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Headache
7 notes available
1 Last Minute Review 2 Migraine 3 TTH, NDPH & Other Primary Headaches 4 Trigeminal Autonomic Cephalalgias 5 Secondary Headache Red Flags 6 Headache Pharmacology 7 Headache Mimics & Facial Pain
Clinical › Headache

Headache Mimics & Facial Pain

Headache Mimics & Facial Pain

What You'll Learn

  • GCA: Age >50, new headache, jaw claudication (most specific symptom), ESR/CRP elevated; temporal artery biopsy remains the gold standard in the US (ACR/VF 2021), while EULAR 2023 prefers temporal artery ultrasound (halo sign) where expertise is available; skip lesions → get ≥1 cm; start steroids BEFORE biopsy
  • RCVS: Recurrent thunderclap headaches, “string of beads” on angiography, normal CSF, resolves <3 months; distinguish from PACNS (insidious onset, abnormal CSF, progressive course)
  • Trigeminal neuralgia: V2/V3 > V1, brief electric shock-like pain triggered by light touch, carbamazepine first-line, MRI for neurovascular compression (SCA most common vessel)
  • Glossopharyngeal neuralgia: Ear/throat lancinating pain triggered by swallowing; can cause syncope via vagal reflex; rule out Eagle syndrome
  • Tolosa-Hunt syndrome: Painful ophthalmoplegia from granulomatous cavernous sinus inflammation; dramatic steroid response
  • Key distinction: Thunderclap + normal CSF + reversible = RCVS; insidious + abnormal CSF + progressive = PACNS
HighYield Pearls
  • Trigeminal neuralgia in a young woman: think MS — demyelinating plaque at root entry zone; MRI mandatory in all TN patients (classic neurovascular conflict vs secondary)
  • Carbamazepine = first-line for both trigeminal AND glossopharyngeal neuralgia; oxcarbazepine alternative; microvascular decompression is the surgical gold standard for classic TN
  • Raeder paratrigeminal syndrome: V1 facial pain + ipsilateral Horner without anhidrosis (post-ganglionic, carotid plexus) → carotid dissection until proven otherwise — image urgently (CTA/MRA)
  • Tolosa-Hunt: painful ophthalmoplegia + dramatic steroid response, but ALWAYS exclude pituitary apoplexy, cavernous sinus thrombosis, dissection, tumor, GCA before labeling it
  • Glossopharyngeal neuralgia + syncope: vagal cross-talk causing asystole — pacemaker may be needed; image to rule out Eagle syndrome / posterior fossa lesion
  • Eagle syndrome: throat/submandibular pain + foreign-body sensation worsened by head turn → CT of styloid (>3 cm or calcified stylohyoid ligament); surgical styloidectomy
  • Ramsay Hunt = VZV CN VII + VIII: ear vesicles + facial palsy + hearing loss/vertigo → acyclovir + steroids early; Hutchinson sign (nose-tip vesicle) → ophthalmic zoster → urgent ophtho
  • Post-herpetic neuralgia prevention: recombinant zoster vaccine + early antiviral within 72h of rash; treat established PHN with gabapentin/pregabalin, TCA, topical lidocaine, capsaicin
  • “Sinus headache” is usually migraine — true bacterial sinusitis needs purulent rhinorrhea + fever + facial tenderness + CT confirmation
  • Burning mouth syndrome: postmenopausal women + bilateral oral burning + normal mucosa — idiopathic; clonazepam lozenges, α-lipoic acid, low-dose TCA
🔍 Quick ReferenceClinical phenotype · Localization / etiology · Treatment
Clinical phenotype
  • “Tic douloureux” / electric shock in cheek triggered by wind, shaving, chewing, light touch → Trigeminal neuralgia (V2/V3)
  • Lancinating pain in throat / tonsillar fossa / ear triggered by swallowing or talking, ± syncope → Glossopharyngeal neuralgia
  • Dermatomal burning pain persisting >3 months after shingles rash → Post-herpetic neuralgia
  • Paroxysmal stabbing pain over the occiput with tender greater occipital nerve → Occipital neuralgia
  • Continuous nagging facial pain without identifiable cause, normal exam/imaging → Atypical odontalgia / persistent idiopathic facial pain
  • Postmenopausal woman with bilateral oral burning + normal mucosa → Burning mouth syndrome
  • Painful ophthalmoplegia (CN III/IV/V1/VI) responsive to steroids → Tolosa-Hunt syndrome
  • Unilateral headache provoked by neck movement + tender cervical facets → Cervicogenic headache
  • Ear vesicles + ipsilateral facial palsy + hearing loss/vertigo → Ramsay Hunt syndrome (VZV)
Localization / etiology
  • Superior cerebellar artery loop compressing trigeminal root entry zone on MRI → Classic trigeminal neuralgia (neurovascular conflict)
  • Demyelinating plaque at pontine trigeminal root entry zone in a young woman → Secondary TN due to MS
  • Granulomatous inflammation of the cavernous sinus / superior orbital fissure with enhancement on MRI → Tolosa-Hunt syndrome
  • Elongated styloid process (>3 cm) or calcified stylohyoid ligament on CT → Eagle syndrome
  • V1 facial pain + ipsilateral Horner without anhidrosis (post-ganglionic carotid plexus) → Raeder paratrigeminal syndrome → rule out carotid dissection
  • TMJ click + bite malocclusion + preauricular tenderness → TMJ disorder
  • Purulent rhinorrhea + fever + facial tenderness + CT air-fluid levels → True bacterial sinusitis (most “sinus HA” = migraine)
  • Hutchinson sign (vesicles on nose tip) → Ophthalmic (V1) zoster — nasociliary involvement → eye at risk
Treatment / pearls
  • Carbamazepine / oxcarbazepine first-line → Trigeminal AND glossopharyngeal neuralgia
  • Microvascular decompression (Jannetta procedure) — gold-standard surgery → Classic trigeminal neuralgia refractory to meds
  • IV fosphenytoin for crisis; gamma knife / RF rhizotomy / balloon compression as alternatives → Trigeminal neuralgia (crisis & refractory)
  • Gabapentin / pregabalin / TCA / topical lidocaine / capsaicin → Post-herpetic neuralgia
  • Greater occipital nerve block (diagnostic + therapeutic) → Occipital neuralgia
  • High-dose corticosteroids with rapid (24–72h) pain relief → Tolosa-Hunt syndrome
  • Acyclovir + corticosteroids started early → Ramsay Hunt syndrome (and ophthalmic zoster)
  • Clonazepam topical lozenges + α-lipoic acid + low-dose TCA → Burning mouth syndrome
  • Surgical styloidectomy → Eagle syndrome
  • TCA / SNRI + multidisciplinary pain management → Persistent idiopathic facial pain / atypical odontalgia
Giant Cell Arteritis (GCA)

Epidemiology

  • Age >50 (mean onset ~70 years); almost never occurs before age 50
  • Female:male ratio 2–3:1
  • Highest incidence in Northern European descent (Scandinavian populations)
  • Polymyalgia rheumatica (PMR) overlap: 40–60% of GCA patients have PMR; ~15% of PMR patients develop GCA

Clinical Features

  • New-onset headache: Temporal region, often unilateral, persistent; most common presenting symptom (~75%)
  • Jaw claudication: Pain with chewing that resolves with rest — most specific clinical feature (LR+ ~34); caused by ischemia of masseter/temporalis muscles
  • Scalp tenderness: Pain when brushing hair or resting head on pillow
  • Vision loss: Most feared complication; anterior ischemic optic neuropathy (AION) — pale disc edema; permanent if untreated
  • Diplopia: CN3, CN4, or CN6 palsy from vasa nervorum ischemia
  • Constitutional symptoms: Fever, weight loss, malaise, fatigue
  • Tongue claudication: Less common but highly specific

Laboratory & Diagnostic Studies

Test Findings Key Points
ESR Typically >50 mm/hr (often >100) Sensitivity ~85%; can be normal in 5–10% (“occult GCA”)
CRP Elevated More sensitive than ESR alone; combined ESR + CRP sensitivity >95%
CBC Thrombocytosis, normocytic anemia Thrombocytosis correlates with disease activity
Temporal artery biopsy Granulomatous inflammation with giant cells, intimal hyperplasia, fragmented internal elastic lamina Remains gold standard in the US (ACR/VF 2021); get ≥1 cm (ideally 2 cm); skip lesions reduce sensitivity (~85%); bilateral biopsy if unilateral negative + high suspicion
Temporal artery ultrasound Halo sign — hypoechoic wall thickening Sensitivity ~77%, specificity ~96%; operator-dependent; EULAR 2023 prefers temporal artery ultrasound as the first imaging test where expertise is available (US biopsy remains gold standard per ACR/VF 2021)
18F-FDG PET/CT Vascular wall uptake Best for large-vessel GCA (aorta, subclavian, axillary); complements temporal biopsy

ACR/EULAR 2022 Classification Criteria

  • Requires age ≥50 + score ≥6 from: temporal artery abnormality, ESR ≥50 or CRP ≥10, morning stiffness, sudden visual loss, jaw/tongue claudication, temporal artery biopsy/halo sign
  • Designed for classification (research), not diagnosis — but clinically useful framework

Treatment

  • High-dose prednisone 40–60 mg/day: Start IMMEDIATELY upon clinical suspicion — do NOT wait for biopsy
  • IV methylprednisolone 1 g/day × 3 days: If vision threatened (acute visual loss, impending second eye involvement)
  • Tocilizumab (IL-6 receptor inhibitor): GiACTA trial demonstrated superior relapse-free remission vs. prednisone alone at 52 weeks; used as steroid-sparing agent
  • Slow prednisone taper over 12–24 months; monitor ESR/CRP for relapse
  • Low-dose aspirin: consider for prevention of ischemic complications
  • Biopsy remains positive for up to 2 weeks after steroid initiation
💎 Board Pearl
  • Jaw claudication is the most specific clinical feature of GCA (LR+ ~34)
  • Start steroids BEFORE biopsy — temporal artery biopsy remains positive for up to 2 weeks after steroid initiation; delaying treatment risks permanent vision loss
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