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Hemifacial Spasm

1.       What is Hemifacial spasm?

Hemifacial spasm is a type of movement disorder characterized by involuntary unilateral tonic and clonic contractions of the face. Most of the patients have unilateral Hemifacial spasm, but some could have bilateral symptoms. 

2.       How common is Hemifacial spasm?

Hemifacial spasm is more common in women (2:1), with overall prevalence of about 10/100,000 but more common in Asian communities.  Most of the cases are sporadic, i.e., not spread in hereditary pattern.  It should be differentiated from other movement disorders of face such as tics, myokymia, tardive dyskinesia and psychogenic facial spasm.  

3.       Why does Hemifacial spasm occur?

In most of the patients no clear cause can be attributed.  But it has been noted that many patients have an aberrant vessel compressing the VII cranial nerve (Facial nerve).   However, it has also been noted that upto 25% of normal controls also have an aberrant vessel, suggesting multiple factors playing the role in pathogenesis.  These factors include abnormal signal transmission in the nerve.  In few patients secondary causes may be the culprit and include meningioma, schwannoma, parotid gland tumor or pilocytic astrocytoma of the fourth ventricle.    

4.       Is any type of investigation required for diagnosing the disorders?

Hemifacial spasm is a clinical diagnosis which does not require any specific investigation.  However, few patients do require brain imaging.  Imaging of the brain (MRI with special sequences to trace facial nerve) is usually indicated in patients who have atypical features such as facial weakness,  numbness, or other associated findings.

          5. How do you treat Hemifacial spasm?

Treatment of Hemifacial spasm

Blepharospasm

What is Blepharospasm?

Blepharospasm is a type of focal dystonia in Movement disorders.  

This is characterized by excessive contraction of Orbicularis oculi muscle (muscle around the eye which controls the blink and eye closure),

leading to excessive blinks / prolonged contraction.  

In severe cases it can lead to functional blindness (Inability to see due to excessive eye closure)

How common is Blepharospasm?

Blepharospasm is increasingly being recognized as cause of disability.   

It affects about 32/100,000 people.  

It typically starts in 5th or 6th decade of life and much more common in women (3:1)

Why does Blepharospasm occur?

There has been no clear clues on imaging of the brain to indicate the cause of Blepharospasm.  

It has been attributed to abnormal neuronal activity and transmission for this abnormality.

Is any type of investigation required for diagnosing the disorders?

Blepharospasm is a clinical diagnosis which does not require any specific investigation. 

 In some suspected cases with other associated features, blood tests or imaging of the brain(MRI) could be required

What are the treatment options for Blepharospasm?

Treatment options are varied and include medications, botulinum injections and surgical procedures. 

Currently Botulinum injections (Botox, Dysport, Xeomin, and other brands)are the preferred and commonly used first line of treatment for patients with blepharospasm.  

Commonly used medications include Carbamazepine, clonazepam, gabapentin, and baclofen.

Is Botulinum (Botox) effective and safe treatment for Blepharospasm?

Botulinum injections (Botox, Dysport, Xeomin and other brands) for Blepharospasms, is a safe and effective treatment in existing literature.  

In various published literature, it has been well documented to have excellent subjective and objective improvement.  

Most of the patients have the benefit for prolonged periods.  

Most of the patients who receive botulinum injections do not have any adverse effects, but some may develop effects such as ptosis, lid edema, diplopia, ecchymosis. 

 All these symptoms are transient and complete reversible.

How frequently one should take Botulinum (Botox) for blepharospasm?

The frequency of injections and dosage is tailored upon individual response.  

Injections are frequently given in a frequency of once in 3-4 months.  

However based upon response this can be prolonged to 6 months to one year. 

Other Hyperkinetic disorders

In addition to above commonly recognized hyperkinetic disorders, there are various other hyperkinetic disorders which can be described based upon the movement characteristics. Most of these disorders are caused by various etiologies and can be managed by drug therapies.

  • Tics: Involuntary abrupt jerk like movements which are voluntary
  • Myoclonus: Sudden, involuntary and brief jerky movements of the body or specific body parts.
  • Stereotypy: Repetitive purposeless movements
  • Restless legs syndrome : Intense desire to move the legs often associated with uncomfortable sensations in legs especially in evenings/night
  • Ataxia: Inco-ordination of limbs (drunkard like)

Chorea

Chorea is characterized by irregular, non-sustained muscle contractions. They appear as if person is making free dance like movements. It is caused by various disorders, but mostly commonly recognized in India is due to its association with Rheumatic heart disease, which is common in young age population. The other most easily recognized choreic disorder is Huntington’s disease, which runs in family and most commonly seen in adults.