Case 364 · ICHD-3 6.4.3
6.4.3 Headache attributed to secondary angiitis of the central nervous system (SACNS)
- Origin
- Synthetic
- Language
- English — also available in German
- Contributed by
- Imported case — HeadAI synthetic vignettes (de Andrade JBC, da Silva Costa TB, Vasconcelos JL, Lopes TLM, Balsells MD, Cristofolini VL, Querobin SO, Filho FMR. Validation of an AI-based platform for structured diagnosis of headache disorders using ICHD-3 criteria. BMC Neurology 25, 489 (2025). doi:10.1186/s12883-025-04473-1. Dataset: Andrade J. Head.AI Platform. Mendeley Data, V2 (2025). doi:10.17632/4fms6cw2s5.2)
Anamnesis
Identification: Female patient, 42 years old, previously healthy, presenting with a subacute progressive headache for 3 weeks.
Current Illness History:
Diffuse headache (7/10), constant, with paroxysmal exacerbations
Characteristics:
Worsens at night
Resistant to common analgesics
Associated with:
Fluctuating mental confusion
Progressive cognitive deficit (recent memory)
Episodes of transient diplopia
Constitutional symptoms:
Weight loss (4kg in 3 weeks)
Evening fever (37.8°C)
No history of:
Recent infections
Known autoimmune diseases
Illicit drug use
Neurological Examination:
Mental state:
Disorientation in time and space
Psychomotor slowing
Focal signs:
Mild right hemiparesis (4+/5)
Asymmetric reflexes (R>L)
NIHSS: 4 (mainly due to cognitive deficit)
Complementary Exams:
Brain MRI:
Multiple areas of T2/FLAIR hyperintensity (bilateral subcortical)
Post-contrast leptomeningeal enhancement
MR Angiography: Segmental stenoses in middle cerebral arteries
Lumbar puncture:
Clear CSF, normal opening pressure
Lymphocytic pleocytosis (25 cells/mm³)
Elevated proteins (80mg/dL)
Increased IgG, positive oligoclonal bands
Leptomeningeal biopsy (gold standard):
Transmural inflammatory infiltrate
Fibrinoid necrosis of small/medium vessels
Report this case — e.g. suspected re-identification risk.