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.

Disorders of facial motor function.

This review deals with a variety of disorders of facial movement. Recent publications on blepharospasm, facial spasm, facial myokymia, apraxia of lid opening and facial paralysis are referenced and discussed. In blepharospasm, carefully performed electromyographic studies reveal a variety of abnormal patterns of contraction of the oribicularis oculi and the levator palpebrae muscles confirming the clinical impression that blepharospasm is not a homogeneous disease. Similar studies are furthering the understanding of apraxia of lid opening, which may involve sustained contraction of the orbicularis oculi with or without failure of levator palpebrae inhibition. In both conditions botulinum toxin A injected pretarsally appears to be the preferred treatment. Of interest with respect to apraxia of lid opening is that it may be the result of a failure to sustain eye opening as well as an inability to open the eyes voluntarily. Although the fact that bilateral facial spasm was found in only five of 702 patients with spasm suggests that it is infrequent, it is also possible that bilateral facial spasm may be underdiagnosed because of lack of awareness of its existence. Despite the continued enthusiasm of neurosurgeons for microvascular decompression, the preferred treatment for facial spasm continues to be botulinum toxin A injections. It appears that a lesion of the dorsolateral pontine postgenu portion of the facial nerve is responsible for the facial myokymia of patients with multiple sclerosis. Interest in the study of lid function has increased as a result of the advent of the search coil technique for recording lid movements. Recordings with this technique have proven useful in facial nerve palsy for monitoring the degree and course of recovery and as a means of judging the effectiveness of therapy.

She could not look to either side.

A young woman presented with the recent onset of an inability to move the eyes on a horizontal plane. Examination revealed limited horizontal gaze to the right and a complete left ophthalmoplegia. The limitation of eye movement could not be overcome by the doll's head maneuver. Other signs included bilateral orbicularis oculi weakness and brisk deep tendon reflexes. Her condition gradually improved and two months following the onset of the illness she was symptom free.

Botulinum toxin: preferred treatment for hemifacial spasm.

This paper retrospectively analyses the clinical data of 65 consecutive cases of hemifacial spasm. A benign etiology was clinically evident in the majority of cases. Neuroradiologic investigation ruled out serious intracranial pathology in atypical cases. Trials of oral medication in 26 patients were consistently unsuccessful. Fifty-one patients had repeated lid and facial muscle injections of botulinum toxin during a period of 8 years. The mean duration of effective relief from spasm following an initial treatment was 18.9 weeks. This effect did not diminish with repeated injections.

Effectiveness of botulinum toxin in the treatment of spasmodic torticollis.

Thirty-two patients with spasmodic torticollis were assessed quantitatively for posture deformity, tremor and range of neck movement, and qualitatively for pain and global subjective disability. All patients were then treated with intramuscular botulinum toxin injections into appropriate neck muscles. Fifty-three treatments were administered using dosages of toxin in the range of 50-100 U per muscle. The maximum dose administered at a single sitting was 280 U. The progress of the patients was assessed during an 18-month period. Seventy-five percent of patients showed documented improvement in both subjective and objective parameters and were considered treatment successes. Pain improved in 65%, posture in 65%, tremor in 50% and range in 46%. The side effects that occurred were transient and included fatigue, dysphagia, neck weakness, hoarseness and local pain. This study demonstrates that treatment with botulinum toxin is of significant benefit for the majority of patients with spasmodic torticollis.

Pupillary areactivity in hydrocephalus of recent onset.

A patient who presented with bilateral loss of all pupillary reactions and normal ocular motor function is reported. Investigation revealed the presence of massive hydrocephalus. The syndrome developed shortly after transsphenoidal surgery for a suprasellar craniopharyngioma. Pupillary function returned to normal following the insertion of a ventriculo-peritoneal shunt. It is suggested that the syndrome was due to compression of the visceral oculomotor nuclei by a dilated sylvian aqueduct.

Visual function improvement in patients with macroprolactinomas treated with bromocriptine.

We studied eight patients who had visual field defects secondary to prolactin-secreting macroadenomas and who had improved visual function with bromocriptine treatment without surgery or radiation. We recommend bromocriptine as a primary treatment for prolactin-secreting macroadenomas. If therapy is effective, continued regular neuro-ophthalmologic, endocrine, and imaging studies are necessary, because treatment with bromocriptine must be continued indefinitely.

Paraneoplastic optic neuritis and encephalomyelitis. Report of a case.

A 63-year-old man developed bilateral paresis of horizontal and upward eye movements. He was found to have a small oat cell carcinoma of the lung. Four months later he experienced acute visual blurring on the right side. Examination of the right eye at that time revealed a visual acuity of 3/200 and a central scotoma. There was swelling of the right optic disc. Three weeks after the onset of the visual loss, the acuity of the right eye spontaneously improved to 20/60, the field deficit lessened, and there was a decrease in the swelling of the optic disc. Subsequently, his neuro-ophthalmologic condition remained unchanged but his general health deteriorated, and he died nine months after the onset of the disease. Neuropathologic examination showed mild perivascular lymphocytic infiltration and fibrosis of the meninges throughout the central nervous system, loss of neurons and gliosis in the third and fourth cranial nerve nuclei, perivascular inflammation and gliosis of the optic nerves, and chiasm and central demyelination of the right optic nerve. No tumor cells were seen. These findings were consistent with a diagnosis of paraneoplastic optic neuritis and paraneoplastic encephalomyelitis. The present case confirms the existence of paraneoplastic optic neuritis and illustrates the clinical course of the disease.

Electromyographic studies of congenital mirror movements.

We studied two patients with congenital mirror movements. In one, the movements were associated with impaired sensation and a cervicodorsal meningocele. In the other, no abnormality of the nervous system was found. In an EMG study, the normal temporal characteristics, response latency, duration, and amount of EMG on the normal and mirror sides suggest that similar motor commands are responsible for both voluntary and mirror movements.

The treatment of the restless legs syndrome with clonazepam: a prospective controlled study.

The effect of clonazepam on the restless legs syndrome was studied in a group of 6 patients. Following a drug-free period, 3 patients received clonazepam for 4 weeks followed by placebo for 4 weeks thereafter and 3 patients received the same medication and for the same length of time but in reverse order. The effectiveness of the medication was evaluated by means of a self-rating system in which patients assigned a score daily to the degree of discomfort experienced in the previous 24 hours. Three patients improved on clonazepam but 2 of these also improved on placebo. Clonazepam was not shown to be significantly more effective than placebo in the treatment of RLS.

Familial restless legs with periodic movements in sleep: electrophysiologic, biochemical, and pharmacologic study.

Restless legs syndrome was present in nine members of a family over a span of five generations. In three subjects, the diagnosis was confirmed by all-night sleep recordings and concomitant EMG. Two of these three subjects also had periodic movements in sleep. The frequency of leg movements decreased from wakefulness to stages 1 and 2 non-REM sleep. There was an increase of free dopamine and homovanillic acid in CSF of the propositus. Clonazepam effectively controlled restless legs in the propositus and his mother.

Background vascular headache: relief with indomethacin.

A patient with long standing sustained unilateral headache ("background vascular") and occasional multiple jabs received prompt and lasting relief from indomethacin. The effectiveness of the drug was tested in a placebo controlled double-blind trial. Indomethacin may be of value in some types of sustained headache.