WordNet

of or pertaining to the outside surface of an object
care for the face that usually involves cleansing and massage and the application of cosmetic creams
cranial nerve that supplies facial muscles (同)facial_nerve, nervus_facialis, seventh_cranial_nerve
of or concerning the face; "a facial massage"; "facial hair"; "facial expression"
a painful and involuntary muscular contraction (同)cramp, muscle_spasm
(pathology) sudden constriction of a hollow organ (as a blood vessel)

PrepTutorEJDIC

顔の;顔用の / (マッサージや化粧品による)顔の手入れ
(筋肉などの)けいれん / (感情・活動などの)発作《+『of』+『名』》
鉱泉,温泉 / 温泉場

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/03/04 23:15:52」(JST)

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Hemifacial spasm (HFS) is a rare neuromuscular disease characterized by irregular, involuntary muscle contractions (spasms) on one side (hemi-) of the face (-facial).^[1] The facial muscles are controlled by the facial nerve (seventh cranial nerve), which originates at the brainstem and exits the skull below the ear where it separates into five main branches.

This disease takes two forms: typical and atypical. In typical form, the twitching usually starts in the lower eye lid in orbicularis oculi muscle. As time progresses, it spreads to the whole lid, then to the orbicularis oris muscle around the lips, and buccinator muscle in the cheekbone area.^[2] The reverse process of twitching occurs in atypical hemifacial spasm; twitching starts in orbicularis oris muscle around the lips, and buccinator muscle in the cheekbone area in the lower face, then progresses up to the orbicularis oculi muscle in the eyelid as time progresses.^[2] The most common form is the typical form, and atypical form is only seen in about 2–3% of patients with hemifacial spasm.^[3] The incidence of hemifacial spasm is approximately 0.8 per 100,000 persons.^[4]

This disorder occurs in both men and women, although it affects middle-aged or elderly women more frequently.^[5] Hemifacial spasm is much more common in some Asian population.^[1] It may be caused by a facial nerve injury, a tumor, or it may have no apparent cause. Individuals with spasm on both sides of the face are very rare.

History[edit]

The earliest descriptions about hemifacia spasm is by Shultze in 1875 and Gowers in 1899. The etiology of hemifacial spasm and location of the abnormality have been debated for more than a century.^[4] Surgical treatment for hemifacial spasm in the early 20th century included neurolysis (destruction of nerve tissue), stretching the facial nerve (seventh cranial nerve), and high-pressure irrigation of the nerve with lactate ringer's solution. The medical regimens of that time involved injection of the nerve with ethanol, electrical stimulation, application of toxic compounds (nitrate of silver, zinc, arsenic, bromides) as well as medications such as Dilantin or other anticonvulsants.^[4]^[6]^[7]

Additional advances in understanding the etiology and improving treatments for hemifacial spasm did not occur until the mid-seventies. In 1977, 47 cases of hemifacial spasm underwent microvascular decompression of the facial nerve using the operating microscope. The results illustrated nerve-vessel conflicts (or cholesteatoma) to be located at the root exit zone of the facial nerve in all cases.^[8]^[9] The root exit zone is where the central glial axonal insulation of the nerve ends and the peripheral nerve axonal myelination begins. Biopsies of the root exit zone demonstrated degeneration of axons, denuded axis cylinder and interrupted myelin. The results of the experiment strengthened the theory that vascular compression of the facial nerve was the primary cause of hemifacial spasm, and proposed a specific region of the facial nerve where the effects of longstanding compression results in nerve dysfunction.^[9]

Causes[edit]

The Facial Nerve ( The Seventh Cranial Nerve)

Three theories exist to explain the facial nerve dysfunction found in hemifacial spasm. The first proposed theory is ephaptic transmission, which is electrical activity crossing from one demyelinated neuron to another resulting in a false synapse.^[7] The second theory involves abnormal activity of axons at the facial nerve root end zone secondary to compressive damage/demyelination.^[10]

The third theory or "Kindling theory" involves increased excitability of the facial nerve nucleus due to feedback from a damaged facial nerve.^[10]

It is generally accepted as compression of the facial nerve by vessels of the posterior circulation.^[10] In detail compression of the seventh cranial nerve by a dolichoectatic (a distorted, dilated, and elongated) vertebral artery^[11] is accepted to be the general cause of hemifacial spasm. Less than 1% of cases are cause by tumor.^[12]^[13] Hemifacial spasm is much more common in some Asian population.^[1]

Several families with hemifacial spasm have been reported, suggesting a genetic etiology or predisposition in some cases. There appears to be an autosomal dominant pattern of inheritance in these families with low penetrance, and except for a younger age at onset, the clinical features overlap with the idiopathic cases.Evaluation of single-nucleotide polymorphisms in genes related to vascular change causing compression of blood vessles did not show an association with hemifacial spasm. Clarifying the role of genetic susceptibility in hemifacial spasm may help to better understand the pathogenesis of this disease.^[14]

Signs & Symptoms[edit]

The two forms of Hemifacial Spasm

The first sign of hemifacial spasm is typically muscle movement in the patient's eyelid and around the eye. It can vary in intensity.^[9] The intermittent twitching of the eyelid, which can result in forced closure of the eye which gradually spreads to the muscles of the lower part of the face (Typical form- See Image). In atypical form the spasms start in the cheekbone area and spreads to the eyelid.^[2] Ultimately, all the muscles on that side are affected, nearly all the time. This sometimes causes the mouth to be pulled to the side. Experts have linked hemifacial spasm to facial nerve injury, Bell's palsy and tumors. Although the most frequent cause is a blood vessel pressing on the facial nerve at the spot where it leaves the patient's brain stem, sometimes there is no known cause. When the affected individual is younger than 40, doctors suspect an underlying cause such as multiple sclerosis.^[15]

Diagnosis[edit]

There are several tests done to diagnose hemifacial spasm. Diagnosing a case of hemifacial spasm begins with a complete neurological exam, including an Electromyography (EMG – a test that measures and records electrical activity generated in muscle at rest and in response to muscle contraction), Magnetic resonance imaging (MRI – a test that uses magnetic waves to make pictures of structures inside the head), Computed tomography (CT scan – a type of x-ray that uses a computer to make pictures of structures inside the head), and Angiography (an x-ray exam of the blood vessels when they are filled with a contrast material).^[16]

Studies have shown that the most effective method of hemifacial spasm screening is MRI. In one study only 25% of the CT scans showed the abnormality in hemifacial spasm patients, whilst more than half of the MRI imaging demonstrated a vascular anomaly. MRI imaging should be the initial screening procedure in the assessment of patients with hemifacial spasm.^[17]

Treatments & Side Effects[edit]

Mild cases of hemifacial spasm may be managed with sedation or carbamazepine (an anticonvulsant drug).^[18] Microsurgical decompression and botulinum toxin injections are the current main treatments used for hemifacial spasm.^[17]

Microvascular Decompression[edit]

Microvascular decompression appears to be the most popular surgical treatment at present. Microvascular compression relieves pressure on the facial nerve, which is the cause of most hemifacial spasm cases. Excellent to good results are reported in 80% or more cases with a 10% recurrence rate.^[19] In the present series approximately 10% had previously failed surgery. Serious complications can follow microsurgical decompressive operations, even when performed by experienced surgeons. These include cerebellar haematoma or swelling, brain stem infarction (blood vessel of the brain stem blocked), cerebral infarction (ischemic stroke resulting from a disturbance in the blood vessels supplying blood to the brain), subdural haematoma and intracerebral infarction (blockage of blood flow to the brain). Death or permanent disability (hearing loss) can occur in 2% of patients of hemifacial spasm.^[20]

Botulinum Toxin[edit]

Botulinum toxin is highly effective in the treatment of hemifacial spasm.^[21] It has a success rate equal to that of surgery, but repeated injections may be required every 3 to 6 months. The injections are administered as an outpatient or office procedure. Whilst side effects occur, these are never permanent. Repeated injections over the years remain highly effective.^[22] Whilst the toxin is expensive, the cost of even prolonged courses of injections compares favourably with the cost of surgery.^[21] Patients with HFS should be offered a number of treatment options. Very mild cases or those who are reluctant to have surgery or Botulinum toxin injections can be offered medical treatment, sometimes as a temporary measure.In young and fit patients microsurgical decompression and Botulinum injections should be discussed as alternative procedures. In the majority of cases, and especially in the elderly and the unfit, Botulinum toxin injection is the treatment of first choice. Imaging procedures should be done in all unusual cases of hemifacial spasm and when surgery is contemplated.^[17]

Prevention[edit]

There is no known way to prevent hemifacial spasm.^[23]

Epidemiology[edit]

The incidence of hemifacial spasm is approximately 0.8 per 100,000 persons.^[4] Hemifacial spasm is more prevalent among females over 40 years of age.^[24]^[25] The estimated prevalence for women is 14.5 per 100,000 and 7.4 per 100,000 in men.^[26] Prevalence for hemifacial spasm increase with age, reaching 39.7 per 100,000 for those aged 70 years and older.^[27] One study divided 214 hemifacial patients based on the cause of the disease. The patients who had a compression in the facial nerve at the end of the brain stem as the primary hemifacial spasm and patients who had peripheral facial palsy or nerve lesion due to tumors, demyelination, trauma, or infection as secondary hemifacial spasm. The study found that 77% of hemifacial spasm is due to primary hemifacial spasm and 23% is due to secondary hemifacial spasm. The study also found both sets of patients to share similar age at onset, male to female ratios, and similar affected side.^[28] Another study with 2050 patients presented with hemifacial spasm between 1986 and 2009, only 9 cases were caused by a cerebellopontine angle syndrome, an incidence of 0.44%.^[13]

References[edit]

^ ^a ^b ^c Kong, Doo-Sik; Kwan Park (2007). "Hemifacial Spasm: A Neurological Perspective". Journal of Korean Neurological Society. 5 42 (5): 355–362. doi:10.3340/jkns.2007.42.5.355. Cite uses deprecated parameters (help)
^ ^a ^b ^c Jannetta, PJ (1998). "Typical or atypical hemifacial spasm". Journal of Neurosurgery. 2 88: 346–7.
^ Ryu, H.; Yamamoto S., Miyamoto T. (1998). "Atypical hemifacial spasm". Acta Neurochir 40 (11): 1173–76. doi:10.1007/s007010050233. Cite uses deprecated parameters (help)
^ ^a ^b ^c ^d Fukushima, T (1995). "Microvascular decompression for hemifacial spasm: Result in 2890 cases". Neurovascular surgary (New York: McGraw Hill): 1133–45.
^ "NINDS Hemifacial Spasm Information Page". National Institute for Neurological Disorder and Strokes. Missing or empty |url= (help)
^ Wilkins, RH (1991). "Hemifacial spasm: a review". Surgical Neurology 36 (4): 251–77. doi:10.1016/0090-3019(91)90087-P. PMID 1948626.
^ ^a ^b Gardner, J.W.; Sava, Gerard A. (1962). "Hemifacial Spasm - A reversible pathophysiologic state". Journal of Neurosurgery 27 (3): 240–47. doi:10.3171/jns.1962.19.3.0240.
^ Jannetta, PJ (1975). "Trigeminal neuralgia and hemifacial spasm--etiology and definitive treatment". Trans Am Neurol Assoc 100: 89–91. PMID 1226647.
^ ^a ^b ^c Jannetta, PJ; Abbasy M, Maroon JC, Ramos FM, Albin MS. (1977). "Etiology and definitive microsurgical treatment of hemifacial spasm. Operative techniques and results in 47 patients". Journal of Neurosurgery 47 (3): 321–8. doi:10.3171/jns.1977.47.3.0321. PMID 894338. Cite uses deprecated parameters (help)
^ ^a ^b ^c Eby, Joesph; Sung Tae Cha,Hrayr K. Shahinian (2002). "Fully endoscopic vascular decompression of the glossopharyngeal nerve". The Journal of Craniofacial Surgery 13 (1): 90–95. doi:10.1097/00001665-200201000-00021. PMID 11887002. Cite uses deprecated parameters (help)
^ Rahman, M.D. Ersalan; Jonathan D. Trobe, and Stephen S. Gebarski (June 2002). "Hemifacial Spasm Caused by Vertebral Artery Dolichoectasia". American Journal of Ophthalmology 133 (6): 854–855. doi:10.1016/S0002-9394(02)01387-9. Cite uses deprecated parameters (help)
^ Caces, F; Chays A, Locatelli P, Bruzzo M, Epron JP, Fiacre E, Magnan J. (1996). "Neuro-vascular decompression in hemifacial spasm: anatomical, electrophysiological and therapeutic results apropos of 100 cases". Rev Laryngol Otol Rhinol (Bord). 5 117: 347–51. Cite uses deprecated parameters (help)
^ ^a ^b Lee SH, Rhee BA, Choi SK, Koh JS, Lim YJ; Jankovic J (2010). "Cerebellopontine angle tumors causing hemifacial spasm: types, incidence, and mechanism in nine reported cases and literature review". Acta Neurochir (Wien). 11 150 (11): 1901–8. doi:10.1007/s00701-010-0796-1. Cite uses deprecated parameters (help)
^ Toby C. Yaltho, MD 1 and Joseph Jankovic, MD (4/5/2011). "The Many Faces of Hemifacial Spasm: Differential Diagnosis of Unilateral Facial Spasms". Movement Disorders 29 (9). Cite uses deprecated parameters (help); Check date values in: |date= (help)
^ Office of Communications and Public Liaison. "NINDS Hemifacial Spasm Information Page". National Institute of Neurological Disorders and Strokes. Retrieved 11/03/2012.
^ Zappia JJ; Wiet RJ; Chouhan A; Zhao JC, JJ; Wiet, RJ; Chouhan, A; Zhao, JC (1997). "Pitfalls in the diagnosis of hemifacial spasm". The Laryngoscope. 4 107 (4): 461–465. doi:10.1097/00005537-199704000-00007. PMID 9111374.
^ ^a ^b ^c I. T. Lorentz (04/1995). "Treatment of hemifacial spasm with botulinum toxin". Journal of Clinical Neuroscience. 2 2. Check date values in: |date= (help)
^ Alexander GE, Moses H. (1982). Carbamazepine for hemifacial spasm Neurology 32: 286–287.
^ PiattJH,J'r, Wilkins RH., JH; Wilkins, RH (1984). "Treatment of tic douloureux and hemifacial spasm by posterior fossa exploration: therapeutic implications of various neurovascular relationships". Neurosurgery 14 (4): 462–71. doi:10.1227/00006123-198404000-00012. PMID 6728149.
^ HanakitaJ, Kondo A., J; Kondo, A (1988). "Serious complications of microvascular decompression operations for trigeminal neuralgia and hemifacial spasm". Neurosurgery 22 (2): 348–52. doi:10.1227/00006123-198802000-00012. PMID 3352885.
^ ^a ^b Tan, Eng-ing; Stephanie Fook-Chong, Sau-Ying Lum, Erle Lim (16 January 2004). "Botulinum toxin improves quality of life in hemifacial spasm: validation of a questionnaire (HFS-30)". Journal of the Neurological Sciences 219 (1–2): 151–155. doi:10.1016/j.jns.2004.01.010. PMID 15050451. Cite uses deprecated parameters (help)
^ LoeserJD, ChenJ., John D.; Chen, James (1982). "Hemifacial spasm: treatment by microsurgical facial nerve decompression". Neurosurgery 13 (2): 141–46. doi:10.1227/00006123-198308000-00006.
^ Toby C. Yaltho, and Joseph Jankovic, MD (2011-04-04). "The Many Faces of Hemifacial Spasm: Differential Diagnosis of Unilateral Facial Spasms". Movement Disorders 26 (9).
^ Wang, A.; Jankovic J. (1998). "Hemifacial spasm: clinical findings and treatment". Muscle and Nerve. 12 21 (12): 1740–7. doi:10.1002/(SICI)1097-4598(199812)21:12<1740::AID-MUS17>3.0.CO;2-V. PMID 9843077. Cite uses deprecated parameters (help)
^ Tan, N.C.; Chan, L.L, Tan E.K. (2002). "Hemifacial spasm and involuntary facial movements". QJM. 8 95 (8): 493–500. doi:10.1093/qjmed/95.8.493. PMID 12145388. Cite uses deprecated parameters (help)
^ Kemp, LW; Reich SG (6 2004). "Hemifacial spasm". Curr Treat Options Neurol 3 (3): 175–9. doi:10.1007/s11940-004-0009-4. Cite uses deprecated parameters (help); Check date values in: |date= (help)
^ Auger, RG; Whisnant JP (1990). "Hemifacial spasm in Rochester and Olmsted County, Minnesota, 1960 to 1984". Arch Neurol 47 (11): 1233–4. doi:10.1001/archneur.1990.00530110095023. PMID 2241620. Cite uses deprecated parameters (help)
^ Colosimo, C; Bologna M, Lamberti S (2006). "A comparative study of primary and secondary hemifacial spasm". Arch Neurol. 6 63 (3): 441–4. doi:10.1001/archneur.63.3.441. PMID 16533973. Cite uses deprecated parameters (help)

"Hemifacial Spasm Information Page". National Institute of Neurological Disorders and Stroke. October 11, 2011. Retrieved January 6, 2012.

External links[edit]

Centre for Cranial Nerve Disorders.
Hemifacial Spasm Association support site.

UpToDate Contents

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1. 思春期および成人における非てんかん性痙攣疾患 nonepileptic paroxysmal disorders in adolescents and adults
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English Journal

Adverse events associated with botulinum toxin injection: A multidepartment, retrospective study of 5310 treatments administered to 1819 patients.

Kim BW, Park GH, Yun WJ, Rho NK, Jang KA, Won CH, Chang SE, Chung SJ, Lee MW.Author information Department of Dermatology, Asan Medical Center, University of Ulsan College of Medicine , Seoul , Korea.AbstractBackground: The injection of botulinum toxin is now commonly used for many therapeutic and cosmetic purposes but because of its increased use more adverse events are being reported. Objective: To retrospectively evaluate and analyze the safety of botulinum toxin injections in terms of purpose and the type of toxin administered. Materials and methods: Data were collected on 1819 patients who underwent a total of 5310 treatments between 2005 and 2011 at a single tertiary medical center. Information on the dosage, treatment purpose, type of botulinum toxin, and any adverse events associated with these treatments were collected and analyzed. The generalized estimating equation (GEE) with the logistic link function was used to estimate the overall frequencies of adverse events. A multivariable GEE with the logistic link function was used to identify the factors associated with adverse events. Results: Among the 5310 botulinum toxin treatments in our study cohort, 2258 of which (42.5%) were used to treat hemifacial spasm, 184 adverse events (3.73%) were recorded, 114 (2.26%) muscle-related, and 71 (1.47%) muscle-unrelated. The highest number of adverse events (8.29%) was associated with the treatment of blepharospasm and the lowest (1.07%) with masseter hyperplasia. By multivariate analysis, the odds ratio for females was 1.577 (95% confidence interval [CI] = 1.052-2.364; p = 0.027) and for the dose was 1.006 (95% CI = 1.002-1.010; p = 0.005). When compared with upper face wrinkles, the odds ratio was 2.510 (95% CI = 1.400-4.499; p = 0.002) for blepharospasm, 0.375 (95% CI = 0.202-0.695; p = 0.002) for cervical dystonia, and 0.114 (95% CI: 0.015-0.862; p = 0.035) for masseter hyperplasia. Conclusion: When injecting botulinum toxin for cosmetic purposes, practitioners should be cautious, especially when targeting the areas around the eyes, as these treatments are prone to cause adverse events.
The Journal of dermatological treatment.J Dermatolog Treat.2014 Aug;25(4):331-6. doi: 10.3109/09546634.2013.789473. Epub 2013 Apr 24.
Background: The injection of botulinum toxin is now commonly used for many therapeutic and cosmetic purposes but because of its increased use more adverse events are being reported. Objective: To retrospectively evaluate and analyze the safety of botulinum toxin injections in terms of purpose and th
PMID 23537074

3D CT-Guided Pulsed Radiofrequency Treatment for Trigeminal Neuralgia.

Fang L, Ying S, Tao W, Lan M, Xiaotong Y, Nan J.Author information Department of Anesthesiology and Pain Management, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.AbstractTrigeminal neuralgia (TN) is a pain syndrome characterized by pain in the trigeminal area often accompanied by a brief facial spasm or tic. The purpose of our study was to investigate the efficacy and clinical utility of CT-guided pulsed radiofrequency (PRF) for treatment of TN. Patients who were diagnosed with severe TN between September 2010 and October 2010 at Beijing Tiantan Hospital were included. Pulsed radiofrequency treatment (PRFT) was employed to treat TN. To verify the accurate needle position, a thin-section cranial CT scan was performed by using a multidetector CT scanner. Three-dimensional reconstruction was performed to visualize the location of the needle and the foramen ovale. A total of 20 patients were included in the study. Seven patients (35%) had favorable outcome 1 year after the PRFT. The numeric rating scale in the 7 patients with good outcome was significantly lower than the 13 patients with bad outcome at 1 day, 1 week, and 2 weeks after the treatment. The remaining 13 patients had residual pain 2 weeks after the PRFT and had to receive radiofrequency thermocoagulation (RFTC). In conclusion, the results of our study demonstrate that CT-guided PRFT is not an effective method of pain treatment for idiopathic TN as compared with conventional RFTC. However, CT-guided PRFT is associated with less complication than RFTC.
Pain practice : the official journal of World Institute of Pain.Pain Pract.2014 Jan;14(1):16-21. doi: 10.1111/papr.12041. Epub 2013 Feb 21.
Trigeminal neuralgia (TN) is a pain syndrome characterized by pain in the trigeminal area often accompanied by a brief facial spasm or tic. The purpose of our study was to investigate the efficacy and clinical utility of CT-guided pulsed radiofrequency (PRF) for treatment of TN. Patients who were di
PMID 23433058

[Changes in Dose and Injection Pattern in the Botulinum Toxin Long-term Therapy of Facial Dyskinesis.]

Laskawi R, Niemczewska A, Schneider S, Winterhoff J, Beutner C, Rohrbach S.Author information HNO-Klinik, Universitätsmedizin Göttingen, Göttingen.AbstractRare information exists about comparative long-term observations of patients with facial movement disorders. This retrospective analysis deals with the course of different parameters of injection over the time.In this study we compared the development of long-term botulinum toxin treatments of patients with blepharospasm, hemifacial spasm and synkinesis. 80 patients (n=30 blepharospasm, n=31 hemifacial spasm, n=19 synkinesis), who had at least 10 consultations for BTA-injections, were included in the retrospective analysis. The development for each entity in total dosage, increase in the number of injection points and change in dosages for each point were evaluated.The over-all dosage in all 3 clinical disorders and for each single disease itself increased continuously over the time. The amount of injection points increased in the treatment of hemifacial spasm and synkinesis. The dosage per point increased most in blepharospasm between the 1. and 25. injection, but was distinctly lower in patients with hemifacial spasm and synkinesis. The increase in dosage in blepharospasm is therefore, in contrast to the other indications, mostly caused by an increase in dosage per point. In patients with hemifacial spasm and synkinesis the escalation of dosage is mainly caused by an increase of the number of injection points.These new aspects of the dynamic in the treatment with botulinum toxin enable the physician to understand better the dynamic of these diseases, to optimize treatment protocols.
Laryngo- rhino- otologie.Laryngorhinootologie.2013 Dec 9. [Epub ahead of print]
Rare information exists about comparative long-term observations of patients with facial movement disorders. This retrospective analysis deals with the course of different parameters of injection over the time.In this study we compared the development of long-term botulinum toxin treatments of patie
PMID 24323508