History

Fact Explanation
Excessive blinking Excessive and uncontrollable blinking occurs in blepharospasm. [1,3]
Twitching of the eye lids Patients complain of twitching of the muscles surrounding the eyes. This often progress to face and neck as well. [5]
Difficulty in opening the eyes Patients often find it difficult to open the eyes and they may complain that they need to put a great effort to open the eyes due to spasm of the muscles surrounding the eyes. [4]
Risk factors Although the exact cause is not known in primary blepharospasm, fatigue, stress, and irritants are known risk factors of primary blepharospasm. Pain in and around the eye causes reflex blepharospasm. Patients with a history of dry eyes and sensitivity to the sun and bright light can also develop blepharospasm. Wind, air pollution, noise, and movements of the head or eyes can also cause blepharospasm. [4]
Blindness Functional blindness can develop in severe cases, because of the inability to open the eyes. [1,4]
Drug history Patients who had neuroleptics, antihistamines, calcium channel blockers, noradrenaline and serotonin reuptake inhibitors can have blepharospasm as a side effect of treatment. Benzodiazepines (alprazolam, bromazepam, clonazepam, diazepam, ethyl loflazepate, or triazolam) can also result in blepharospasm after prolonged use and in acute withdrawal. [6,8]
Photophobia Some patients may complain of photophobia which is useful in making the diagnosis of blepharospasm. [6,7]
Irritation of the eyes This is one of the significant complain of blepharospasm. [6]
Family history Positive family history can be detected in some patients with blepharospasm. [8]
History of Parkinson disease Secondary blepharospasm as well as treatment with dopaminergic agents can be associated with Parkinson disease. [2,6]
Psychiatric symptoms Patients can have depression and anxiety associated with blepharospasm. [6]
History of head trauma Some patients may develop blepharospasm after head trauma. [8]
References
  1. TRUONG DD, GOLLOMP SM, JANKOVIC J, LEWITT PA, MARX M, HANSCHMANN A, FERNANDEZ HH, XEOMIN US BLEPHAROSPASM STUDY GROUP. Sustained efficacy and safety of repeated incobotulinumtoxinA (Xeomin®) injections in blepharospasm J Neural Transm [online] 2013, 120(9):1345-1353 [viewed 12 September 2014] Available from: doi:10.1007/s00702-013-0998-9
  2. KHOOSHNOODI MA, FACTOR SA, JINNAH HA. Secondary Blepharospasm Associated with Structural Lesions of the Brain J Neurol Sci [online] 2013 Aug 15, 331(0):98-101 [viewed 12 September 2014] Available from: doi:10.1016/j.jns.2013.05.022
  3. DEFAZIO G, HALLETT M, JINNAH HA, BERARDELLI A. Development and validation of a clinical guideline for diagnosing blepharospasm Neurology [online] 2013 Jul 16, 81(3):236-240 [viewed 12 September 2014] Available from: doi:10.1212/WNL.0b013e31829bfdf6
  4. HWANG WJ. Demographic and clinical features of patients with blepharospasm in southern Taiwan: a university hospital-based study. Acta Neurol Taiwan [online] 2012 Sep, 21(3):108-14 [viewed 12 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/23196730
  5. AQUINO CC, FELíCIO AC, CASTRO PC, OLIVEIRA RA, SILVA SM, BORGES V, FERRAZ HB. Clinical features and treatment with botulinum toxin in blepharospasm: a 17-year experience. Arq Neuropsiquiatr [online] 2012 Sep, 70(9):662-6 [viewed 12 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/22990720
  6. EMOTO Y, EMOTO H, OISHI E, HIKITA S, WAKAKURA M. Twelve cases of drug-induced blepharospasm improved within 2 months of psychotropic cessation Drug Healthc Patient Saf [online] :9-14 [viewed 12 September 2014] Available from: doi:10.2147/DHPS.S20691
  7. DIGRE KB, BRENNAN KC. Shedding Light on Photophobia J Neuroophthalmol [online] 2012 Mar, 32(1):68-81 [viewed 12 September 2014] Available from: doi:10.1097/WNO.0b013e3182474548
  8. PECKHAM EL, LOPEZ G, SHAMIM EA, RICHARDSON SP, SANKU S, MALKANI R, STACY M, MAHANT P, CRAWLEY A, SINGLETON A, HALLETT M. Clinical features of patients with blepharospasm: a report of 240 patients. Eur J Neurol [online] 2011 Mar, 18(3):382-6 [viewed 12 September 2014] Available from: doi:10.1111/j.1468-1331.2010.03161.x

Examination

Fact Explanation
Frequent blinking Patients with blepharospasm blink frequently. [1]
Narrow eye lids The eye lids of the affected patients can be narrowed or closed. [1]
Muscle spasms Spasmodic and stereotyped contractions can be seen in muscles surrounding the eyes. These can not be voluntarily suppressed. [1]
Sensory tricks Patients with blepharospasm can have sensory tricks, which are the manoeuvres used to relieve pain due to muscle spasms. Patient may touch or rub the eyes. [1]
References
  1. DEFAZIO G, HALLETT M, JINNAH HA, BERARDELLI A. Development and validation of a clinical guideline for diagnosing blepharospasm Neurology [online] 2013 Jul 16, 81(3):236-240 [viewed 12 September 2014] Available from: doi:10.1212/WNL.0b013e31829bfdf6

Differential Diagnoses

Fact Explanation
Myasthenia Myasthenia is an autoimmune neuromuscular disease causing fluctuating muscle weakness and fatigue. Common muscle groups that are affected are muscles of facial expressions, chewing, talking, and swallowing. Varying degrees of ptosis and fatiguability aids in diagnosis. [3]
Chorea Chorea causes abnormal involuntary muscle contractions, which might involve muscles of the eyes and facial muscles. [1]
Bell palsy Bell palsy leads to the development of partial ptosis. Further examination will be helpful to detect the paralysis of muscles of facial expression. [1]
Tics Tics can be voluntarily suppressed by the patient, [1]
Meige's syndrome Meige's syndrome is a neurological disorder characterized by oromandibular dystonia and blepharospasm. [2]
References
  1. DEFAZIO G, HALLETT M, JINNAH HA, BERARDELLI A. Development and validation of a clinical guideline for diagnosing blepharospasm Neurology [online] 2013 Jul 16, 81(3):236-240 [viewed 12 September 2014] Available from: doi:10.1212/WNL.0b013e31829bfdf6
  2. DEBADATTA M, MISHRA AK. Meige's Syndrome: Rare Neurological Disorder Presenting as Conversion Disorder Indian J Psychol Med [online] 2013, 35(3):317-318 [viewed 12 September 2014] Available from: doi:10.4103/0253-7176.119493
  3. SATHASIVAM SIVAKUMAR. Diagnosis and management of myasthenia gravis. Prog. Neurol. Psychiatry [online] December, 18(1):6-14 [viewed 12 September 2014] Available from: doi:10.1002/pnp.315

Investigations - for Diagnosis

Fact Explanation
Electromyogram (EMG) EMG of the muscles of the eyelids (orbicularis oculi and levator palpebrae superioris) is helpful in early diagnosis of blepharospasm. [2]
Functional MRI (fMRI) fMRI can demonstrate abnormal activity in the somatosensory and cingulate cortices, thalamus, caudate, cerebellum, and brainstem. [1]
PET scan PET scan can also demonstrate findings similar to fMRI. [1]
References
  1. KHOOSHNOODI MA, FACTOR SA, JINNAH HA. Secondary Blepharospasm Associated with Structural Lesions of the Brain J Neurol Sci [online] 2013 Aug 15, 331(0):98-101 [viewed 12 September 2014] Available from: doi:10.1016/j.jns.2013.05.022
  2. PARDAL-FERNáNDEZ JM, MANSILLA-LOZANO D. [Value of simultaneous electromyographic recording of the levator palpebrae and the orbicularis oculi muscles as an early diagnostic marker for blepharospasm]. Rev Neurol [online] 2012 Dec 1, 55(11):658-62 [viewed 12 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/23172092
  3. DEFAZIO G, HALLETT M, JINNAH HA, BERARDELLI A. Development and validation of a clinical guideline for diagnosing blepharospasm Neurology [online] 2013 Jul 16, 81(3):236-240 [viewed 13 September 2014] Available from: doi:10.1212/WNL.0b013e31829bfdf6

Investigations - Fitness for Management

Fact Explanation
Renal function test Assessment of the renal function is indicated if surgical intervention is planned. Basic assessment of the renal function includes serum electrolytes and serum creatinine levels. [2]
Full blood count Full blood count enables the diagnosis of anemia and infections. [2]
ECG ECG asseses the cardiovascular fitness of the patient. [1]
References
  1. FLEISHER LA. The Preoperative Electrocardiogram: What Is the Role in 2007? Ann Surg [online] 2007 Aug, 246(2):171-172 [viewed 13 September 2014] Available from: doi:10.1097/SLA.0b013e31811eb927
  2. KUMAR A, SRIVASTAVA U. Role of routine laboratory investigations in preoperative evaluation J Anaesthesiol Clin Pharmacol [online] 2011, 27(2):174-179 [viewed 13 September 2014] Available from: doi:10.4103/0970-9185.81824

Management - General Measures

Fact Explanation
Health education Patients with light sensitivity are advised to wear dark glasses and hats during out door activities. This is effective as a mode of camouflage as well. Blepharospasm caused by wind can be avoided by using spectacles and by avoiding riding motor cycles. Maintenance of lid hygiene is important in treating blepharospasm if the causative factor is irritants. Patients with stress related blepharospasm can be educated to practice relaxation methods. Good quality and adequate sleep is also helpful in relieving symptoms. [1]
References
  1. HWANG WJ. Demographic and clinical features of patients with blepharospasm in southern Taiwan: a university hospital-based study. Acta Neurol Taiwan [online] 2012 Sep, 21(3):108-14 [viewed 12 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/23196730

Management - Specific Treatments

Fact Explanation
Withhold the offending drug Drug induced blepharospasm usually resolve with the cessation of the offending drug. [3]
Medical management Anticholinergics (trihexyphenidyl, benztropine, biperiden, atropine) are indicated for the medical management of blepharospasm. They are known to inhibit the nicotinic acetylcholine receptors in the skeletal muscles which inhibit the propagation of the nerve impulse causing muscle relaxation. [4]
Botulinum toxin injections Botulinum toxin is injected to induce localized, partial paralysis of the affected muscles. [1,2,4]
Surgery If the patient is not responding to both medical management and to botulinum toxin injections, protractor myectomy, bilateral facial nerve avulsions are done. [4]
Ganglion block Ganglion block can be done by injecting botulinum toxin in to the ganglion, which inhibits the transmission of nerve impulses. [5]
Nerve block By blocking the selected nerve fibers of the facial nerve, spasmodic contractions of the muscles can be abolished. [6]
References
  1. TRUONG DD, GOLLOMP SM, JANKOVIC J, LEWITT PA, MARX M, HANSCHMANN A, FERNANDEZ HH, XEOMIN US BLEPHAROSPASM STUDY GROUP. Sustained efficacy and safety of repeated incobotulinumtoxinA (Xeomin®) injections in blepharospasm J Neural Transm [online] 2013, 120(9):1345-1353 [viewed 12 September 2014] Available from: doi:10.1007/s00702-013-0998-9
  2. HWANG WJ. Demographic and clinical features of patients with blepharospasm in southern Taiwan: a university hospital-based study. Acta Neurol Taiwan [online] 2012 Sep, 21(3):108-14 [viewed 12 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/23196730
  3. EMOTO Y, EMOTO H, OISHI E, HIKITA S, WAKAKURA M. Twelve cases of drug-induced blepharospasm improved within 2 months of psychotropic cessation Drug Healthc Patient Saf [online] :9-14 [viewed 12 September 2014] Available from: doi:10.2147/DHPS.S20691
  4. GRANDAS F, ELSTON J, QUINN N, MARSDEN CD. Blepharospasm: a review of 264 patients. J Neurol Neurosurg Psychiatry [online] 1988 Jun, 51(6):767-772 [viewed 12 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1033145
  5. LIM SJ, PARK HJ, LEE SH, MOON DE. Ganglion Impar Block With Botulinum Toxin Type A for Chronic Perineal Pain -A Case Report- Korean J Pain [online] 2010 Mar, 23(1):65-69 [viewed 12 September 2014] Available from: doi:10.3344/kjp.2010.23.1.65
  6. SANCHEZ-CAPUCHINO A, MEADOWS D, MORGAN L. Local anaesthesia for eye surgery without a facial nerve block. Anaesthesia [online] 1993 May, 48(5):428-31 [viewed 13 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/8317654