Neurovascular Cases

dwi1
dwi2
t2flai

What’s the diagnosis?

Central nervous system vasculopathy including diffuse vascular beading with segmental stenosis and dilation of vessels causing multifocal cerebral infarctions

dwi1

DWI sequences show large regions of restricted diffusion in the bilateral posterior temporo-parietal lobes and also in the left frontal lobe, consistent with ischemic strokes

dwi2

t2flai

T2 FLAIR sequences show edema in these same territories.

Note the diffuse vascular “beading,” or segmental stenosis and dilation of vessels that effects the entire intracranial circulation.

Differential Diagnosis

  1. Primary CNS vasculitis
  2. Reversible cerebral vasoconstriction syndrome
  3. Diffuse intracranial atherosclerosis

Background

  • Central nervous system vasculopathy is a catchment term that includes a broad array of diseases. It can be divided into primary and secondary CNS vasculopathy, where primary CNS vasculopathy refers to disease of only the CNS while secondary CNS vasculopathy refers to disease of the CNS in the context of systemic disease like a connective tissue disorder or chronic inflammatory state.
  • The radiographic hallmark of CNS vasculopathy is “beading,” or alternating areas of segmental stenosis and dilation.
  • The pathophysiology of CNS vasculitis is an inflammatory process within the cerebral vessel walls.
  • In contrast, the pathophysiology of reversible cerebral vasoconstrictive syndrome is felt to be related to impaired autoregulation of cerebral arterial tone. It is often associated with exposure to vasoactive drugs (e.g., Triptans used for migraines) and cannabis.
  • CNS vasculopathy can follow a fulminant course that → multifocal strokes, cerebral edema, and death.

What's your treatment plan?

  • Because CNS vasculopathy encompasses so many different disease processes, it is difficult to narrow the precise diagnosis. Treatment options are often limited and supportive care is the common denominator.

In this case, treatment included supportive care and oral nimodipine, a calcium channel antagonist that selectively dilates cerebral arteries.