Hemifacial spasm consists of painless irregular involuntary

contractions on one side of the face. Symptoms may

develop as a sequela to Bells palsy but may also be due
to an irritative lesion of the facial nerve (e.g., an acoustic
neuroma, an aberrant artery that compresses the nerve,
or a basilar artery aneurysm).However, in the most common
form of hemifacial spasm, the cause and pathology
are unknown. Mild cases can be treated with carbamazepine,
gabapentin, or, if these drugs fail,with baclofen.
Local injections of botulinum toxin into affected muscles
can relieve spasms for 34 months, and the injections can
be repeated. Refractory cases due to vascular compression usually respond to
surgical decompression of the facial
nerve. Blepharospasm is an involuntary recurrent spasm of
both eyelids that usually occurs in elderly persons as an
isolated phenomenon or with varying degrees of spasm
of other facial muscles. Severe, persistent cases of blepharospasm
can be treated by local injection of botulinum
toxin into the orbicularis oculi. Facial myokymia
refers to a fine rippling activity of the facial muscles; it
may be caused by multiple sclerosis or follow GuillainBarr syndrome (Chap. 41).
Facial hemiatrophy occurs mainly in women and is
characterized by a disappearance of fat in the dermal
and subcutaneous tissues on one side of the face. It usually
begins in adolescence or early adult years and is
slowly progressive. In its advanced form, the affected side
of the face is gaunt, and the skin is thin, wrinkled, and
brown. The facial hair may turn white and fall out, and
the sebaceous glands become atrophic. Bilateral involvement
may occur. A limited form of systemic sclerosis
(scleroderma) may be the cause of some cases.Treatment
is cosmetic, consisting of transplantation of skin and subcutaneous
fat.
Harison

Hemifacial spasm has

many similarities to trigeminal neuralgia. Both are often
caused by minor anatomical variations of blood vessels
overlying the nerve. Hemifacial spasm consists of continuous
twitching movements usually maximal around the eye
and the mouth. The condition is often more annoying and
embarrassing than unpleasant. It may occur at any age but
is more commonly found in older age groups. Tumours at
the cerebellopontine angle, basilar artery aneurysms and
basal meningitis may all be responsible for this condition,

but in most cases no definite cause is found.

Clinical neuro anatomi

Hemifacial spasm is characterized by clonic spasms of the facial muscles, usually

starting around the eye and often spreading to other muscles of one side of the
face. It increases in intensity during stress and may occur in sleep. The characteristic
EMG findings include bursts of muscle action potentials that occur either
regularly or irregularly at 5 to 20 per second. Synkinetic motor responses in muscles
innervated by the facial nerve follow stimulation of the ipsilateral fifth nerve (blink
reflex). Hemifacial spasm does not have the ominous implications of myokymia, but the
cosmetic effects may be distressing. The cause is usually obscure, but it may
follow facial nerve trauma. Treatment with anticonvulsant medication, such as
carbamazepine, may be effective. Jannetta (1977) reported relief of the involuntary
movements by exposing the facial nerve in the posterior fossa and decompressing
vessels at the root entry zone. Botulinum toxin is also effective.

Pathology
After nerve damage, the pathologic changes depend on the nature of the injury, which
also affects the regenerative response and the prognosis for recovery.
According to Seddon (1954), mechanical nerve injuries are classified as follows: (1)
complete severing of a nerve ( neurotmesis), (2) axonal interruption with distal
degeneration but an intact endoneurium ( axonotmesis), or (3) conduction block at the
site of the lesion but normal distal conduction without degeneration of distal
fibers (neurapraxia).
Within the first 24 hours of injury, focal swelling occurs adjacent to the damaged site
with fragmentation of endoplasmic reticulum, neurotubules, and neurofilaments,
and accumulation of organelles. The axolemma becomes discontinuous; axons swell at
some sites and narrow at others to give a beaded appearance. This process
begins between the nodes of Ranvier and appears first in smaller fibers. Changes in
myelin sheaths lag behind those in axons but progress in a similar way along the
entire distal stump, again affecting small fibers first. The myelin surrounding the
fragmented axons breaks up to form rows of elliptoids. Finally, Schwann cells and
macrophages degrade the axon and myelin debris. In addition to these distal nerve
changes, a retrograde axon reaction or chromatolysis is seen, with retraction of
axons proximal to the lesion and alterations in the somata of neurons, such as cell body
swelling, disruption of Nissl substance, migration of the cell nucleus, and
increase in the size of the nucleolus. Presynaptic terminals gradually withdraw from the
soma and dendrites; synaptic transmission is reduced until dorsal root
stimulation fails to excite the motor neuron and evoke a reflex discharge in the ventral
root. The pathologic distal changes of degeneration and retrograde axon
reaction are similar in crush injury or complete nerve transection.

If a nerve has been completely severed, the orderly process just described is interfered
with in proportion to the length of the discontinuity between proximal and distal
ends. If this distance is great, regeneration is not possible, unless the ends are apposed
at operation. If the distance is small, the fine processes of the axon penetrate
the fibrin and connective tissue in the scar and enter the distal end of the nerve. Some
of these may be deflected from the proper path by the scar and become
entangled to form a neuroma.
merrit