eMedicine Specialties > Neurology > Headache and Pain

Persistent Idiopathic Facial Pain

Author: Stanley J Krolczyk, DO, RPh, Assistant Professor, Director of Multiple Sclerosis Center, Department of Neurology, University of South Florida College of Medicine
Coauthor(s): Kavita Kalidas, MD, Fellow, Department of Neurology, University of South Florida; Martin A Myers, University of South Florida College of Medicine
Contributor Information and Disclosures

Updated: Jan 14, 2010

Introduction

Background

The term atypical facial pain was first introduced by Frazier and Russell in 1924. It has since been renamed persistent idiopathic facial pain (PIFP). PIFP refers to pain along the territory of the trigeminal nerve that does not fit the classic presentation of other cranial neuralgias.¹ The duration of pain is usually long, lasting most of the day (if not continuous). Pain is unilateral and without autonomic signs or symptoms. It is described as a severe ache, crushing sensation, or burning sensation. Upon examination and workup, no abnormality is noted.

The International Headache Society defines PIFP as follows (Headache Classification Subcommittee of the International Headache Society, 2004)² :

• Pain is in the face.
• Pain is present daily and persists for all or most of the day.
• Pain is confined at onset to a limited area on one side of the face, deep ache, and poorly localized.
• In addition, the pain is not associated with sensory loss or other physical signs, with no abnormalities in laboratory or imaging studies.

Within the group of chronic facial pain syndromes, PIFP represents a diagnostic challenge. Patients frequently are misdiagnosed or attribute their pain to a prior event such as a dental procedure or facial trauma. Psychiatric symptoms of depression and anxiety are prevalent in this population and compound the diagnostic conundrum. Treatment is less effective than in other facial pain syndromes and requires a multidisciplinary approach to address the many facets of this pain syndrome.

Frequency

United States

Accurate figures are difficult to obtain because of the lack of agreement on classification criteria. Estimated incidence is 1 case per 100,000 population, although this number may be underestimated.³

Sex

PIFP affects both sexes approximately equally, but more women than men seek medical care.³

Age

The disorder mainly affects adults and is rare in children.³

Clinical

History

PIFP is essentially a diagnosis of exclusion. Daily or near-daily headaches are a widespread problem in clinical practice.^1,3 According to population-based data from the United States, Europe, and Asia, chronic daily headache affects a large number (approximately 4-5% of the population) of patients.^3,4,5

Importantly, PIFP must be distinguished from various other chronic daily headache syndromes, including hemicrania continua,^3,4 temporomandibular joint (TMJ) syndrome, side-locked migraine, chronic cluster headache, SUNCT (short-lasting, unilateral, neuralgiform headache attacks with conjunctival injection and tearing) syndrome, trigeminal neuralgia, and many others.^5,6,7,8 A careful history and physical examination, including a dental consultation, laboratory studies, and imaging studies, may be necessary to rule out occult pathology. Underlying pathology such as malignancy, vasculitis, infection, and central or peripheral demyelination may manifest early as neuralgia, and not until focal neurologic deficits, imaging abnormalities, or laboratory abnormalities are discovered does the diagnosis become evident. Rare cases of referred pain must also be considered.

The following are examples of common facial pain or headache syndromes, along with some of the classic characteristics:

• Trigeminal neuralgia
  • This is characterized by severe bursts of lancinating pain in one or more branches of the trigeminal nerve. Bursts are quick, repetitive, electric shock–like sensations.
  • Each pain episode is seconds in duration, occurs irregularly, and is not related to the patient's pulse.
  • Treatments include antiepileptic drugs (AEDs), antidepressants (eg, tricyclic antidepressants [TCAs], selective serotonin reuptake inhibitors [SSRIs]/norepinephrine reuptake inhibitors [NeRIs]), injections, and/or surgical intervention.⁹
• Postherpetic neuralgia
  • This is defined as pain that persists for 1-6 months after an acute herpes zoster infection.
  • The pain is neuropathic in nature. It is associated with allodynia and hyperalgesia, most commonly affecting the V1 distribution of the trigeminal nerve.
  • The mainstay of treatment is AEDs, TCAs, and SSRIs.¹⁰
• TMJ syndrome
  • This is characterized by focal tenderness to one or both TMJs and is usually aggravated by chewing, talking, and jaw movement.
  • The quality of the pain is similar to that of PIFP (ie, dull, aching, crushing, or burning).
  • The treatment for TMJ syndrome is often directed either at the articular joint itself or at fatigue of the period and temporalis muscles.
• Cluster headache
  • The onset of pain is sudden.
  • Episodes last 30-180 minutes.
  • The pain is characterized as severe, boring, and burning.
  • It awakens the patient from sleep and does not improve with rest. Many individuals pace and may injure themselves because of the pain severity.
  • Associated symptoms include ipsilateral conjunctival injection, tearing, and nasal congestion.
  • The male-to-female ratio is 6:1.
  • Abortive treatment includes oxygen (8-15 L), sumatriptan injections, and/or dihydroergotamine.
  • Preventative treatment includes verapamil, lithium, divalproex sodium, and topiramate.¹¹
• Cluster-tic syndrome
  • This is a difficult diagnosis and can be easily confused with other facial pain syndromes.
  • The 3 types of pain include clusterlike features, trigeminal neuralgia features, and a combination of these 2 types.
  • Treatment includes AEDs, TCAs, and SSRIs/NeRIs.¹²
• SUNCT syndrome
  • This is possibly a variation of the cluster-tic syndrome.
  • It is characterized by brief (15-120 seconds) bursts of pain in the eyes, temple, or face.
  • The pain is usually unilateral and is described as burning, stabbing, or electric. It occurs frequently in a 24-hour period (>100 episodes).
  • Neck movements can trigger the pain.
  • SUNCT syndrome is refractory to medical therapy.¹³
• Jabs and jolts syndrome, primary stabbing headache, or ice-pick headache
  • Head pain occurs as a single stab or a series of stabs.
  • The pain is exclusive or predominantly felt in the distribution of V1.
  • Stabs last for up to a few seconds and recur with irregular frequency ranging from one to many per day
  • No other accompanying symptoms are noted, and it cannot be attributed to another disorder.
  • This syndrome is refractory to medical treatment.¹⁴
• Raeder syndrome
  • It is a V1 distribution of a unilateral burning facial pain associated with hyperesthesia, ptosis, and miosis.
  • The pain may be caused by trauma, a middle cranial fossa mass lesion, syphilis, or sinusitis.
  • In the absence of these underlying conditions, the pain is self-limited.¹⁵
• Thalamic pain syndrome
  • This is described as unilateral facial pain and dysesthesias and is attributed to a lesion of the ventral-medial thalamic nuclei.
  • It is typically a severe, burning, or aching pain to the contralateral side of the face.
  • Diagnosis can be made with imaging studies or can be based on other associated symptoms in the trunk or limbs.
• Hemicrania continua
  • This condition is rare.
  • It is characterized by unilateral headache and facial pain.
  • The daily head pain is continuous (24 h/d, 7 d/wk), with pain exacerbation periods that occur with varying frequency from multiple times per week to every third month or less.
  • The pain at baseline is mild to moderate, and exacerbations are moderate to severe in intensity.
  • It is associated with migraine or cluster features (eg, photophobia, nausea, aura, lacrimation, eye injection).
  • It responds to indomethacin, which aids in diagnosis.^11,16,17
  • It awakens the patient from sleep and does not improve with rest. Many individuals pace and may injure themselves because of the severity of the pain.
  • Associated symptoms include ipsilateral conjunctival injection, tearing, and nasal congestion.
  • The male-to-female ratio is 6:1.
  • Abortive treatment includes oxygen (8-15 L), sumatriptan injections, and dihydroergotamine.
  • Preventative treatment includes verapamil, lithium, divalproex sodium, and topiramate.¹¹
• Migraine
  • Migraine is a common condition.
  • The female-to-male ratio is 3:1.
  • It is unilateral but can be bilateral.
  • The pain has a throbbing quality and feels as if it is associated with a pulse.
  • Photophobia, phonophobia, and osmophobia are features of migraine, as is nausea.
  • The pain worsens with exertion and improves with sleep.
  • The patient may or may not experience aura.
  • Pharmacologic therapy includes abortive and preventative medications, depending on the frequency and severity of the headaches.
  • Abortive agents include serotonin agonists, ergotamine, isometheptene, and anti-inflammatories. Preventative agents include AEDs, beta-blockers, calcium channel blockers, TCAs, SSRIs/NeRIs, and angiotensin receptor blocking agents.

Physical

Neurological and physical examination findings, by definition of PIFP, should be normal. Pain evoked responses after stimulation are less common than with trigeminal neuralgia. Palpation- and manipulation-induced tenderness of the TMJ is associated with TMJ syndrome and less so with other cephalgias or facial pain syndromes.

Causes

PIFP usually does not have a specific cause; however, injury of the trigeminal nerve proximally or distally may lead to this disorder. Demyelination, either central or peripheral, may initiate PIFP symptoms and infectious etiologies should also be considered.

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