Another disorder similar to both cluster headache and trigeminal neuralgia is Raeder's paratrigeminal neuralgia. y This syndrome is characterized by intense pain in the distribution of V1, lacrimation, conjunctival injection, rhinorrhea, and ipsilateral pupillary mydriasis (Horner's syndrome). This syndrome may be idiopathic or may result from pathology affecting the ipsilateral carotid artery or structures within the middle cranial fossa. In the idiopathic form, patients tend to be middle-aged or elderly males who develop a severe throbbing orbital or retro-orbital headache upon awakening. Additional signs such as lacrimation, conjunctival injection, and oculosympathetic dysfunction develop later. Therapy includes ergotamine tartrate, methysergide, and systemic corticosteroids. In both cluster headache and Raeder's syndrome, the pain in V1, duration of attacks, and associated features help distinguish the condition from trigeminal neuralgia. Raeder's paratrigeminal neuralgia may not actually represent a distinct clinical entity because those patients with an isolated Horner's syndrome likely have cluster headaches, whereas those with ocular motility impairment have a cavernous sinus process until proven otherwise. Furthermore, it is important to consider the possibility of a carotid dissection in a patient who presents with an isolated painful Horner's syndrome.
The skull base and exit points of V1 to V3 (i.e., at the superior orbital fissure, foramen ovale, or foramen rotundum) can be diseased and can result in focal sensory or sensorimotor trigeminal dysfunction. For example, acute or resolved bacterial, tuberculous, carcinomatous, or granulomatous
(sarcoid) meningitis can result in inflammation, infiltration, or congestion of the basal meninges through which V1 to V3 nerve roots exit the skull. In Paget's disease, narrowing of the skull foramina can also lead to cranial (trigeminal) neuropathy. If V3 is affected, motor and sensory manifestations may be identified (see Fig 10.-.5 )
in one or more branches of the trigeminal system.
Various pathological processes including tumors, aneurysms, infarctions, trauma, and infections can damage the ophthalmic division of the trigeminal nerve. y Superior orbital fissure involvement is characterized by numbness, paresthesias, or pain in the distribution of V1 and within the orbit, Horner's syndrome, and ophthalmoparesis. Involvement of the optic nerve suggests extension into the orbital apex. If, on funduscopic examination, there is evidence of venous congestion, cavernous sinus thrombosis is likely. Cavernous sinus thrombosis is almost always caused by spread of an infection from the face, nose, or mouth. Patients may initially complain of fever, malaise, and frontal headache, but they subsequently develop proptosis, ptosis, ophthalmoparesis, and vasocongestion. Initially the CSF test result may be normal, but findings characteristic of meningitis may occur if treatment is delayed. Mortality is linked to spread of bacteria to the meninges, which warrants early and intensive antibiotic therapy. In contrast, carotid-cavernous sinus arteriovenous fistulae may be congenital in children but in adults are more commonly the result of trauma. Carotid-cavernous sinus arteriovenous fistulae also occur in women during pregnancy or at childbirth. On examination, pulsating proptosis, conjunctival erythema, ophthalmoparesis, and a bruit over the globe may be found. Diagnosis by CT or angiography is usual, and treatment with neurosurgical or invasive radiological approaches is warranted.
In addition, there are several syndromes involving the peripheral trigeminal branches. Cluster headache appears as multiple attacks of severe head or facial pain and may be confused with trigeminal neuralgia. y Cluster headache typically occurs in middle-age men and is more prominent at night. Attacks are of short duration; they occur in clusters that recur with variable frequency. Unlike trigeminal neuralgia, trigger points are not a characteristic feature. The associated symptoms of cluster headache such as lacrimation, conjunctival injection, sweating, ipsilateral nasal blockage, miosis, and ptosis are quite distinct from symptoms of trigeminal neuralgia. Studies have suggested, however, that disruption of normal autoregulatory trigeminovascular innervation may be responsible for cluster headache. y
Crescendo orbital pain or frontal headache can herald impending internal carotid artery occlusion presumably from irritation or ischemia to peripheral trigeminal branches. Similarly, a cluster of symptoms including facial, orbital, or neck pain or facial paresthesias in association with an ipsilateral Horner's syndrome may reflect dissection of the cervical portion of the internal carotid artery. y These symptoms may also be prodromal. Excruciating pain in the supraorbital headache in association with a pupil involving third nerve palsy is almost pathognomonic for an intracranial (especially posterior communicating artery) aneurysm. Ipsilateral orbital or ocular pain has also been reported in association with posterior cerebral artery occlusion, which may reflect ischemic damage to regions of the tentorium adjacent to the occipital lobes that are innervated by V1.
Circumscribed facial paresthesias in V1 to V3 distributions have been identified in patients with diabetes mellitus, Guillain-Barre syndrome, hereditary sensory motor neuropathy I,y chronic inflammatory demyelinating polyneuropathy, nutritional deficiencies, and other peripheral neuropathological disorders. Vascular infarction of the nerve branches, for example, in vasculitis can also result in sensory loss. Compressive or infiltrative processes affecting any of the peripheral trigeminal branches result in focal, well circumscribed sensory loss or paresthesias (see numb chin syndrome, later). Dental trauma and exposure to various toxic substances such as trichloroethylene and trichloroacetic acid can also result in circumscribed facial sensory loss or paresthesias in the trigeminal distribution.
The temporomandibular joint (TMJ) syndrome refers to recurrent pain in the region of the jaw, ear, occiput, and supraorbital regions, which is believed to result from degeneration or malocclusion of the TMJ. Erosion of the bony surfaces within the glenoid fossa may cause irritation of several adjacent nerves including the auriculotemporal and chorda tympani trigeminal nerves. Patients may report an increase in pain in the evening and pain referred to the oropharynx. Rarely, a sensation of hearing dullness or auricular congestion may be noted. The TMJ syndrome may result from local trauma, neoplastic invasion, ankylosis, or inflammation, although some cases reflect nonorganic or ill-defined joint pain syndromes. On examination, the TMJ may appear lax and may be painful to passive motion or palpation. Correction of an underlying malocclusion may be curative, but other measures include analgesics, jaw exercises, soft diet, and tricyclic antidepressants. y
The numb chin syndrome (mental neuropathy) often reflects a bony lesion affecting the mental foramen through which V3 passes to innervate the chin and mandible.y , y Patients often report numbness or pain on one or both sides of the chin, which may extend to the lip or submandibular region. Frequent causes include granulomatous diseases such histiocytosis X; primary bony malignancies such as osteosarcoma, fibrosarcoma, and plasmacytoma; and metastatic lesions from lung, breast, and prostate carcinomay as well as lymphoma (especially Burkitt's lymphoma). Development of a numb chin in a patient with cancer in remission may indicate relapse. Nonmalignant etiologies include collagen vascular disorders, trauma, periodontal disease, benign bony cyst, focal idiopathic osteolysis (Gorham's disease),y and sickle cell disease. A variant of the numb chin syndrome is the numb cheek syndrome, which results from a bony lesion affecting the infraorbital foramen or the maxillary sinus and trigeminal branch V2. y On rare occasions, focal motor weakness affecting the masticatory muscles results from damage to motor branches of V3.
Evaluation of patients with focal sensory loss over the chin or malar region begins with a full, careful sensory examination to determine whether the sensory loss results from a distal or proximal trigeminal lesion. Specifically, mental neuropathy causes focal sensory loss over the lower lip and chin, whereas more proximal V3 dysfunction results in more widespread sensory disturbance and may be associated with dysfunction of other cranial nerves. Motor
involvement is characterized by ipsilateral jaw deviation, flaccidity of the floor of the mouth, and wasting of the ipsilateral temporalis muscle. Weakness of the tensor tympani results in difficulty detecting low-pitched sounds. Careful examination of the oropharynx, dentition, and skin over the chin should also be performed because erythema and edema may be present over a focal bony lesion. Plain radiographs of the mandible may be very useful in establishing the diagnosis because they may be revealing in about 50 percent of cases. If the result is abnormal, bone scan and biopsy of the lesion itself may be indicated. A search for occult malignancy employing CT of the chest and abdomen, prostate-specific antigen (in men), and bone marrow biopsy may be necessary. If radiographs are normal, evaluation of the brain and cranial nerves by MRI (with contrast) and lumbar puncture for cytology is indicated because a brain stem lesion or carcinomatous meningitis may cause focal trigeminal dysfunction.
Corneal hypesthesia and orbital pain may result from local corneal dystrophies or reflect damage to branches of V1, which innervate these structures. Viral (herpetic) infections, diabetes, leprosy, and vitamin A deficiency can result in unilateral or bilateral corneal hypesthesia. In addition, the pain associated with anterior uveitis, acute angle-closure glaucoma, and optic neuritis is mediated through V1 orbital sensory fibers. Clinically, a diminished corneal reflex may be detected. In the orbit, inflammatory conditions such as cellulitis, pseudotumor, or neoplasms (lymphoma, metastatic tumors) may present as orbital pain in association with ophthalmoparesis. Trigeminal involvement in orbital pseudotumor is uncommon. Infections within the orbit including those from bacterial and fungal pathogens such as Streptococcus and Mucor, respectively, can also result in a painful ophthalmoplegia. The Tolosa-Hunt syndrome (painful ophthalmoplegia) is characterized by steady, unremitting retro- and supraorbital pain (in the cutaneous V1 distribution) in association with paresis of nerves III, IV, and VI, and a diminished corneal reflex (for review, see reference 42). Sensory loss and pain in V2 distribution may also occur. Less frequently, the optic nerve and oculosympathetic fibers may be affected as well. Symptoms may persist for weeks to months. Pathologically, a low-grade, granulomatous, noninfectious, inflammatory process adjacent to the cavernous sinus or within the superior orbital fissure has been identified consisting of lymphocytes and plasma cells. The Tolosa-Hunt syndrome typically responds dramatically to systemic corticosteroids, although symptoms may recur over months to years. Spontaneous remissions have also been reported.
Other causes of decreased sensation within the globe and conjunctiva are orbital surgery or orbital trauma, which affect nasociliary and frontal branches of V1. In herpes zoster ophthalmicus (.Fig.; 10.16 ), inflammation and vesicular eruption involving all branches of V1 as well as small arterioles within the gasserian ganglion may result in excruciating, lancinating pain in the periorbital region. y Symptoms of herpes zoster ophthalmicus typically begin 2 to 3 days before the appearance of vesicles and may diminish after 2 to 3 weeks. Hypalgesia and paresthesias may be noted during and after lesions heal. Pain may persist after the rash is gone only to evolve into post-herpetic neuralgia. This syndrome consists of burning, lancinating, aching pain in the V1 territory often in association with paresthesias and hyperpathia. As in trigeminal neuralgia, trigger points can evoke pain in response to cutaneous stimuli. y , y
Atypical facial pain can also reflect trigeminal branch disorders. y , y Some patients experience persistent facial pain that is not confined to the distribution of V1 to V3
Figure 10-6 Acute (A) and resolving B) herpes zoster arrows). In A, there is selective involvement of the nasociliary branch of the trigeminal nerve.
and that differs in character from classic trigeminal neuralgia. Many of these atypical facial pain syndromes including Charlin's nasociliary neuralgia, Sluder's pterygopalatine ganglion syndrome, and Vail's vidian neuralgia, all involve portions of the trigeminal nerve. They are also characterized by numerous autonomic symptoms such as lacrimation, conjunctival injection, altered sweating, salivation, facial flushing, and nasal congestion, which are believed to result from involvement of the autonomic ganglia (ciliary, pterygopalatine) in the face. These atypical facial neuralgias are additionally characterized by nondermatomal localization of pain; bilateral symptoms; continual instead of paroxysmal pain; lack of clear trigger zones; and deep, poorly localized pain. y Appropriate therapy for these debilitating and often refractory disorders is often unsatisfactory and has consisted of surgical ablation of peripheral pain fibers, peripheral or sympathetic nerve blockade, transcutaneous electrical stimulation, tricyclic antidepressants, and narcotic and non-narcotic analgesics.
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