Introduction to Neuropathic Pain Syndromes




Chronic pain impairs the quality of life for millions of individuals and therefore presents a serious ongoing challenge to clinicians and researchers. Debilitating chronic pain syndromes cost the US economy more than $600 billion per year. This article provides an overview of the epidemiology, clinical presentation, and treatment outcomes for craniofacial, spinal, and peripheral neurologic pain syndromes. Although the authors recognize that the diagnosis and treatment of the chronic forms of neuropathic pain syndromes represent a clinical challenge, there is an urgent need for standardized classification systems, improved epidemiologic data, and reliable treatment outcomes data.


Key points








  • Chronic pain impairs the quality of life for millions of individuals and therefore presents a serious ongoing challenge to clinicians and researchers.



  • Debilitating chronic pain syndromes cost the US economy more than $600 billion per year. This article provides an overview of the epidemiology, clinical presentation, and treatment outcomes for craniofacial, spinal, and peripheral neurologic pain syndromes.



  • Although the authors recognize that the diagnosis and treatment of the chronic forms of neuropathic pain syndromes represent a clinical challenge, there is an urgent need for standardized classification systems, improved epidemiologic data, and reliable treatment outcomes data.




Chronic pain impairs the quality of life for millions of people, and therefore presents a serious ongoing challenge to clinicians and researchers. Debilitating chronic pain syndromes cost the US economy more than $600 billion per year. The annual cost of pain treatment is greater than the combined annual costs of heart disease, cancer, and diabetes. A recent epidemiology review reports that prevalence rates of neuropathic pain as a global clinical entity range from 0.9% to 17.9%.


For many years, clinicians have understood that nociceptive pain sensation serves as a crucial, adaptive physiologic response to noxious stimuli through primary nociceptive afferent activation. In contrast, neuropathic pain arises by activity generated within the somatosensory system without adequate stimulation of peripheral afferents. This maladaptive plasticity in the neuropathic pain state is often a consequence of lesions to the peripheral or central nervous system. Alterations such as ectopic generation of action potentials, facilitation and disinhibition of synaptic transmission, loss of synaptic connectivity and formation of new synaptic circuits, and neuroimmune interactions contribute to the multifaceted pathogenesis of complex neuropathic pain syndromes.


Historically, neuropathic pain syndromes have been classified based on their cause or on the anatomic distribution of pain. Although this classification has some use for diagnostic purposes, it offers no good framework for the clinical management of pain or for the evaluation of the available therapies. A range of positive and negative neurosensory symptoms usually characterize these syndromes. This clinical heterogeneity makes the development of standardized diagnostic and evaluation tools for pain increasingly challenging. Consequently, patients are frequently misclassified and treatment outcomes are not recorded in a reliable and efficient manner. Modern pain research has explored genetic and molecular modulation of nociceptive systems to develop new analgesic strategies. Such examples of backward translation from the clinic to basic science are starting to become increasingly important. For this approach to be successful, it is of utmost importance to first develop an effective way of assessing treatment outcomes in the clinic. Successful exploration of genetic and molecular tools to better define neuropathic pain syndromes requires the tandem improvement of clinical outcome measurements. This article categorizes the existing literature for neuropathic pain, focusing on craniofacial, spinal, and peripheral pain syndromes.




Craniofacial pain syndromes


Epidemiologists estimate that approximately 39 million adult Americans are suffering from chronic craniofacial pain. Despite the high prevalence, in the last few years, the classification of craniofacial pain disorders has been a matter of active debate, and there are no established criteria to evaluate the efficacy or effectiveness of available pharmacologic and nonpharmacologic therapies. This section details recently reported epidemiology and neurosurgical treatment outcomes for craniofacial pain syndromes.


Primary Headaches


Primary headaches, such as tension-type, migraine, and cluster headaches, are prevalent conditions that affect the US population. Table 1 provides a comprehensive overview of the available neurosurgical interventions for cluster and migraine headaches.



Table 1

Clinical presentation, epidemiology and treatment outcomes for primary headaches














































Pain Syndrome Epidemiology Clinical Presentation Treatments Outcomes References
Cluster headaches Prevalence of 53 per 100,000
Male to female ratio is 1.7:1 for chronic 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



  • Chronic cluster headache has attacks occurring for >1 y without remission or with remissions lasting <1 mo



  • Episodic form is 6 times more common than the chronic form

Occipital nerve stimulation Significant, long-term benefit in 67% of patients with refractory chronic cluster headache
Sphenopalatine ganglion stimulation Pain relief seen in 67.1% of attacks treated with full stimulation. Mean attack frequency decreased from 17.4 per week at baseline to 12.5 per week during treatment
Radiofrequency ablation of sphenopalatine ganglion Reported to reduce attack intensity and frequency in patients with refractory chronic cluster headache
A case series with 15 patients showed decrease in mean attack intensity (8.6 vs 4.2) and mean attack frequency (17 vs 8.3 attacks/wk) at 18 mo follow-up vs baseline
On comparing complete pain relief vs partial pain relief (medication <50% preoperative requirement) vs no pain relief, in a study of 10 patients with medication-resistant chronic cluster headache, outcome was 30% vs 30% vs 40%
Gamma Knife stereotactic radiosurgery to trigeminal nerve root A review of 43 patients followed for 12–40 mo reported pain relief rated as excellent in 28%, good in 21%, fair in 12%, and poor in 39%
Hypothalamic deep brain stimulation Total positive response rate of around 60%
Transection of the nervus intermedius High early success rate for pain relief, with potential for long-lasting pain relief. Rowed reported a 75% success rate
Migraine headaches Prevalence in the US population
Acute: 18% (women) and 6% (men)
Chronic: 4%
Moderate to severe headache attacks, with or without accompanying vegetative symptoms Botulinum toxin A prophylactic injections Small to modest pain reduction
Trigger site deactivation surgery Pain elimination or significant reduction in 29%–57%
Occipital nerve stimulation Overall improvement in 50 to >90%


Patients suffering from cluster or migraine headache often do not find pain relief from conventional management. Considerable progress has been made in neurostimulative and neuroablative approaches to treat chronic headache syndromes. However, the effectiveness of each treatment approach varies widely in terms of pain relief. Some of the studies that were reviewed report moderate to significant pain reduction, whereas others report decrease in frequency of pain attacks. The level of evidence for the treatment outcomes ranges from case reports to quasi-randomized studies, thus unmasking a clear need for prospective randomized controlled studies to rigorously evaluate current surgical treatment interventions effective for craniofacial neuropathic pain.


Cranial Neuralgias


Cranial neuralgias comprise various painful paroxysmal disorders of the head. Although trigeminal neuralgia has an incidence that ranges from 3 to 5 new cases for 100,000 persons per year and a prevalence that ranges from 12.6 to 28.9 per 100,000 persons, it is still a rare disease that is easily misdiagnosed among the duplicative and inconsistent nomenclature of craniofacial neuralgias ( Table 2 ).



Table 2

Clinical presentation, epidemiology and treatment outcomes for cranial neuralgias
































































































Pain Syndrome Epidemiology Clinical Presentation Treatments Outcomes References
Occipital neuralgia


  • Prevalence and incidence estimates not available



  • The major occipital nerve is more frequently involved (90%) as compared with the minor occipital nerve (10%)

Paroxysmal stabbing pain, with or without persistent aching between paroxysms, in the distribution of the greater, lesser, and/or third occipital nerves Peripheral neurectomy Outcome of 95 neurectomies: excellent 15%, good 71%, poor 9%
Initial pain relief reported to be durable in only approximately 50% of cases in a series
C2 dorsal root ganglionectomy One series reports that >80% of patients with trauma as a precipitant achieved good or excellent results. Results were poorer with nontraumatic occipital pain. >50% of these patients did not experience worthwhile improvement (>50% reduction in pain) with their surgery. In patients with a history of migraine headaches, surgery produced a satisfactory result in only 30%
Extended follow-up of another series noted pain recurrence in 65% of patients by 12 mo and no recurrences after 24 mo
Microvascular decompression Pain relief: complete in 89.5% and significant in 6.6%. Recurrence in 3.9% (89 procedures performed in 76 Chinese patients)
Percutaneous neurolysis of C-2 nerve root Case report of a patient being free of pain for more than 12 mo, after alcohol-induced rhizotomy
Occipital nerve stimulation Success rates of 70%–100% (mean 88%)
Radiofrequency lesioning of greater and lesser occipital nerves Traumatic cause: 72%; excellent pain relief. Nontraumatic cause: 76%; excellent
Botulinum toxin type A Reports of effectiveness exist, but botulinum toxin cannot be firmly recommended as an evidence-based treatment in secondary headaches or cranial neuralgias
Nerve decompression and fixation of the atlantoaxial joint In patients with subluxation of the atlantoaxial joint causing compression of the C2 nerve, reliable benefit was achieved
Partial posterior rhizotomy at C1–3 or selective posterior rhizidiotomy or microsurgical DREZ-otomy Likelihood of long-term pain relief ∼71%
Complete pain relief in 44% (4 of 9) of patients who underwent intradural C1-C3 and C1-C4 selective dorsal rhizotomies
Chambers reported 22 patients, 6 of whom had 75% improvement and 10 complete relief
Pulsed radiofrequency treatment of occipital nerve 19 patients included in the prospective trial, 68.4%, 57.9%, and 52.6% reported an improvement of 50% or more 1, 2, and 6 mo after pulsed radiofrequency treatment, respectively.
Pulsed radiofrequency treatment of C-2 dorsal root ganglion 2 of 4 patients had a long-term effect (18 and >24 mo), no improvement observed in the other 2 patients
Trigeminal neuralgia Prevalence: 12.6–28.9 per 100,000


  • Brief strong, sharp, unilateral shooting pain in 1 or more branches of cranial nerve. Quality of pain is sharp, shooting, electric-like



  • Distribution: V1, 4%; V2, 17%; V3, 15%; V2 + V3, 32%; V1 + V2, 14%; V1 + V2 + V3, 17%

Gasserian ganglion radiofrequency thermocoagulation, glycerol, balloon compression Pain relief: significant in 50% of cases for 5 y
Gamma Knife Pain relief: significant in 52% of cases for 3 y
Microvascular decompression Pain relief: significant in 73% of cases for 5 y
Glossopharyngeal neuralgia


  • Incidence: 0.2 and 0.7 per 100,000 people per year



  • Average annual recurrence rate for a second episode is low (3.6%)




  • 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 in the ear



  • Triggers: swallowing, chewing, talking, coughing, and yawning

Microvascular decompression Highest initial and long-term success rates. Complete pain relief in the range of 76%–97%
Rhizotomy of cranial nerve IX and with or without upper rootlets of X Long-term relief is 96.4%
Gamma Knife 13 of 15 (87%) reported patients treated have achieved significant pain relief
Motor cortex stimulation >50% pain relief noted to last for 18–72 mo
Computed tomography–guided percutaneous trigeminal tractotomy-nucleotomy 14 patients, half of them experienced recurrent pain during the long-term follow-up period (5–12 y); 4 underwent a second procedure, 3 of them with rhizotomy; 3 of the 4 in this recurrent group had no pain after the second surgical intervention
Selective trigeminal tractotomy All 6 patients were cured of neuralgia. Tractotomy was repeated in 1 within 12 d because the analgesia was not complete. 1 patient has been followed up for 6 y, 2 for 3 y, 1 for 1 y, and 2 for a few months
Percutaneous radiofrequency neurolysis (pulsed-mode, low-temperature, and conventional radiofrequency) 5 patients pain free 4 mo to 3 y. About 10 other patients have been reported to be pain free after the procedure a
Geniculate neuralgia Data not available because it is an uncommon condition 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) An author reports that in 64 patients who underwent nervus intermedius transaction and geniculate ganglion removal, excellent results were obtained, except in 1 patient
Microvascular decompression Based on case reports and small series, long-term pain control can be seen after nerve sectioning or microvascular decompression, but no prospective studies exist

Abbreviation: DREZ, dorsal root entry zone.

a Because of the high incidence of complications, it was recommended that this procedure be reserved for patients whose condition is secondary to malignancy or who are unable to withstand intracranial procedures.



Despite the low incidence and prevalence of cranial neuralgias, a wide range of surgical and nonsurgical interventions are available, most of which have good success rates. Classification criteria (eg, International Headache Society, International Classification of Headache Disorders 2nd edition) have long been purely based on the clinical presentation of pain, which can sometimes be subjective. Thus, it makes it easy to misdiagnose or misclassify patients with chronic conditions. To have more reliable outcomes data, one must first have a more effective classification system for these conditions. Classification systems, such as the one proposed by Burchiel in 2003, attempt to reduce misclassification by standardizing the nomenclature. However, this system is based on empirical observations rather than on prospective data.


Other Types of Craniofacial Pain


Other types of craniofacial pain, such as temporomandibular joint (TMJ) disorders, are not uncommon; however, most of the available treatment options do not fall in the scope of the neurosurgery practice. Persistent idiopathic facial pain (PIFP), also known as atypical facial pain, is a rare condition that includes facial pain that does not have the characteristics or distribution of any of the cranial neuralgias. Likewise, anesthesia dolorosa (AD) is a pain syndrome that arises as a complication of the surgical treatments of neuralgias and trauma, among other causes. PIFP and AD are rare chronic pain conditions that are commonly treatment refractory; consequently, there is a limited literature on success rates of the available treatments ( Table 3 ).



Table 3

Clinical presentation, epidemiology, and treatment outcomes of other types of craniofacial pain
























































Pain Syndrome Epidemiology Clinical Presentation Treatments Outcomes References
Persistent idiopathic facial pain Prevalence: 3.2–5.9 per 100,000 people


  • Persistent facial pain that does not have the characteristics of cranial neuralgias or cannot be attributed to another disorder



  • Described as throbbing pain situated deep in the eye and malar region, often radiating to the ear, neck, and shoulders

CT-guided percutaneous trigeminal tractotomy-nucleotomy Largest case series shows pain relief in 16 of 17 patients
Nucleus caudalis DREZ lesioning Not available
Experimental procedures: pulsed radiofrequency to the sphenopalatine ganglion Pain relief: complete in 21% of cases and significant in 65% of cases
Anesthesia dolorosa Incidence rate: 0.8%


  • Uncommon complication of surgical treatments for neuralgias



  • Described as excruciating pain perceived in an insensate region of the face

Neuromodulation by motor cortex stimulation Pain relief: minimal, high recurrence
Deep brain stimulation Mixed reports of efficacy
Nucleus caudalis DREZ lesioning Pain relief: significant in >60% of cases. Postprocedure ataxia of 90%
TMJ disorders Estimated 15%–16% prevalence of treatment need for temporomandibular disorder in general adult population


  • Recurrent pain in one or more regions of the head and/or face



  • Pain is precipitated by jaw movements and/or chewing of hard or tough food



  • Reduced range of or irregular jaw opening



  • Noise from one or both TMJs during jaw movements



  • Tenderness of the joint capsule of one or both TMJs



  • Headache resolves within 3 mo, and does not recur, after successful treatment of the TMJ disorder

Botulinum toxin 90% patients treated with botulinum toxin had mean 3.2-point reduction in pain on visual analog scale. Pain significantly reduced in patients with botulinum toxin compared with placebo ( P <.01)
Arthrocentesis and arthroscopy Both affect mandibular movement, reduce pain intensity and mandibular functioning to the same degree. Success rates were often high, independent of treatment mode. The effect of maxillofacial surgery is still unclear
Open surgery: disk repositioning, disk repair, discectomy, discectomy with graft replacement Improvement in pain is generally seen
Joint replacement Intended primarily at restoration of form and function, and any pain relief gained is only a secondary benefit
Denervation of TMJ Pain free for 12 mo after surgery (patient with recurrent dislocations of TMJ articular disc. History of 2 arthroscopic surgeries and 1 open attempt to treat TMJ pain)

Abbreviations: CT, computed tomography; DREZ, dorsal root entry zone.


Neuroablative and neurostimlative approaches used to treat these conditions have had questionable success rates that can be attributed to the rarity of these conditions, as well as the limited body of published literature on successful intervention. The level of evidence that supports the effectiveness of the available treatments for PIFP, AD, and TMJ disorders ranges from case reports to prospective studies lacking randomization or control cohorts.




Craniofacial pain syndromes


Epidemiologists estimate that approximately 39 million adult Americans are suffering from chronic craniofacial pain. Despite the high prevalence, in the last few years, the classification of craniofacial pain disorders has been a matter of active debate, and there are no established criteria to evaluate the efficacy or effectiveness of available pharmacologic and nonpharmacologic therapies. This section details recently reported epidemiology and neurosurgical treatment outcomes for craniofacial pain syndromes.


Primary Headaches


Primary headaches, such as tension-type, migraine, and cluster headaches, are prevalent conditions that affect the US population. Table 1 provides a comprehensive overview of the available neurosurgical interventions for cluster and migraine headaches.



Table 1

Clinical presentation, epidemiology and treatment outcomes for primary headaches














































Pain Syndrome Epidemiology Clinical Presentation Treatments Outcomes References
Cluster headaches Prevalence of 53 per 100,000
Male to female ratio is 1.7:1 for chronic 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



  • Chronic cluster headache has attacks occurring for >1 y without remission or with remissions lasting <1 mo



  • Episodic form is 6 times more common than the chronic form

Occipital nerve stimulation Significant, long-term benefit in 67% of patients with refractory chronic cluster headache
Sphenopalatine ganglion stimulation Pain relief seen in 67.1% of attacks treated with full stimulation. Mean attack frequency decreased from 17.4 per week at baseline to 12.5 per week during treatment
Radiofrequency ablation of sphenopalatine ganglion Reported to reduce attack intensity and frequency in patients with refractory chronic cluster headache
A case series with 15 patients showed decrease in mean attack intensity (8.6 vs 4.2) and mean attack frequency (17 vs 8.3 attacks/wk) at 18 mo follow-up vs baseline
On comparing complete pain relief vs partial pain relief (medication <50% preoperative requirement) vs no pain relief, in a study of 10 patients with medication-resistant chronic cluster headache, outcome was 30% vs 30% vs 40%
Gamma Knife stereotactic radiosurgery to trigeminal nerve root A review of 43 patients followed for 12–40 mo reported pain relief rated as excellent in 28%, good in 21%, fair in 12%, and poor in 39%
Hypothalamic deep brain stimulation Total positive response rate of around 60%
Transection of the nervus intermedius High early success rate for pain relief, with potential for long-lasting pain relief. Rowed reported a 75% success rate
Migraine headaches Prevalence in the US population
Acute: 18% (women) and 6% (men)
Chronic: 4%
Moderate to severe headache attacks, with or without accompanying vegetative symptoms Botulinum toxin A prophylactic injections Small to modest pain reduction
Trigger site deactivation surgery Pain elimination or significant reduction in 29%–57%
Occipital nerve stimulation Overall improvement in 50 to >90%


Patients suffering from cluster or migraine headache often do not find pain relief from conventional management. Considerable progress has been made in neurostimulative and neuroablative approaches to treat chronic headache syndromes. However, the effectiveness of each treatment approach varies widely in terms of pain relief. Some of the studies that were reviewed report moderate to significant pain reduction, whereas others report decrease in frequency of pain attacks. The level of evidence for the treatment outcomes ranges from case reports to quasi-randomized studies, thus unmasking a clear need for prospective randomized controlled studies to rigorously evaluate current surgical treatment interventions effective for craniofacial neuropathic pain.


Cranial Neuralgias


Cranial neuralgias comprise various painful paroxysmal disorders of the head. Although trigeminal neuralgia has an incidence that ranges from 3 to 5 new cases for 100,000 persons per year and a prevalence that ranges from 12.6 to 28.9 per 100,000 persons, it is still a rare disease that is easily misdiagnosed among the duplicative and inconsistent nomenclature of craniofacial neuralgias ( Table 2 ).



Table 2

Clinical presentation, epidemiology and treatment outcomes for cranial neuralgias
































































































Pain Syndrome Epidemiology Clinical Presentation Treatments Outcomes References
Occipital neuralgia


  • Prevalence and incidence estimates not available



  • The major occipital nerve is more frequently involved (90%) as compared with the minor occipital nerve (10%)

Paroxysmal stabbing pain, with or without persistent aching between paroxysms, in the distribution of the greater, lesser, and/or third occipital nerves Peripheral neurectomy Outcome of 95 neurectomies: excellent 15%, good 71%, poor 9%
Initial pain relief reported to be durable in only approximately 50% of cases in a series
C2 dorsal root ganglionectomy One series reports that >80% of patients with trauma as a precipitant achieved good or excellent results. Results were poorer with nontraumatic occipital pain. >50% of these patients did not experience worthwhile improvement (>50% reduction in pain) with their surgery. In patients with a history of migraine headaches, surgery produced a satisfactory result in only 30%
Extended follow-up of another series noted pain recurrence in 65% of patients by 12 mo and no recurrences after 24 mo
Microvascular decompression Pain relief: complete in 89.5% and significant in 6.6%. Recurrence in 3.9% (89 procedures performed in 76 Chinese patients)
Percutaneous neurolysis of C-2 nerve root Case report of a patient being free of pain for more than 12 mo, after alcohol-induced rhizotomy
Occipital nerve stimulation Success rates of 70%–100% (mean 88%)
Radiofrequency lesioning of greater and lesser occipital nerves Traumatic cause: 72%; excellent pain relief. Nontraumatic cause: 76%; excellent
Botulinum toxin type A Reports of effectiveness exist, but botulinum toxin cannot be firmly recommended as an evidence-based treatment in secondary headaches or cranial neuralgias
Nerve decompression and fixation of the atlantoaxial joint In patients with subluxation of the atlantoaxial joint causing compression of the C2 nerve, reliable benefit was achieved
Partial posterior rhizotomy at C1–3 or selective posterior rhizidiotomy or microsurgical DREZ-otomy Likelihood of long-term pain relief ∼71%
Complete pain relief in 44% (4 of 9) of patients who underwent intradural C1-C3 and C1-C4 selective dorsal rhizotomies
Chambers reported 22 patients, 6 of whom had 75% improvement and 10 complete relief
Pulsed radiofrequency treatment of occipital nerve 19 patients included in the prospective trial, 68.4%, 57.9%, and 52.6% reported an improvement of 50% or more 1, 2, and 6 mo after pulsed radiofrequency treatment, respectively.
Pulsed radiofrequency treatment of C-2 dorsal root ganglion 2 of 4 patients had a long-term effect (18 and >24 mo), no improvement observed in the other 2 patients
Trigeminal neuralgia Prevalence: 12.6–28.9 per 100,000


  • Brief strong, sharp, unilateral shooting pain in 1 or more branches of cranial nerve. Quality of pain is sharp, shooting, electric-like



  • Distribution: V1, 4%; V2, 17%; V3, 15%; V2 + V3, 32%; V1 + V2, 14%; V1 + V2 + V3, 17%

Gasserian ganglion radiofrequency thermocoagulation, glycerol, balloon compression Pain relief: significant in 50% of cases for 5 y
Gamma Knife Pain relief: significant in 52% of cases for 3 y
Microvascular decompression Pain relief: significant in 73% of cases for 5 y
Glossopharyngeal neuralgia


  • Incidence: 0.2 and 0.7 per 100,000 people per year



  • Average annual recurrence rate for a second episode is low (3.6%)




  • 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 in the ear



  • Triggers: swallowing, chewing, talking, coughing, and yawning

Microvascular decompression Highest initial and long-term success rates. Complete pain relief in the range of 76%–97%
Rhizotomy of cranial nerve IX and with or without upper rootlets of X Long-term relief is 96.4%
Gamma Knife 13 of 15 (87%) reported patients treated have achieved significant pain relief
Motor cortex stimulation >50% pain relief noted to last for 18–72 mo
Computed tomography–guided percutaneous trigeminal tractotomy-nucleotomy 14 patients, half of them experienced recurrent pain during the long-term follow-up period (5–12 y); 4 underwent a second procedure, 3 of them with rhizotomy; 3 of the 4 in this recurrent group had no pain after the second surgical intervention
Selective trigeminal tractotomy All 6 patients were cured of neuralgia. Tractotomy was repeated in 1 within 12 d because the analgesia was not complete. 1 patient has been followed up for 6 y, 2 for 3 y, 1 for 1 y, and 2 for a few months
Percutaneous radiofrequency neurolysis (pulsed-mode, low-temperature, and conventional radiofrequency) 5 patients pain free 4 mo to 3 y. About 10 other patients have been reported to be pain free after the procedure a
Geniculate neuralgia Data not available because it is an uncommon condition 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) An author reports that in 64 patients who underwent nervus intermedius transaction and geniculate ganglion removal, excellent results were obtained, except in 1 patient
Microvascular decompression Based on case reports and small series, long-term pain control can be seen after nerve sectioning or microvascular decompression, but no prospective studies exist

Abbreviation: DREZ, dorsal root entry zone.

a Because of the high incidence of complications, it was recommended that this procedure be reserved for patients whose condition is secondary to malignancy or who are unable to withstand intracranial procedures.



Despite the low incidence and prevalence of cranial neuralgias, a wide range of surgical and nonsurgical interventions are available, most of which have good success rates. Classification criteria (eg, International Headache Society, International Classification of Headache Disorders 2nd edition) have long been purely based on the clinical presentation of pain, which can sometimes be subjective. Thus, it makes it easy to misdiagnose or misclassify patients with chronic conditions. To have more reliable outcomes data, one must first have a more effective classification system for these conditions. Classification systems, such as the one proposed by Burchiel in 2003, attempt to reduce misclassification by standardizing the nomenclature. However, this system is based on empirical observations rather than on prospective data.


Other Types of Craniofacial Pain


Other types of craniofacial pain, such as temporomandibular joint (TMJ) disorders, are not uncommon; however, most of the available treatment options do not fall in the scope of the neurosurgery practice. Persistent idiopathic facial pain (PIFP), also known as atypical facial pain, is a rare condition that includes facial pain that does not have the characteristics or distribution of any of the cranial neuralgias. Likewise, anesthesia dolorosa (AD) is a pain syndrome that arises as a complication of the surgical treatments of neuralgias and trauma, among other causes. PIFP and AD are rare chronic pain conditions that are commonly treatment refractory; consequently, there is a limited literature on success rates of the available treatments ( Table 3 ).



Table 3

Clinical presentation, epidemiology, and treatment outcomes of other types of craniofacial pain
























































Pain Syndrome Epidemiology Clinical Presentation Treatments Outcomes References
Persistent idiopathic facial pain Prevalence: 3.2–5.9 per 100,000 people


  • Persistent facial pain that does not have the characteristics of cranial neuralgias or cannot be attributed to another disorder



  • Described as throbbing pain situated deep in the eye and malar region, often radiating to the ear, neck, and shoulders

CT-guided percutaneous trigeminal tractotomy-nucleotomy Largest case series shows pain relief in 16 of 17 patients
Nucleus caudalis DREZ lesioning Not available
Experimental procedures: pulsed radiofrequency to the sphenopalatine ganglion Pain relief: complete in 21% of cases and significant in 65% of cases
Anesthesia dolorosa Incidence rate: 0.8%


  • Uncommon complication of surgical treatments for neuralgias



  • Described as excruciating pain perceived in an insensate region of the face

Neuromodulation by motor cortex stimulation Pain relief: minimal, high recurrence
Deep brain stimulation Mixed reports of efficacy
Nucleus caudalis DREZ lesioning Pain relief: significant in >60% of cases. Postprocedure ataxia of 90%
TMJ disorders Estimated 15%–16% prevalence of treatment need for temporomandibular disorder in general adult population


  • Recurrent pain in one or more regions of the head and/or face



  • Pain is precipitated by jaw movements and/or chewing of hard or tough food



  • Reduced range of or irregular jaw opening



  • Noise from one or both TMJs during jaw movements



  • Tenderness of the joint capsule of one or both TMJs



  • Headache resolves within 3 mo, and does not recur, after successful treatment of the TMJ disorder

Botulinum toxin 90% patients treated with botulinum toxin had mean 3.2-point reduction in pain on visual analog scale. Pain significantly reduced in patients with botulinum toxin compared with placebo ( P <.01)
Arthrocentesis and arthroscopy Both affect mandibular movement, reduce pain intensity and mandibular functioning to the same degree. Success rates were often high, independent of treatment mode. The effect of maxillofacial surgery is still unclear
Open surgery: disk repositioning, disk repair, discectomy, discectomy with graft replacement Improvement in pain is generally seen
Joint replacement Intended primarily at restoration of form and function, and any pain relief gained is only a secondary benefit
Denervation of TMJ Pain free for 12 mo after surgery (patient with recurrent dislocations of TMJ articular disc. History of 2 arthroscopic surgeries and 1 open attempt to treat TMJ pain)

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Oct 12, 2017 | Posted by in NEUROSURGERY | Comments Off on Introduction to Neuropathic Pain Syndromes

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