Craniofacial Muscles

290 J. Park et al.
Table 16.2 Jankovic Rating Scale (JRS) severity score
Blepharospasm severity
0 = None
1 = Minimal, increased blinking present only with external stimuli (e.g., bright light, wind,
reading, driving, etc.)
2 = Mild, but spontaneous eyelid fl uttering (without actual spasm), de fi nitely noticeable, possibly
embarrassing, but not functionally disabling
3 = Moderate, very noticeable spasm of eyelids only, mildly incapacitating
4 = Severe, incapacitating spasm of eyelids and possibly other facial muscles
Blepharospasm frequency
0 = None
1 = Slightly increased frequency of blinking
2 = Eyelid fl uttering lasting less than 1 second (s) in duration
3 = Eyelid spasm lasting more than 1 s, but eyes open more than 50% of the waking time
4 = Functionally “blind” due to persistent eye closure (blepharospasm) more than 50% of the
waking time
and partial remissions. Remission rates of 1.2–11.4% have been reported (Castelbuono
and Miller 1998 ; Grandas et al. 1988 ) .
The involuntary movement ranges from increased blink frequency to severe,
sustained spasms of the protractor muscles causing the eyelids to clamp tightly shut.
It is very helpful in evaluating the effect of treatments for blepharospasm to grade
the symptoms based on the severity and frequency. The Jankovic Rating Scale (JRS)
is probably the most widely used current clinical scale which differentiates the
severity and frequency of blepharospasm into grades of 0–4 (Jankovic and Orman
1987 ) . As seen in Table 16.2 , the JRS primarily focuses on the objective signs of
blepharospasm but does incorporate subjective symptoms such as whether the
increased blinking and spasms affect quality of life.
Rarely, only one eyelid is affected in blepharospasm patients, but eventually in
almost all cases both eyelids are involved within weeks to months. However, the
degree of involvement may remain asymmetric. Most patients present with sensory
complaints of dry eye symptoms (ocular irritation, foreign body sensations, grittiness,
photophobia, and tearing) that may precede or occur simultaneously with the
development of the eyelid spasms (McCann et al. 1999 ) . The initial motor sign may
be an increased frequency in blinking, particularly in response to a variety of common
stimuli including wind, sunlight, and air pollution. Many blepharospasm
patients wear sunglasses, even inside and on cloudy days, and have dif fi culty with
reading and driving.
As the disease progresses, excessive blinking is seen early and late due to overaction
of the eyelid protractor muscles, particularly the orbital portion of the orbicularis
oculi and the corrugators. Once the contractions are well established they may
be tonic and sustained, brief and clonic, or regular and rhythmic. Patients frequently
complain of retro-orbital discomfort at the time of the spasms (Shorr et al. 1985 ) .
Spreading of the spasms to midfacial and lower facial muscles is often seen.