Apraxia Of Eyelid Opening Research Articles

Botulinum toxin in the treatment of tremors, dystonias, sialorrhea and other symptoms associated with Parkinson’s disease

Botulinum toxins are an effective treatment modality for a growing number of neurologic conditions. Although there has been varied interest and success in their use, they have been studied for a variety of conditions associated with Parkinson’s disease. Conditions reviewed in this paper include hand and jaw tremor, dystonia, blepharospasm and apraxia of eyelid opening, bruxism, camptocormia, freezing of gait, sialorrhea and constipation. We will make comments when applicable on our unique experience with botulinum toxin in these conditions. Other conditions associated with Parkinson's disease, which will not be reviewed here, but may benefit from botulinum toxin treatment include anterocollis (also known as dropped head syndrome), hyperhidrosis, seborrhea and overactive bladder.

Botulinum toxin A injections improve apraxia of eyelid opening without overt blepharospasm associated with neurodegenerative diseases

Botulinum toxin A injections improve apraxia of eyelid opening without overt blepharospasm associated with neurodegenerative diseases

Modified Lundie Loops Improve Apraxia of Eyelid Opening

Current treatments are unsatisfactory for improving apraxia of eyelid opening, defined as a delay or inability to open closed eyelids voluntarily in the presence of intact motor pathways. Improvement in functional health was assessed using the Blepharospasm Disability Scale (BDS) in five consecutive patients with apraxia of eyelid opening treated with wire loops affixed behind ordinary spectacles (Lundie loops) and modified to provide pressure on the brow as a stimulus to keep the eyelids elevated. All five patients showed improvement in BDS scores. The mean percentage of normal activity of the study population improved from 25% to 37.6%. Outdoor activities were not significantly altered with the use of the device. Modified Lundie loops appear to be helpful in improving the functional health of patients with eyelid apraxia. These results will need to be verified in larger trials.

Dystonia redefined as central non-paretic loss of control of muscle action: A concept including inability to activate muscles required for a specific movement, or ‘negative dystonia’

Dystonia is defined as a syndrome of sustained muscle contractions, frequently causing twisting and repetitive movements, or abnormal postures. Although this definition comprises an essential feature of dystonia, the clinical observation indicates that there is an additional aspect of dystonia; failure to adequately activate muscles required for specific movement, exemplified by the lack of contractions of the levator palpebrae superioris muscles in apraxia of lid opening, as well as by inability to activate appropriate muscles in cervical dystonia or in the paretic form of writer's cramp, and possibly by dropped head syndrome or camptocormia seen in parkinsonian patients without apparent truncal dystonia or rigidity. Taking this "negative dystonia" into consideration, the author proposes a revised definition of dystonia as a symptom characterized by the central non-paretic loss of voluntary control of muscle activities, which may result in either excessive or deficient contractions of muscles, frequently causing twisting and repetitive movements, limitation of movements, or abnormal postures.

Apraxia of Eyelid Opening: Clinical Features and Therapy

Botulinum toxin injection is the treatment of choice in cases of benign essential blepharospasm. However, about 10% of the patients do not get sufficient effect from this treatment, and many of them have concomitant apraxia of lid opening. Over a 3-year period we treated 12 patients. Three had pure apraxia of lid opening and in the other nine it was associated with blepharospasm. All patients were initially treated with botulinum toxin injections with poor results. They underwent surgical treatment like blepharoplasty, limited myectomy, aponeurosis repair, and/or frontalis suspension. Some of them needed post operative botulinum toxin injections in the pretarsal part of orbicularis oculi muscles. This combined therapy gave good functional and aesthetic results. The specific causes of blepharospasm and apraxia of lid opening are unknown, but these two conditions coexist in some patients and can be difficult to treat. It is important to make a correct diagnosis, and a combined surgical and botulinum toxin treatment can be very effective.

Apraxia of lid opening

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.

Stimulation of the subthalamic nucleus in Parkinson’s disease: a 5 year follow up

Background: The short term benefits of bilateral stimulation of the subthalamic nucleus (STN) in patients with advanced levodopa responsive Parkinson’s disease (PD) are well documented, but long term benefits are...

Apraxia in movement disorders

The definition of apraxia specifies that the disturbance of performed skilled movements cannot be explained by the more elemental motor disorders typical of patients with movement disorders. Generally this does not present a significant diagnostic problem when dealing with 'higher-level' praxic disturbances (e.g. ideational apraxia), but it can be a major confound in establishing the presence of limb-kinetic apraxia. Most motor disturbances characteristic of extrapyramidal disorders, particularly bradykinesia and dystonia, will compromise the ability to establish the presence of loss of dexterity and deftness that constitutes this subtype. The term 'apraxia' has also been applied to other motor disturbances, such as 'gait apraxia' and 'apraxia of eyelid opening', that perhaps are misnomers, demonstrating the lack of a coherent nomenclature in this field. Apraxia is a hallmark of corticobasal degeneration (CBD) and historically this has received the most attention among the movement disorders. Corticobasal degeneration is characterized by various forms of apraxia affecting limb function, particularly ideomotor apraxia and limb-kinetic apraxia, although buccofacial and oculomotor apraxia can be present as well. The syndrome of parkinsonism and prominent apraxia, designated the 'corticobasal syndrome' (CBS), may be caused by a variety of other central nervous system pathologies including progressive supranuclear palsy (PSP), Alzheimer's disease, dementia with Lewy bodies and frontotemporal dementias. Distinct from the CBS, PSP and Parkinson's disease can demonstrate varying degrees of apraxia on selected tests, especially in those patients with more severe cognitive dysfunction. Diseases that cause the combination of apraxia and a primary movement disorder most often involve a variety of cerebral cortical sites as well as basal ganglia structures. Clinical-pathological correlates and functional imaging studies are compromised by both this diffuse involvement and the confusion experienced in the clinical evaluation of apraxia in the face of the additional elemental movement disorders. Finally, although apraxia results in clear disability in patients with the CBS, it is not clear how milder ideomotor apraxia found on specific testing contributes to patients' overall day-to-day motor disability.

Assessment of Blepharospasm Surgery Using an Improved Disability Scale: Study of 138 Patients

To assess a new scale for grading functional disability in a series of patients operated on for blepharospasm resistant to treatment by botulinum toxin (BT) injections. The bicentric study included 138 patients: 80 (58%) with essential blepharospasm; 46 (33%) with apraxia of eyelid opening; and 12 (9%) with intermediate forms. They had undergone orbicularis myectomy (92 cases, 67%), frontalis suspension (31 cases, 22%), or both (15 cases, 11%). Functional disability was assessed by using an original scale covering 6 daily life activities, each graded using 5 levels from 0 (no disability) to 4 (activity impossible). The score obtained was related to the total of activities actually performed to produce a functional disability score (FDS) from 0 to 100. Each patient's FDS was prospectively determined, before surgery and 3 months after surgery (M3). In patients who, after surgery, required a further course of BT injections, the FDS was assessed immediately before the third injection. The mean FDS fell significantly, from 78 +/- 15 (standard deviation) before surgery to 45 +/- 21 at M3 (p < 0.01%), whatever the clinical form of essential blepharospasm or surgical procedure. The mean postoperative FDS was significantly lower in the 62 patients (45%) weaned off BT than in the unweaned group, respectively (31 +/- 17 versus 56 +/- 18; p < 0.01). In the latter group, the mean FDS after BT reinjection (34 +/- 19) fell to a level comparable with that of the weaned patients. This accurate, easy-to-use scale makes it possible to quantify in patients with blepharospasm functional improvement as the result of surgery and, where applicable, of BT reinjection.

Clinical Analysis of Blepharospasm and Apraxia of Eyelid Opening in Patients with Parkinsonism

Background and PurposeBlepharospasm (BSP) and apraxia of eyelid opening (AEO) have been reported as dystonia related with parkinsonism. However, systematic analysis of clinical characteristics of BSP and AEO in parkinsonism has been seldom reported. To investigate the clinical characteristics of BSP and AEO in parkinsonism and to find out the clinical significance to differentiate parkinsonism.MethodsWe enrolled 35 patients who had BSP with or without AEO out of 1113 patients with parkinsonism (913 IPD, idiopathic Parkinson's disease; 190 MSA, multiple system atrophy, 134 MSA-p, 56 MSA-c and 10 PSP, progressive supranuclear palsy). We subdivided MSA into MSA-p (predominantly parkinsonism) and MSA-c (predominantly cerebellar) according to the diagnostic criteria proposed by Quinn. We analyzed the clinical features of BSP and parkinsonism including onset age, onset interval to BSP, characteristics of BSP, presence of AEO, coexisted dystonias on the other body parts, severity of parkinsonism and relationship with levodopa treatment.ResultsBSP with or without AEO were more frequently observed in atypical parkinsonism (PSP, 70%; MSA-p, 11.2%; MSA-c, 8.9%) than in IPD (0.9%). Reflex BSP was observed only in atypical parkinsonism (4 MSA-p, 1 MSA-c and 2 PSP). BSP preceding parkinsonism (Pre-BSP) was observed mainly in atypical parkinsonism (2 MSA-p, 1 MSA-c, 1 PSP and 1 IPD). The presence of AEO was more frequent in atypical parkinsonism than in IPD, but isolated AEO was not detected. BSP related to levodopa ('off' symptom or 'peak-dose' effect) were observed only in IPD.ConclusionsReflex BSP, Pre-BSP and the presence of AEO may be a unique feature of atypical parkinsonism. BSP related to levodopa might be representative of IPD. No differences were found in the clinical features of BSP between MSA-p and MSA-c.

Blefaroespasmo y blefarocolisis. Caras diferentes de una misma moneda

The eyelid movements are mediated mainly by the orbicularis oculi (OO) and the levator palpebrae superioris (LPS) muscles. Dissociated upper lid functions exhibit different counterbalanced action of these muscles, and in blinking they show a strictly reciprocal innervation. The disturbance of this close LPS-OO relationship likely leads to many of the central lid movement disorders.Two major lid postural disorders, the blepharospasm (BSP) and the blepharocolysis (BCO), share two different clinical aspects: the involuntary eyelids' closure along with the inability to open the eyes. BSP consists of an involuntary overactivity of the OO, with LPS co-contraction activity, and is expressed as frequent and prolonged blinks, clonic bursts, prolonged tonic contraction or a blend of all of them. BCO (commonly named 'so-called lid opening apraxia') is an involuntary overinhibition of the LPS muscles with no evidence of ongoing OO activity; it exists a co-inhibition of these muscles. BSP and BCO occur in many instances of idiopathic dystonias and basal ganglia diseases and, less frequently, in rostral brainstem lesions. Both may coincide in the same patient.BSP and BCO should be considered different expressions of the dystonic focal eyelid movement disorders, related to the increase of the two counteracting components of the normal eyelid motor and blinking patterns: the excessive OO activation in the BSP; the excessive LPS inhibition in the BCO.

Eyelid movements in health and disease. The supranuclear impairment of the palpebral motility

The eyelid movements are mediated mainly by the orbicularis oculi (OO) and the levator palpebrae superioris (LPS) muscles. Dissociated upper lid functions exhibit different counterbalanced action of these muscles, and in blinking they show a strictly reciprocal innervation. The disturbance of this close LPS–OO relationship likely leads to many of the central lid movement disorders. Three groups of supranuclear motor impairment of lid movements are considered: the disorders of the lid-eye movements’ coordination, the disturbances of blinking and lid “postural” maintenance, and the alteration of voluntary lid movements. Nuclei of the posterior commissure control the inhibitory modulation of LPS motor-neuronal activity and they are involved in the lid-eye coordination disorders such as lid retraction, which is observed in the Parinaud’s syndrome and also in parkinsonism and progressive supranuclear palsy. Spontaneous (SB) and reflex blinking consist of two components: the inhibition of the basal tonic LPS activity, which keeps the eyes open, and the concurrent activation of the OO muscles. LPS inhibition precedes and outlasts the OO activation. This normal configuration is impaired in parkinsonism and blepharospasm (BSP). SB shows a highly interindividual rate variation (among 10–20 per minute in adults) and abnormal blink rates occur in neurological diseases related to dopaminergic transmission impairments. Lid postural abnormalities include involuntary eyelid closure, which is usually associated with inability to open the eyes. Two major disorders share these two aspects: BSP and blepharocolysis (BCO). BSP consists of an involuntary overactivity of the OO, with LPS co-contraction activity, and is expressed as frequent and prolonged blinks, clonic bursts, prolonged tonic contraction or a blend of all of them. BCO (commonly named “so-called lid opening apraxia”) is an overinhibition of the LPS with no evidence of ongoing OO activity. BSP and BCO occur in many instances of idiopathic dystonias and basal ganglia diseases and, less frequently, in rostral brainstem lesions. Both may coincide in the same patient. Voluntary lid movement disorders comprise the impairment of Bell’s phenomenon, the voluntary eyelid closure palsy and the so-called cerebral ptosis, all related to lesions of frontal cortical areas and/or the corticospinal system.

Post-encephalitic segmental dystonia with apraxia of eyelid opening

We describe a 34 year old woman presenting with a 25 year history of segmental dystonia with apraxia of eyelid opening, orofacial and focal upper limb dystonia following an episode of meningoencephalitis. Post-encephalitic apraxia of eyelid opening has not been previously reported in the absence of parkinsonian features.

Primary blepharospasm: diagnosis and management.

Primary blepharospasm is an adult-onset focal dystonia characterised by involuntary contractions of the orbicularis oculi muscles. Patients may have various types of movements arising from the different parts of the orbicularis oculi muscle. These include typical blepharospasm associated with Charcot's sign, pretarsal blepharospasm and flickering of the eyelids. Primary blepharospasm may be associated with so-called apraxia of eyelid opening as well as dystonia in the lower face, jaw or cervical muscles. Unless there are clinical clues to a symptomatic cause, adults presenting with blepharospasm do not require extensive aetiological investigation because the condition is rarely due to an identifiable condition. As the aetiology of primary blepharospasm is largely unknown, therapeutic approaches are symptomatic, with type A botulinum toxin being the treatment of choice.

Glucose hypometabolism in medial frontal cortex of patients with apraxia of lid opening.

To determine whether regional cerebral functional abnormalities exist in patients with apraxia of lid opening (ALO). Cerebral glucose metabolism was examined by positron emission tomography (PET) in 11 patients (8 women and 3 men, age 48-69 years); 10 with ALO accompanied by blepharospasm and 1 patient with pure ALO. Eleven normal volunteers (6 women and 5 men, age 45-66 years) were examined as controls. A comprehensive ophthalmological examination, magnetic resonance imaging (MRI), and PET were performed. The cerebral glucose metabolism was evaluated by the relative uptake of [fluorine-18]fluorodeoxyglucose by PET. The mean +/- two standard deviations of the normal controls was defined as the normal range for cerebral glucose metabolism. MRI revealed no particular lesion except for an infarction in the unilateral basal ganglia in two patients. Decreased glucose metabolism was observed in a wide area of the medial frontal lobe (six cases) and primary visual cortex (PVC) (four cases). Group multiple comparisons revealed a significant decrease ( P<0.0035) in the bilateral anterior cingulate gyrus, left supplementary motor area (SMA), and bilateral PVC. The results support the hypothesis that ALO is associated with hypofunction in the SMA and/or anterior cingulate gyrus.

Frequency of apraxia of eyelid opening in the general population and in patients with extrapyramidal disorders.

We ascertained the prevalence of apraxia of eyelid opening (AEO) in a community located in Puglia, a region of southern Italy. The crude prevalence rate was 59 per million (95% confidence interval, 24-173). AEO coexisted with adult onset blepharospasm in 75% of cases, with atypical parkinsonism in 25% of cases. Among the overall patient population seen at our movement disorders clinic from 1987 to 1997, AEO was isolated in 10 otherwise healthy individuals, associated with adult-onset dystonia in 13 cases, and associated with a parkinsonian syndrome in 9 cases. The frequency of AEO was 10.8% in the dystonia group, and 2.1% in the overall parkinsonian group (Parkinson's disease, 0.7%; progressive supranuclear palsy, 33.3%). In two patients with possible progressive supranuclear palsy, AEO worsened after increasing levodopa dosage or acute apomorphine challenge and disappeared following levodopa discontinuation. AEO developing in the setting of a parkinsonian syndrome may be either disease- or drug-related.

Tozlovanu V, Forget R, Iancu A, et al. Prolonged orbicularis oculi activity. A major factor in apraxia of lid opening.

Tozlovanu V, Forget R, Iancu A, et al. Prolonged orbicularis oculi activity. A major factor in apraxia of lid opening.

Prolonged orbicularis oculi activity. A major factor in apraxia of lid opening.

Background: Apraxia of lid opening (ALO) is a nonparalytic inability to open the eyes at will in the absence of visible contraction of the orbicularis oculi (OOc) muscle. Objective: To test the hypothesis that sustained activity of the OOc is a major factor in the difficulty in opening the eyes in this condition. Methods: Lid movement detected in an electromagnetic field and electromyogram activity of the septal and pretarsal portions of the OOc were recorded in 12 healthy control subjects and 12 patients with a clinical diagnosis of ALO. The latencies to onset and to complete eye opening and the time during which eye opening was sustained were measured and analyzed in relation to OOc activity. Results: The lid opening latencies and the lid movement duration were significantly increased in patients compared with control values. An abnormal persistence of OOc activity was present in 10 of the 11 patients with a delay in complete lid opening. The complete lid opening delay showed a strong positive correlation with the time it took to inhibit the OOc activity. This relationship fit the same linear regression in both groups. Conclusions: Patients with ALO show significant delays in eye opening. An abnormal persistence of OOc activity, detectable electromyographically but not clinically, could be the main factor contributing to the delay in lid opening in these patients.

An unusual case of paroxysmal kinesigenic dyskinesia

Paroxysmal kinesigenic dyskinesia (PKD) is an uncommon neurological disorder characterised by abnormal episodic brief movements induced by sudden movements of the body. The recognition and understanding of this disorder has increased over the past few decades. While most cases are idiopathic, the as sociation of PKD with various disorders, including metabolic abnormalities has also been reported. We report an interesting case of a 52 year old male who presented with PKD manifesting as subtle facio-brachial movements and apraxia of eyelid opening (AL O) secondary toidiopathic hypoparathyroidism.

Prolonged orbicularis oculi activity: a major factor in apraxia of lid opening.

Apraxia of lid opening (ALO) is a nonparalytic inability to open the eyes at will in the absence of visible contraction of the orbicularis oculi (OOc) muscle. To test the hypothesis that sustained activity of the OOc is a major factor in the difficulty in opening the eyes in this condition. Lid movement detected in an electromagnetic field and electromyogram activity of the septal and pretarsal portions of the OOc were recorded in 12 healthy control subjects and 12 patients with a clinical diagnosis of ALO. The latencies to onset and to complete eye opening and the time during which eye opening was sustained were measured and analyzed in relation to OOc activity. The lid opening latencies and the lid movement duration were significantly increased in patients compared with control values. An abnormal persistence of OOc activity was present in 10 of the 11 patients with a delay in complete lid opening. The complete lid opening delay showed a strong positive correlation with the time it took to inhibit the OOc activity. This relationship fit the same linear regression in both groups. Patients with ALO show significant delays in eye opening. An abnormal persistence of OOc activity, detectable electromyographically but not clinically, could be the main factor contributing to the delay in lid opening in these patients.