6.4.3 Headache attributed to secondary angiitis of the central nervous system (SACNS)

Identification: Male patient, 38 years old, architect, history of migraine without aura (well-controlled), no other relevant medical history.

History of Present Illness:

Progressive holocranial headache for 6 weeks (5-8/10)

Atypical characteristics:

Cluster-like pattern (2-3 episodes/day, 45-90 minutes each)

Partially responsive to sumatriptan

Associated with:

Transient visual scotomas

"Migrating" paresthesias (upper limbs > lower limbs)

Executive dysfunction (difficulty planning projects)

Constitutional symptoms:

Marked asthenia

Anorexia (3kg weight loss)

Alert:

Denies use of vasoconstrictors or recreational drugs

Recent travel to a Lyme disease endemic area (6 months ago)

Neurological Examination:

Mental status:

Preserved verbal fluency

Mild ideomotor apraxia

Difficulty with complex sequences

Focal signs:

Bilateral indifferent plantar reflex

Mild dysmetria on finger-to-nose test

NIHSS: 2 (due to cognitive deficit only)

Complementary Exams:

3 Tesla MRI + contrast:

Punctate lesions in the basal ganglia (hyperintense on T2)

Meningeal enhancement in the right Sylvian fissure

SWI: Bilateral thalamic microhemorrhages

3D high-resolution MR Angiography:

Concentric stenosis of A2 (anterior cerebral artery)

"Beading" in distal branches of the MCA

Lumbar puncture (2x):

1st: 18 lymphocytes/mm³, proteins 65mg/dL

2nd (1 week later): 32 lymphocytes/mm³, oligoclonal bands +

Cortical-leptomeningeal biopsy (MRI-guided):

Perivascular lymphomonocytic infiltrate

Multinucleated giant cells (granulomas)

Immunohistochemistry: predominance of CD4+ T cells