There is an unusually rare headache subtype known as contact point headache also known as Sluder's neuralgia, anterior ethmoid neuralgia, pterygopalatine ganglion neuralgia, or sphenopalatine ganglion neuralgia.

This extremely frustrating headache has caused untold suffering for numerous patients who suffer from unrelenting sharp or stabbing pain in a single localized spot on their face. It is not unusual for a patient to go for years without a diagnosis or good treatment plan that works.

They may have been seen by a number of neurologists, ENTs, dentists, oral surgeons, spine specialists, all without pain resolution. Medications that may have been tried include antibiotics, nasal sprays, neuropathy medications, steroids, narcotics, muscle relaxants, etc which don't seem to help very much. Oddly enough, the one medication that may provide the single best relief is an over-the-counter decongestant like sudafed.

MRI and CT scans do not reveal any tumor or sinus infection. In the end, patients may even be told it is all psychological or a very bad type of neuropathy.

The typical patient with contact point headaches classically have a history as follows that may include some or all of the following elements:

• Usually the pain started after an upper respiratory infection
• Pain localized to a single area on the face on just ONE side
• Pain classically described localized to the cheek and/or between nose and eye (red areas in diagram) but can radiate to other locations.
• Pain can also be localized to upper teeth and roof of mouth.
• Pain described as sharp or shooting; less commonly as pressure (like someone driving a high-heel into the face)
• May be associated with light and noise sensitivity; not uncommon for these patients to be erroneously diagnosed as migraines without aura (MWOA)
• Decongestants seem to work the best to resolve headache, but only temporarily

This rare headache has even been published as a medical mystery in the Washington Post and portrayed on TV in the hit drama Grey's Anatomy (Season 5 Episode 3: Here Comes the Flood) during which Dr. Sloan diagnosed this condition in a man who had this headache for 7 years.

What Causes It?

To put it simply, there's an anatomic spot inside the nose where a nerve is getting compressed between two structures. It's analogous to leg sciatica, but of the nose/face. The nerve that is getting pinched is either the anterior ethmoid nerve which is a branch off the ophthalmic division of the trigeminal nerve or one of the nerves branching off the sphenopalatine ganglion (also known as pterygopalatine ganglion).

As such, pain involves regions where one of these nerves get pinched. The diagram illustrates a side profile of the nose where both nerves are located (anterior ethmoid nerve in green and pterygopalatine ganglion in purple). Both nerves innervate the septum which divides the left and right nasal cavities as well as lateral aspects of the nose. The pterygopalatine ganglion additionally innervates the roof of the mouth and upper teeth.

As such, patients suffering from contact point headache with additional symptoms of upper teeth and gum pain and/or odd sensations of the roof of the mouth typically have sphenopalatine ganglion involvement, but not the anterior ethmoid nerve (note the purple distribution of the pterygopalatine ganglion).

The culprit internal nasal structure that often leads to nerve compression is a deviated septum or a septal spur that digs or juts into the side of the nose (middle or superior turbinate). Think of it as a bunion inside the nose. The septum is a wall that divides the right and left nasal cavities and is supposed to be straight. However, when it becomes deviated, it may lead to narrowing on one side. If severe enough, with any nasal mucosal swelling, it can dig into the lateral nasal wall where the middle and superior turbinates are located leading to the pinpoint headache.

normal
deviated
spur
Normal Straight Septum
Deviated Septum (Green Arrow)
Septal Spur (Arrowhead)

Here are corresponding CT scans showing normal and the septal abnormalities:

normal
spur
Normal Straight Septum
Deviated Septum (Red Arrow)
Septal Spur (Red Arrow)


Here is an actual picture of a septal spur after and before nasal decongestion. Pain obviously occurs with congestion in the 2nd picture.

spur 

Besides the septum, there are other possible structures that can less commonly cause nerve compression leading to contact point headaches including concha bullosa (aerated middle turbinate) or abnormally positioned turbinates. Turbinates (inferior one shown by the arrow below) are normal internal nasal structures that warm and humidify the air as it passes through the nose.

turbinates

What is the Workup?

When contact point headaches are suspected, a patient unfortunately needs to go through a workup which includes:

• ENT evaluation
• Nasal Endoscopy to confirm presence of a structural problem
• CT scan of the sinus cavity is THE key test to obtain to ensure no underlying sinus pathology or anatomic boney irregularities.
• CT Neck may additionally be recommended if sphenopalatine ganglion neuropathy is present with endoscopic suggestion of a possible skull base tumor. 
• MRI Scan usually obtained by a neurologist to ensure no underlying brain/spine pathology.

Depending on the nasal endoscopy and CT scan findings, a diagnostic test of sorts can be performed in the office whereby the nasal endoscope can "touch" the contact point area to see if it reproduces or exacerbates the contact point headache. A follow-up test would be to apply numbing medicine in the area of the contact point and see if it resolves the pain.

What is the Treatment?

Given the basic problem leading to contact point headaches is a STRUCTURAL problem, there is no pill or nasal spray that will "cure" this issue just like a broken bone can not be fixed with an oral pill.

Nasal sprays and decongestants can help temporarily (hours to a few days) as these medications reduce any mucosal swelling that is present thereby creating room and relieving any nerve compression. However, the moment the musocal swelling recurs, the pain comes back.

As such, the standard treatment to treat the source problem is surgery to address the underlying anatomic structures leading to nerve compression. Surgery usually involves straightening the septum or removing the septal spur. On rare occasions, sinus surgery may even be required. The goal is to create as much room as possible to prevent any nerve compression when mucosal swelling occurs.

In rare cases, there are less invasive options that can be considered as well (injections), though again, usually only temporary in duration (months).

Why Now?

A common question I hear is WHY did this headache occur now since a deviated septum or septal spur probably has been present for years even before the headaches started.

There is no good answer to this question. However, one thought is that during a viral upper respiratory infection, for whatever reason, the nerve has become hypersensitized leading to a type of neuropathy. With this neuropathy, any physical irritation of the nerve including physical compression leads to pain whereas before the viral nerve injury, pain wouldn't have occurred.

But that's just an educated guess.

What if There Are NO Anatomic Abnormalities?

If there are no anatomic abnormalities, it becomes less likely for a contact point headache to be present. Differential diagnosis at this point would be a dental issue, atypical migrainecluster headachetrigmeninal neuralgia, or a true sphenopalatine ganglion neuralgia without a physical basis.

It is still possible there may be a sino-nasal physical basis for the headache for which surgical treatment may be curative, but it just becomes less likely when there are no identifiable sino-nasal structural problems.

arrow
arrow
    全站熱搜

    怪醫 發表在 痞客邦 留言(0) 人氣()