Vessel wall calcification and vascular event: Are we concerned enough?

Area of Science:

• Neurology
• Neuroimmunology
• Vascular Neurology

Background:

• Primary central nervous system (CNS) vasculitis is a rare condition affecting blood vessels in the brain and spinal cord.
• Early diagnosis and treatment are crucial for favorable outcomes, but presentation can be variable and delayed.

Observation:

• A 49-year-old female with hypertension and dyslipidemia initially presented with thalamic bleed and multiple micro-hemorrhages.
• After a 3-year symptom-free interval, she developed complex partial seizures, with MRI revealing increased microbleeds and ischemic changes.
• Cerebrospinal fluid analysis and digital subtraction angiography confirmed small vessel primary CNS vasculitis.

Findings:

• The patient experienced a significant increment in brain microbleeds and periventricular ischemic changes.
• Diagnostic workup, including CSF studies and angiography, was consistent with primary CNS vasculitis.
• The case demonstrated a late presentation of primary CNS vasculitis after a 3-year latency period.

Implications:

• This case underscores the necessity of maintaining a high index of suspicion for primary CNS vasculitis, even with delayed or atypical presentations.
• Stringent follow-up protocols are essential for patients with a history of neurological events and risk factors for vasculitis.
• Prompt immunosuppressive therapy led to clinical improvement, emphasizing the treatable nature of this condition.