Weber Syndrome: Treatment Approach
Critical Clarification: Two Distinct Conditions
Weber syndrome refers to a midbrain stroke syndrome (superior alternating hemiplegia), NOT Sturge-Weber syndrome or Parkes Weber syndrome—these are completely different entities that should never be confused. 1
Treatment of Weber Syndrome (Midbrain Stroke)
Weber syndrome requires acute stroke management with antiplatelet therapy, strict blood pressure control, and supportive care, with an excellent prognosis when treated early. 2
Acute Management
- Antiplatelet therapy is the cornerstone of treatment for this ischemic midbrain infarction 2
- Strict blood pressure control is essential to prevent extension of the infarct and reduce risk of recurrence 2
- Piracetam has been used as adjunctive neuroprotective therapy with good results 2
Clinical Features to Monitor
Weber syndrome presents with the classic triad of:
- Ipsilateral oculomotor nerve palsy (ptosis, medial gaze palsy) 2
- Contralateral hemiparesis due to corticospinal tract involvement 2
- This "crossing hemiplegia" pattern is pathognomonic for midbrain infarction 2
Prognosis
Despite being a brainstem stroke, Weber syndrome carries a favorable prognosis with early treatment and risk factor control. 2
- Massive clinical improvement can occur within 3 weeks of medical treatment 2
- The key to good outcomes is early recognition and aggressive management of vascular risk factors, particularly hypertension 2
Diagnostic Workup
- Diffusion-weighted MRI is the imaging modality of choice, showing millimetric diffusion restriction in the mesencephalon consistent with acute infarct 2
- The etiology is typically occlusion of paramedian branches of the posterior cerebral artery or perforating branches of the basilar bifurcation 2
Common Pitfall: Terminology Confusion
Never confuse Weber syndrome with:
- Sturge-Weber syndrome: A congenital neurocutaneous disorder with facial port-wine stain, leptomeningeal angioma, and epilepsy requiring completely different management (laser therapy, anticonvulsants, possible hemispherectomy for refractory epilepsy) 1, 3, 4
- Parkes Weber syndrome: A congenital vascular malformation with arteriovenous malformations requiring embolization or surgical resection 5
- Osler-Weber-Rendu syndrome (HHT): Hereditary hemorrhagic telangiectasia with liver vascular malformations requiring intensive medical therapy or transplantation for symptomatic cases 6