trigeminal cephalgia

1. Hemicrania continua
Trigeminal neuralgia
Glossopharyngeal neuralgia
DR BHAVIN J PATEL
SR NEUROLOGY
GMC, KOTA

2. Hemicrania continua(HC)
Indomethacin sensitive primary headache disorder.
Term hemicrania continua coined by sjaasatad and spierings.
Classified under trigeminal autonomic cephalgias(TAC).
Considered as rare headache disorder ????

3. Epidemiology
Exact prevalence not known
1.7% patient attending headache or neurology opd.
2nd most common TAC and 4th mc side locked headache.
Mean age of onset is approx. 40 year.(10-80 years)
Female:male:- 1.8:1
No genetic susceptibility.

4. Etiology and Pathophysiology
HC is considered as idiopathic disorder.
Disinhibition of posterior hypothalamus with subsequent release of trigeminal-
autonomic reflex.
Studies with functional MRI showed activation of the contra-lateral posterior
hypothalamus and ipsilateral dorsal pons in cases of HC

5. Clinical features
Characterized by a strictly unilateral, continuous headache of moderate
intensity, with superimposed exacerbations of severe intensity.
The exacerbations are associated with cranial autonomic features, restlessness
and migrainous features.
Laterality:- unilateral, right> left
Site of pain:-
 All TACs usually present with pain in ophthalmic division of trigeminal nerve.

6. Clinical features
Site of pain

7. Clinical features
Pain characteristic and pattern:-
Two components:-
1) Continuous unilateral headache
2) Superimposed variable exacerbations

8. Clinical features
Pain characteristic and pattern:-
Background pain is usually have mild intensity dull and pressure type. (VAS- 5)
Exacerbation phases are largely throbbing or stabbing in character.(VAS-9)
Background pain usually dose not hamper physical activity while exacerbation
dose.
Few patients only have continuous background pain without exacerbation.

9. Clinical features
Duration and frequency of exacerbation:-
 It do not have any boundary.
 Duration ranges from few seconds to 2 weeks while frequency ranges from
many attacks per day to once a month.
Triggers:-
 MC trigger:- stress
 Alcohol ingestion, irregular sleep and menstruation are other known stressor till
date.

10. Clinical features
Cranial autonomic symptoms:-
65% patients have autonomic symptoms.
Tearing mc autonomic symptom followed by Conjunctival injection, ptosis, nasal
congestion and rhinorrhea.
Others are eyelid edema, meiosis, aural fullness and facial flushing with
sweating.
Felling of foreign body sensation is peculiar to HC.
Sometime it is found on objective assessment.

11. Clinical features
Migraneous features:-
Nausea, vomiting, photophobia and phonophobia are called as migrainous features.
60% patient have these features while exacerbation phase.
Visual and olfactory aura also reported.
Restlessness or agitation:-
It is important feature of TAC.
Recently restlessness has also included as alternative to CAS in HC diagnostic
criteria.

12. ICHD 3 Diagnostic criteria
3.4 HC

13. Types of HC

14. Secondary HC
Posttraumatic HC is mc secondary HC.(40%)
Followed by postcraniotomy, postpartum and postoperatives HC.
Other cause:-
Intracranial sol:- pituitary tumor, CP angle tumor
Vascular pathology:- ICA dssection, CVT, post stroke
Extracranial tissue:- sinusitis, nasopharyngeal carcinoma
Substance or withdrawal:- analgesic rebound
Neuroimaging should be perform in unilateral headache to rule out secondary causes.

15. Associated headache disorder
1) Both HC and other primary headache disorder existing simultaneously:-
 CH is the most common associated headache.
 Other reported headaches are migraine, TTH
2) HC evolving from other primary headache: -
 Again CH is the most common entity
3) HC evolving into other primary headache:-
 Withdrawal effective drug developed PH.

16. Diagnosis delay

17. Diagnosis
All TACs share some common features.
Mnemoniic 3 A’s:
1) Anteriorly located (orbital, frontal and temporal) pain
2) Autonomic features in the same area (ipsilateral) during attacks/exacerbations
3) Agitation during attacks or exacerbation
If all 3 a’s are present then most likely it is one of the TAC.

18. Diagnosis
Differentiating feature between HC and other TAC:-
1) All TAC are episodic except HC.
2) Duration and frequency of PH and CH are predictable
3) Migraneous features are more common in HC.

19. Diagnosis
Indotest:-
Intramuscular 50 mg indomethacin usually given.
Response usually occurs within 2-3 hrs.
In case of partial response 100mg indomethacin can be given.
Because of inavaibility of injectable indomethacin oral test can perform.

20. Treatment
Two options are available:
1) Medical management
2) Surgical management

21. Medical management
Indomethacin:-
 Drug of choice.
Dose:- start at 25 mg TDS f/b 25 mg increase every 3-5 days.
Usual dose required ranges from 50-500mg/day.
It takes 3-4 week for complete response.

22. Medical management
Indomethacin:-
Dose reduction can be done after 3-6 month of stable period.
Treatment failure considered when patient not responsive to more than 300 mg
daily dose.
Side effects:-gastritis with ulcer(mc), bleeding, dizziness
Contraindication:- renal impairment, gastric ulcer, bleeding diathesis.

23. Alternative drugs
Other drugs:- Ibuprofen, verapamil, ASA, methysergide

24. Surgical management
1) Peripheral nerve block:-
Supraorbital nerve, greater occipital nerve block and trochlear nerve block with
local anesthetics with or without steroids has been tried.
Response started immediately and last for 2-10 month.
Complete response achieved in half patients only.
2) Sphenopalatine ganglion block:-
Used in one trial with significant improvement
Perform twice initially followed by once a 4-5 week.

25. Surgical management
3) Radiofrequency ablation:-
Ablation of C2 ventral ramus, C2 dorsal root ganglion and SPG has been done
with longer relief period of 1 to 2 year.
4) Occipital nerve stimulation:-
Pain has been relived with ONS but cranial autonomic manifestation persist.
Newer wireless devices has also used to avoid complications like lead migration
or infection.
>50% reduction in headache days was observed in 50% patients.

26. Surgical management
5) Vagal nerve stimulation:-
Noninvasive VNS devices used during exacerbation result in reduction in
intensity of pain.
6) Botulinum toxin:-
Miller et al reported case series of 9 patients treated with onabotulinum toxin A
injections.
Five subjects had a >50% reduction in headache days.
Median duration of response was 11 weeks.

27. Trigeminal neuralgia(TN)

28. Introduction
Facial pain syndrome characterized by sudden, unilateral, sharp shooting pain In
area supplied by trigeminal nerve.
Synonyms:- Tic doulourex
Trifacial neuralgia
Fothergill’s disease
1677 John Locke:- the first full description with its treatment.
1756, Nicolaus Andre :– tic douloureux
1773 John Fothergill :- published detailed description of TN

29. Etiology
Idiopathic
Vascular compression
Secondary causes:-
Infection
Demyelination
Dental problem
Neoplasm
Post traumatic
Other vascular pathology

30. Classification
Pattern of pain:-
1) Typical:- episodic pain
2) Atypical:- constant pain
Etiological:-
1) Idiopathic
2) Classical
3) Secondary

31. Pathogenesis
Focal demyelination due to
vascular compression leads to
electrical spread of excitation
between adjacent sensory axons.
This is known as emphatic
transmission.

32. Trigeminal convergence projection theory
Continuous or recurrent nociceptive inputs from head and
neck converges on spinal trigeminal nucleus
Release of neurotransmitter and vasoactive
substance
Decrease threshold of adjacent second order
neuron
Signals from excited neuron transmitted to
thalamus ,limbic system

33. Bioresonance hypothesis
Vibration frequency of a structure surrounding the trigeminal nerve
becomes close to its natural frequency, the resonance of the
trigeminal nerve occurs
Bioresonance can damage trigeminal nerve fibers and lead to the
abnormal transmission of the impulse.

34. Ignition therapy
Innocuous injury to trigeminal nerve
Increased proportion of A beta fibers with
subthreshold occilations and ectopic discharges
Transient depolarization in neighbouring passive c
neurons
Stimulation of nociceptor by injured low
threshold mechanoreceptor

35. Epidemiology
Incidence: 8 to 13 : 1,00,000
Age: 5th – 6th decade of life
Sex: Female > male ; 1.6 > 1.0
Affliction for side: Right > left
Division of trigeminal nerve involvement: V2 > V3 > V1

36. Clinical features
Site of pain:-
Unilateral
Bilateral 3%
Along distribution of trigeminal nerve
Severity:-
Moderate to severe

37. Clinical features
Pain characteristic and pattern:-
Electric shock-like, sharp, shooting
Episodic and sudden onset of pain
Duration and frequency:-
Lasting a few seconds to minutes and
stopping suddenly
Many attacks a day to remission for
weeks to months.

38. Clinical features
Triggers :-
Washing of face
Shaving
Brushing teeth
Applying make up
Vibrations from walking
Going out in cold air
Chewing and talking

39. Red flags
Age of onset under 40 year
Bilateral and ophthalmic division
involvement
Sensory deficit
History of skin lesion
 Poor response to treatment

40. ICHD 3 diagnostic criteria
13.1.1 Trigeminal neuralgia

41. Investigation
MRI:-
Extracranial masses along the course of the trigeminal nerve
Pathological enhancement of the trigeminal nerve
 Cavernous sinus masses
 Demyelination plaques
 Intrinsic brain lesions in the thalamus or trigeminal brain stem pathways such as
lacunar infarctions
 Cerebellopontine angle mass lesions.

43. Neurophysiology
Trigeminal reflexes include blink reflex and masseter inhibitory
reflex.
It assess function of trigeminal afferents and central circuit.
Abnormality usually observed in division of trigeminal nerve that
appear clinically affected.
It is commonly abnormal in secondary TN.
Sensitivity 95% specificity 93%

44. Differential diagnosis
Migraine:- severe type persistent headache for hours, no trigger zone
Sinusitis:- pain is persistent and associated with nasal symptom
Dental pain:- localized, related to biting or hot/cold food, abnormal oral
examination
Post herpetic neuralgia:- persistent pain, usually in ophthalmic division, history
of rashes
Tumor of nasopharynx:-pain in lower jaw associated with tongue, restricted soft
palate mobility,Unable to open mouth.

45. Treatment
Pharmacological
management
surgical
management

47. Medical treatment
Classes of drugs used in TN
1. AED’ s :- Carbazepine,oxcarbazine, lamotrigine.
2. Antidepressants :- Amitryptiline/ Nortryptiline
3. Muscle relaxants :- baclofen
4. Opioid

48. Medical treatment
First line
Carbamazepine
Phenytoin
Baclofen
Gabapentin
Oxcarbazepine
Lamotrigine
Second line
Valproate
Topiramate
Zonisamide
Levetiracetam
Amitryptiline
Nortryptiline

49. Carbamazepine
Use in TN first described in 1962.
Sodium-channel modulator
 Initial response is virtually universal (If no response-then reconsider diagnosis)
 Initial response rate- 80%; By 10 yrs it drops to 50%
Dosing: Start at 200 mg/d. Add up to 200 mg in intervals of 4-5 days until pain
relief. Typical dose 1200 mg/day

50. Carbamazepine
Adverse reaction:-
 Neurologic- Ataxia, Dizzniess, Diplopia, vertigo
 Systemic- GI irritation, hyponatremia, hypersensitivity, Asymptomatic
elevation of liver enzymes , rarely severe hepatotoxicity
 Rare- Aplastic anemia, agranulocytosis, thrombocytopenia, and Stevens-
Johnson syndrome
Monitoring: CBC, LFT,RFT
 2 weekly for 2 months
 Later, 3 monthly

51. Baclofen
Analogue of GABA
Synergism with CBZ/phenytoin
30% develop resistance in long term
Dose: Start with 10 mg TDS, increase gradually; Typical maintainence dose: 50-
60 mg/day
Side effects: Somnolence/ dizziness/ GI distress
Withdraw gradually (or else Seizures and hallucinations can occur)

52. 2nd generation AED
Gabapentin:-
GABA analogue
Effective in cases resistant to traditional treatment modalities
Effective daily dose: upto 3 gm/day
Adverse effects: Dizziness, weight gain, peripheral edema, mood swings
Obermann et al: Cephalgia 2008

53. 2nd generation AED
Lamotrigine:
Acts presynaptically on voltage-gated sodium channels to decrease glutamate
release.
Effective in refractory TN (as an add on drug to the combination)
Usually well tolerated
Dose:- start as 25 mg od then 5o mg od followed by 50 mg increase every week,
Max.dose 400 mg/day
Most serious A/E- Stevens- Johnson syndrome

54. 2nd generation AED
Oxcarbazepine :-
Prodrug
As effective as CBZ
Less toxic, no hepatic enzyme induction, improved side effect profile
Dose:- usually started with 600mg/day in two divided doses,
 max. dose range from 1200-1800mg/day.

55. Botulinum toxin A
Various literature it has shown efficacy of 70%-100% in pain reducing by 60-
80%.
No major adverse event.
Can be Use in refractory cases
To decide optimal dose,time and indication of repeat dose required further
study.

56. Other medications
Pimozide
Topical lidocaine
Tizanidine
Phenytoin or fosphenytoin
Valproic acid
Topiramate

57. Surgical treatment
Gasserian ganglion-level procedures
Microvascular decompression (MVD)
Ablative treatments
◦ Radiofrequency thermocoagulation (RFT)
◦ Glycerol rhizolysis (GR)
◦ Balloon compression (BC)
Stereotactic radiosurgery (SRS)
Peripheral procedures
Peripheral neurectomy
Cryotherapy (cryonanlgesia)
Alcohol block

58. Microvascular decompression
(Jannetta procedure)
 Gardner and Sava- 1959 first developed MVD
Janetta-1977 perfected and popularized the technique
Indications:- Relatively young pts with definite vascular loop and no other
major co-morbidities
Contraindications:-
Only absolute C/I- Patients unfit for GA
Relative C/I- Multiple sclerosis
Elderly pts- not a C/I: equally good outcome as compared to young pts
Gunther et al: Neurosurgery Sep 2009

59. Microvascular decompression
(Jannetta procedure)
Offending vessels:
Arterial: 85%: Venous-68%, sole venous- only- 12%; Both- 55%
Arterial
SCA- 75%; AICA- 10%
Others: VA, Basilar (more in elderly, males and HTN), PICA and unnamed
arteries
Lower TN (V3)- SCA commonly found compressing anterosuperiorly
Upper TN (V1/ V2)- Arterial compression caudo-laterally
Isolated V2- Medial/ lateral venous compression

60. Microvascular decompression
(Jannetta procedure)
Complications:-
 Mortality: < 0.5%
Facial weakness- < 1%; Hearing loss- 1-2%
Facial numbness- 1.5%
Facial palsy, brainstem/ cerebellar infarct, CSF leak/ meningitis
At 2 yrs: 75% 10 yrs: 70% 20 yrs: 63%
Kabara EC et al: JNS 2002 Mar

61. Microvascular decompression
(Jannetta procedure)
Recurrence rates: 10-15%
Factors associated with long term recurrence:
Female patients
Symptoms> 8 yrs
Venous compression
Failure of immediate post op pain relief
If immediate recurrence- re-explore
Delayed recurrence- medical line; If fails- redo-MVD

62. Microvascular decompression
(Jannetta procedure)
Advantages:
Consistent long term success rates
Lower recurrence rates
Substantially lower incidence of facial dysesthesias
Ability to restore normal/ near normal function of the nerve itself

63. Stereotactic Radiosurgery
Attractive option for elderly patients and those who do not tolerate the more
invasive surgical procedures available.
Gamma knife
 Developed by Lars Leksell
 First use of GK was for TN
in 1971

64. Stereotactic Radiosurgery
Initial response rates: 80%-90%
Median time to response:2 wks-1 month
Long term response rates:
65-70% at 3 yrs; 50-55% at 5 yrs
Response with dose:
Better response with 90 Gy than 70 Gy
Adverse effects: Facial sensory loss (< 10%)
More with high doses (>90 Gy)
Dhople et al: Long term outcomes of GKRS for TN: JNS 2009

65. Percutaneous Techniques
Under LA/ short GA on outpatient basis
Commonly are performed in debilitated persons or those older
than 65 years.
Thermal ablation
Glycerol rhizolysis
Balloon compression
Standard landmarks for foramen ovale
2.5 cm lat to angle of lip, 3 cm ant to EAM, just below the
medial aspect of pupil

66. Percutaneous retrogasserian glycerol
rhizotomy (PRGR)
Hakanson introduced in 1981.
0.3 ml injected into the trigeminal cistern
Outcomes:
Initial pain relief: 90%
Recurrence rates: 30-70% after 2 yrs
Mild hypesthesia: 10-70%

67. Percutaneous thermal
rhizotomy
 Thermocoagulation probe is used.
Benefits depends upon how much numbness is created
Dense hypalgesia rather than analgesia is recommended
Outcomes:
90% initial pain relief; 50% at 2 yrs, 25% at 4 yrs.
Complications: Dysesthesia, motor weakness, keratitis

68. Percutaneous balloon
compression
Under short GA
Balloon catheter directed towards f.ovale
Balloon is inflated 1.3 to 1.5 atmospheres using insufflation
syringe
Compression for not> 1.5 min
Outcome:
Initial success rate: 95%
Recurrence: 25% at 2 yrs

69. Peripheral Procedures
 Goal is to denervate the trigger zone region in contrast to denervating the area
of pain distribution.
Both chemical and surgical
Supraorbital, supratrochlear, infraorbital and inferior alveloar nerves are
targeted.
Have been superseded by other safe and effective methods

70. Peripheral Procedures
Indications:
Elderly patients, cognitively impaired pts who cannot co-operate with
physicians to undergo percutaneous procedures.
Drawbacks:
High incidence of recurrence
Near total/ total anaesthesia in distribution of ablated nerves

71. Peripheral Procedures
Alcohol injections: -
Absolute alcohol is highly neurotoxic.
Under LA; needles oriented for the respective foramina
0.5-1.5 ml injected
Average duration of pain relief: 8-16 months

72. Peripheral Procedures
Surgical neurectomy
For V1 distribution: Supraorbital and supratrochlear
For V2: Infraorbital neurectomy
For V3: Inferior alveolar neurectomy
Pain relief: 26-30 months
Recurrence rates: 30% at 5 yrs

73. Surgical Treatment
Choice of surgical treatment
Relatively young patients with no co-morbidities: MVD
Patients unable to tolerate GA:
 Percutaneous procedures
 Stereotactic radiosurgery
Multiple sclerosis: SRS/ Percutaneous techniques/MVD
Final choice based on patient’s preference and ability to tolerate GA

75. Pediatric onset TN
 Extremely uncommon: 0.2/100000
Onset before 18 yrs
MVD is an effective option: (venous compression more)
Lower outcome rates than adults-
 55% at a mean f/u of 105 months
 30% recurrence in 1 year
Co-existent TN and hemifacial spasm
MVD is an effective option

76. TN with multiple sclerosis-
Symptomatic TN
2% of pts with MS, earlier onset, more atypical pain, frequently B/L
Recurrences are high
GKS is an effective treatment for refractory TN in Ms.
MVD also performed
50-75% good outcome at f/u of 50 months
Neurovascular conflict found in 58% in MRA and 90% intra-op
Veins more common
High rate of hearing dysfunction (13%)
Sandell et al: Neurosurgery Sep 2010

77. Glossopharyngeal neuralgia (GPN)
Weisenburg first described GPN in 1910.
It is characterized by unilateral, sudden, severe, brief, reccurent
pain in the anatomical distribution of the glossopharyngeal nerve.
Proposed term Vagoglossopharyngeal neuralgia

78. Epidemiology
Its rare disorder with 0.7/ 1 lakh prevalence
Female>male
Left side> right.
Occur more commonly in patient aged over 50 year

79. Etiology
Most cases are considered secondary to nerve compression by vessel.
Some patients may have other secondary causes like:-
Trauma:- skull bone #, penetrating injury
Neoplasm:- cp angle tumor, ca tongue or neck malignancy
Infections:- pharyngitis, petrositis
Vascular malformation
Demyelination:- MS
Eagle’s syndrome

80. Classifiication
Anatomical location:-
1) Otitic type:- commoner, pain around ear
2) Oropharyngeal type:- pain around throat
IHS classification:-
1) Classical:- secondary to nerve compression by vessel
2) Secondary type
3) Idiopathic

81. Clinical feature
Location of pain:-
It usually occurred in area supplied by glossopharyngeal and vagus nerve
Ear
Tonsil
Base of tongue
Larynx and oropharynx
Angle of jaw
Combination of sites are common than single site alone.
MC

82. Clinical feature
Pain characteristic:-
Sharp, shooting or stabbing pain in character
Substantial number of patient experienced dull aching or burning pain
Some patient reported unpleasant sensation like sticking, scratching or foreign
body sensation before pain started.
Laterality:-
Unilateral mc, bilateral (20%)
Left> right

83. Clinical feature
Duration and frequency of pain:-
Usually pain last for few seconds to minutes.
Sometime dull aching pain may persist for hours.
Spontaneous remission can occur from few months to years.
Triggers:-
 Swallowing(mc) of highly spiced , sweetened or cold food.
 Chewing, talking, cleaning of throat, sneezing
Cleaning of ear canal
Sudden head movement or lateral movement of jaw.

84. Clinical feature
Associated symptoms:-
Cough ,hoarseness of voice or inspiratory stridor.
Excessive salivation or lacrimation after pain
Serious complication are bradycardia, hypotension, syncope and convulsion.
GPN can present in combination with TN(2-5%)

85. ICHD 3 Diagnostic criteria
13.2.1 Glossopharyngeal neuralgia

86. Diagnosis
Cocaine test:-
10% solution of cocaine or other local anesthetic agent applied to reion of pain.
Pain should be relieved for 1-2 hour after application.
During this phase patient should be able to drink, eat or tolerate probing into
trigger zone
Positive test aid in diagnosis but negative test dose not rule it out.

87. Diagnosis
Investigations:-
CBC with ESR
 serum chemistry
MR angio or 3D CT angiography
MRI brain.

88. Treatment
Pharmacological management

89. Treatment
Glossopharyngeal nerve block:-
Can be done by non paralytic(local anesthetic with or without steroids or
paralytic agents.
Two approaches:-
1) Intraoral
2) Extraoral :-preferred because of simplicity and comfortable to patient.
It has excellent response and rapid relief of pain.
Complications:-intraarterial injection, difficulties in deglutition or huskiness of
voice.

90. Treatment
Surgical management:-
1) Peripheral procedures:-
Extracranial:- surgical neurotomies or percutaneous thermal rhizotomy
Intracranial:- direct section of glossopharyngeal nerve and vagus in cp angle
2) Central procedure:;-
Percutaneous or open trigeminal tractotomy-nucleotomy
3) Microvascular decompression
4) Pulsed radiofrequency neurolysis or Gamma knife surgery.

92. References
Bradely’s neurology in clinical practice, 7th edition
Ann Indian Acad Neurol. 2013 Jan-Mar; 16 An uncommonly common:
Glossopharyngeal neuralgia,P. M. Singh,
Journal of Pain Research 2017:10 1493–1509, Hemicrania continua: clinical
review, diagnosis and Management,sanjay Prakash
Cephalalgia 2018, Vol. 38(1) 1–211
Hindawi Pain Research and Management Volume 2017, Trigeminal Neuralgia,
Glossopharyngeal Neuralgia, and Myofascial Pain Dysfunction Syndrome: An
Update
uptodate.com

93. Thank you

94. Linear accelerator (LINAC)
Another option for TN
Produces radiation that is referred to as high energy X-ray.
Similar to the one used in Radiotherapy (IMRT)
Good outcome in TN- 80% pain relief with a mean f/u of 28 months
Mean duration of initial relief- 2weeks to 2 months
Increased dose (90Gy v/s 70 Gy), increased isodose to brainstem (30% v/s 50%)
had better pain relief but with increased risk of numbness (35% v/s 50%)
Smith ZA et al: Int J Radiat Onco Biol Phy 2011 Sep