apraxia of eyelid opening

Lid watching, a lost art!

Dr. Marie-Hélène Marion

 

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The diagnosis of blepharospasm is not always easy, in particular in case of pretarsal blepharospasm, also called Apraxia of eyelid opening, mistaken quite often for ocular myasthenia.

1- what is ocular myasthenia?

Myasthenia is an autoimmune disease, responsible of muscle weakness, affecting the all body and when localized to the muscles around the eyes, called ocular myasthenia.

Ocular myasthenia can present with isolated droopy eyelids (medically called ptosis), due to a weakness of the levator muscles of the eyelids, the Levator Palpebrae, but is often associated with double vision, due to weakness of the oculomotor muscles. The weakness is worst at the end of the day, due to the fatigue of the myasthenic muscles.

The diagnosis of ocular myasthenia is based on clinical findings, the presence of antibodies in the serum (30 to 60% cases) and the abnormal response of the muscles on repetitive electrical stimulation. Clinically the doctor can look for the “peek sign” by asking the patient to perform a sustained gentle eyelid closure; the fatigue of the orbicularis oculi will be responsible of a slight opening of the eye, as the patient peeking.! ( J. Glaser).

2- why so many patients with Blepharospasm are misdiagnosed with ocular myasthenia?

-Blepharospasm patients can have a misleading presentation of ptosis, with intermittent droopy eyelids, due to the spasms of the pretarsal portion of the orbicularis oculi muscles (also described as pretarsal BSP) pulling down the eyelid like the string of a roller blind down to the window.

-The words that patients used to describe their symptoms such as “my eyes are tired”, “my eyelids feel heavy”, “I feel more comfortable eyes closed” can indicate wrongly a muscle weakness of the eyelids, and reflect in fact the loss fight of the patients against the dystonic spasms closing their eyes.

– the variability of the symptoms through the day ; in myasthenia the patient is worst at the end of the day; in BSP the patient is worst walking outdoors, driving, watching TV, looking up and with any bright light and dazzy winter light.

– A levator palpebre detachement can occurs on some patients after years of spasms, and pulling on the muscle insertion

3-the art of lid watching

The famous neuro-ophtalmologist, J Glaser talked about “ Lid watching, a lost art!” Joel Glaser in Handbook of neuro-ophthalmology, 1999

-Lid watching is optimal when patients are keeping silent; BSP patients have an increased rate of blinking when being quiet and vice versa a low blinking rate when speaking. It’s the opposite of what observed in normal subjects. That may explain why patients find easier to keep her eyes opened when speaking and choose to sing when driving.

-In the case of pretarsal BSP careful lid watching along the eyelashes, looking for a pulling down spasms of the eyelids is a good indicator of pretarsal spasms.

– In addition, the lid closing spasms may be associated with Bell’s phenomenon with elevation of the eyeball, so it’s important to look not only at the eyelid but also the eyeball

– In case of levator palpebre detachment, the forceful opening of the eyes on command will be limited;

– Also the patients sometimes can’t reopen their eyes, after a spontaneous blink or after a closing spasm. It can be for a fraction of second or for up to few minutes; the patient will flicker his eyelashes, or his eyelids to kick them opened. Sometimes it will be a forceful pulling of the eyelids with his fingers, stretching the skin around the eyes, even sometimes resulting in bruising.

All these signs found on careful lid watching are good indicators of eyelids spasms, and not eyelid droopiness. They also direct the hands of the injector to the maximum spasms to achieve optimal results.

In the movement disorders field, most of the diagnosis are made by watching the movement; it’s particular true for eyelid dystonia and vocal cords dystonia where immobility does not always equal paralysis but also permanent tension due to the dystonia; one day I will ask my ENT colleagues to tell us about the art of vocal cords watching!!!

Marie-Helene Marion

 

The diagnosis of Blepharospasm is always delayed!

Dr. Marie-Hélène MarionLeave a comment

     The diagnosis of Blepharospasm is always delayed!

Blepharospasm is a focal adult- onset dystonia, responsible of an involuntary eye closure. It can start with an increased blinking explained by dry eyes, gritty eyes or intolerance to bright lights. Gradually the patient, more often a woman around her 60’s, complains of difficulty to watch TV, to drive at night or just to walk outdoors on a cloudy day

At that stage, surprisingly the diagnosis is not easily done. Why?

1-    The GP refers this lady to the eye clinic; the diagnosis of blockage of the lacrymal ducts, or of blepharitis (with an inflamation of the eyelids) are much more common conditions and often the first to be considered.

2-    The patient does not spontaneously mention that the eyes are involuntary closing. They more often talk about their heavy eyelids, or tired eyes, or intolerance to bright light (also called photophobia)

3-    The patient has often the eyes well opened when speaking and the doctor can’t document any forceful eye spasms during the clinic.

This explains that the diagnosis of dystonia (Blepharospasm, Cervical dystonia) is usually made after 5.4 years on average after onset of symptoms and at least after seeing 3 different consultants (Canadian survey of Dr Jog ).

The patient will benefit to come to the first clinic with a relative or a friend who may describe it more accurately as an external observer. I also ask my patients to stop talking and be silent for few minutes, fixing a visual target in the room; the spasms with forceful eye closure will occur 2 to 3 minutes later. It’s worthwhile waiting as it’s a great opportunity not only to make the diagnosis but also to identify the type of Blepharospasm.

 Hope this blog will contribute to an earlier diagnosis of Blepharospasm!

 Reference: Causes of treatment delays in dystonia and hemifacial spasm: a canadian survey. Jog M et al, Can J Neurol Sci 2011: 38:704-11

Are all the blepharospasms the same?

Dr. Marie-Hélène MarionLeave a comment

Are all Blepharospasms the same?

 BSP can vary from one patient to another and also change overtime after few sessions of Botulinum toxin injections.In daily life, we can close our eyes in different ways; for instance when we are in the shower and we get shampoo into our eyes (by the way I am not sure that shampoo are still stinging), we spontaneously frown, screw and close our eyes very tight. On the other hand when we go to sleep, we pulled down very gently our eyelids, which feel heavy. In case of a patient with BSP, there is the same diversity; the eyes can closed very tight with a forceful spasm of or closed with only the eyelids pulling down, despite sometimes one finger trying to hold it up.

The orbicularis oculi muscle which is the muscle responsible of closing the eyes are organized in 3 circular parts: an inner circle (the pretarsal part responsible of the eyelid going down), a middle circle (the preseptal part) and an external circle (the orbital part); these 3 parts can contract independently.In the situation of the shampoo in the eyes, it’s the orbital part, which is contracting. When sleeping, it‘s only the pretarsal portion of the orbicularis oculi, which is involved, pulling down the eyelid.

When treating the patient with injection of Botulinum toxin, the sites of the injections have to be placed in the right portion of the orbicularis oculi muscle depending of the type of BSP.  The treatment needs to be customized for each individual, not very far from the world of the haute couture!