Asymmetric Ptosis Research Articles

A severe case of facioscapulohumeral muscular dystrophy (FSHD) with some uncommon clinical features and a short 4q35 fragment

Severe and early facioscapulohumeral muscular dystrophy (FSHD) is relatively rare. In this report, we describe a case of severe, infantile onset FSHD in a patient with asymmetric progressive ptosis and early hyperlordosis. DNA analysis revealed a very short 4q35 allele of 8.6 kb and a somatic mosaicism for the 4q35 deletion detected in a subclinically affected parent of the patient. This case demonstrates difficulties in the management of abnormal posture, especially early hyperlordosis in children with this disorder.

Ptosis in patients with hemispheric strokes. Averbuch-Heller L. Leigh RJ. Mermelstein V. Zagalsky L. Streifler JY. Neurology 2002;58:620–624

Ptosis caused by lesions of the cerebral hemispheres without involvement of the third cranial nerves or oculosympathetic pathways is considered rare. So-called cerebral ptosis has been most often reported with unilateral, usually right hemispheric lesions. However, the frequency of cerebral ptosis in patients with strokes has not received systematic study. The purpose of this study was to determine the frequency of ptosis in patients with acute hemispheric stroke and to identify stroke features associated with ptosis. Eyelid function was studied in 64 consecutive patients with acute hemispheric stroke and 40 age-matched subjects with no known neurologic disease. All underwent comprehensive neuroophthalmologic and general neurologic examination within 48 hours of admission, including measurement of palpebral fissures, marginal reflex distance, and range of upper lid movement. Only patients who could cooperate with eyelid testing were included Brain CT scans were obtained for all patients. Neurogenic ptosis was found in 24 patients (37.5%) with stroke, which was bilateral in 10 patients and unilateral in 14. None of the control subjects had neurogenic ptosis. All patients with stroke with ptosis had a hemiparesis, most often contralateral to the side of the ptosis in those patients with unilateral or highly asymmetric ptosis. CT evidence of right-sided hemispheric cortical infarction was more common in patients with strokes with ptosis. Rightward gaze deviation and upgaze paresis were more common (P < .05) in the patients with ptosis compared with others who had had strokes. In five patients with large hemispheric infarction, complete bilateral or asymmetric ptosis was the first sign of imminent herniation, preceding pupillary dilatation and ocular motor deficits. The authors conclude that ptosis occurs frequently in patients with hemispheric strokes, especially in association with right hemispheric lesions. Complete bilateral ptosis is usually caused by large infarctions and may be a premonitory sign of an impending herniation.—Nancy J. Newman

Ptosis in patients with hemispheric strokes

Cerebral ptosis is considered rare; it has been reported with unilateral, usually right hemispheric lesions. However, the frequency of cerebral ptosis in patients with strokes has not received systematic study. To determine the frequency of ptosis in patients with acute hemispheric stroke and to identify stroke features associated with ptosis. Eyelid function was studied in 64 consecutive patients with acute hemispheric stroke and 40 age-matched subjects with no known neurologic disease. All underwent comprehensive neuro-ophthalmologic and general neurologic examination within 48 hours of admission, including measurement of palpebral fissures, marginal reflex distance, and range of upper lid movement. Only patients who could cooperate with eyelid testing were included. Brain CT scans were obtained for all patients who had had strokes. Twenty-four (37.5%) of the patients with strokes had neurogenic ptosis, which was bilateral in 10 and unilateral in 14. None of the control subjects had neurogenic ptosis. All patients with strokes with ptosis had a hemiparesis. Rightward gaze deviation and upgaze paresis were more common (p < 0.05) in the patients with ptosis compared with others who had had strokes. CT evidence of right-sided hemispheric cortical infarction was more common in patients with strokes with ptosis (p < 0.05). In five patients with large hemispheric infarction, complete bilateral or asymmetric ptosis was the first sign of imminent herniation, preceding pupillary dilation and ocular motor deficits. Ptosis occurs frequently in patients with hemispheric strokes, especially in association with right hemispheric lesions. Complete bilateral ptosis is usually caused by large infarctions and may be a premonitory sign of an impending herniation.

Hering's law and eyebrow position.

A patient with bilateral but asymmetrical brow ptosis is presented in whom the frontalis action on the relative position of the eyebrows could be demonstrated to obey Hering's law of equal innervation. The neurological basis for this finding is discussed, along with the implications for the surgical management of brow ptosis.

Unilateral Ptosis and Eye Dominance

We studied the relationship between eye dominance (Scobee test) and upper lid level (MRD) in 54 patients with unilateral ptosis or asymmetric bilateral ptosis (> or = 2 mm difference in MRD) using the phenylephrine test. A contralateral lid drop was measured in 12/54 patients after instillation of 2.5% phenylephrine in the more ptotic eye and occurred in both congenital (3) and acquired (9) ptosis cases. A contralateral lid drop was seen in 7/14 (50%) patients whose ptosis was greater in the dominant eye and in 5/40 (12.5%) patients whose ptosis was greater in the nondominant eye (p = .01). Contralateral lid drops > or = 1 mm were present in 7/54 patients, and 6/14 (43%) of them had greater ptosis on the dominant side and 1/40 (2.5%) on the non-dominant side (p = .001). There was no association between contralateral pseudoretraction and either visual acuity or severity of ptosis. These results suggest that levator muscle tone is influenced by eye dominance when ptosis is present. The preoperative evaluation of unilateral or asymmetrical bilateral ptosis cases should include the Scobee and phenylephrine tests to unmask contralateral upper lid pseudoretraction.

Visual Development in the Blepharophimosis Syndrome

One hundred and one cases of the blepharophimosis syndrome presenting over a decade are reviewed with particular attention to the factors influencing their visual development. Three distinct clinical patterns emerge--severe bilateral ptosis, moderate bilateral ptosis, and asymmetric ptosis--and their differing incidence of amblyopia and strabismus is discussed. The risk of amblyopia is much higher than previously believed (56.4% in our series) and preventive management is discussed.

Visual development in the blepharophimosis syndrome.

One hundred and one cases of the blepharophimosis syndrome presenting over a decade are reviewed with particular attention to the factors influencing their visual development. Three distinct clinical patterns emerge--severe bilateral ptosis, moderate bilateral ptosis, and asymmetric ptosis--and their differing incidence of amblyopia and strabismus is discussed. The risk of amblyopia is much higher than previously believed (56.4% in our series) and preventive management is discussed.

The Measurement and Definition of Ptosis

Measurements of 242 ptotic and normal eyelids were recorded clinically and compared with algebraically derived measurements from projected 35 mm photographs. Accuracy to 1.5 mm or better was obtained in 84% of clinical as compared with photographic measurements. The following definition is suggested: ptosis is present when the upper eyelid is less than 2 mm from midpupil. Reduction of the upper field of vision to 30 degrees or less is present in 97% of eyes with ptosis so defined. Asymmetric ptosis is present in the lower of the two upper eyelids when there is 2 mm or more asymmetry between the levels of the upper eyelids, even if both eyelids are 2 mm or more from midpupil. Although ptosis is best defined in terms of the midpupil to upper lid distance, the diagnosis of ptosis rests with the examining physician based on the clinical evaluation of the patient.

Clinical Features of Types A and B Food-borne Botulism

Medical records of 55 patients with type A and type B food-borne botulism reported to the Centers for Disease Control during 2 years were reviewed to assess the clinical features and severity of illness, diagnostic test results, nature of complications, amd causes of death. Some patients had features not usually associated with botulism including paresthesia (14%), asymmetric extremely weakness (17%), asymmetric ptosis (8%), slightly elevated cerebrospinal fluid protein values (14%), and positive responses to edrophonium chloride(26%). Several observation suggest that type A was more severe than type B disease. Although the case-fatality ratio was not significantly greater, patients with type A disease saw a physician earlier in the course of illness, were more likely to need ventilatory support, and were hospitalized longer. Patients who died were older than those who survived. Deaths within the first 2 weeks resulted from failure to recognized the severity of the disease or from pulmonary or systemic infection whereas the three late deaths were related to respirator malfunction.

Enhanced ptosis in myasthenia gravis.

The ocular manifestations of myasthenia gravis result from a dysfunction at the myoneural junction. Histories of diurnal variation, exacerbation during fatigue, periodic remission, and responsiveness to edrophonium chloride (Tensilon) are characteristic.1Common signs are asymmetrical ptosis and ophthalmoplegia. Less common findings are lid retraction and lid oscillations.2Abnormal saccadic movements,3-6gaze palsies, vertical nystagmus,7lid twitch, and pseudointernuclear ophthalmoplegia8have also been reported. The question of pupillary dysfunction has not been fully resolved.This article describes enhanced ptosis as an unusual manifestation of ocular myasthenia gravis and discusses possible mechanisms. TECHNIQUE Enhanced ptosis is demonstrated during ocular upgaze in patients with bilateral ptosis by elevating and maintaining the more ptotic eyelid in a fixed position (Figure). The opposite eyelid slowly falls and may close completely. In this latter state, eyelid tone is greatly reduced. A variation of this response has also been observed; in