When a headache is a pain! About primary and secondary headaches

When a headache is a pain! About primary and secondary headaches

Nitin K. Sethi, MD

Assistant Professor of Neurology

New York-Presbyterian Hospital

Weill Cornell Medical Center

New York, NY 10065

 

 

Headaches are a very common reason that patients consult a neurologist like me.  The intensity of the headache, its character and duration are some of the factors which determine if and when patients seek medical attention. So in this post I shall go over the different types of headaches and list their main differentiating features.

Broadly speaking we can divide headaches into primary and secondary headaches. Primary headaches are those headaches for which there is no structural/ secondary cause.  For example a migraine headache is an example of a primary headache. The migraine may be severe and frequently disabling but if you scan (CT or MRI) the patient’s brain no underlying structural lesion like a tumor is found. Secondary headaches as the name implies are secondary to a another cause. For example headache due to a large brain tumor or headache due to an infection (meningitis) represent secondary headaches.

Types of primary headaches:

1)      Migraine: common migraine and classical migraine

2)      Tension type headache

3)      Cluster headache

Types of secondary headaches:

1)      Headache secondary to a brain tumor or other masses (space occupying lesions) in the brain

2)      Headache secondary to an infectious and inflammatory process in the brain (example headache associated with meningitis)

Headaches can be a cause of concern and distress. Patients frequently worry that may have a brain tumor. So let us go over some of the signs and symptoms which suggest that the headache is due to a secondary cause . These are the signs and symptoms which should make you seek urgent medical attention:

  1. Sudden onset of severe headache. When asked patients rate their headache as the “worse headache of their lives”. I shall be concerned if I encounter such as patient in the ER, more so if the patient tells me that they have never had a  headache in their life before. When accompanied with other signs such as nuchal (neck) rigidity and depressed sensorium (level of alertness), the question of subarachnoid hemorrhage (SAH) comes to mind. A common cause of non-traumatic SAH is the rupture of an aneurysm or arteriovenous malformation (AVH) in the brain. SAH is associated with high morbidity and mortality especially if the patient presents to medical attention late.

Caveat: Not all “worse headaches of my life” are due to SAH due to rupture of an intracranial aneurysm. Many times (especially if there is a past history of migraine headaches) these patients are suffering from a particularly debilitating/ severe attack of migraine. Thunderclap/ explosive headaches have also been reported at the time of coitus. They are referred to as post coital or orgasmic headache.

  1. Headaches in extreme of ages:  headaches which occur in the extreme of ages (such as in a young child or after middle age especially if there is no past history of headaches) deserve a more closer look. Signs such as neck stiffness or depressed level of sensorium may be masked or difficult to elicit in these age groups. As a neurologist my threshold of investigating these patients with tests such as MRI of the brain and more invasive tests such as spinal tap (lumbar puncture) is lower.

Caveat: small children frequently cannot express headache (meaning they cannot tell you that they are bothered by headache).  Primary headaches such as migraine may be missed in children as a result. The child may be agitated and parents may think he is acting up. So a high index of suspicion has to maintained by the physician to timely identify migraines in small children. Also it is well known that migraine is not “typical” in the pediatric age group. Various migraine variants have been identified in children such as abdominal migraine (the child complains of episodic abdominal pain), cyclical vomiting and so forth.

  1. Onset of headache in middle age (especially if there is no prior history of headaches): when a middle aged patient comes to me and says he is bothered by headaches for the past 3-4 months, never had headaches when he was in his teens, I pay close attention to him. A thorough neurological examination shall frequently tell me if there is anything “brewing” inside the brain aka does he have a mass lesion/ tumor? As a neurologist I have several tools to help me in this endeavor. I can look for signs of asymmetry: weakness/ numbness on one side of the body, problems with coordination on one side of the body, is the gait steady, are the reflexes symmetrical and so forth. I can further look into his eyes with an ophthalmoscope to see if there is increased pressure inside the brain (remember the eyes are the window into the brain).  Based on my exam, I may or may not order a MRI scan or CT scan to confirm my suspicion.
  2. Headaches associated with fever: are always a cause of concern. Various disease processes such as meningitis (bacterial, viral or fungal), brain abscesses and so forth come into the differential. Patients are admitted into the hospital and further management depends upon the underlying etiology.
  3. Recent onset of headache in an immunocompromised patient: recent onset of headache in a patient who is immunocompromised (example a HIV positive patient or a patient who is receiving chemotherapy for cancer) always warrant a thorough work-up. Such patients are frequently found to have a secondary cause for their headaches. So again my threshold for imaging such patients is low.  My threshold for doing a spinal tap in these patients is also low (provided I am confident that the pressure in the brain is normal).
  4. Headache/ temporal pain  in a patient past 65: temporal arteritis (Giant cell arteritis) is a disease entity which is seen in the elderly and is due to inflammation of the temporal arteries. Patients may not complain of headache per-se. When you examine them you find they have tenderness over the temporal arteries and the artery may feel beaded and thickened.  It is imperative that temporal arteritis be identified in a timely fashion and treated otherwise the patient may suffer loss of vision.

In the next post I shall talk about the primary headaches.

Brain tumors: meningioma

Let us talk here about a relatively common brain tumor called meningioma. I shall try to keep this simple yet cover some important aspects. Meningiomas are brain tumors which do not arise from the cells of the brain (as against glioms which arise from glial cells and other tumors which arise from neural cells). As they do not arise from “brain” cells, they actually are extra-axial in location. By that I mean, they are located outside the brain but inside the skull. So meningiomas do not actually “invade” the brain, on the other hand as they grow in size they press on the brain from outside inwards.

This is how meningiomas cause their effects. Depending upon which location the tumor is, as it grows in size it exerts pressure on surrounding structures. Pressure on the surface of the brain may cause seizures (so many patients may present with seizures and when a MRI scan is done the tumor is found), if they are near the optic nerve or tracts patient may present with slowly progressive loss of vision, if near the motor tracts with weakness in the arm and leg, if near the cerebellum with gait and balance problems.

Meningiomas are slow growing tumors and as I stated earlier they usually do not invade the brain (though they may be locally invasive at times and these tumors are called atypical or malignant meningiomas). As these are slow growing, if they are small in size and discovered accidently (as in you went for a MRI for some other reason and a meningioma is found but is not the cause of your symptoms), your doctor may decide not to do anything and just wait and watch and follow you with serial MRI scans. Frequently patients outlive their tumors and die of natural causes without the tumor ever becoming symptomatic.  If for some reason it starts increasing in size and becomes symptomatic then a surgical option can be explored.

So now that we know something about these tumors, we can discuss how to treat them. The treatment option pursued depends upon the size and location of the tumor. If the tumor is the right size and in a surgically accessible location, then it is easy take it out surgically if it is symptomatic. However if the tumor is symptomatic but in a surgically inaccessible location like near the optic nerves then other options like sterotactic radiotherapy may be tried. The management decisions need expert opinion and hence one should consult a specialist.

Personal Regards,

Nitin Sethi, MD

Awake craniotomy during brain tumor surgery

As many of you may have read by now Senator Kennedy was awake at the time of his surgery to remove the malignant tumor from his brain. What does this mean? What is awake craniotomy (been awake during the time the cranium/ skull is open?)

I thought in this post I shall discuss awake craniotomy. As you know during most surgeries, patients are under deep general anaesthesia. We try to reach a plane of anaesthesia so that they feel no pain and also have no recollection of any events during the surgery. Sometimes though especially in brain surgeries we actually want them to be awake during at least a part of the surgery.

Why you may ask. Well take the case of Senator Kennedy, his tumor was in a location which is very near to eloquent areas of the brain (parts of the brain which control language/ memory/ movements of the opposite arm and leg that is his tumor was near the language and motor cortex). So in cases like these the surgeon wants you awake during a part of the procedure so that he can test that these functions are indeed intact. The patient is asked to speak, talk aloud or move the opposite arm or leg). This assures the surgeon that he is not near these vital areas of the brain and once he maps them out he can avoid them.

At times we do what is called cortical mapping. With the help of an electric probe, the surgeon touches parts of the brain near the region of interest (in this case a brain tumor), if when the probe is touched the patient has a language arrest or his hand or leg move, we know these are vitals parts of the brain. In this way we are able to map all the parts of the brain hence the name cortical mapping.

Awake craniotomy requires special type of anaesthesia and preparation. At times what is done is that the anaesthesia is turned off after the surgeon has cut open the skull and then the patient is woken up. Once the mapping has been carried out, the anaesthesia is restarted and the patient falls back asleep and then the surgeon continues with the surgery. Awake craniotomy is also carried out during deep brain stimulation (DBS) surgery for Parkinson’s disease.

I shall discuss DBS in a separate post.

 

Personal Regards,

Nitin Sethi, MD

 

 

Brain Tumors

Let us now turn our attention to brain tumors. My aim here shall be to discuss how brain tumors present in different age-groups, the most common type of tumors in different age groups, how brain tumors are diagnosed ( what work-up doctors do in the hospital) and finally touch on the management of different types of brain tumors.

Brain tumors can present in any age-group right from the neonatal and pediatric age-groups till the adult ages. Different types of brain tumors are seen in different age-groups.

First what exactly is a brain tumor?  A tumor refers to a group of cells which can multiply and proliferate relentlessly. Normally the growth of cells in the body occurs in a controlled and programmed fashion. At times for reason which are still been elucidated this control is lost and a group of cells in a tissue/organ may start to multiply in a haphazard fashion. When this occurs in the brain it leads to a brain tumor. Different types of brain tumors may arise depending upon which cell in the brain starts to multiply. Tumors can arise from neurons in the brain, the cells of the lining of the brain (meninges) and from the supporting cells in the brain (glial cells),

Each of these tumor type has its own natural history meaning the age when it presents (pediatric Vs adult), where it arises in the brain (location) and its aggressiveness ( how quickly it grows in the brain, how much does it spread, how much it invades the surrounding healthy brain tissue). As you can now imagine each of these tumors is thus treated in its own unique way–some with anti-tumor (chemotherapy) drugs, some solely by surgery and others by radiation therapy ( not all tumors are sensitive to radiation). Some require a combination of the above mentioned modalities: surgery, chemotherapy and radiotherapy.

Tumors are also classified in another way: benign Vs malignant. What does it mean when your doctor tells you “ you have a benign brain tumor”.  When we as doctors refer to a tumor as benign we mean that its natural history is one of slow growth (the tumor may either never increase in size or may grow only over years), its invasive potential is very low ( the tumor does not invade into surrounding healthy brain tissue) and it is not aggressive. Benign brain tumors like meningiomas may not warrant any treatment, they may be discovered incidentally when you have an imaging study (MRI or CT scan) for some reason and your doctor may decide not to do anything apart from keeping you under observation and periodically repeating  your imaging study.

There is a catch to this though. Even though your tumor may be benign it might be in a place (location in the brain) where it leads to some deficits. If it is near your brain stem and cerebellum it may press on your auditory nerve and lead to loss of hearing, some benign brain tumors can cause seizures. So the treatment has to be tailored to each individual case. Some benign brain tumors may warrant treatment.

Its Friday, lets take a short break. When I come back I shall discuss how tumors present ( what are the warning signs that someone has a brain tumor).

Personal Regards,

Dr. S

So lets now talk a little about how brain tumors present clinically. Brain tumors may present in a variety of ways. They may be completely silent and discovered only accidentally. Let me give you an example. Person is asymptomatic, he has a minor fall and doctor orders a CT scan or MRI and a brain tumor is found incidentally. Maybe the same tumor would have presented clinically a little while later after having enlarged in size.

Common ways that brain tumors present are as follows:

1) Headache: there is no particular pattern of headache which is specific for a brain tumor. No brain tumor is found in the majority of patients who are worked up for headaches. Most of the headaches are due to migraines, tension headaches and other common causes of headaches. But new onset headache in a middle aged person, headache which wakes one up from sleep or occurs in the morning on awakening, headache with nausea and projectile vomiting are warning signs that something might be brewing up. Also change in the character or frequency of headache in a person who has had headaches in the past makes doctor think about the possibility of brain tumor.

2) Seizures: frequently brain tumors may present with seizures. Thus whenever a person especially middle aged or elderly presents with an unprovoked seizure, a neuroimaging study is warranted to rule out the possibility of brain tumor.

3) Focal neurological signs: let me explain what I mean by that. New appearance of neurological signs like weakness or numbness in an arm or leg, unsteadiness of gait, loss of vision in one part of the visual field, difficulty with speech or weakness of one side of face all warrant work up for the possibility of brain tumor under the correct circumstances.

4) A brain tumor may be discovered as a work up of malignancy somewhere else in the body. Let me explain. Someone has lung cancer, as a work up a MRI scan of the brain is done and metastatic spread of cancer is noted to the brain (metastatic tumor: the tumor did not arise in the brain, rather spread to the brain from the primary source, in the above example it is the lung).

Next we shall turn our attention to the different kinds of brain tumors.

Personal Regards,

Dr. S

Lets now talk about the different kinds of brain tumors. As I stated earlier different kinds of tumors can arise in the brain ( depending upon the cell of origin). The most common primary brain tumors are those that arise from t glial cells. These tumors include astrocytomas.

Astrocytomas are graded as per a classification developed by the World Heath Organization (WHO). On the basis of the WHO classificiation these tumors are graded from I to IV.

Grade I and II astrocytomas are also called low grade astrocytomas ( what do you mean by low grade? Well the tumor is not so malignant, it lacks the invasive character of a high grade tumor, grows more slowly and increases in size over  years)

 

Grade III and IV astrocytomas are also called high grade astrocytomas. (these tumors are more malignant, invasive, increase in size over months and thus life expectancy is shorter)

 

Grade IV astrocytoma is more commonly referred to as Glioblastoma multiforme or rather as GBM. This tumor has a poor prognosis and a short life expectancy.

There are other types of brain tumors. I shall list a few of them out here and comment on them as we go along.

1) Meningiomas

2) Acoustic Neuromas/ vestibular schwannoma

3) Ependymomas

4) Oligodendrogliomas

5) Optic nerve gliomas

6) Craniopharyngiomas

7) Germ cell tumors.

8) Pineal glad tumors

9) Pituitary gland tumor-pituitary microadenoma and macroadenomas

10) Medulloblastoma

11) Neuroblastoma

12) central neurocytoma

13) Ganglioglioma

14) Retinoblastoma

Next we shall go over some aspects of diagnosis and management of brain tumors.

I hope you all are enjoying this Saturday morning.

Personal Regards,

Dr. S

Let us now discuss a few aspects regarding the management of brain tumors. First what are the tests usually carried out to detect and diagnose a brain tumor? As you can imagine the most commonly done tests are the ones that involve imaging the bain. Either a CT scan or an MRI brain is done. Usually during these tests a dye (also called contrast) is given. The dye/ contrast helps to highlight the tumor from the surrounding background and aids in visualization of the extent of its spread and the surrounding brain edema. Some tumors enhance, that is they take up the dye and look bright while others do not enhance.

If we are dealing with a primary brain tumor, by that I mean a tumor which arises from the brain itself and stays in the brain (does not spread) then nothing else may be needed. But if we are dealing with a metastatic brain tumor (for example a lung tumor which has spread to the brain), more imaging may need to be done. This includes imaging the chest, abdomen and pelvis to look for how far the tumor has spread.

Sometimes tests may be more invasive. The CT scan or MRI brain only shows the mass lesion, it does not tell what kind of tumor it is histopathologically. Remember not all masses in the brain  are tumors, infections too can present with mass lesions (brain abscesses). For example a patient is noted to have a mass/ lesion on MRI brain. The doctor cannot tell what it is and may like to get a biopsy of the lesion. Biopsy means getting a small amount of the tissue and looking under the microscope to identify what it is. This procedure is carried out in the OR by a neurosurgeon.

So to summarize some of the investigations which may be carried out during the work-up of a brain lesion include:

1) blood tests

2) Chest X-ray

3) EKG (electrocardiogram)

4) CT scan of the brain with and without contrast

5) MRI of the brain with and without contrast.

6) EEG (electroencephalogram): remember some patients present with seizures and are then detected to have a brain tumor on testing.

7) CT scan of the chest, abdomen and pelvis.

 

Treatment of brain tumors: the treatment of brain tumors is complex and frequently involves multiple specialities like neurology, neurosurgery, neuro-oncology and radiation oncology. Simply put the treatment depends upon a number of factors. These are:

1) Location of the tumor in the brain–is it easily accessible surgically or is it deep in the brain and cannot be approached surgically?

2) Size of the brain tumor–is it small or is it large? Can it be safely removed without causing weakness, loss of memory or speech problems?

3) Number of tumors in the brain–a solitary lesion usually can be surgically removed while multiple lesions cannot.

4) Type of the brain tumor: as I stated earlier different tumors behave in different ways. Some are slow growing while others grow quite fast. Some are sensitive to radiation while others are not. Some respond to one type of chemotherapy (cancer drugs) while others do not.

 

The different modalities used in the treatment of brain tumors are:

 

1) Surgery–the neurosurgeon resects the tumor. For some tumors especially those caught early, this may be all that is needed. In others after surgery you may need radiation to the brain and/or chemotherapy.

 

2) Radiation–this may either be whole brain radiation (meaning that the whole brain is radiated) or radiation just to the tumor and the surrounding areas. While radiation involves no surgery, it too carries its own side-effects. Radiation may cause cognitive deficits and at times necrosis of the surrounding brain tissue.

 

3) Chemotherapy: different types of chemotherapy drugs are used in the treatment of brain tumors. Some of these are a group of drugs called nitrosoureas with names like BCNU and CCNU.

 

Other aspects of care include rehablitation–this may include physical therapy, occupational therapy and speech therapy.