Annals of Indian Academy of Neurology
CASE REPORT
Year
: 2006  |  Volume : 9  |  Issue : 3  |  Page : 163--165

Apraxia of lid opening


Y Kingsly Jebasingh, K Bijoy Menon, M Jawahar, S Gobinathan, S Balasubramanian, KR Shanbhogue 
 Institute of Neurology, Madras Medical College, Chennai, India

Correspondence Address:
K R Shanbhogue
Institute of Neurology, Madras Medical College and Govt. General Hospital, Chennai - 600 003
India

Abstract

Apraxia of lid opening is a condition where patients do not have ptosis but have difficulty in overcoming levator palpebrae inhibition. We report a patient who presented with difficulty in opening eyelids with out diurnal variation, ptosis or blepharospasm. The diagnosis of Apraxia of lid opening is confirmed by electro physiology. The possibility of apraxia of lid opening should be considered in patients who present with difficulty in opening eyes. Various causes of Apraxia of lid opening are discussed.



How to cite this article:
Jebasingh Y K, Menon K B, Jawahar M, Gobinathan S, Balasubramanian S, Shanbhogue K R. Apraxia of lid opening.Ann Indian Acad Neurol 2006;9:163-165


How to cite this URL:
Jebasingh Y K, Menon K B, Jawahar M, Gobinathan S, Balasubramanian S, Shanbhogue K R. Apraxia of lid opening. Ann Indian Acad Neurol [serial online] 2006 [cited 2021 Jan 16 ];9:163-165
Available from: https://www.annalsofian.org/text.asp?2006/9/3/163/27659


Full Text

 Introduction



Apraxia of eyelid opening refers to inability to open the eyes voluntarily in the absence of ptosis or blepharospasm.[1] Patients who suffer from this problem do not have true ptosis but have difficulty in overcoming levator palpabrae inhibition. To overcome this problem patients must thrust their head backwards or should open their eyes manually. We report a case of apraxia of eyelid opening; which was confirmed by electrophysiology studies.

 Case Report



A 38-year-old lady presented with a 4 years history of difficulty in opening eyes after voluntary closure of eyes. There was no history of diurnal variation, fatiguability, diplopia periorbital pain or swelling of eyelids. There was no history of motor weakness sensory, bladder, bowel or autonomic disturbances. Patient did not suffer from headache, vomiting or seizures. She was treated with neostigmine elsewhere as there was a suspicion of Myasthenia gravis. There was no improvement hence the patient was referred to our center and evaluated.

General examination did not reveal any abnormality or contributory clinical findings. Neurological evaluation showed normal higher mental function and cranial nerves. Extra ocular movements, pupil, optic fundi were normal. Spinomotor system was normal and there was no fatiguability. Sensory examination was normal. There were no cerebellar signs or involuntary movements. Patient had excessive wrinkling of fore head while she attempted to open the eyes, which showed over action of frontalis.

Investigations showed normal haemogram. Other routine laboratory investigations including blood glucose, electrolytes, renal and liver function tests were normal. Serum copper and ceruloplasmin were within normal limits. K.F ring and peripheral smear for acanthocytes were negative. E.C.G was within normal limits. Chest X-ray was normal. MRI brain both plain and contrast were normal as seen in [Figure 1]. Nerve conduction study showed normal distal latency, compound muscle action potential amplitude and F wave latency and conduction velocity in all nerves bilaterally. Sensory nerve action potentials were normal. Repetitive nerve stimulation of the facial nerve showed no decremental response. EMG with surface electrodes showed normal MUPs and recruitment of orbicularis oculi during eye closure, on attempted eye opening there was no MUP or recruitment. Surface EMG of the Frontalis muscle during eye closure did not evoke any MUPs. Unlike healthy persons there was recruitment of MUPs from Frontalis Muscle on attempted eye opening.

 Discussion



Normally during the closure of eyes the orbicularis oculi contracts and levator palpabrae muscle will be in the inhibited state. During attempted eye opening, the levator palpabrae muscle contracts and orbicularis oculi will be in the inhibited state without any activity in the Frontalis.

Patients with apraxia of eyelid opening have normal eyelids, but have difficulty in opening the eyelids due to persistent levator palpabrae inhibition or due to an abnormal persistence of orbicularis activity, detectable electromyographically.[2] During eye opening, the orbicularis oculi will be in a relaxed state and levator palpabrae inhibition persists, hence the frontalis overacts to open the eyes. Apraxia of eyelid opening is commonly associated with blepharospasm. Pure Apraxia of eyelid opening is very rare. A patient with Apraxia of lid opening with associated blepharospasm will typically have spasmodic closure of eyelids, which is beyond the control of the patient. The blepharospasm and the associated levator palpepbrae inhibition together prevent the patient from opening his eyes.[3]

Ever since Liepmann's original descriptions at the beginning of the century, apraxia has usually been attributed to damage confined to the cerebral cortex and or cortico-cortical connecting pathways. However, there have been suggestions that apraxia can be due to deep subcortical lesions, which raises the question as to whether damage to the basal ganglia or thalamus can cause apraxia. The main conclusion drawn from a meta-analysis of 82 cases[4] reported in the literature are that lesions confined to the basal ganglia rarely cause apraxia, a basal ganglia lesion that intrudes into the adjacent lateral white matter leads to apraxia. This suggests that apraxia was most commonly seen when there were lesions in the periventricular or peristriatal, white matter that involve association fibres, in particular those of the superior longitudinal fasciculus and frontostriatal connections.

Surface EMG in our patient shows normal orbicularis oculi activity and a normally absent frontalis activity during eyelid closure. However, in the eyelid opening task, there is no activity in the orbicularis oculi. This is expected normally and rules out blepharospasm. Activity in the frontalis alone during eyelid opening suggests that our patient has apraxia of eye lid opening alone without blepharospasm. The repetitive nerve stimulation studies in the orbicularis oculi and nasalis showed a normal response. These electrophysiological tests in our patient suggest the possibility of pure apraxia of eyelid opening, though in the absence of needle EMG studies of the levator palpebrae during eyelid opening and closure, they are not confirmatory. We could not do needle EMG studies in the levators as it is technically very demanding. Our patient was treated with Clonazepam 1 mg at bedtime, which showed a good response as shown in [Figure 2].

These are Idiopathic, Secondary right (or) bihemispherical lesions,[5] Parkinson's disease, Huntington's disease, Pallido ponto nigral degeneration. Shy drager syndrome, Wilson's disease, Hallerverden Spatz disease, Neuroacanthocytosis, Motor neuron disease, Post thalamotomy syndrome. In Cortico basal ganglionic degeneration patient may have apraxia of lid opening and closure.[6] In our patient all the secondary causes of apraxia of eyelid opening were reasonably ruled out with relevant investigations. It is likely that this patient has idiopathic apraxia of lid opening with out blepharospasm. This case is presented for its rarity.

References

1Lepore FE, Duovoison RC. Apraxia of eyelid opening: An involuntary levator inhibition. Neurology 1985;35:423-7.
2Prolonged orbicularis oculi activity: A major factor in apraxia of lid opening Viorika Tozlovanu, Robert Forget andreea Iancu; and Dan Boghen, Neurology 2001;57:1013-8.
3Paul W, Brazis MD. Localization in clinical neurology. 4th ed. p. 253.
4Pramstaller PP, Marsden CD. The basal ganglia and apraxia. Brain 1996;119:319-40.
5Johnston JC, Rosenbaum DM, Picone CM, Grotta JC. Apraxia of eyelid opening secondary to right hemispherical infarction. Ann Neurol 1989;25:622-4.
6Riley DE, Land AE, Lewis A, Resch L, Ashby P, Hornykiewicz O, et al . Cortical-basal ganglionic degeneration. Neurology 1990;40:1203-12.