eMedicine Specialties > Neurology > Neuromuscular Diseases
Hemifacial Spasm: Treatment & Medication
Updated: Feb 3, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
Treatment
Medical Care
- Use medications in patients with noncompressive lesions and early idiopathic hemifacial spasm.
- Response to medication varies but can be satisfactory in early or mild cases.
- The most helpful agents are carbamazepine and benzodiazepines (eg, clonazepam).
- Often, medication effects attenuate over time, necessitating more aggressive treatment.
- Medications may be used in early hemifacial spasm (when spasms are mild and infrequent) or in patients who decline botulinum toxin injection.
Surgical Care
- Treat compressive lesions surgically.
- Ectatic blood vessels cause hemifacial spasm by compressing the facial nerve as it exits the brainstem.
- Surgical decompression of these blood vessels can yield excellent results.
- Patients with apparently idiopathic hemifacial spasm may benefit from posterior fossa exploration and microvascular decompression.
- Myectomy rarely is required.
Medication
The goal of pharmacotherapy is reduction of abnormal muscle contractions. Botulinum toxin type A is the treatment of choice. Carbamazepine, benzodiazepines, and baclofen also may be used in patients who refuse BTX injections or who are not surgical candidates.
Toxins
Botulinum toxin type A is the drug of choice. It causes presynaptic paralysis of the myoneural junction and reduces abnormal contractions. Therapeutic effects may last 3-6 months.
Botulinum toxin type B is useful in reducing excessive, abnormal contractions associated with blepharospasm; binds to receptor sites on the motor nerve terminals and after uptake inhibits release of acetylcholine, blocking transmission of impulses in neuromuscular tissue; 7-14 d after administering initial dose, assess patients for a satisfactory response; increase doses 2-fold over previously administered dose for patients who experience incomplete paralysis of the target muscle.
Botulinum toxin type A (BOTOX®)
Useful in reducing excessive, abnormal contractions associated with blepharospasm; binds to receptor sites on the motor nerve terminals and after uptake inhibits release of acetylcholine, blocking transmission of impulses in neuromuscular tissue; 7-14 d after administering initial dose, assess patients for a satisfactory response; increase doses 2-fold over previously administered dose for patients who experience incomplete paralysis of the target muscle.
Adult
Initial dosing: Inject 1.25-2.5 U (0.05-0.1 mL) IM into abnormally contracting muscles via hollow EMG needle; not to exceed 25 U when given as a single injection or 200 U when given as a cumulative dose in a 30 d period
Pediatric
<12 years: Not established
>12 years: Administer as in adults
Caution in patients taking aminoglycoside antibiotics or any other drug that interferes with neuromuscular transmission as they may potentiate effect of botulinum toxin
Documented hypersensitivity
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
Do not exceed recommended dosages and frequencies of administration; presence of antibodies may reduce effectiveness of therapy
Botulinum toxin type B (Myobloc)
When botulinum toxin injection is indicated and type A toxin is ineffective, injection with type B (Myobloc) should be considered.
Adult
Not established: This author suggests starting dose of 500-1000 U IM, divided among abnormally contracting muscles
Pediatric
Not established
Caution in patients taking aminoglycoside antibiotics or any other drug that interferes with neuromuscular transmission because they may potentiate effect of botulinum toxin
Documented hypersensitivity to drug; patients with hypersensitivity to type A toxin, hypersensitivity to type B is significant concern, and use of type B in these patients is not recommended
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
Likely to cause pain at injection site for a few seconds immediately following administration
Benzodiazepines
May potentiate effects of GABA and facilitate inhibitory GABA neurotransmission. May act in the spinal cord to induce muscle relaxation. Individualize treatment for each patient.
Clonazepam (Klonopin)
Useful in suppressing muscle contractions by facilitating inhibitory GABA neurotransmission and other inhibitory transmitters.
Adult
Initial dose: 1.5 mg PO in 3 divided doses
Maintenance dose: Increase initial dose by 0.5-1 mg PO q3d to a dose range of 0.05-0.2 mg/kg
Alternative maintenance dose: 7-12 mg/d PO; not to exceed 20 mg/d
Pediatric
<10 years or <30 kg:
Initial dose: 0.01–0.03 mg/kg/d PO bid/tid
Maintenance dose: Increase initial dose by 0.5 mg PO q3d to a dose range of 0.1-0.2 mg/kg/d divided tid; not to exceed 0.2 mg/kg/d
>10 years: Administer as in adults
Phenytoin and barbiturates may increase clonazepam clearance and reduce its effects; toxicity in the CNS is significantly increased when used concurrently with CNS depressants
Documented hypersensitivity; severe liver disease; acute narrow-angle glaucoma
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 with chronic respiratory disease or impaired renal function; withdrawal symptoms can result from abrupt discontinuation
Muscle relaxants
May inhibit transmission of monosynaptic and polysynaptic reflexes at the spinal cord level.
Baclofen (Lioresal)
May induce hyperpolarization of afferent terminals and inhibit both monosynaptic and polysynaptic reflexes at the spinal level.
Adult
Administer 5 mg PO tid for 3 d; 10 mg PO tid for 3 d; 15 mg PO tid for 3 d; 20 mg PO tid for 3 d; thereafter, additional increases may be necessary; not to exceed 80 mg/d PO divided qid
Pediatric
Not established
Opiate analgesics, benzodiazepines, hypertensive agents, alcohol, tricyclic antidepressants, guanabenz, MAOIs, and clindamycin may increase effects
Documented hypersensitivity
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Caution when spasticity is used to obtain increased function and with patients with a history of autonomic dysreflexia; withdrawal can cause autonomic dysreflexia
Anticonvulsants
Used to manage severe muscle spasms and provide analgesia and mild sedation. Anticonvulsants are probably the best medications in terms of efficacy and long-term safety when BTX and/or surgery are not an option.
Carbamazepine (Tegretol)
Effective in treatment of HFS and complex partial seizures; appears to act by reducing polysynaptic responses and blocking posttetanic potentiation; once a response is attained, attempt to reduce dose to the minimum effective level or discontinue at least once every 3 mo; in patients who cannot tolerate carbamazepine, consider oxcarbazepine (dosage not yet established).
Adult
200 mg PO bid (100 mg qid susp)
Increase at weekly intervals by no more than 200 mg/d using a tid/qid regimen (bid with extended release) until the best response is obtained; not to exceed 1600 mg/d
Pediatric
<12 years: Not established
>12 years: Administer as in adults
Do not use concomitantly with MAOIs; discontinue MAOIs at least 14 d before administration; may alter hepatic metabolism, causing decrease in primidone and phenobarbital serum concentrations and increase in carbamazepine concentrations; plasma levels may increase and toxicity may result when taken concurrently with cimetidine; appears to be more significant when cimetidine is added to carbamazepine during the first 4 wk of therapy; levels increase significantly within 30 d of danazol administration; avoid concomitant administration if possible
Documented hypersensitivity; patients with history of bone marrow depression
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Not a simple analgesic; do not use for relief of minor aches or pains; caution in patients with increased intraocular pressure; obtain CBC and serum-iron baseline prior to initiating treatment, then monthly CBCs and iron during the first 2 mo; thereafter, obtain CBC, differential, and platelet count yearly or every other year; can cause drowsiness, dizziness, and blurred vision; patients should observe caution while driving or performing other tasks requiring alertness
Trileptal (Oxcarbazepine)
Effective in partial complex epilepsy, Oxcarbazepine shows promise in HFS. Oxcarbazepine may be considered when first-line agents (eg, botulinum toxin, carbamazepine) have failed or are contraindicated.
Adult
Monotherapy: 150 mg or 300 mg PO bid initially; dose may be increased by 300 mg/d q3d; maximum recommended daily dose of 1200-2400 mg in divided dosing; elderly patients may require slower titrations
Pediatric
<4 years
Not established
4-16 years
Adjunctive therapy: 8-10 mg/kg/d PO divided bid initially, not to exceed 600 mg/d; gradually increase to target dose over 2 wk; target adjunctive dose is based on body weight; 20-29 kg = 900 mg/d, 29.1-39 kg = 1200 mg/d, >39 kg = 1800 mg/d
Conversion to monotherapy: 8-10 mg/kg/d PO divided bid initially; gradually reduce the dose of concomitant anticonvulsants over 3-6 wk; may gradually increase oxcarbazepine dose if clinically indicated by increments not to exceed 10 mg/kg/d at qwk to recommended monotherapy dose; monitor patients closely during this transition phase for anticonvulsant adverse effects
Monotherapy: 8-10 mg/kg/d PO divided bid; may increase by 5 mg/kg/d q3d to recommended daily dose; maintenance monotherapy dose is based on body weight; 20-24 kg = 600-900 mg/kg/d, 25-34 kg = 900-1200 mg/kg/d, 35-44 kg = 900-1500 mg/kg/d, 45-49 kg = 1200-1500 mg/kg/d, 50-59 kg = 1200-1800 mg/kd/d, 60-69 kg = 1200-2100 mg/kg/d, >70 kg = 1500-2100 mg/kg/d
May decrease levels of dihydropyridine calcium antagonists and oral contraceptives; can reduce serum concentrations of carbamazepine, phenobarbital, phenytoin and valproic acid; when oxcarbazepine is given in doses above 1200 mg/d may increase phenytoin and phenobarbital serum concentrations significantly; oxcarbazepine can reduce serum concentrations of oral contraceptives and make oral contraceptives ineffective; can increase clearance of felodipine
Documented hypersensitivity
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
May decrease levels of dihydropyridine calcium antagonists and oral contraceptives; can reduce serum concentrations of carbamazepine, phenobarbital, phenytoin and valproic acid; when oxcarbazepine is given in doses above 1200 mg/d may increase phenytoin and phenobarbital serum concentrations significantly; oxcarbazepine can reduce serum concentrations of oral contraceptives and make oral contraceptives ineffective; can increase clearance of felodipine
More on Hemifacial Spasm |
| Overview: Hemifacial Spasm |
| Differential Diagnoses & Workup: Hemifacial Spasm |
 Treatment & Medication: Hemifacial Spasm |
| Follow-up: Hemifacial Spasm |
| References |
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References
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Jannetta PJ, Abbasy M, Maroon JC, et al. Etiology and definitive microsurgical treatment of hemifacial spasm. Operative techniques and results in 47 patients. J Neurosurg. Sep 1977;47(3):321-8. [Medline].
Kraft SP, Lang AE. Cranial dystonia, blepharospasm and hemifacial spasm: clinical features and treatment, including the use of botulinum toxin. CMAJ. Nov 1 1988;139(9):837-44. [Medline].
Mauriello JA, Leone T, Dhillon S, et al. Treatment choices of 119 patients with hemifacial spasm over 11 years. Clin Neurol Neurosurg. Aug 1996;98(3):213-6. [Medline].
Moller AR. The cranial nerve vascular compression syndrome: I. A review of treatment. Acta Neurochir (Wien). 1991;113(1-2):18-23. [Medline].
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Further Reading
Keywords
hemifacial spasm, craniofacial movement disorders, facial myoclonus, facial dystonia, botulinum toxin, BTX therapy
