Craniofacial pain

Pathways for facial pain include

Trigeminal nerve

Facial nerve (usually deep facial pain),

Eighth nerve

Aetiology

Cephalic neuralgias

Trigeminal neuralgia

Vascular compression of Cr. N. V by the SCA at root entry zone: the most common cause
MS: plaque within Cr. N. V nucleus

Glossopharyngeal neuralgia: pain usually in base of tongue and adjacent pharynx

Geniculate neuralgia: otalgia and deep prosopalgia

Tic convulsif: geniculate neuralgia with hemifacial spasm

Occipital neuralgia

Superior laryngeal neuralgia: a branch of the vagus, results primarily in laryngeal pain and occasionally pain on the auricle

Sphenopalatine neuralgia

Herpes zoster: pain is continuous (not paroxysmal). Characteristic vesicles and crusting usually follow pain, most often in distribution of V1 (isolated V1 TGN is rare). In rare cases without vesicles, diagnosis may be difficult

Postherpetic neuralgia

Ramsay-Hunt syndrome: reactivation of herpes zoster in the geniculate ganglion (AKA herpes zoster oticus). Triad: ispilateral facial paralysis, ear pain and veiscles on the face, ear, or in the ear

Supraorbital neuralgia (SON)

Trigeminal neuropathic pain (AKA trigeminal deafferentation pain): may follow injuries from sinus or dental surgery, head trauma

Trigeminal deafferentation pain: follows trigeminal denervation, including therapeutic measures to treat trigeminal neuralgia

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT)

Ophthalmic pain

Tolosa-Hunt syndrome: painful ophthalmoplegia

(Raeder’s) paratrigeminal neuralgia: unilateral Horner syndrome+ trigeminal neuralgia

Orbital pseudotumor: proptosis, pain, and EOM dysfunction

Diabetic (oculomotor) neuritis

Optic neuritis

Iritis

Glaucoma

Anterior uveitis

Otalgia (see below)

Masticatory disorders

Dental or periodontal disease

Nerve injury (inferior and/or superior alveolar nerves)

Temporo-mandibular joint (TMJ) dysfunction

Elongated styloid process

Temporal & masseter myositis

Vascular pain syndromes

Migraine headaches: see Migraine

Simple migraine: includes classic migraine, common migraine
Complicated migraine: includes hemiplegic migraine, ophthalmoplegic migraine

Cluster H/A; subtypes: episodic, chronic, chronic paroxysmal hemicrania

Giant cell arteritis (temporal arteritis). Tenderness over STA

Toxic or metabolic vascular H/A (fever, hypercapnia, EtOH, nitrites, hypoxia, hypoglycemia, caffeine withdrawal)

Hypertensive H/A

Aneurysm or AVM (due either to mass effect or hemorrhage)

Carotidynia: e.g. with carotid dissection

Basilar dolichoectasia with fifth n. compression or indentation of the pons

Sinusitis (maxillary, frontal, ethmoidal, sphenoidal)

Dental disease

Neoplasm: may cause referred pain or fifth nerve compression

Extracranial

Intracranial tumor: primarily posterior fossa lesions, neoplastic compression of trigeminal nerve usually causes sensory deficit

Atypical facial pain (AFP) (prosopalgia):

Traditionally a “wastebasket” category used for many things. It has been proposed to reserve this term for a psychogenic disorder

Primary (nonvascular) H/A: including

Tension (muscle contraction) H/A

Posttraumatic H/A

Headaches

Definition

Pain due to irritation of intra- or extracranial structures (e.g. meninges, blood vessels).

Types of headaches

Primary headaches

Classification

Tension-type

Migraine

Cluster

Localization

Bilateral

Unilateral

Image

Duration

> 30 min (typically 4–6 hr); constant

4–72 hr

15 min–3 hr; repetitive

Description

Steady, “bandlike” pain. No nausea or vomiting. No more than one of photophobia or phonophobia. No aura. Most common primary headache; more common in females.

Pulsating pain with nausea, photophobia, and/or phonophobia. May have “aura.” Due to irritation of CN V, meninges, or blood vessels (release of vasoactive neuropeptides [eg, substance P, calcitonin gene-related peptide]). More common in females.
POUND–Pulsatile, One-day duration, Unilateral, Nausea, Disabling.

Excruciating periorbital pain (“suicide headache”) with autonomic symptoms (eg, lacrimation, rhinorrhea, conjunctival injection). May present with Horner syndrome. More common in males.

Associated factors

Sometimes photophobia, phonophobia; nausea absent

Nausea, vomiting, photophobia, phonophobia, aura in 30%, either visual (flickering, zig-zagging lines), motor (paresthesias of hand, arm, or face, or language dysfunction).

Autonomic symptoms: lacrimation, rhinorrhea, miosis, ptosis, eyelid edema, conjunctival injection

Factors that aggravate or provoke

Sustained muscle tension, as in driving or typing

Alcohol, certain foods, or stress may provoke; also menses, high altitude; provoked by noise and bright light

During attack, sensitivity to alcohol may increase

Factors that relieve

Possibly massage, relaxation

Quiet, dark room; sleep; sometimes transient relief from pressure on the involved artery

Treatment

- Acute: analgesics, NSAIDs, acetaminophen.
- Prophylaxis: TCAs (e.g., amitriptyline), behavioral therapy.

- Acute: NSAIDs, triptans, dihydroergotamine, antiemetics (eg, prochlorperazine, metoclopramide).
- Prophylaxis: lifestyle changes (eg, sleep, exercise, diet), β-blockers, amitriptyline, topiramate, valproate, botulinum toxin, anti-CGRP monoclonal antibodies.

- Acute: sumatriptan, 100% O₂.
- Prophylaxis: verapamil.

ㅤ	Migraines	Tension	Cluster
Process	Neuronal dysfunction, possibly of brainstem origin, involving low serotonin, spreading cortical depression and trigemino-vascular activation. Types: with aura; without aura; variants.	Unclear—possibly heightened CNS pain sensitivity. Involves pericranial muscle tenderness. Etiology also unclear.	Unclear—possibly hypothalamic then trigemino-autonomic activation.
Location	Unilateral—in ~70%; bifrontal or global—in ~30%	Usually bilateral; may be generalized or localized to the back of the head and upper neck or to the frontotemporal area.	Unilateral, usually behind or around the eye or temple
Quality & Severity	Throbbing or aching, variable in severity	Steady; pressing or tightening; nonthrobbing. Mild to moderate intensity	Deep, continuous, severe
Timing—Onset	Fairly rapid, reaching a peak in 1–2 hours	Gradual	Abrupt; peaks within minutes
Timing—Duration	4–72 hours	30 minutes to 7 days	Up to 3 hours
Timing—Course	Peak incidence early to mid-adolescence; prevalence is ~6% in men and ~15% in women. Recurrent—usually monthly, but weekly in ~10%	Episodic; may be chronic; Annual prevalence ~40%	Episodic, clustered in time, with several each day for 4–8 weeks and then relief for 6–12 months; prevalence <1%, more common in men

Secondary headaches

Medication overuse
Meningitis
Subarachnoid hemorrhage
Hydrocephalus
Neoplasia
Giant cell arteritis

General information

Headache (H/A) may be broadly categorized as follows:

Chronic recurring headaches

Vascular type (migraine):
Muscle contraction (tension) headaches

Headache due to pathology

Systemic pathology
Intracranial pathology: a wide variety of etiologies including:

Subarachnoid hemorrhage: sudden onset, severe, usually with vomiting, apoplexy, focal deficits possible; see differential diagnosis of paroxysmal H/A
Increased intracranial pressure from any cause (tumor, communicating hydrocephalus, inflammation, pseudotumor cerebri…)
Irritation or inflammation of meninges: meningitis
Tumor: with or without elevated ICP
Local pathology of the eye, nasopharynx, or extracranial tissues (including giant cell arteritis
Following head trauma: postconcussive syndrome
Following craniotomy: “syndrome of the trephined”

Note

A severe new H/A, or a change in the pattern of a longstanding or recurrent H/A (including developing associated N/V, or an abnormal neurologic exam) warrants further investigation with CT or MRI.
Unilateral H/A that never changes side over a period ≥ 1 year warrants an MRI; this would be atypical in migraine and may be a presentation of an occipital AVM.

Cranial neuralgias and surgical options

Syndrome	Comments	Surgical Options
Cluster headache	Pain attacks of severe or very severe unilateral orbital, supraorbital, and/or temporal pain lasting 15-180 min if untreated. May be accompanied by ipsilateral conjunctival injection and/or lacrimation, nasal congestion, and/or rhinorrhea, eyelid edema, forehead and facial sweating, miosis and/or ptosis and a sense of restlessness or agitation. Attacks have a frequency from 1 every other day to 8 per day. Episodic form is six times more common than the chronic form (attacks occurring for >1 year without remission or with remissions lasting <1 month).	Occipital nerve stimulation; Sphenopalatine ganglion stimulation; Radiofrequency ablation of sphenopalatine ganglion; Gamma knife SRS to trigeminal root; Hypothalamic DBS; Transection of nervus intermedius
Migraine	Usually a throbbing headache with or without sensory aura.	Botulinum toxin A prophylactic injections; Trigger site deactivation surgery; Occipital nerve stimulation
Occipital neuralgia	Paroxysmal stabbing pain, with or without persistent aching between paroxysms, in the distribution of the greater, lesser, and/or third occipital nerves.	Peripheral neurectomy; C2 dorsal root ganglionectomy; Microvascular decompression (C2 root); Percutaneous neurolysis C2 root; Occipital nerve stimulation; RF lesioning of greater/lesser occipital nerves; Pulsed RF treatment C2 dorsal root ganglion
Trigeminal neuralgia	Brief strong, sharp, unilateral shooting pain in one or more branches of trigeminal nerve. Combined V2 and V3 symptoms commonest, V1 symptoms alone rarest presentation.	Microvascular decompression; Gamma knife surgery to trigeminal DREZ; Peripheral ablation of gasserian ganglion
Glossopharyngeal neuralgia	Paroxysmal attacks of facial pain lasting from a fraction of a second to 2 min. Characterized by unilateral, sharp, stabbing, and severe pain in the distribution within the posterior part of the tongue, tonsillar fossa, pharynx, or beneath the angle of the lower jaw and/or ear. Triggers include swallowing, chewing, talking, coughing, and yawning.	MVD; Rhizotomy of IX (± upper rootlets of X); Gamma knife surgery; Motor cortex stimulation; Trigeminal tractotomy ± nucleotomy
Geniculate neuralgia	Brief paroxysms of pain felt deep in the auditory canal. A trigger area is present in the posterior wall of the auditory canal.	Transection of nervus intermedius (with or without geniculate ganglion removal); Microvascular decompression
Persistent idiopathic facial pain	Persistent facial pain that does not have the characteristics of cranial neuralgias or cannot be attributed to another disorder. Usually a throbbing pain situated deep in the eye and malar region, often radiating to the ear, neck, and shoulders.	CT-guided percutaneous trigeminal tractotomy-nucleotomy; Nucleus caudalis DREZ lesioning; Pulsed radiofrequency to the sphenopalatine ganglion
Anesthesia dolorosa	Uncommon complication of surgical treatments for neuralgias: excruciating pain perceived in an insensate region of the face.	Motor cortex stimulation; Deep brain stimulation; Nucleus caudalis DREZ lesioning
Temporomandibular joint disorder	Recurrent pain in one or more regions of the head and/or face precipitated by jaw movements and/or chewing of hard or tough food. Other findings are a reduced range or irregular jaw opening, noise from one or both TMJs during jaw movements, tenderness of the joint capsule.	Botulinum toxin; Arthrocentesis/arthroscopy; TMJ disc surgery or joint replacement; Denervation of TMJ