Article Text

Download PDFPDF
Letter
Apraxia of lid opening due to a small lesion in basal ganglia: two case reports
  1. Masaki Hirose1,2,
  2. Hitoshi Mochizuki1,
  3. Mari Honma3,
  4. Toru Kobayashi4,
  5. Masatoyo Nishizawa2,
  6. Yoshikazu Ugawa1
  1. 1Department of Neurology, Fukushima Medical University, Fukushima, Japan
  2. 2Department of Neurology in Brain Research Institute, Niigata University, Niigata, Japan
  3. 3Department of Neurology, Masu Memorial Hospital, Fukushima, Japan
  4. 4Department of Neurosurgery, Hoshi General Hospital, Fukushima, Japan
  1. Correspondence to Dr Masaki Hirose, Department of Neurology, School of Medicine, Fukushima Medical University, 1 Hikarigaoka, Fukushima 960-1295, Japan; masakihirose1977{at}yahoo.co.jp

Statistics from Altmetric.com

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

The apraxia of lid opening (ALO), a synonym for apraxia of eye opening, is a disabling syndrome characterised by an inability to open the eyes at will, and patients sometimes try to open their eyes by contracting the frontalis muscles and touching their eye lids with their own fingers (sensory trick). This symptom is often associated with blepharospasm and/or ptosis and sometimes seen in patients with Parkinson's disease or other movement disorders. These reports suggest that ALO may be produced by some lesions at the basal ganglia.

To determine specific sites responsible for some symptoms, patients with the target symptoms resulting from a small cerebrovascular lesion usually can provide us with useful information. However, the ALO is rarely produced by a single cerebrovascular lesion.1 A few patients with ALO had a large ischaemic lesion at the non-dominant side in the middle cerebral artery territory.1 One case report described a small lesion at the putamen causing ALO.2 Here, we present two patients with ALO due to a small lesion in the basal ganglia and discuss mechanisms for ALO.

Patient 1: a 60-year-old woman

On 10 December 2008, patient 1 developed right hemiparesis and had difficulty in opening her eyes. A brain CT showed an intracerebral haemorrhage positioned between the left globus pallidus and thalamus (figure 1A). Her right hemiparesis was improved in a few days, but the difficulty in eye lid opening remained.

Figure 1

(A) Case 1. The brain CT demonstrated a intracerebral haemorrhage between the left globus pallidus and thalamus (volume of bleeding: 2 ml). (B) Case 2. The diffusion-weighted MRI (TE 1649 TR 664) showed two small infarctions (indicated by arrows) in the right thalamus (arrow 1) and a posterior limb of the right internal capsule (arrow 2). The high signals at the bilateral internal capsule (arrows 3) were not considered to be pathological, because such signals are often seen in normal subjects. (C, D) T2-weighted (TE 2048 TR 1024) and fluid-attenuated inversion recovery (TE 2048 TR 1024) MRIs 2 months after the onset. Two months later, a single high-signal lesion was demonstrated in the right thalamus. r, right side.

On neurological examination, she had difficulty in bilateral lid opening (especially after closing her eyes tightly). Neither blepharospasm nor ptosis was present. The surface electromyographic recordings when opening eyes after tight eye closure showed remarkable contraction of the frontalis muscles without any contraction of the orbicularis oculi muscles. This finding was compatible with the ALO. No extrapyramidal findings or signs of underlying Parkinsonism were observed. The Edinburgh handedness test revealed her right handedness.

Patient 2: an 86-year-old woman

Patient 2 had been regularly visiting the department of cardiovascular diseases of our hospital for atrial fibrillation and heart failure since 1989. On 30 June 2008, she noticed the left hemiparesis. The neurological examination revealed mild left hemiparesis and difficulty in bilateral lid opening. Neither blepharospasm nor ptosis was seen. The diffusion-weighted MRI showed small cerebral infarctions at the right thalamus and posterior limb of the internal capsule (figure 1B). The high signals in the bilateral internal capsule were not considered to be pathological because such signals are often seen bilaterally in normal subjects. Two months later, T2-weighted and FLAIR MRIs (figure 1C,D) showed a single high signal at the right thalamus.

The surface electromyographic recordings when opening eyes after tight closure showed remarkable contraction of the frontalis muscle without any contraction of the orbicularis oculi muscle. The Edinburgh handedness test revealed her right handedness.

We described two patients with ALO having neither blepharospasm nor ptosis. Our two cases showed bilateral apraxia of eye-lid opening. No other extrapyramidal findings or signs of underlying Parkinsonism were observed. It is conspicuous that a unilateral focal lesion produced bilateral ALO. Here, we discuss pathomechanisms for ALO from our patients.

The direct motor command for voluntary eye opening should be mediated by the corticobulbar tract from the primary motor cortex (M1). Some motor-related cortical areas including supplementary motor area (SMA) or premotor cortex may send a command to M1 for eye-lid opening. The basal ganglia may regulate the eye-opening system mainly in the SMA through the thalamus. Dysfunction of this regulation from basal ganglia may produce ALO because the ALO is often observed in Parkinson's disease or other diseases with Parkinsonian features and also in some patients with functional imbalance within the basal ganglia. The fact that l-DOPA sometimes improves the ALO3 also supports this hypothesis that some basal ganglia dysfunction causes ALO. A previous positron emission tomography (PET) study4 also supports the hypothesis of basal ganglia dysfunction. It showed hypometabolism in the medial frontal lobe in four patients with ALO, and the authors speculated that the abnormal interactions between the basal ganglia and the medial frontal lobe may cause ALO. The lesion sites in our two patients (globus pallidus and thalamus) and one reported patient (putamen)2 with ALO are also compatible with the notion that some basal ganglia system abnormality should cause ALO.

Another conspicuous finding of our patients is a unilateral lesion producing bilateral ALO. To the best of our knowledge, no patients have been reported to have unilateral ALO. The unilateral lesion for ALO was not always in the same side. Namely, it was in the non-dominant hemisphere in some patients and in the dominant hemisphere in others. One of our patients had a lesion at the non-dominant hemisphere. A few patients with ALO had large ischaemic lesions in the middle cerebral artery territory in the non-dominant hemisphere.1 Furthermore, the right (non-dominant) hemispheric supranuclear area controlled the bilateral levator palpebrae superioris.5 In contrast, two of four patients with ALO showed significant hypometabolism at the dominant-side basal ganglia,4 and one of our patients had a lesion at the dominant side. Based on these arguments, we cannot make any conclusion about the laterality about a lesion for ALO.

Acknowledgments

We thank Y Tanji and K Shimizu, Fukushima Medical University, for their technical assistance.

References

  1. 1.
  2. 2.
  3. 3.
  4. 4.
  5. 5.

Footnotes

  • Competing interests None.

  • Patient consent Obtained.

  • Ethics approval Ethics approval was provided by the Fukushima Medical University.

  • Provenance and peer review Not commissioned; externally peer reviewed.