Posts Tagged "occipital neuralgia"

Last updated on April 30th, 2021 at 11:16 pm

WAKING UP WITH HEADACHES: 6 TYPES OF WAKE UP HEADACHES.

@Neuralgroover

 

WAKE UP HEADACHE

Do you frequently wake up with a headache? This is a common feature with several types of headache disorders, but they are all distinct headache types with completely different treatments. Let’s discuss the 6 most common types of wake up headaches and the reasons why you may be waking up with headaches.

 

1) MIGRAINE

Migraine commonly causes wake up headaches for many patients, and is by far THE most common cause of wake up headaches. Sleep stage transitions can be a trigger for migraine attacks in many patients. Migraine is also susceptible to changes in sleep patterns. So for many, sleeping in (such as on the weekends or on vacation) can be a common (and cruel) migraine trigger leading to waking up with headaches. Thus, trying to maintain a similar sleep schedule on the weekends and weekdays can help with this type of trigger.

 

Other causes of wakeup headache commonly occur in patients that are stuck in chronic migraine (15-30 days per month with at least 8 headache days with migraine features), particularly if they are in rebound headache (medication overuse headache) from excess pain or “as-needed” medications. This occurs when a person with migraine is using triptans, OTCs (over-the-counter pain meds), or NSAIDs (non-steroidal anti-inflammatory drugs) more than 10 days per month, opiates or opioids more than 8 days per month, or butalbital medications such as fioricet or fiorinal more than 5 days per month, on average. Rebound headache occurs because as the patient is sleeping, the overused medication is being metabolized and eliminated from the body and the headache (typically migraine) is triggered as a result of withdrawal from the medication and the need to take more. Patients in this cycle will often notice that after they take their overused medication, the headache calms back down again. It starts to worsen again as they are due for another dose and it is wearing off. This pattern is characteristic for rebound headache. Caffeine withdrawal headache can also be a cause of wake up headaches, for similar reasons as described for rebound headache.

 

Treatments for migraine are discussed here. The key for abortive (as-needed) migraine treatment for waking migraines is that is must be something fast acting to have a chance to catch the migraine. The difficulty with waking migraines is that you are already “behind the ball” by the time you wake with the migraine because you’ve missed the early treatment window where most medications such as the triptans would normally be most effective. So for waking migraines, injectable Sumatriptan (Imitrex), nasal Zolmitriptan (Zomig) or Sumatriptan (Imitrex), are typically going to be the most effective triptans. With that said, sometimes patients can get away with a fast-acting oral triptan such as Rizatriptan (Maxalt) as well. Other options for waking migraines would be DHE (Migranal nasal spray or injection), or one of the new gepants (Nurtec ODT, Ubrelvy) since they can still be effective if taken up to 4 hours past the migraine onset, which is really great and expands the migraine onset treatment window. A neuromodulatory device could also be considered.

 

If you are averaging more than 4 migraines per month, a daily preventive treatment is generally recommended. There are many options for this including a daily pill, natural supplements, a once monthly or quarterly CGRP monoclonal antibody (Aimovig, Ajovy, Emgality, Vyepti), Botox, or a neuromodulatory device.

 

 

2) CLUSTER HEADACHE

Cluster headache is another classic cause of wake up headaches. It is a very distinct form of headache that is easy to pick out with its characteristics. Cluster headache is classified as a trigeminal autonomic cephalalgia (TAC). There are 4 types of TAC syndromes, and cluster headache is the most common of them. The other 3 TAC syndromes are hemicrania continua, paroxysmal hemicrania, and SUNCT/SUNA, none of which are waking headache types. There are some overlapping characteristics between all 4 of these TAC headache types, but cluster headache is the only one that often wakes the patient from sleep.

 

Cluster headaches can occur anytime during the day, but classically occur at the same time every night, often waking the patient up from sleep, many times shortly after falling asleep within an hour or two. Men tend to be affected 3 times more than women, but it is seen in both men and women. It is a severely painful headache, and has been termed “suicide headache” at times because of the pain severity.

 

Cluster headache is characterized by attacks of severe unilateral (one-sided) orbital (around the eye), supraorbital (above the eye), and/or temporal pain lasting 15 to 180 minutes if untreated. There is either agitation/restlessness with the headache attack and/or at least 1 autonomic sign or symptom on the side of the headache [lacrimation (runniness/tearing of the eye), conjunctival injection (redness of the eye), facial sweating or flushing (skin turning blushed), nasal congestion, rhinorrhea (runniness of nose), sense of ear fullness, eyelid edema (swelling), or partial Horner’s syndrome (miosis (pupil becomes small)) and/or ptosis (droopiness of the eye)]. Headache attacks typically occur from 1 every other day to 8 per day for more than half the time during a cluster cycle. Chronic cluster headache is defined by attacks that occur for more than 1 year without remission, or with remission periods lasting less than 1 month.

 

Cluster headache attacks occur in “clusters”, or cycles, of frequent headache attacks. These cycles of cluster attacks may last for weeks or months before they go away completely. Remission periods can last months to years. Cluster cycles often occur at a predictable time of year, such as season changes (Fall and Spring are most common).

 

Treatments for cluster headache are discussed here. In general, at the onset of a cluster cycle, a course of high dosed Prednisone is often started over 1-2 weeks to try to break up or shorten the cycle. An abortive option is also mandatory, and the most effective options are oxygen by a face mask, injectable Sumatriptan (Imitrex), nasal Zolmitriptan (Zomig) or Sumatriptan (Imitrex), or DHE (Migranal nasal spray or injection). A preventive daily treatment is also typically started at the onset of a cluster cycle and there are a variety of options for this.

3) HYPNIC HEADACHE

Hypnic headache has also been called “alarm clock” headache because it often wakes the person up at almost exactly the same time every night. These recurrent attacks occur only during sleep, causing wakening. They typically occur on 10 or more days per month for more than 3 months. The headache lasts 15 minutes and up to 4 hours after waking. This headache usually begins after age 50, but can occur in younger ages too.

 

The pain is typically mild to moderate, but can be severe occasionally. The pain usually occurs on both sides of the head (as opposed to cluster headache which is 1 sided). There is no restlessness during the headache (as opposed to cluster headache). Hypnic headache is NOT associated with autonomic symptoms [lacrimation (runniness/tearing of the eye), conjunctival injection (redness of the eye), facial sweating or flushing (skin turning blushed), nasal congestion, rhinorrhea (runniness of nose), sense of ear fullness, eyelid edema (swelling), or partial Horner’s syndrome (miosis (pupil becomes small)) and/or ptosis (droopiness of the eye)] (as opposed to cluster headache which requires these autonomic symptoms for diagnostic criteria).

 

Treatments for cluster headache are discussed here. The most common treatments are some caffeine before bed (in those who can tolerate it and not cause insomnia), or upon waking. Indomethacin taken before bed is also a common treatment.

 

4) OCCIPITAL NEURALGIA

Occipital neuralgia is a miserable nagging soreness, pain, and headache in the back of the head. I tell patients to think of occipital neuralgia as “sciatica of the head”. It is sometimes associated with cervicogenic headache (headache originating from the cervical spine with associated prominent neck pain), but more commonly occurs by itself. It is typically felt in the suboccipital region (where the base of the skull meets the top of the neck) and radiates variably into the back and top of the head and behind the ears. It can less commonly even radiate to the frontal areas (by the trigeminocervical circuitry in the upper cervical spinal cord and brainstem). It can be one sided or both sides. The pain is often described as an intense stabbing, sharp, shooting, shocking, or burning pain. It often occurs in attacks of pain which may last seconds to minutes, but can also be a continuous unrelenting pain. Sometimes it may not be as intense and may be a lower-level pain such as pressure, aching, soreness or throbbiness. Some patients may have a sensation of numbness or tingling in the back of the head. Associated neck pain is typically in the mix too. The back of the head in the area where the skull meets the neck often feels very sore or tender along the ridge of the skull bone. The pain and tenderness often increase by pushing on the back of the head and along the skull base, or lying on the back of the head. Thus, for some patients, when they lie on the back of the head during sleep, it puts pressure on the occipital nerves and they continue to get more irritated and painful until they may wake the person up from sleep due to the pain.

 

Treatment for occipital neuralgia is discussed in much greater deal here and here. In general, first line options are neck physical therapy to this area, as well as an anti-neuritic pain medication such a tricyclic antidepressant (TCA) of Amitriptyline (Elavil) or Nortriptyline (Pamelor), an anticonvulsant such as Gabapentin (Neurontin), or an SSRI such as Duloxetine (Cymbalta) or Venlafaxine XR (Effexor XR).

 

 

5) SLEEP APNEA HEADACHE:

Sleep apnea is a common cause of a headache present upon waking in the morning. However, in comparison to the headache types listed above, this headache does not “wake you up”, but rather, you “wake up with it”. It generally fades away as the morning goes on and most often has tension type headache characteristics. So if you snore, often feel unrefreshed when you wake up in the morning, and this is associated with a headache, wake with a sore throat or dry mouth, a conversation with your doctor about possible obstructive sleep apnea evaluation should be pursued. If your bed partner witnesses times where you seem to stop breathing during sleep, then this is very likely. Sleep apnea is associated with elevated high blood pressure and increased risk of stroke and heart attack, so it is important to not let it go untreated. During the deep stages of sleep, your brain is replenishing its neurotransmitters. So, if you are not getting into those deep stages because the sleep apnea is disrupting progression through normal sleep stages, fatigue, memory and cognitive complaints are common.

 

Treatment varies depending on the severity of the sleep apnea. This is determined by an overnight sleep study called a polysomnogram. These have historically been done in a controlled setting such as a hotel room, but they are now commonly done remotely in your own bed from home too.

 

 

6) HEADACHE ATTRIBUTED TO INTRACRANIAL NEOPLASM (BRAIN TUMOR)

Lastly, brain tumor is always in the differential (and at the very top of everyone’s mind when they come in the office), depending on age, prior headache history, and other clinical symptoms. These headaches are typically associated with some other neurological complaints or findings on neurological exam such as vision deficit, imbalance, speech dysfunction, memory or cognitive impairment, or one-sided numbness or weakness. However, this isn’t an absolute, and headaches can certainly present by just themselves as well. With all of that said, this is an uncommon reason for wake up headache or headache in general, surprisingly. Thus, why I have listed it last. However, it is still a reason that you should always be evaluated by your doctor for not only wake up headaches, but for any headache, especially if you don’t have a prior history of headaches, it is a different type of headache from your prior headaches, or you have any associated neurological symptoms.

 

These are certainly not the only causes of nocturnal headaches, but they are typically the top 6 that are evaluated for first. Disorders such as nocturnal bruxism (teeth grinding and jaw clenching) and TMJ dysfunction, or headache attributed to temporomandibular disorder can also be a contributor to headaches. However, these types of disorders don’t typically cause the patient to wake up with the pain. In addition, the pain is primarily in the temples, in the areas in front of the ear, into the face, and in the master muscles in the jaw. The headaches related to this are more often a tension type headache in description and not severe, and an ache and soreness in the jaw muscles and around the TMJ regions. A dentist should be able to easily diagnose if there is significant nocturnal bruxism happening by evaluating the teeth. Bed partners are also good historians on observations of teeth grinding during sleep.

 

IF YOU HAVE HEADACHE, MIGRAINE, OR FACIAL PAIN AND ARE LOOKING FOR ANSWERS ON ANYTHING RELATED TO IT, A HEADACHE SPECIALIST IS HERE TO HELP, FOR FREE!

FIRST, LET’S DECIDE WHERE TO START:

IF YOU HAVE AN EXISTING HEADACHE, MIGRAINE, OR FACIAL PAIN DIAGNOSIS AND ARE LOOKING FOR THE LATEST INFORMATION, HOT TOPICS, AND TREATMENT TIPS, VISIT OUR FREE BLOG OF HOT TOPICS AND HEADACHE TIPS HERE. THIS IS WHERE I WRITE AND CONDENSE A BROAD VARIETY OF COMMON AND COMPLEX  MIGRAINE AND HEADACHE RELATED TOPICS INTO THE IMPORTANT FACTS AND HIGHLIGHTS YOU NEED TO KNOW, ALONG WITH PROVIDING FIRST HAND CLINICAL EXPERIENCE FROM THE PERSPECTIVE OF A HEADACHE SPECIALIST.

 

IF YOU DON’T HAVE AN EXISTING HEADACHE, MIGRAINE, OR FACIAL PAIN DIAGNOSIS AND ARE LOOKING FOR POSSIBLE TYPES OF HEADACHES OR FACIAL PAINS BASED ON YOUR SYMPTOMS, USE THE FREE HEADACHE AND FACIAL PAIN SYMPTOM CHECKER TOOL DEVELOPED BY A HEADACHE SPECIALIST NEUROLOGIST HERE!

 

IF YOU HAVE AN EXISTING HEADACHE, MIGRAINE, OR FACIAL PAIN DIAGNOSIS AND ARE LOOKING FOR FURTHER EDUCATION AND SELF-RESEARCH ON YOUR DIAGNOSIS, VISIT OUR FREE EDUCATION CENTER HERE.

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Last updated on July 13th, 2021 at 07:08 am

WHAT A PAIN IN THE BACK OF THE HEAD! OCCIPITAL NEURALGIA, TREATMENTS, AND TECHNIQUE FOR OCCIPITAL NERVE BLOCK.

@Neuralgroover

HEADACHE IN THE BACK OF THE HEAD – OCCIPITAL NEURALGIA SYMPTOMS

Occipital neuralgia is a miserable nagging soreness, pain, and headache in the back of the head, often described as a base of skull headache. I tell patients to think of occipital neuralgia as “sciatica of the head”. This back of head pain is typically felt in the suboccipital region (where the base of the skull meets the top of the neck) and occipital region (back of head) and radiates variably into the back and top of the head (by the greater occipital nerve) and there is often pain behind the ear on the effected side as well (by the lesser occipital nerve). This headache in the base of the skull can less commonly even radiate to the frontal areas (by the trigeminocervical circuitry in the upper cervical spinal cord and brainstem). It can be one sided or both sides. The pain is often described as an intense stabbing, sharp, shooting, shocking, or burning pain. It often occurs in attacks of pain which may last seconds to minutes, but can also be a continuous unrelenting pain. Sometimes it may not be as intense and may be a lower-level pain such as pressure, aching, soreness or throbbiness. The back of the head often feels very sore or tender. The pain and tenderness often increases by pushing on the back of the head and along the skull base, or lying on the back of the head. Some patients may have a sensation of numbness or tingling in the back of the head. These headaches and neck pain are commonly intermingled. There may or may not be associated cervicogenic headache (headache coming from the neck) associated with occipital neuralgia.

Although patients often have isolated occipital neuralgia, I see many patients with occipital neuralgia who also have associated migraine and chronic migraine. This always creates an even more miserable feedback loop between frequent exacerbations of both the occipital neuralgia and migraine. The reason is because these two headache types influence and feed into each other. For example, 70% of patients with straightforward episodic migraine will get neck pain and tightness at the beginning of a migraine attack. So, if someone is stuck in a smoldering cycle of chronic migraine (15-30 days per month), neck pain and occipital neuralgia are commonly associated. The flip side is if someone has structural abnormalities in the cervical spine (herniated disc, injury, whiplash, etc.), it can also be a contributor to frequent or daily headaches (especially if they have a history of migraine).

 

OCCIPITAL NEURALGIA CAUSES

What are the causes of occipital neuralgia? The cause of occipital neuralgia is most commonly idiopathic, meaning there isn’t a specific cause. If you have had surgery or an injury to the back of the head, this can cause scarring of the tissues in the back of the head and base of the skull where the occipital nerves travel. This scarring can pull, twist, and tangle up the occipital nerves over time which causes persistent occipital pain in the back of the head. Sometimes the cause can be from a lot of arthritis in the upper cervical spine, tight muscles through the upper neck and skull base, or following a viral illness which can cause them to become inflamed.

 

TREATMENT FOR OCCIPITAL NEURALGIA

What is the best occipital neuralgia treatment? Neck physical therapy should always be considered as a first line treatment. The physical therapist can instruct you on the best exercises for occipital neuralgia. The goal is to stretch out and loosen the muscles below the skull base and neck. The occipital nerves pierce directly through these muscles as they travel to the back of the scalp. So imagine what is happening to those nerves if these muscles are in a state of constant tightness and spasm. The muscles will continually squeeze and irritate the nerves traveling through them, which keeps them irritated and keeps the pain going.

 

Along with the neck physical therapy, I typically suggest a daily preventive medication until the patient is consistently doing much better for several months. A more detailed list of commonly used medications for occipital neuralgia can be read here. In general, typical first line options are the anticonvulsants with Gabapentin (Neurontin) being a first line medication, or the antidepressant/anxiety class of medications, with the most effective ones being Amitriptyline (Elavil), Nortriptyline (Pamelor), Duloxetine (Cymbalta) and Venlafaxine XR (Effexor XR).

 

If there is associated chronic migraine or frequent migraine, a migraine preventive medication or treatment should be chosen, optimally one that can be helpful for both disorders. Preventive migraine treatments include , CGRP monoclonal antibodies (mAbs), neuromodulation devices, and Botox injections. Botox injections in particular are a great consideration for the combination of chronic migraine and occipital neuralgia because it can be very effective for both. I always give additional dosing over the occipital nerves in this scenario.

 

If there is a migraine component (as there often is), it’s always important to make sure to have a good abortive option for migraine exacerbations. NSAIDs (non-steroidal anti-inflammatory drugs) can be helpful for both migraine and the occipital neuralgia component. Diclofenac potassium tends to be one that I prefer, but some people do better with one or another, so it can be a trial-and-error process. If NSAIDs are not effective for aborting the migraine, then a more migraine specific option should be used such as a triptan, one of the new gepant options such as Nurtec ODT (Rimegepant) or Ubrelvy (Ubrogepant), or the new ditan medication Reyvow (Lasmiditan). A neuromodulation device could also be considered.

 

Other treatment options include occipital nerve blocks, occipital nerve stimulators, and occipital nerve decompression to detangle the occipital nerves through the scalp tissue. Most centers have moved away from occipital nerve stimulators since insurance rarely covers them and there are common problems with lead migration (leads move out of place) or lead infection. If there is a strong cervicogenic component along with the occipital neuralgia, pain management procedures such as cervical facet blocks can also be helpful. However, if the patient has only occipital neuralgia and not much of a neck pain component, cervical facet blocks and other procedures targeting the cervical spine are typically not helpful.

OCCIPITAL NERVE BLOCKS

Occipital nerve blocks should also be considered and offered. They are easy to perform in the office, can provide quick dramatic relief, and only take a minute or so to do. These can act as both an abortive option to cool down an ongoing flare of both migraine and occipital neuralgia. It can also help to prevent the pain returning for a period of time, or making it much more tolerable. However, they are typically more of a temporary benefit (days to weeks to months). However, I have quite a few patients that can get 3 months of relief until they wear off. I have occasionally seen patients break the cycle of occipital neuralgia for much longer, or even indefinitely, but this should not be the expectation. Unfortunately, it is hard to predict how much benefit one may have, or how long it may last. Pain relief typically occurs rapidly, often within minutes of the procedure, but full benefit should be seen by 2-3 days. There may or may not be some associated temporary numbness in the back of the scalp for part of the day as well. If they are done with steroids, they should not be repeated at intervals any less than 3 months. If they are done without steroids, there is no limitation on how frequent they can be done.

 

For these injections, I prefer to have the patient sitting up on the exam table with their legs hanging over 1 side. Some physicians have them sit backwards on a chair, resting their arms and head on the back of the chair. I stand on the opposite side of the exam table behind them. These are done with an anesthetic (numbing) medication such as Bupivacaine, Lidocaine, or Ropivacaine. Some physicians combine these injections with a steroid, most commonly Triamcinolone (Kenalog) or Betamethasone (Celestone). The existing evidence suggests steroids do not add much value to occipital nerve blocks, unless they are done for cluster headache. However, anecdotally many physicians still feel patients tend to do better with steroids, which makes sense given the inflammatory component of occipital neuralgia and potent anti-inflammatory effect of steroids. I prefer to use a small ½ inch 30-gauge needle (same as for Botox) to minimize the temporary pain of injection, although it takes a bit more force to inject the medicine through the needle.

 

I prefer to do 3 injections per side of occipital neuralgia (or both sides if both are affected) to ensure the occipital nerve is treated at all major points. These spots are illustrated on the photograph below. Some physicians do only 1 or 2, so this varies between physicians. The 1st injection site is located by feeling the occipital protuberance, or inion, (bump in the middle along the skull base), and going 2 fingerbreadths down and 1 over. This is the region where the greater occipital nerve pierces through the musculature. The 2nd injection site is located just lateral to the occipital protuberance, about 1/3rd of the way over between the occipital protuberance and the mastoid process behind the ear. Feel for a small groove in this area. This is the occipital groove, or notch, where the occipital nerve travels. The patient typically has the most tenderness over this spot as well, so it is typically quite easy to find. The occipital nerve travels along side the occipital artery, so it’s important to withdraw the syringe in this location, as well as all locations, prior to injection to ensure there is no blood retraction into the syringe. The 3rd site is located just behind the ear, posterior to the mastoid bone in another palpable groove. This also happens to be where the lesser occipital nerve travels.

 

Side effects of occipital nerve blocks are typically minimal and well tolerated. There are no limitations to activity afterwards and they will not make you drowsy. Some temporary tenderness in the sites is possible. Dizziness and nausea are infrequent brief side effects. Some patients can develop slight divots in the area of injection if steroids are used. If steroids are used, some patients can also feel more energized for a couple days, and sometimes some flushing.

 

Here is an example of how I do a typical bilateral (both sides) occipital nerve block procedure (following discussion of risks, benefits, alternatives, informed consent, etc.). If only one side is done, dosing can be split in two.

 

PROCEDURE

A combination of Triamcinolone (Kenalog) 40 mg (1 cc) OR Betamethasone (Celestone) 6 mg (1 cc) and 9 cc of 0.25% Bupivacaine was prepared in a single syringe and the injection sites were sterilized with alcohol swabs.

For both sides, the greater occipital nerve was injected 3 cm caudal and 1.5 cm lateral to the inion where the main trunk of the occipital nerve penetrates the semispinalis muscle. The needle was placed perpendicular and the needle advanced 1 cm. After aspiration to ensure no obstruction or presence of blood, the area was injected with 2 cc. The needle was then repositioned at the greater occipital nerve at the level of the occipital groove. After aspiration to ensure no obstruction or presence of blood, the area was injected with 2 cc.

For both sides, the needle was repositioned at the posterior border of the sternocleidomastoid at the level of the angle of the jaw, where the lesser occipital nerve is located. After aspiration to ensure no obstruction or presence of blood, the area was injected with 1 cc.

IF YOU HAVE HEADACHE, MIGRAINE, OR FACIAL PAIN AND ARE LOOKING FOR ANSWERS ON ANYTHING RELATED TO IT, A HEADACHE SPECIALIST IS HERE TO HELP, FOR FREE!

FIRST, LET’S DECIDE WHERE TO START:

IF YOU HAVE AN EXISTING HEADACHE, MIGRAINE, OR FACIAL PAIN DIAGNOSIS AND ARE LOOKING FOR THE LATEST INFORMATION, HOT TOPICS, AND TREATMENT TIPS, VISIT OUR FREE BLOG OF HOT TOPICS AND HEADACHE TIPS HERE. THIS IS WHERE I WRITE AND CONDENSE A BROAD VARIETY OF COMMON AND COMPLEX  MIGRAINE AND HEADACHE RELATED TOPICS INTO THE IMPORTANT FACTS AND HIGHLIGHTS YOU NEED TO KNOW, ALONG WITH PROVIDING FIRST HAND CLINICAL EXPERIENCE FROM THE PERSPECTIVE OF A HEADACHE SPECIALIST.

 

IF YOU DON’T HAVE AN EXISTING HEADACHE, MIGRAINE, OR FACIAL PAIN DIAGNOSIS AND ARE LOOKING FOR POSSIBLE TYPES OF HEADACHES OR FACIAL PAINS BASED ON YOUR SYMPTOMS, USE THE FREE HEADACHE AND FACIAL PAIN SYMPTOM CHECKER TOOL DEVELOPED BY A HEADACHE SPECIALIST NEUROLOGIST HERE!

 

IF YOU HAVE AN EXISTING HEADACHE, MIGRAINE, OR FACIAL PAIN DIAGNOSIS AND ARE LOOKING FOR FURTHER EDUCATION AND SELF-RESEARCH ON YOUR DIAGNOSIS, VISIT OUR FREE EDUCATION CENTER HERE.

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Last updated on April 30th, 2021 at 11:29 pm

CHIARI MALFORMATION HEADACHE, AND WHY YOU MAY STILL HAVE A DAILY HEADACHE FOLLOWING CHIARI DECOMPRESSION SURGERY.

@Neuralgroover

 

 

Chiari malformation is a common anatomical variation, specifically type I which this blog summarizes. It is most often a benign and asymptomatic finding found incidentally during routine imaging of the brain when an MRI or CT is done for other reasons, especially headache. The difficulty is often trying to associate the likelihood of a patient’s symptoms with the Chiari malformation vs. other headache disorder such as migraine, chronic migraine, and occipital neuralgia which can all have overlapping characteristics. Internet searching will give you a very long list of reported symptoms caused by Chiari malformation, many of which are inaccurate. Chiari malformation that is truly related to a patient’s symptoms typically include a “pegged” appearance of the cerebellar tonsils (back and bottom part of the cerebellum) which are pointed rather than rounded, suggesting compression at the cervicomedullary junction (area where the brainstem and upper cervical spinal cord merge between the bottom of the skull and upper cervical spine). The illustration above highlights this appearance compared to a normal brain. When this appearance is present, the patient often does have symptoms that correlate to the Chiari. Unfortunately, most of the time the Chiari malformation is not as extensive, making it more difficult to determine if some of the patient’s symptoms are correlated or not. A contrast brain MRI which includes a cine flow (cine-phase contrast) study can be helpful in determining the extent of compression and subsequent blockage of normal cerebrospinal fluid (CSF) flow throughout the craniocervical junction. A cervical MRI without contrast is also recommended to rule out a cervical syrinx (enlarged area in the center of the spinal cord), which can sometimes be associated with Chiari. If a cervical syrinx is found, an MRI of the remaining thoracic and lumbar spine should also be performed.

 

In general, Chiari malformation cerebellar tonsillar herniation is considered to be within normal anatomical variation in the following:
-First decade (0-10 years): 6mm or less
-Second and third decades (10-30 years): 5mm or less
-Fourth-eighth decades (30 to 80 years): 4mm or less
-Ninth decade (greater than 80 years): 3mm or less

 

Some mild or borderline Chiari malformations can be associated with extensive symptoms, while other times an extensive Chiari malformation is found, but the patient lacks any Chiari symptoms. So, a detailed history of symptoms including headache and associated features is crucial in determining whether a Chiari malformation is clinically relevant or not. This is more useful than basing treatment decisions purely on the extent of tonsillar herniation in Chiari. History is also important in excluding other disorders which can cause a reversible “pseudo-Chiari”, caused by a different disorder such as intracranial hypotension CSF leak, or low-pressure headache) or idiopathic intracranial hypertension (IIH) (previously known as pseudotumor cerebri).

 

According to the International Classification of Headache Disorders 3rd Edition (ICHD3), Chiari headache caused by Chiari type I malformation is usually occipital or suboccipital, of short duration (less than 5 minutes) and provoked by cough or other Valsalva-like maneuvers (straining in the abdominal region such as when having a bowel movement). It remits after the successful treatment of the Chiari malformation. Here are the ICHD3 diagnostic criteria and a Chiari malformation symptoms checklist:

 

Diagnostic criteria require Chiari malformation to have at least two of the following:

1. Either or both of the following:
a) Headache has developed in temporal relation to the Chiari or led to its discovery
b) Headache has resolved within 3 months after successful treatment of the Chiari

 

2. Headache has one or more of the following three characteristics:
a) Precipitated by cough or other Valsalva-like maneuver
b) Occipital or suboccipital location
c) Lasting less than 5 minutes

 

3. Headache is associated with other symptoms and/or clinical signs of brainstem, cerebellar, lower cranial nerve and/or cervical spinal cord dysfunction. (These may include symptoms such as hoarseness, slurred speech, swallowing or choking difficulty, unsteadiness, dizziness, vertigo, tongue weakness, trigeminal or glossopharyngeal neuralgia, tinnitus, absent gag reflex, facial numbness, autonomic symptoms (syncope, slow heart rate (bradycardia), drop attacks), loss of pain and temperature sensation of the upper torso and arms (from syrinx), loss of muscle strength in the hands and arms (from syrinx).

According to ICHD3 criteria, diagnosis of Chiari malformation by MRI requires a 5-mm caudal descent of the cerebellar tonsils or 3-mm caudal descent of the cerebellar tonsils plus crowding of the subarachnoid space at the craniocervical junction as evidenced by compression of the CSF spaces posterior and lateral to the cerebellum, or reduced height of the supraocciput, or increased slope of the tentorium, or kinking of the medulla oblongata.

 

Unfortunately, we see many patients who have had Chiari decompression, but they continue to have chronic daily headache which often resembles their pre-surgery headaches. When you delve deeper into their pre-existing headaches, many times they describe headaches which had/have migrainous features (throbbing, pounding, pulsating pain quality with nausea (+/- vomiting) and/or photophobia and phonophobia (sensitivity to bright light and loud sound with bad headache flares)). These pre-surgical headaches often fit criteria for chronic migraine, many times of which were likely sustained as chronic daily headache and chronic migraine due to medication overuse headache (rebound). So, if any of the history is suggestive of a migrainous component, this should empirically treated for first to ensure they won’t get a cranial surgery/decompression simply for chronic migraine! With that said, if it is an obvious prominent Chiari with clear Chiari headache type symptoms, this can certainly expedite the treatment plan.

 

Most of the time, the chronic daily headaches that patients continue to have after decompression surgery are associated with some variable degree of these migrainous characteristics. They typically resemble a chronic migraine pattern, and many times treating the headaches as chronic migraine rather than being distracted and treating only as ongoing Chiari headache can provide significant improvement. If the Chiari has been decompressed, then it is certainly no longer a “Chiari headache” at that point, and treatment and diagnoses should be reconsidered. However, as mentioned above, even more important is screening for these migrainous features prior to surgery, and if present, treatments targeting migraine and chronic migraine should always be exhausted first because pure Chiari headache is not going to cause migrainous features of throbbing, pounding, pulsating headache with nausea (+/- vomiting) and/or photophobia and phonophobia. Pure Chiari headache just doesn’t cause those symptoms. Those symptoms are migraine. It is common that patients can have both Chiari and migraine. The key is differentiating which is which and eliminating the migrainous component to get more clarity of how much of the symptoms are truly Chiari related, if any.

 

In addition to a chronic migraine appearing headache, patients who have had Chiari decompression frequently have associated occipital neuralgia in the back of the head and a component of chronic post-craniotomy headache. This is related to scarring of the tissues in the back of the head and base of the skull where the occipital nerves travel. This scarring can pull, twist, and tangle up the occipital nerves over time which causes persistent occipital pain in the back of the head. Post-craniotomy headache is technically similar to chronic post-traumatic headache since decompression surgery is, well, certainly a form of trauma to the head. Chronic post-traumatic headache itself commonly has a chronic migraine clinical appearance (with or without pre-existing migraine history), and treating as such can often be very beneficial. For example, we often seen concussion patients that develop chronic daily headache and chronic migraine which is “turned on” by the injury or head trauma.

 

Successful treatment with significant improvement of chronic daily headache with chronic migraine characteristics following Chiari decompression surgery is often a difficult task requiring patience and a good headache specialist. Daily medications used in migraine prevention should be considered, particularly ones that are good for not only migraine, but also occipital neuralgia and musculoskeletal pain such as anticonvulsants (topiramate, gabapentin, etc.), TCAs (amitriptyline, nortriptyline), or SNRIs (duloxetine, venlafaxine ER). Neck physical therapy can often be very helpful at stretching out the suboccipital tissues and lessening tension on the occipital nerves. If there are any migraine or chronic migraine features, then more aggressive migraine preventives such as Botox (OnabotulinumtoxinA) injections or the CGRP monoclonal antibodies should also be considered. As of 2010, Botox is still the only truly FDA approved treatment for “chronic migraine”, although all of the other treatments are still used for it as well. It should be done according to the “PREEMPT protocol”. I prefer to do additional dosing over the occipital nerves and often add numbing medicine such as bupivicaine which can provide additional temporary relief as the Botox starts to kick in over the next couple weeks. If there is an ongoing chronic daily headache driver from rebound headache (medication overuse headache), it is also crucial to eliminate this factor. Improvement will not happen while this is an ongoing factor (especially if there is a chronic migraine component). If there are migrainous features to headache exacerbations, then using more migraine specific abortive (as-needed) meds such as triptans, gepants (such as Nurtec or Ubrelvy) or ditans (Reyvow) should also be considered. Notably, the gepants do not cause medication overuse headache (rebound headache).

 

IF YOU HAVE HEADACHE, MIGRAINE, OR FACIAL PAIN AND ARE LOOKING FOR ANSWERS ON ANYTHING RELATED TO IT, A HEADACHE SPECIALIST IS HERE TO HELP, FOR FREE!

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