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Biomedical subjects

J S Elston

Publications and source records attributed to J S Elston.

At least 19 recordsLinked to original sources

The management of blepharospasm and hemifacial spasm.

The aetiology of blepharospasm and hemifacial spasm is different, but both produce involuntary eye closure and facial movements which do not respond to systemic drug treatment. The introduction of therapeutic focal muscle weakening with botulinum toxin injections in the early 1980s appeared to offer great promise in the management of these conditions. In this paper the results of botulinum toxin treatment of 234 patients with blepharospasm and 73 patients with hemifacial spasm over a 7-year period have been analysed. Most patients receive sustained benefit from repeated injections whilst side-effects become less frequent. A clinically recognisable subgroup of patients with blepharospasm respond poorly and may be better treated surgically.

Blepharoptosis

Amaurosis fugax in young people.

Nine young adults (median age 19.5 years) who suffered from amaurosis fugax (AF) are described. The attacks of AF were short in duration and preceded by premonitory symptoms in five cases and by a migrainous headache in two. In five patients the visual loss progressed in a lacunar pattern unlike the 'curtain' pattern characteristic of AF in older patients. Investigation revealed no evidence of an embolic or atheromatous aetiology. In two cases a minor abnormality was found on echocardiography. We conclude that AF in young adults has a different clinical pattern and may have a different aetiology, possibly migrainous, compared with that seen in older patients. The pattern of visual loss in some of the cases suggests that the choroidal circulation rather than the retinal circulation is primarily affected.

Adolescent

One stage adjustable sutures: practical aspects.

Strabismus surgery using one stage adjustable sutures was carried out under local anaesthesia in six patients with good results. The criteria for patient selection and the surgical technique are discussed.

Adult

Clinical evidence for the onset of the sensitive period in infancy.

Seven neonates had a IIIrd or VIth nerve palsy or afferent visual pathway pathology at birth. These abnormalities resolved within 6 weeks and the children have developed normal visual acuity, motor fusion, and stereopsis. We conclude that there is a latent period of 6 weeks before the onset of the sensitive period.

Abducens Nerve

A new variant of blepharospasm.

Ten patients who were unable to initiate or sustain eye opening in the absence of overt spasm of the orbicularis oculi, were investigated. In five, the problem was isolated. Three had Parkinson's disease and two progressive supra-nuclear palsy for between one to six years before the eye opening difficulty developed. The clinical features and electrophysiological investigation suggested that the disorder is a variant of blepharospasm due to abnormal contraction in the pre-tarsal orbicularis oculi.

Aged

Heterogeneity in dominant anterior segment malformations.

Peters' anomaly is usually a sporadic or autosomal recessive condition. We present three families whose members had dominantly inherited anterior segment anomalies with variable expression, including typical Peters' anomaly in at least one family member. Slit-lamp examination of parents and family members of children with Peters' anomaly is therefore important to exclude dominant inheritance.

Abnormalities, Multiple

Extraocular muscle surgery in myasthenia gravis.

Myasthenia gravis is typically a disease of young people in active employment who need a field of binocular single vision. Although it is systemically controllable with a good chance of spontaneous remission, persistent loss of binocularity may cause chronic disability. We report our experience of extraocular muscle surgery in five patients with stable myasthenia gravis and persistent double vision. Extraocular muscle involvement was selective, giving rise to incomitant and concomitant squints, with individual muscle overactions as well as underactions. Treatment was by conventional recession and resection procedures with the additional use of Faden and adjustable sutures where appropriate. In all five cases a larger, stable field of binocular single vision was established. It is concluded that extraocular muscle surgery may be beneficial in selected cases of myasthenia gravis.

Adolescent

Saccadic eye movements in essential blepharospasm.

To provide evidence of an organic pathology for essential (idiopathic) blepharospasm, reflex saccadic eye movements in response to randomly stepped visual targets were assessed in seven affected patients and seven age-matched controls using the magnetic scleral search coil technique. The results indicate a significant prolongation in latency and a reduction in gain of horizontal saccades, and an increase in latency and reduction of peak velocity of large downward saccades. These findings suggest an organic component to the aetiology of blepharospasm, the pathology of which also appears to involve the oculomotor system. The pattern of the oculomotor disorder does not allow specific localisation but is consistent with the underlying pathology being localised in the basal ganglia.

Adult

Botulinum toxin A in clinical medicine.

The results of the treatment with botulinum toxin A injection of 234 patients with blepharospasm and 73 with hemifacial spasm were reviewed. Visual function improved in the majority of patients with blepharospasm, and the improvement was sustained for up to 40 sets of injections. Mid and lower facial movements were also reduced in a minority of patients. However, a sub-group with pre-tarsal blepharospasm or persistent levator inhibition after treatment had a poor response. An average 75% reduction in abnormal movements was seen in cases of hemifacial spasm. Side effects of the treatment were usually mild and short lived.

Blepharospasm

Botulinum toxin.

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Botulinum Toxins

The relationship between eye-winking tics, frequent eye-blinking and blepharospasm.

A family is reported in which three generations were affected with eye-winking tics and/or blepharospasm. The proband developed eye-winking tics in childhood and then developed excessive blinking progressing to blepharospasm by the age of 21 years. His mother presented with Meige's syndrome and spasmodic torticollis at the age of 59 years; his uncle had blinked excessively from his early forties. His eldest son developed an eye-winking tic with facial grimacing at the age of 8 years, and in another son, a self-limiting period of eye-blinking occurred at the age of 4 years. The recovery cycle of the blink reflex was abnormal in all three generations. Three other children with eye-winking tics have a parent or close relative with frequent eye-blinking or blepharospasm. Five patients with adult-onset blepharospasm or Meige's syndrome are also described who had excessive eye-blinking dating back to childhood. It is suggested that eye-winking tics, frequent blinking and blepharospasm may share common pathophysiological mechanisms; the clinical expression may be age-related.

Adolescent

The significance of ophthalmological symptoms in idiopathic blepharospasm.

Two hundred and seventy-two patients with idiopathic blepharospasm were reviewed to establish the role of local eye disease in their illness. The majority of patients (57%) had symptoms at the onset of their illness such as dryness of the eyes, grittiness, irritation or photophobia suggesting eye lid or ocular surface disease. Detailed ophthalmological examination at the time of presentation had been carried out in 170 of the 272 cases; 64% of these patients had ocular symptoms, and 40% had demonstrable ocular surface or eye lid pathology. Such pathology was usually bilateral, chronic and resistant to local treatment. Blepharospasm developed in these patients after a long latent period, often of many years. Unilateral pathology was acute, normally responded well to local treatment, but was followed by the development of bilateral blepharospasm usually within six months. Amongst all 272 patients, those without ocular symptoms at presentation rarely developed them subsequently; if they did, there were no abnormal signs. The data suggest that ophthalmological disorders may trigger idiopathic blepharospasm in a substantial proportion of cases predisposed to develop this condition.

Adolescent

Long-term results of treatment of idiopathic blepharospasm with botulinum toxin injections.

One hundred and one patients with idiopathic blepharospasm have been treated with injections of botulinum toxin A into the orbicularis oculi. Ten had previously had facial nerve avulsions and responded well, normal visual function being restored in the majority (7/10) for an average of 14 weeks. Without prior surgical treatment the response was more variable, but 71/91 regained normal or near normal vision. Older patients, those with a family history of the condition, and those without oromandibular dystonia responded slightly better. The severity of the blepharospasm, the length of the history, and spontaneous resolution of an episode of focal dystonia in the past had no influence on the outcome. Results were poor in the presence of an associated neurological disorder. Side effects, particularly a temporary partial ptosis, were common but were well tolerated. The average duration of improvement was eight weeks in men, nine in women, and there was no evidence of any increase in duration after multiple injections. Eighty nine patients continued with injections, 11 opted for surgical treatment, and one resumed drugs.

Adult