Trigeminal Neuralgia Neuropathic disorder characterized by episodes of intense pain in the face originating from the trigeminal nerve.

Also known as tic douloureux (painful tic), the Suicide disease and Fothergills disease - One of the most painful conditions known - 4-13 per 100,000 (15,000 new cases per year) - usually occurs after the age of 50 (has been seen in patient as young as 3) - All three of the trigeminal nerve branches can be affected (ophthalmic, maxillary, or mandibular) - 10-12% can be bilateral History: - first full and accurate description written in 1773 by John Fothergill (recorded symptoms seen as early as the 200 AD (Aretaeus of Cappodocia, pupil of Hippocrates), with the 11th century physician Jajuni postulating that the cause may be the proximity of the artery to the nerve, though debate still remains if this was a mistranslation.) - Wells cathedral holds the tomb of Bishop Button (died in 1274) that was a popular pilgrimage for people who suffered from toothaches, now theorized to have actually been facial neuralgias due to the relative rarity of toothaches at the time. The site was so famous for attracting this particular type of pilgrim that the pillars of the cathedral bear carvings of people suffering from facial neuralgias. - Reports of cases so severe that resulted in people being unable to eat or swallow for fear of pain, some succumbing to malnutrition or committing suicide - Phenytoin was first used to treat trigeminal neuralgia in 1942 Sx: intense facial pain that lasts from seconds to hours, occurring paroxysmally The areas can be so sensitive, even air currents can trigger reactions (look for the area that is no shaved or purposely concealed Reports of stabbing electric shocks, burning, pressing, crushing, exploding or shooting pain the becomes intractable Worsen in frequency or severity over time, usually developing pain in one branch Some report pain specific to the waking hours (pillow contact?) Atypical trigeminal neuralgia Severe, relentless underlying pain similar to a migraine in addition to shock-like pain Sometimes the pain is a combination of shock-like sensations, migraine-like pain, and burning or prickling pain (even unrelenting boring, piercing pain) Differential Diagnosis: - Cavernous sinus syndrome - Cerebral aneurysms - Cluster headache Causes: - Previous theory believed etiology was due to nerve compression - New research indicates an enlarged blood vessel (possibly superior cerebellar artery) compressing or throbbing against the microvasculature of the trigeminal nerve near its connection with the pons - Such a compression can injure the nerves protective myelin sheath and cause erratic hyperactive functioning of the nerve - Lowers threshold for nerve activation and impairs nerves ability to shut off pain signals after the simulation ends - Similar types of injury may occur with aneurysm, tumor, arachniod cyst in the cerebellopontine angle or traumatic event (even tongue piercing) - Correlation with MS: large portion of individuals with MS have TN (but not everyone with TN also has MS)

Hemifacial spasm Hydrocephalus Intracranial hemorrhage

Migraine headache Multiple sclerosis Postherpetic neuralgia SAH

- ophthalmic division of the trigeminal nerve innervates the proximal portions of large intracranial vessels, and the meninges in the supratentorial region. Consequently, painful stimuli in these structures are referred to the eye or retro-orbital area that are innervated by V1 - rostral cervical roots innervate structures in the posterior fossa, so that noxious stimuli in this region are experienced as coming from the neck or posterior base of the skull

Diagnosis: y Paroxysmal attacks of pain lasting from a fraction of a second to two minutes, affecting one or more divisions of the trigeminal nerve y Pain has at least one of the following characteristics: o o y y y y y Intense, sharp, superficial or stabbing Precipitated from trigger areas or trigger factors

Attacks are stereotyped in the individual patient There is no clinically evidence neurologic deficit Not attributed to another disorder Intense, sharp, superficial, or stabbing Precipitated from trigger areas or by trigger factors

Management: Carbamazepine (most effective) (sodium Oxcarbazepine Baclofen Lamotrigine Pimozide Surgical therapy (ablative procedures) channel blocker)

Headache of SAH - Abrupt headache with altered mentation (seen in 50%) Spectrum of Presentation: - warming headache (20-50%) o distinct, unusually severe headache in the days or weeks before the index episode of bleeding - thunderclap, the worse headache of my life (develops in seconds, last hours to days) o altered consciousness with confusion, buckling of legs with vomiting soon to follow o physical exam can show: o retinal hemorrhage, nunchal rigidity, restlessness, a diminished level of consciousness and focal neurologic signs o sparing the soma but devastating the psyche = absence of focal signs o less common signs:  CN III (posterior communicating artery)  CN VI palsy (posterior fossa)  nystagmus or ataxia (posterior fossa)  bilateral weakness in legs or abulia (lack of initiative or will) (anterior communicating artery)  aphasia, hemiparesis, or left-sided visual neglect (middle cerebral artery) Chemical meningitis o Hemorrhage into the subarachnoid space produces irritation of the meninges and of the structures traversing the subarachnoid space with resultant:  Headache  Stiff neck  meningeal stretch signs  Possible cranial nerve palsies (II or VI with double vision) o Pia mater prevents direct extension of the blood into the brain parenchyma the thus prevents focal neurological signs Risk factors: - cigarette smoking - Heritable connective-tissue diseases - hypertension o Ehlers-Danlos syndrome (type - alcohol consumption (especially after a IV) recent binge o Pseudoxanthoma elasticum - personal or family history of SAH (fragmentation and - polycystic kidney disease mineralization of elastic fibers) - Sickle cell anemia o Fibromuscular dysplasia - Alpha-antitrypsin deficiency (narrowing of arteries) Diagnosis: CT first (just to rule out mass) Gold-standard is blood LP tap Angiogram to follow Do as little as possible to make the diagnosis