- Skew deviation and ocular tilt reaction
- Multiple types of nystagmus (horizontal, torsional, mixed)
- Gaze-holding abnormalities
- Smooth pursuit defects
The Triad
The clinical triad of Horner syndrome + ipsilateral ataxia + contralateral hypalgesia is the best identifier of lateral medullary infarction.
Rare/Additional Features
Described in extensive case series:
- Wild arm ataxia (lateral cuneate nucleus involvement)
- Central neuropathic pain with allodynia
- Loss of taste (nucleus tractus solitarius - rostral/lateral zone)
- Inability to sneeze (sneezing center in spinal trigeminal nucleus)
- Autonomic dysfunction - tachycardia, blood pressure lability
- Ondine's curse (central hypoventilation syndrome) - failure of automatic breathing; life-threatening if not recognized; due to lesions of nucleus ambiguus and adjacent reticular formation
- Contralateral hyperhidrosis with ipsilateral anhidrosis (sympathetic disruption)
- Opalski syndrome - Wallenberg + ipsilateral hemiparesis (lesion extends to pyramidal decussation at the caudal medulla)
Sensory Pattern Variants
The crossed pattern is the classic presentation, but variants occur based on lesion location:
- Far lateral lesion: Ipsilateral face + contralateral foot/leg only (selective sacral/lumbar spinothalamic fibers hit, which are located most laterally)
- Mediolateral lesion: Ipsilateral face + contralateral entire hemibody (unilateral pattern) - ventral trigeminothalamic tract also affected
- Rostral medullary lesion: More severe dysphagia, hoarseness, facial paresis
- Caudal medullary lesion: More marked vertigo, nystagmus, gait ataxia
Diagnosis
Imaging
- MRI with diffusion-weighted imaging (DWI) is the investigation of choice - confirms the infarct in the lateral medulla
- DWI positive from day 1 (earliest indicator)
- T2-weighted MRI may not show the infarct initially; becomes visible after several days
- MRA (MR Angiography) - shows loss of flow in the vertebral artery or PICA; identifies arterial dissection
- T1-weighted axial MRI through the vertebral arteries - bright intramural signal (crescent sign) indicates vertebral dissection
Treatment
| Aspect | Management |
|---|
| Acute ischemic stroke | IV thrombolysis (tPA) within 4.5 hours if eligible (high risk in this region - use with caution) |
| Vertebral dissection | Antiplatelet agents (aspirin); some use anticoagulation (controversial) |
| Atherosclerotic disease | Antiplatelet therapy (aspirin, clopidogrel), statins, blood pressure control |
| Dysphagia | Nasogastric feeding, speech therapy, swallowing rehabilitation |
| Nausea/vertigo | Antiemetics, vestibular suppressants short-term |
| Neuropathic pain | Gabapentin, TCAs, or other neuropathic pain agents |
| Ondine's curse | Mechanical ventilation, close respiratory monitoring |
| Rehabilitation | Physiotherapy (ataxia, lateropulsion), speech therapy, occupational therapy |
Prognosis
Recovery is generally good for most deficits. In the case example from the textbook, at 11-day follow-up, the patient no longer had nausea, vertigo, or nystagmus and could walk, with only mild residual Horner's, slight sensory deficit, and trace ataxia. Dysphagia and ataxia typically improve over weeks to months. Neuropathic pain can be a persistent, troublesome long-term complication.
- Wallenberg syndrome is the most common posterior circulation stroke syndrome, accounting for ~2-3% of all ischemic strokes
- It is the most prevalent syndrome related to intracranial vertebral artery occlusion
Memory Aid
"AEIOU" or think of the lateral medulla contents:
Ipsilateral side (same as lesion): Horner's + facial pain/temp loss + cerebellar ataxia + bulbar palsy (dysphagia, hoarseness)
Contralateral side (opposite): body pain/temp loss
Spared (medial medulla): Motor power of limbs, tongue, vibration/proprioception
Sources: - Localization in Clinical Neurology, 8e - Neuroanatomy through Clinical Cases 3rd Ed. - K.J. Lee's Essential Otolaryngology