Subjective
Date of visit
Reason for visit (follow-up for PACNS, evaluation of new symptoms)
Current symptoms:
Neurological deficits (depending on affected vessels):
Headaches (common)
Weakness (focal or generalized)
Seizures
Speech or language difficulties
Cognitive impairment (memory problems, confusion)
Visual disturbances
Sensory changes (numbness, tingling)
Gait abnormalities
Constitutional symptoms (may be present):
Fever
Fatigue
Weight loss
Recent changes in symptoms or medication use
Stressful events since last visit
Objective
Vital signs (temperature, blood pressure, heart rate) – fever may suggest active inflammation
Physical exam:
General (assessing for signs of systemic illness)
Neurological exam (evaluating for specific deficits based on reported symptoms)
Laboratory Tests
Complete blood count (CBC): may show elevated white blood cells if inflammation is active
Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP): inflammatory markers, often elevated in PACNS
Antineutrophil cytoplasmic antibodies (ANCA): positive in some PACNS patients, but not specific for PACNS diagnosis
Electrolytes and kidney function tests
Lumbar puncture (spinal tap) with cerebrospinal fluid (CSF) analysis:
Elevated opening pressure (suggestive of inflammation)
Increased white blood cells in CSF
Elevated protein levels in CSF
Imaging Tests
Magnetic resonance imaging (MRI) of the brain and/or spinal cord:
To identify areas of inflammation or damage in the CNS
May show abnormal blood vessel signals
Magnetic resonance angiography (MRA) of the brain and/or spinal cord (may be used):
To visualize blood flow and assess for vessel narrowing or occlusion
Assessment
Primary angiitis of the central nervous system (confirmed or suspected based on clinical presentation, laboratory, and imaging findings)
Disease activity (active vs. inactive)
Severity of symptoms and neurological deficits
Identification of potential complications (e.g., stroke, cognitive decline)
Plan
Treatment plan based on disease activity and severity:
Induction therapy with corticosteroids (e.g., prednisone) to suppress inflammation
Immunosuppressive medications (e.g., azathioprine, methotrexate) to prevent relapses
Anti-seizure medication (if seizures are a problem)
Pain management (for headaches or other pain)
Rehabilitation therapy (physical, occupational, speech) – to address neurological deficits and improve function
Education
Explain PACNS, a rare inflammatory disease affecting the brain and spinal cord blood vessels.
Discuss the treatment plan, potential side effects, and importance of medication adherence.
Importance of recognizing and reporting any new symptoms or worsening of existing ones.
Provide information about the disease course, potential for relapses, and long-term management.
Offer resources for support groups or patient advocacy organizations.
Follow-up
Schedule for follow-up appointments:
Monitor response to treatment (clinical improvement, imaging findings)
Adjust medication regimen as needed
Address any ongoing challenges or concerns
Monitor for long-term complications
Disclaimer: This information is for educational purposes only and should not be interpreted as medical advice. Please consult with a qualified healthcare professional for diagnosis and treatment of primary angiitis of the central nervous system.