Differential Diagnosis for Diffuse Intracranial Vasculopathy

The patient's presentation with acute left MCA infarct and multiple intracranial vasculopathies, including long segment stenosis of the right ICA, absent both ACA, and occluded left MCA, suggests a complex vascular pathology. The following differential diagnoses are considered:

• Single Most Likely Diagnosis

  • Atherosclerosis: Given the patient's age, history of dyslipidemia, and the presence of long segment stenosis and occlusions, atherosclerosis is the most likely cause of her diffuse intracranial vasculopathy. Atherosclerosis is a common cause of cerebral vasculopathy, especially in older adults with risk factors for vascular disease.

• Other Likely Diagnoses

  • Vasculitis (e.g., Giant Cell Arteritis, Primary Central Nervous System Vasculitis): Although less common, vasculitis can cause diffuse intracranial vasculopathy. The patient's age and the absence of other systemic symptoms make this less likely but still a consideration.
  • Moyamoya Disease: This is a rare condition but can cause significant intracranial vasculopathy. It is more common in younger individuals but can present at any age.
  • Fibromuscular Dysplasia: This condition can cause stenosis and occlusion of cerebral arteries, although it is more commonly associated with extracranial carotid and vertebral artery disease.

• Do Not Miss Diagnoses

  • Reversible Cerebral Vasoconstriction Syndrome (RCVS): Although the patient's presentation does not strongly suggest RCVS, missing this diagnosis could lead to inappropriate treatment and poor outcomes. RCVS typically presents with thunderclap headaches and is often associated with vasoconstriction rather than fixed stenosis or occlusion.
  • Dissections: Carotid or vertebral artery dissections can lead to intracranial vasculopathy and are critical to diagnose due to their potential for significant morbidity and mortality if not properly managed.

• Rare Diagnoses

  • Ehlers-Danlos Syndrome: A genetic disorder that can affect blood vessels and lead to intracranial vasculopathy, although it is rare and typically presents with other systemic manifestations.
  • Neurofibromatosis Type 1: Can cause vascular dysplasia and intracranial vasculopathy, but the patient's age and lack of other characteristic features (e.g., neurofibromas, café-au-lait spots) make this less likely.
  • MELAS Syndrome (Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-like episodes): A mitochondrial disorder that can cause stroke-like episodes and vasculopathy, but it typically presents at a younger age and with other systemic and neurological symptoms.