eMedicine Specialties > Neurology > Headache and Pain

Raeder Paratrigeminal Syndrome: Treatment & Medication

Author: Steven H Schechter, MD, Clinical Assistant Professor, Department of Neurology, Wayne State University School of Medicine; Consulting Staff, Division of Neurology, Assistant Medical Director of Stroke Unit, William Beaumont Hospital
Contributor Information and Disclosures

Updated: Sep 11, 2008

Treatment

Medical Care

  • Unless intracranial pathology exists, treatment of Raeder paratrigeminal syndrome remains predominantly symptomatic.
  • Avoidance of vasodilators and alcohol is recommended due to potential for exacerbation of pain.
  • When parasellar involvement is absent, steroids (either oral or intravenous) may be beneficial.
  • Nolph and Dion also have suggested analgesics, ergotamines, and vitamin B therapy.3

Surgical Care

Surgery is not indicated for most patients unless a secondary cause is found that justifies surgical intervention.

Medication

For management of pain associated with Raeder paratrigeminal syndrome, gabapentin, pregabalin, carbamazepine, other anticonvulsants, or baclofen may be effective. Anti-inflammatory agents can also be effective, and, at times, narcotic analgesics may be necessary. The efficacy of TCAs has been demonstrated in controlled trials for idiopathic facial pain and appears to be independent of the antidepressant effect. Steroids may also be effective in some patients.

Anticonvulsants

Many agents in this class are effective in the treatment of chronic pain syndromes.


Gabapentin (Neurontin)

Effective in treating neuralgia and chronic pain, likely due to immunomodulatory effect. Structurally related to GABA but does not interact with GABA receptors. Not converted metabolically into GABA or GABA agonist. Not an inhibitor of GABA uptake or degradation. Does not exhibit affinity for other common receptor sites.

Adult

100-300 mg PO tid initial; titrate dose pending clinical response; not to exceed 3600 mg

Pediatric

<12 years: Not established
>12 years: Administer as in adults

Antacids significantly may reduce bioavailability (administer at least 2 h following antacids); may increase norethindrone levels significantly

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Adjust dose in renal failure; adverse effects include dizziness, somnolence, ataxia, tremor, and GI upset


Carbamazepine (Tegretol, Carbatrol, Epitol)

DOC that may reduce polysynaptic responses and block posttetanic potentiation.
May depress activity of nucleus ventralis of thalamus or decrease synaptic transmission or summation of temporal stimulation, leading to neural discharge by limiting influx of sodium ions across cell membrane or other unknown mechanisms.
Target blood serum concentration is 4-12 mg/L.

Adult

Initial dose: 200 mg PO bid; increase dose gradually prn over 2-wk interval to 200 mg PO tid
Sustained-release form: Administer therapeutic dose bid

Pediatric

<6 years: 10-20 mg/kg/d PO initially; titrate prn
6-12 years: 100 mg PO bid initially; titrate prn
>12 years: 200 mg PO bid; titrate prn

Serum levels may increase significantly within 30 d of danazol coadministration (avoid whenever possible); do not coadminister with MAOIs; cimetidine may increase toxicity, especially if taken in first 4 wk of therapy; carbamazepine may decrease primidone and phenobarbital levels (coadministration may increase carbamazepine levels)

Documented hypersensitivity; bone marrow suppression; MAOIs

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

MAOIs should be discontinued for a minimum of 14 d before starting carbamazepine; do not use to relieve minor aches or pains; caution with increased intraocular pressure; obtain CBC counts and serum iron baseline prior to treatment, during first 2 mo, and yearly or every other year thereafter; can cause drowsiness, dizziness, and blurred vision; caution while driving or performing other tasks requiring alertness


Pregabalin (Lyrica)

Similar to gabapentin but faster clinical effect without the slow titration. Reduces calcium-dependent release of several neurotransmitters, possibly by modulation of calcium channel function. Twice-daily dosing may improve compliance.

Adult

75 mg PO bid initially; titrate to clinical efficacy, not to exceed 600 mg/d

Pediatric

Not established

May cause additive effects on cognitive and gross motor functioning when coadministered with drugs that cause dizziness or somnolence

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Discontinue gradually (over a minimum of 1 wk) to minimize increased seizure frequency in patients with seizure disorders; may cause insomnia, nausea, headache, or diarrhea with abrupt withdrawal; common adverse effects include dizziness, somnolence, blurred vision, weight gain, and peripheral edema; may elevate creatinine kinase level, decrease platelet count, and increase PR interval; doses >300 mg/d associated with higher rate of adverse effects and treatment discontinuation; decrease dose with renal impairment (ie, CrCl <60 mL/min)

Analgesics

Pain control is essential to quality patient care. Ensure patient comfort, promote pulmonary toilet, and have sedating properties, which are beneficial for patients who have sustained trauma or injuries.


Naproxen (Anaprox, Naprelan, Naprosyn)

Well-absorbed in PO route and not usually associated with rebound headaches. For relief of mild to moderate pain. Inhibits inflammatory reactions and pain by decreasing activity of cyclooxygenase, which is responsible for prostaglandin synthesis. Inexpensive and can be purchased OTC.

Adult

500 mg PO at onset; 250-500 mg after 6 h

Pediatric

2.5-5 mg/kg PO q12h

Coadministration with aspirin increases risk of inducing serious NSAID-related adverse effects; probenecid may increase concentrations and, possibly, toxicity of NSAIDs; may decrease effects of hydralazine, captopril, and beta-blockers; may decrease diuretic effects of furosemide and thiazides; monitor PT closely (instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; phenytoin levels may be increased when administered concurrently

Documented hypersensitivity; peptic ulcer disease; recent GI bleeding or perforation; renal insufficiency

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Acute renal insufficiency, interstitial nephritis, hyperkalemia, hyponatremia, and renal papillary necrosis may occur; patients with preexisting renal disease or compromised renal perfusion risk acute renal failure; leukopenia occurs rarely, is transient, and usually returns to normal during therapy; persistent leukopenia, granulocytopenia, or thrombocytopenia warrants further evaluation and may require discontinuation of drug


Acetaminophen and codeine (Tylenol #3)

Indicated for treatment of mild to moderate pain.

Adult

15-60 mg PO q4h prn

Pediatric

0.5-1 mg/kg/dose PO q4-6h prn; not to exceed 60 mg/dose

Toxicity increases with CNS depressants or TCAs

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Caution in patients dependent on opiates since this substitution may result in acute opiate-withdrawal symptoms; caution in severe renal or hepatic dysfunction

Corticosteroids

Have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli.


Prednisone (Deltasone, Orasone, Sterapred)

Corticosteroids are the most commonly used and most versatile immunosuppressants. Corticosteroids have many complex actions and a broad range of immunosuppressive and anti-inflammatory effects. Induce lymphocytopenia, interfere with production and function of numerous lymphokines, and disrupt intercellular communication among leukocytes.
Use lowest effective dose, weighing benefits against risks in each patient.

Adult

5-80 mg/d PO

Pediatric

0.05-2 mg/kg/d PO

Coadministration with estrogens may decrease prednisone clearance; when used with digoxin, digitalis toxicity secondary to hypokalemia may increase; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics

Documented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections; patients receiving glucocorticoids are at risk of multiple complications, including infections

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use

Tricyclic antidepressants

A complex group of drugs that have central and peripheral anticholinergic effects, as well as sedative effects. They have central effects on pain transmission. They block the active reuptake of norepinephrine and serotonin.


Amitriptyline (Elavil)

Amitriptyline can be used, with a slow titration schedule. This agent can be useful if a mild sedative effect is desired. It can be dosed at night.

Adult

10 mg PO at night, and titrate to efficacy with dosage adjustments at 3-4 wk; dosages of up to 150 mg may be necessary, but response is usually at lower dosages in the 10-75 mg range

Pediatric

5 mg PO depending on weight and tolerability, titrate to efficacy

Phenobarbital may decrease effects; coadministration with CYP2D6 enzyme system inhibitors (eg, cimetidine, quinidine) may increase amitriptyline levels; amitriptyline inhibits hypotensive effects of guanethidine; may interact with thyroid medications, alcohol, CNS depressants, barbiturates, and disulfiram

Documented hypersensitivity; use of MAO inhibitors within 14 d of initiating therapy; history of seizures, cardiac arrhythmias, glaucoma, or urinary retention

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Caution in cardiac conduction disturbances and in history of hyperthyroidism or renal or hepatic impairment; avoid using in elderly persons; drowsiness may occur

Antispasticity agents

Effective in some patients for pain management.


Baclofen (Lioresal)

Centrally acting muscle relaxant; precise mechanism of action is unknown. GABA analog; may exert effects by stimulation of GABA-beta receptor. Inhibits monosynaptic and polysynaptic reflexes at spinal level by hyperpolarization of afferent terminals.

Adult

Initial: 5 mg PO tid; increase pending clinical response
Maintenance: 10-20 mg PO tid

Pediatric

Not established

Opiate analgesics, benzodiazepines, alcohol, TCAs, guanabenz, MAOIs, clindamycin, and hypertensive agents may increase effects

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Caution in patients with history of autonomic dysreflexia and when spasticity is used to obtain increased function; autonomic dysreflexia can result from withdrawal of this medication

More on Raeder Paratrigeminal Syndrome

Overview: Raeder Paratrigeminal Syndrome
Differential Diagnoses & Workup: Raeder Paratrigeminal Syndrome
Treatment & Medication: Raeder Paratrigeminal Syndrome
Follow-up: Raeder Paratrigeminal Syndrome
Multimedia: Raeder Paratrigeminal Syndrome
References
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References

  1. Goadsby PJ. Raeder's syndrome [corrected]: paratrigeminal paralysis of the oculopupillary sympathetic system. J Neurol Neurosurg Psychiatry. Mar 2002;72(3):297-9. [Medline].

  2. Murnane M, Proano L. Raeder's paratrigeminal syndrome: a case report. Acad Emerg Med. Sep 1996;3(9):864-7. [Medline].

  3. Nolph MB, Dion MW. Raeder's syndrome associated with internal carotid artery dilation and sinusitis. Laryngoscope. Oct 1982;92(10 Pt 1):1144-8. [Medline].

  4. BONIUK M, SCHLEZINGER NS. Raeder's paratrigeminal syndrome. Am J Ophthalmol. Dec 1962;54:1074-84. [Medline].

  5. Law WR, Nelson ER. Internal carotid aneurysm as a cause of Raeder's paratrigeminal syndrome. Neurology. Jan 1968;18(1 Pt 1):43-6. [Medline].

  6. Grimson BS, Thompson HS. Raeder's syndrome. A clinical review. Surv Ophthalmol. Jan-Feb 1980;24(4):199-210. [Medline].

  7. Vijayan N, Watson C. Pericarotid syndrome. Headache. Nov 1978;18(5):244-54. [Medline].

  8. Raeder JG. "Paratrigeminal" paralysis of oculo-pupillary sympathetic. Brain. 1924;47:149-158.

  9. Selky AK, Pascuzzi R. Raeder's paratrigeminal syndrome due to spontaneous dissection of the cervical and petrous internal carotid artery. Headache. Jul-Aug 1995;35(7):432-4. [Medline].

  10. Appen RE, Sturm RJ. Raeder's paratrigeminal syndrome. Ann Ophthalmol. Sep 1978;10(9):1181-7. [Medline].

  11. Bajwa ZH, Ho CC. Causes of Facial Pain. UpToDate. Available at www.uptodate.com. Accessed 2004.

  12. Solomon S. Raeder syndrome. Arch Neurol. Apr 2001;58(4):661-2. [Medline].

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Further Reading

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Keywords

Raeder's paratrigeminal syndrome, facial pain, oculosympathetic palsy, Raeder's syndrome, paratrigeminal neuralgia, unilateral face pain, Horner syndrome, ipsilateral oculosympathetic palsy, paratrigeminal oculosympathetic syndrome

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Contributor Information and Disclosures

Author

Steven H Schechter, MD, Clinical Assistant Professor, Department of Neurology, Wayne State University School of Medicine; Consulting Staff, Division of Neurology, Assistant Medical Director of Stroke Unit, William Beaumont Hospital
Steven H Schechter, MD is a member of the following medical societies: American Academy of Neurology, American Medical Association, American Medical Electroencephalographic Association, Michigan State Medical Society, and Oakland County Medical Society
Disclosure: Eli Lilly Honoraria Speaking and teaching; Pfizer Honoraria Speaking and teaching; Novartis Honoraria Speaking and teaching; Forest Honoraria Speaking and teaching

Medical Editor

Jorge E Mendizabal, MD, Consulting Staff, Corpus Christi Neurology
Jorge E Mendizabal, MD is a member of the following medical societies: American Academy of Neurology, American Headache Society, National Stroke Association, and Stroke Council of the American Heart Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

James H Halsey, MD, Professor, Department of Neurology, University of Alabama Medical Center
James H Halsey, MD is a member of the following medical societies: American Academy of Neurology, American Heart Association, American Medical Association, American Neurological Association, American Society of Neuroimaging, Medical Association of the State of Alabama, New York Academy of Sciences, Pan American Medical Association, Sigma Xi, Society for Neuroscience, and Southern Medical Association
Disclosure: Nothing to disclose.

CME Editor

Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital
Matthew J Baker, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Chief Editor

Nicholas Y Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants
Nicholas Y Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology
Disclosure: Nothing to disclose.

 
 
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