Trigeminal neuralgia awareness

Despite the striking strength of this pain, TGN isn’t particularly well known. Most people never hear of it until they or a relative develop it. 

Sometimes the pain comes out of nowhere with no trigger at all. While a classic attack is sudden and sharp and then gone altogether, sometimes a low-grade ache or burning pain will persist in its wake for an hour or more. In some patients, the constant aching, burning pain is their initial complaint.

It was a warm October morning and I was in a merry mood having a good time because of the approaching Durga Pujas. I always try to make most of the time with my mother. The season is very inviting and makes us happy and less anxious. I sat down with a book determined to enjoy it thoroughly but suddenly out of the blue something zapped across my right eye. The jolt of lightening appeared repeatedly. It lasted for a few seconds to a few minutes but it was difficult to keep my right eye open. This continued for the next few days but the pain was gone as abruptly as it appeared. I went to the neuro-ophthalmologist thinking about my optic nerve tumour but he said optic nerve doesn't cause pain and it looks like trigeminal neuralgia and I should visit my neurologist immediately. He did his examination and said it was TGN and asked for an MRI. I underwent an MRI scan the next day and it confirmed the diagnosis of trigeminal neuralgia...“ Thin vascular loop of superior cerebellar artery (SCA) abutting cranial aspect of the right trigeminal nerve at the root entry zone is noted.”

Contorted faces of men suffering from agonizing facial pain have been recorded historically. Greek and Roman physicians, who called it simply cephalalgia, meaning head pain. Persian physician, Avicenna, described it 900 years ago and even suggested that it might have something to do with an artery being near a nerve.

Branches of Trigeminal nerve

The mandibular branch( bottom) of the trigeminal nerve serves the 

• the lower jaw, including the lower teeth, lower lip, side and front of the tongue, lower gums.

• part of the ear. 

The middle or maxillary branch serves the 

• the upper jaw, upper teeth, and gums, upper lip,

• cheeks

•  palate

•  sinuses

•  temples, and most of the nose.

The upper or ophthalmic branch serves 

• the forehead, eyes

• bridge of the nose.

There can be some overlap, especially in the eye itself.

The trigeminal nerves are responsible for the sensations of touch, temperature, and pain in most of the face. A separate branch of the trigeminal nerve also controls the muscles used in chewing.

The three main nerves come together in a ganglion (Greek ganglion ‘ tumour on or near sinews or tendons,’). A ganglion is a collection of specialized cells linked by connection points [synapses] forming a swelling on a nerve fibre. This is the first neuron (primary neuron) in a pathway whose activity will eventually result in your awareness of being touched.

The nerve fibres and the nerve itself are sheathed by a fatty, protective substance called myelin.

The trigeminal nerves are responsible for almost all sensations from the forehead to the lower jaw, including heat, cold, pressure, touch and, of course, pain. The right trigeminal nerve serves the entire right side of the face; the left one, the left side.

It is also known as"suicide disease” as a result of those who killed themselves to escape the pain.

One or both of the trigeminal nerves are misfiring, sending pain signals when they should not. Most of the time it is one trigeminal nerve, so it is one-sided. It is the loss of or damage to the nerve's protective coating, the myelin, is somehow related to the problem.

The myelin damage results from a chronic irritation of the nerve usually a blood vessel compressing it in at least the first 4 millimetres of the trigeminal nerve where it connects to the pons (part of the brain stem).

It's like in TGN  what happens is when a plugged-in electrical wire loses its insulation: When you touch with bare hands, they spark, short-circuit, and the wire stops working as it should. In TGN, the damaged nerve fibres are like bare wires and light touch is the “movement” of the wire that sets off sparking and the short-circuiting.

Genetic changes may also play a role in the susceptibility of the nerve to react to mechanical pressure (vessels, tumours, etc.).

Some doctors and nutritionists have proposed that lack of Vitamin B-12 and possibly other nutritional defects prevent the body from building the myelin.

1. Trigeminal Neuralgia Type 1 (TGN-1) has the sharp, shooting jolts of pain, often occurring in response to some triggering event (wind, touch). 

2. Trigeminal neuralgia Type 2 (TGN-2) refers to idiopathic, spontaneous facial pain that is predominantly constant and can be aching or burning in nature. 

3. Trigeminal neuropathic pain (TGN-3) results from unintentional injury to the trigeminal nerve from trauma or surgery.

4. Trigeminal deafferentation pain (TGN-4) results from intentional injury to the nerve.

5. Symptomatic TGN (TGN-5) results from multiple sclerosis 

6. Postherpetic TGN (TGN-6) follows a cutaneous herpes zoster outbreak in the trigeminal distribution.    

7. Atypical facial pain (TGN-7) is reserved for facial pain secondary to a non-organic process.

Pharmaceutical science has medications to control the pain without any invasive or non-invasive treatment.

The first drug used was phenytoin, a more effective drug—carbamazepine (Tegretol)—was introduced next and even now it remains doctors' top choice. Oxacarbamazepine has similar effectiveness, and patients report they seem to tolerate it better. There are a few add on drugs like Baclofen,  Gabapentin, Lamotrigine, topiramate, levetiracetam. There is also Duolexitine and Pregabalin.

I was initially put on medicine but it mostly didn’t contain the pain. I had fleeting jabbing pain which would fade away at the most in a few hours. Pulses of electricity travelled through my cheeks. Doctors upped and upped my dosage but I was not satisfied.

I had severe side-effects. I went somewhere else for consultation on less invasive procedures like damaging the nerve fibres with a heated electrode known as Radio-frequency Lesioning where an electrode is inserted through the foramen ( hollow or opening inside the skull) or Gamma- knife where beams of cobalt 60 radiation are used to damage the nerve. This was because the treating doctors were telling the haunting medical lore the melancholia of which was that Microvascular decompression (MVD) surgery is the only hope and while doing so they'd do a biopsy of my leptomeningeal hemangioblastomas not caring about the drastic and dramatic effect, not considering the highly vascular nature of the tumours ---that they are richly supplied with blood vessels. A biopsy would make them bleed resulting in a stroke. After my first brain tumour surgery, done in 2006 I woke up with paralysis on the left side. After the craniotomy ( open brain surgery) I experienced total numbness in my arms and hands and I couldn't close my fingers and make a fist. But this was sorted out by proper physiotherapy and exercise and I got back the control of my limbs but even now I don't have sensations in my fingertips. Looking at tumour cells in the cerebrospinal fluid under high-resolution MRI scans are all that is necessary for an accurate diagnosis moreover they are confirmed by Ga-Dotanoc PET scan. A biopsy isn't required for diagnosis as it may cause meningitis and blood loss thereby cell spillage.

There it was confirmed by the doctor that the left-hand side pain which is milder than the right-hand side is also due to trigeminal neuralgia. Thus I have a bilateral manifestation of trigeminal neuralgia. The trigeminal nerve has three branches: ophthalmic, maxillary, and mandibular. According to the doctor it is rarely seen that bilateral trigeminal neuralgia with pain in all three branches on both sides.

I revisited the neurologist who previously diagnosed it. A new medicine was added to my list of medicines. I asked him if it's true I had bilateral trigeminal neuralgia...he gave a knowing wink and said but the pain on the left side is milder...why should he hide it from me? Who is he to decide the intensity of my pain? Mistaking the pain in the jaw for dental pain I sought the help of a dentist. Because of my low immunity, I acquired some infection and got a 105F fever. I wouldn't have suffered if he hadn't held back the truth from me.

Bilateral TGN—the rare incidence of having TGN on both sides of the face—seems to be more common in patients who have a family history of TGN. That occurs in 12-20% of face pain patients. This is in line with the preliminary genetic studies reported thus far. They indicate differences in the pain genes, which may explain why the pain can be so severe.

About 13 per cent of patients have pain in all three branches. Many patients also report having their worst episodes during stressful periods in their life and I have been leading an unnaturally stressful life since my father died after my liver transplant.

Mostly, even now I scream and awaken in pain when I turn onto the right side, the most painful side of my face. Now I just feel a dull pain in the jaw and eye.

I never knew how to cry because I was born without the vital emotion fear but when Trigeminal Neuralgia attacked me I learned to cry as the spontaneous facial pain that is predominantly constant and can be aching or burning in nature made it a tough battle for me every day.

My heart stopped jumping with joy, enjoying the elixir of life, and as I learned to shed tears of wicked pain hugging my mother at night when the soul screamed out with the agony and howling in pain. I hugged my mother and muttered and cried as pain then comes through loud and clear.

Because the length of time a blood vessel has been compressing the nerve may be the most significant concern. Blood vessels that beat on nerves year after year may cause a chronic injury that not only changes the nature of a person's pain but also makes it harder to treat. In that sense, largely, TGN is a progressive disorder.

Nerve injuries also may interfere with the brain's ability to send stop-pain signals. So once an attack begins, it may not stop until the nerve has used up its supply of ions biochemicals and is physically incapable of firing anymore and threshold levels that we know of vary from person to person and from time to time. That may explain why one person with a compressing blood vessel ends up with terrible TGN pain while another person with a similar compression does not. There is a role for the genes to play here as well.

All my life I have kept looking for hope. I have undergone countless surgeries including cancer and a few life-threatening ones but I have never stopped hoping. Hope sustains us. I got this hope from Mumbai who said he could definitely treat me by cyberknife radiosurgery. In a Cyberknife instead of Cobalt-60, a 6 MeV linear accelerator is mounted on a robotic arm that moves around the patient and fires radiation from different angles. The patient is immobilised but does not require a frame to be screwed to the head.

Cluster tic syndrome 

Cluster tic syndrome is a name given to the coexistence of two different pains at the same time namely cluster headaches and TN (tic douloureux). Fortunately, the combination is very rare. This can be very difficult to diagnose because it has both the sharp, stabbing, come-and-go pains of TN and the more searing, boring traits of cluster headaches. 

Treatment may require a combination of the therapies used to treat stand-alone cases of TN and cluster headaches. In the long run, the cluster-headache component may resolve itself, while the TN pain can be addressed by surgery or other methods if anticonvulsant medications fail. In several patients who underwent surgery for cluster tic syndrome, blood vessels were found to be compressing either the trigeminal nerve, the facial nerve, or both.

In the MRI, before the planning, it was found ' A thin loop of anterior inferior cerebellar artery is seen overlying the 7th /8th nerve complex. However, no obvious compression or displacement is seen. The 7th/8th nerve complex is unremarkable.

It's 5 months after the cyberknife but I have new problems even though I have partially achieved the "good numbness” as was told by the doctor is needed to get “good relief.”A strange burning sensation on my tongue on that side. A weird pain creeps outside the trigeminal nerve's territory, maybe into the back of the head, the neck, a bump appearing in the anterior part of the tongue, hearing metallic noises, and unable to swallow pills or having coughing fits while eating? I have chosen mostly nutritious but liquid food or puree but what to do with my essential pills and the bump?

Genetic aspect:

Autosomal Dominant Vascular Disorders

Since familial TGN appears to be AD (autosomal dominant) and may be related to patterns of vascularity, known AD vascular disorders may help guide the search for a genetic locus for familial TGN. Moreover, familial TGN appears with other cranial nerve dysfunctions including those of the seventh and eighth cranial nerves. Autosomal dominant disorders with primary or secondary vascular involvement include hereditary hemorrhagic telangiectasia ([HHT] former eponym, Osler-Weber-Rendu disease), neurofibromatosis type 1 (NF1), cerebral AD arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), von Hippel-Lindau disease (VHL), polycystic kidney disease (PKD), several ophthalmologic syndromes,

and a variety of vascular malformations including cavernous angioma and venous and arteriovenous malformations.

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Familial occurrence of trigeminal neuralgia is more common than previously considered. Although our results demonstrate variants in genes encoding voltage-gated ion channels and transient receptor potential channels within these patients, further study will be needed to determine their roles in the pathogenesis of trigeminal neuralgia.