Atypical trigeminal neuralgia (ATN) is a rare disorder of cranial nerve V that causes facial pain with a heavy, aching, burning, or dull quality. It is difficult to distinguish from classic trigeminal neuralgia, but medications for typical trigeminal neuralgia can provide pain relief. The underlying mechanisms of ATN can result from several conditions including a tumor or malformed blood vessel compressing the nerve, infection, dental procedures, and demyelinating diseases such as multiple sclerosis. Partial relief for ATN can be obtained from the drugs used for its classic counterpart, and surgical decompression of the nerve can be curative.
Atypical trigeminal neuralgia (ATN) is a rare disorder of cranial nerve V (CN V), characterized by facial pain that has a constant or fluctuating timing, with a heavy, aching, burning, or dull quality. It is difficult to distinguish it from classic trigeminal neuralgia or tic douloureux because the latter is also characterized by intense facial pain in cranial nerve V or in the trigeminal distribution. Sometimes, atypical neuralgia is mistaken for migraine, hypochondria, or temporomandibular joint disorder. Medications for typical trigeminal neuralgia can provide pain relief.
Cranial nerve V is the largest nerve in the face that transmits signals for different sensations including pain, heat and pressure. When the trigeminal nerve becomes irritated, a condition called trigeminal neuralgia occurs. It manifests itself as an intense, electric-shock-like or shooting pain on one side of the face, especially the jaw and lower face. In classic or typical trigeminal neuralgia, trigeminal nerve pain occurs in spasms or attacks that usually last several seconds and do not radiate. Classic trigeminal pain is stimulated when facial “trigger points” are touched and is characterized by painless periods called remissions. When a person comes to the doctor complaining of facial pain that does not quite match the characteristics of classic trigeminal neuralgia, a diagnosis of atypical trigeminal neuralgia or trigeminal neuralgia type 2 is made.
The pain of atypical trigeminal neuralgia may be crushing or burning, rather than electric or stabbing. Also, the pain of this variant of trigeminal neuralgia can be constant or continuous, and the patient rarely has periods of remission. Some people who have atypical trigeminal neuralgia complain of constant migraines or pain throughout the face. This pain is made worse by facial movements such as chewing, talking, and smiling, and sometimes by cold sensations. The constancy of excruciating pain has driven some people with ATN to commit suicide, thus giving ATN the nickname “the suicide disease.”
The underlying mechanisms of ATN are inflammation of the nerve, destruction of the myelin sheath, and subsequent increased sensitivity of CN V. These mechanisms can result from several conditions including a tumor or malformed blood vessel compressing the nerve, infection, dental procedures and demyelinating diseases such as multiple sclerosis. Some have hypothesized that ATN is secondary to a compression of a part of the CN V called the portio minor, but others believe that it is a more severe or progressive form of typical trigeminal neuralgia.
Partial relief for atypical trigeminal neuralgia can be obtained from the drugs used for its classic counterpart. These medications, which include anticonvulsants such as carbamazepine and lamotrigine, anesthetics such as lidocaine, and antidepressants such as amitriptyline, are thought to be helpful because they provide relief from neuropathic pain. Surgical decompression of the nerve can be curative. It is important to be able to control pain using these modalities because atypical trigeminal neuralgia can lead to depression and reduce the affected person’s quality of life.
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