Trigeminal Neuralgia: Classification

The following forms of trigeminal neuralgia are distinguished:

  • Idiopathic trigeminal neuralgia without evidence of vascular nerve compression – more common form; occurs predominantly unilaterally
  • Classic trigeminal neuralgia with evidence of vascular nerve compression.
  • Secondary (symptomatic) trigeminal neuralgia – a cause (e.g., multiple sclerosis (MS), space-occupying lesion in the cerebellopontine angle) may be found; rare form; occurs more often bilaterally; other pain may exist between episodes of pain. Sensory disturbances of the facial skin may also occur.

In addition, in the clinical symptomatology, trigeminal neuralgia with purely paroxysmal pain is differentiated from trigeminal neuralgia with additional continuous constant pain in the area supplied by the nerve. Classification of the subtypes of trigeminal neuralgia according to the ICHD-3.

13.1 Pain attributed to a lesion or disease of the trigeminal nerve
13.1.1 Trigeminal neuralgia
13.1.1.1 Classic trigeminal neuralgia
13.1.1.1 Classic trigeminal neuralgia, purely paroxysmal (occurring with attacks)
13.1.1.2 Classic trigeminal neuralgia with continuous pain
13.1.1.2 Secondary trigeminal neuralgia
13.1.1.2.1 Trigeminal neuralgia attributed to multiple sclerosis
13.1.1.2.2 Trigeminal neuralgia attributed to space-occupying lesion
13.1.1.2.3 Trigeminal neuralgia attributed to another cause
13.1.1.3 Idiopathic trigeminal neuralgia
13.1.1.3.1 Idiopathic trigeminal neuralgia, purely paroxysmal
13.1.1.3.2 Idiopathic trigeminal neuralgia with continuous pain
13.1.2 Painful trigeminal neuropathy
13.1.2.1 Painful trigeminal neuropathy attributed to herpes zoster (shingles)
13.1.2.2 Postherpetic trigeminal neuralgia
13.1.2.3 Painful posttraumatic trigeminal neuropathy
13.1.2.4 Painful trigeminal neuropathy attributed to another disorder
13.1.2.5 Idiopathic painful trigeminal neuropathy.

Classification of trigeminal neuralgia: International Headache Society (IHS) 2018 (after).

Classical trigeminal neuralgia
Purely paroxysmal (without persistent background facial pain).
Trigeminal neuralgia in which imaging or surgical intervention demonstrate vascular (vessel-related) compression (atrophy, displacement of the nerve). Mere contact is not sufficient (strictly speaking, this is equivalent to secondary trigeminal neuralgia in neurovascular (nerve-vessel) compression). Classic trigeminal neuralgia usually occurs in the second and third branches (cheek/lower jaw/chin area). Classic trigeminal neuralgia may be preceded by a phase of continuous pain in the area (pra-trigeminal neuralgia). Between paroxysms (seizure-like occurrence of a disease symptom), there is freedom from symptoms.
With continuous pain (with persistent background facial pain).
There is concomitant continuous or almost continuous pain in the affected supply area of the trigeminal nerve between attacks of classic trigeminal neuralgia.
Idiopathic trigeminal neuralgiaNeither vascular (vessel-related) compression nor any other cause of trigeminal neuralgia can be demonstrated.
Secondary trigeminal neuralgiaA cause of trigeminal neuralgia such as plaques of multiple sclerosis, infection, or a space-occupying lesion in the cerebellopontine angle have been demonstrated.

Diagnostic criteria of trigeminal neuralgia.

Criteria Description
A
  • Recurrent paroxysmal unilateral (one-sided) facial pain attacks in the supply area(s) of one or more branches of the trigeminal nerve without radiation beyond, fulfilling criteria B and C.
B Pain exhibits all of the following characteristics:

  • Duration between a fraction of a second to 2 minutes.
  • Strong intensity
  • Surge, shooting in, piercing or sharp quality.
C
D
  • Not better explained by another ICHD-3 diagnosis.