Neurosurgery notes/Functional/Craniofacial pain/Trigeminal autonomic cephalalgias (TACs)/Unilateral neuralgiform headache

Unilateral neuralgiform headache

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Trigeminal autonomic cephalalgias (TACs)

Reference

Types of unilateral neuralgiform headache

  • Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT)
  • Short-lasting unilateral neuralgiform headache with autonomic symptoms (SUNA)
      • SUNCT
      SUNA
      Autonomic Symptoms
      Involves two specific autonomic symptoms: eye redness and tearing
      Can involve any autonomic symptoms
      Attack Duration
      Short-lasting
      Slightly longer-lasting, averaging 10 minutes
      Attack Site
      Most common attack site is the temporal area
      Most common attack site is the parietal area
      Demographics
      Female predominance with a ratio of 1.14:1.
      Mean age at onset is 37.22 years
      Higher female predominance with a ratio of 2.10:1.
      Mean age at onset is 42.45 years

Numbers

  • Annual incidence to be 1.2 per 100,000.
  • Mean age of 48
  • Gender
    • Male predominance in SUNCT
    • Female predominance in SUNA

Diagnostic criteria

  1. Have had a minimum of 20 headache episodes that fulfil the criteria below
  1. Headache is at least of moderate-intensity in the orbital or temporal area on one side and lasts more than one second but less than 600 seconds.
  1. Must have one of the below symptoms on the same side as the headache
      • Tearing and redness of the eyes
      • Stuffy nose or runny nose
      • Eyelid swelling on the same side as the headache
      • Sweating of head and neck area
      • Redness of head and neck area
      • Ear fullness and discomfort
      • Droopy eyelid or pinpoint pupil
  1. At least one attack per day when the disease is active
  • *Lack of response to indomethacin and the response to intravenous lidocaine,

Mechanism

  • Exact pathophysiology of SUNCT and SUNA remains unknown.
  • Hypothalamic dysfunction → overproduction of nociceptive orexin B promotes pain signal in the trigeminal-hypothalamic tract, → pain pathway activated
  • DBS to the ventral tegmental area (VTA) ipsilateral to the side of the attacks showed about 80% improvement in symptoms; this finding supports the involvement of VTA in the pathophysiology of SUNCT and SUNA.
  • Stimulus (stimulus could be simple or a noxious one) to the trigeminal nerve → Trigeminal ganglion activated → projects impulses to the trigeminal-cervical complex (composed of C1/C2 dorsal horns and the trigeminal nucleus caudalis (TNC).) → impulse then travels to the superior salivary nucleus (SSN) in the pons → SSN sends signal through pre ganglionic parasympathetic fibres → sphenopalatine ganglion → innervate the lacrimal gland and the nasal mucosa and palate

Investigation

  • MRI
    • Look for secondary causes of unilateral neuralgiform headaches
      • Pituitary
      • Posterior fossa
      • Local lesions
  • Blood tests for pituitary function

Management

  • Short Term Prevention
    • Trial of IV lidocaine
      • Given in an ITU
        • Requires cardiac and blood pressure monitoring
    • Dose
      • 1.3 mg/kg per hour to 3.3 mg/kg per hour.
  • Long Term Prevention
    • Lamotrigine
      • 80% of patients reporting improvement
      • Dose
        • 100 mg to 400 mg
    • Carbamazepine and oxcarbazepine
      • 900 mg/day
    • Topiramate
    • Gabapentin
  • Invasive
    • Greater occipital nerve block
    • Peripheral nerve blocks, including infra and supraorbital nerve blocks
    • Peripheral nerve stimulation of the occipital nerve

Difference between trigeminal

 
Cluster headache (CH)
Short-lasting Unilateral Neuralgiform Headache Attacks (SUNHA) (previously SUNCT and SUNA)
Description
Unilateral head pain (orbital, supraorbital, temporal, or any combination)
Unilateral head pain
Duration
Lasting 15–180 minutes
Lasting seconds to minutes (1–600 sec)
Frequency
1–8 times a day
At least once a day
Associated features
Ipsilateral cranial autonomic features and/or restlessness or agitation
Usually associated with prominent lacrimation and redness of the ipsilateral eye
Attack pattern
notion image

SUNA

  • Short-acting unilateral neuralgiform headache with cranial autonomic symptoms
    • Prominently pain in V1 more than V2, V3 or in teeth
    • Very brief (5–240 secs) occurring at least 20–200 times a day
    • Conjunctival injection or tearing only but not both
    • Ipsilateral photophobia, phonophobia c.f. bilateral in migraine. Other cranial autonomic symptoms: rhinorrhoea, eyelid oedema, facial sweating/flushing, ear flushing
    • Cutaneous stimulation triggers attacks in 22%
    • No refractory period between attacks
    • High rate of pituitary tumours