trigeminal Neuralgia/ Lo and High pressure HA. Suitable for Internal Medicine residents. Has MKSAP questions at end

2. Low and High Pressure
Headaches and Trigeminal
Neuralgia
Monique Canonico DO
Assistant Professor, John A. Burns School of Medicine
April 2019

3. Intracranial Hypotension Risk Factors
• Young
• F
• Low BMI
• Migraine hx does not increase risk

4. HA from Intracranial Hypotension
• Postural
• Ha; neck pain/stiffness
• Diplopia
• Nausea
• Vertigo
• Tinnitus
• Hyperacusis

5. Intracranial Hypotension Causes
• LP
• Trauma
• Surgery
• Spontaneous

6. MRI in Low Pressure HA

7. Intracranial Pressure Evaluation
• MRI with contrast of brain and spinal cord
• MRI could be normal or show
 Diffuse pachymeningeal enhancement
 Subdurals
 Tonsillar descent
• CT myelography: level of the leak

8. MRI in Low Pressure Headache

9. Pituitary Gland Changes: MRI

10. MRI in Low Pressure HA-Bilat Subdurals

11. MRI in Low Pressure Headaches

12. MRI in Low Pressure HA: Cerebellar Tonsils LOW

13. Intracranial Hypotension: Treatment
• Fluids, rest, caffeine
• Blood patch
• Fibrin glue, repeat blood patch, surgical correction of
the dural tear

15. Pseudotumor Epidemiology
• 1,100/000 general population
• Women aged 15-44= 3.5/100/000
• Women whose BMI > 29 = 20/100,000

16. Pseudotumor Risk Factors
• Hypervitaminosis A
• Tetracyclines
• Isotretinoin
• Pregnancy
• Steroid use

17. Pseudotumor Headache
• Diffuse
• Steady
• Throbbing
• Worse in am or with Valsalva
• TVOs (transient visual obscurations)

18. Pseudotumor-Other Features
• Pulsatile tinnitus
• Dizziness
• Neck pain

19. Diagnosis
• Clinical feature of raised ICP without apparent cause
• Normal cerebral MRI/MRV
• LP
 Check pressure
 Exclude infectious/inflammatory/neoplastic
 Symptomatic improvement

20. Pseudotumor Dx
• Papilledema is almost always present
• Confirmation: CSF OP > 250 mm H20 with normal
fluid composition
• They also need a visual field test at baseline and every
3 mos thereafter until stabilized
 This shows enlarged blind spot and decreased peripheral fields

22. Conditions to Exclude
• Space occupying lesion
• Venous sinus thrombosis
• Hydrocephalus
• Chronic meningitis-infective/inflammatory/neoplastic
• C02 retention
• Malignant htn

26. Pseudotumor MRI-flattened globes

27. MRI in Pseudotumor

28. Preudotumour: Empty Sellla

29. Empty Sella

30. Empty Sella

31. Treatment
• LOSE WEIGHT !
• Stop offending med
• Treat endo problem
• Medical
 Carbonic anhydrase inhibitors (decrease CSF production )-acetazolamide
 Topiramate
 Furosemide
 Surgical
optic nerve sheath fenestration
diversion procedures

32.  Acetazolamide
 Topiramate
 Wt loss (if overwt)
 Optic nerve sheath fenestration
 Lumboperitoneal shunt
Pseudotumor Tx

33. Pseudotumor Summary

35. Trigeminal Neuralgia

36. Trigeminal Neuralgia
• Initiating pathologic events include:
 nerve compression by tortuous arteries of the posterior fossa blood vessels
 demyelinating plaques
 herpes virus infection
 infection of teeth and jaw
 a brainstem infarct

37. Anatomy

38. Functional Anatomy
• GSA – general sensation from head and facial structures
Main sensory nucleus
Descending tract of V to spinal trigeminal nucleus
Functional equivalent of substantia gelatinosa of spinal cord
• GSE – muscles of mastication
• SVE – branchial arch muscles
Tensor veli palatini
Tensor tympani

39. Demographics
• Slight female predominance
 Female 5.9 per 100,000
 Male 3.4 per 100,000
• Right side affected slightly more often
• Occasional familial occurrences
• Slightly elevated risk associated with HTN and multiple
sclerosis

40. TN: Age of Onset
0
5
10
15
20
25
30
2nd 3rd 4th 5th 6th 7th 8th 9th
Decade

41. Distribution of Pain By Division
32
17 17
15 14
4
0.4
0
5
10
15
20
25
30
35
Percent
V2,3 V2 V1,2,3 V3 V1,2 V1 V1,3
Trigeminal Division

42. Clinical Manifestations TN
• Abrupt onset with excruciating pain!!
• Pain described as burning, knifelike, or lightning shock
in the lips, upper or lower gums, cheek, forehead, or
side of the nose.
• Patient may twitch, grimace; frequent blinking and
epiphora may occur

43. Clinical Manifestations TN
• Attacks may be brief (2 or 3 minutes)
• Unilateral
• Episodes may be initiated by triggering mechanism of
light cutaneous stimulation as a specific point (trigger
zone) along nerve branches.

44. Classic TN (Type I)
• Brief (seconds to minutes) episodes of severe, sharp, stabbing,
lancinating, pain
• Almost always unilateral
 Bilateral V1 pain suggestive of MS
• Pain occurs along one or more trigeminal divisions
• Spontaneous or evoked pain
 Cutaneous trigger zones
• Multiple attacks may occur over short periods
• Asymptomatic between attacks
• Normal facial sensation

45. Triggers
Chewing, brushing teeth, hot or cold
blast of air on the face, washing the
face, yawning, or talking.
Patient may eat improperly, neglect
hygiene practices, wear cloth over
face, withdraw from interaction with
others.

46. Atypical / Symptomatic Pain
carotid dissection
giant cell (“temporal” arteritis)
acute V-Z & post-herpetic
neuralgia
Tolosa – Hunt syndrome

47. All Facial Pain is Not TN
• ALL FACIAL PAIN IS NOT TRIGEMINAL NEURALGIA!
• Successful treatment of any patient with facial pain in
general and TN in particular depends on making the
correct diagnosis at the outset

48. TN: DX
Trigeminal Neuralgia: IHS Diagnostic Criteria
A. Paroxysmal attacks of facial or frontal pain which last a few seconds
to less than two minutes
B. Pain has at least 4 of the following characteristics:
(1) distribution along one or more distributions of the
trigeminal nerve.
(2) sudden, intense, sharp, superficial, stabbing or
burning in quality.
C. No neurologic deficit
D. Attacks are stereotyped in the individual patient.
E. Exclusion of other causes of facial pain by history, physician
examination and special investigations when necessary.

49. MRA-Right V is Compressed

50. Pharmacologic Tx of TN
• AEDs are the cornerstone of treatment
• Start low, titrate to relief or side effects
• Monitor side effects and drug interactions
• Monitor levels and blood tests if indicated
• Rotate other AEDs or add as needed
• Carbamazepine remains the gold standard
 Response thought to be diagnostic

51. Before Carbamazepine or Oxcarbamazepine:
• The HLA-B*1502 gene test is used to identify those at
risk for serious side-effects to a medication called
carbamazepine.
• Check in Asians!
• Risk increased for topic epidermal necrolysis

52. Pharmacologic Tx TN
 AEDs
 Tegretol (carbamazepine)
 Trileptal (oxcarbazepine)
 Dilantin (phenytoin)
 Neurontin (gabapentin)
 Lyrica (pregabalin)
 Lamictal (lamotrigine)
 Topamax (topiramate)
 Gabatril (tiagabine)
 Keppra (levetiracetam)
 TCAs
 Elavil (amitriptyline)
 Pamelor (nortriptyline)
 Baclofen
 Opioids

53. Side Effects AEDs
• Cognitive changes
• Sedation
• Nystagmus, ataxia, diplopia, dizziness
• Nausea, vomiting, headache
• Hyponatremia
• Allergic reaction
 Up to 7% with CBZ
 Some cross-reactivity between CBZ and PHT

54. Surgical Tx of TN
• Microvascular decompression (MVD)
• Percutaneous ablative procedures
 Radiofrequency gangliolysis
 Glycerol rhizolysis
 Balloon compression
• Stereotactic radiosurgery
 Gamma knife
• Peripheral ablative procedures (V1 and V2 pain)
 Peripheral branch neurectomy
 Alcohol neurolysis
• Open destructive procedures
 Partial sensory rhizotomy
 Subtemporal ganglionectomy (Frazier-Spiller procedure)

55. Microvascular Decompression Surgery

56. Operative Findings
• Arterial compression
Superior cerebellar artery (SCA) – most common
AICA
PICA
Vertebrobasilar artery
• Venous compression
More common with atypical TN
• Combined arterial and venous compression

57. Gamma Knife

58. Facial Pain Association
• Call 1-800-923-3608 or 1-352-384-3600
Phone support business hours: M-F 9 am – 5 pm
Eastern Time.
• 22 SE Fifth Ave., Suite D
Gainesville, FL 32601

59. Glossophayrngeal Neuralgia
• Pain most often occurs in the territory of the glossopharyngeal nerve
• GSA input from external/middle ear, posterior tongue, and pharnyx
 Classic GPN – pain primarily in tongue and pharnyx
 Otalgic GPN – pain primarily occurs in ear
• Unilateral, paroxysmal, lancinating pain; last seconds to minutes
 Pain may occur in clusters
 Irregular intervals over days, weeks or months
• Spontaneous occurrence or precipitated by swallowing
• Peak incidence : 5th to 7th decade
• Pain relieved by anesthetizing posterior pharynx with 10% cocaine
• 5% - 8% of cases caused by posterior fossa tumor
• Pain may be due to elongated styloid process (Eagle’s syndrome)

60. Associated Factors
• Female> male
• Obesity
• Fe deficiency anemia
• Endocrine
 Hypothyroidism
 Hypoparathyroidism
 PCOS
• Drugs
 Vit A
 Tetracycline

61. MKSAP questions

62. MKSAP17
A 26yo F presents with worsening ha that began intermittently 6 mos and
became qd 3 mo ago. Pain is vise-like, steady and bilateral. She has brief
temporal sharp pains associated with visual dimming. HA are associated with
neck stiffness but no photophobia or nausea. She has PCOS tx with
metformin and takes a combined oral contraceptive. BMI is 30. Partial L sixth
nerve palsy is noted on exam. MRI is nl; LP shows OP of 350 mm H20.
Which is the appropriate treatment?
a) Acetazolamide
b) Amitryptiline
c) Blood patch
d) Optic nerve sheath fenestrations
e) Spironolactone

63. MKSAP17
A 36yo F has 1 wk of recurrent 1-3 sec episodes of facial pain that
occur spontaneously any time throughout the day. The pain is
sharp and severe and located in the R infraorbital region. During
the same time period she has developed worsening bilat LE
weakness and urinary incontinence. The pt has an 18 yr hx of MS
tx with beta interferon 1A; she also takes baclofen to control
spasticity.
On exam, bilateral LE weakness, hyperreflexia and a sensory level at
T6 are noted.
What is the cause of the facial pain?
a)Chronic paroxysmal hemicrania
b)Herpes zoster
c)Primary stabbing headache
d)Trigeminal neuralgia

64. MKSAP18
A 37-year-old woman is evaluated for a 3-day history of recurrent episodes of severe, piercing
right maxillary pain lasting several seconds. Attacks have become progressively more frequent,
now occurring several times per hour, and can either arise spontaneously or be triggered by
washing the face, chewing, or applying facial cosmetics. She has had no associated conjunctival
injection, tearing, or nasal congestion or drainage. The patient has multiple sclerosis.
Medications are glatiramer acetate and an oral contraceptive.
On physical examination, vital signs are normal; BMI is 22. A left afferent pupillary defect is
noted, as is unsteadiness of tandem gait. All other physical examination findings are
unremarkable, including normal facial sensation bilaterally.
A fluid-attenuated inversion recovery MRI reveals periventricular and brainstem hyperintensities
that are not seen with contrast enhancement.
What is the treatment?
A) Acetazolamide
b) Carbamazepine
3) Indomethicin
4) Lamotrigine

65. MKSAP18
A 52-year-old woman is evaluated for a 3-week history of new-onset daily headaches. The
pain is absent nocturnally and on awakening but starts within 15 minutes of the patient’s
arising from bed and becomes progressively severe throughout the day.
The headache is global, steady, and (when severe) associated with photophobia and mild
nausea. Intermittent bilateral tinnitus and brief episodes of horizontal diplopia also have occurred.
The pain improves within 15 to 20 minutes of the patient's lying down. Analgesic agents have been
unhelpful. She has no other medical problems.
On physical examination, vital signs are normal; BMI is 26. Partial right abducens nerve (cranial nerve VI) palsy is noted
An MRI of the brain shows diffuse nonnodular pachymeningeal enhancement,
a cerebellar tonsillar descent of 3 mm, and clinically insignificant bilateral subdural fluid collections.
Which of the following is the most appropriate first step in management?
A) Acetazolamide
B) Blood patch
C) Lumbar puncture
D) Subdural evacuation