Drooping Of Eyelid Research Articles

Myasthenia gravis following chicken pox infection in a Nigerian primary school girl

We report a case of myasthenia gravis occurring post Chicken pox infection in a 9 year old Nigerian girl. The girl was growing and seeing normally until 2weeks after a chicken pox infection when she observed progressive drooping of both upper eye lids which worsens as the day progresses and has persisted for more than one year. There was good response to Neostigmine.

Long-term outcomes of laser assisted blepharoplasty for ptosis: About 104 procedures in 52 patients

Background: Eyelid ptosis or blepharoptosis is defined as an abnormal drooping of the upper eyelid when looking straight ahead. Laser-assisted blepharoplasty (LAB), first introduced by Baker in 1984, presents the following advantages: improved intraoperative haemostasis, decreased operating time and improved appearance in the immediate postoperative periods. This article reports our long-term experience with LAB in ptosis correction surgery and underlines the advantages of the technique. Methods: A total of 52 patients were treated for ptosis with LAB between 2000 and 2011. The patients had an average age of 59.5 ± 9.6 years. Etiologies were senile ptosis in 34 cases, traumatic ptosis in 9 cases and congenital ptosis in 9 cases. The ptosis was classified as mild in 24 cases, moderate in 11 cases, and severe in 17 cases. The surgical technique was similar to the one described by Baker. The laser used in our studies was the CO2 Lumenis Active™ with the following parameters for skin incision: ultrapulse mode, continuous emission, 3-W power (program 1). The laser was then reprogrammed (program 2) with the following parameters for resection of a skin-orbicular muscle flap: regular mode, continuous emission, 9-W power with the beam slightly defocused. Results: Early complications included oedema in 8 patients. More than 1 mm of lid asymmetry was seen in 8 patients with 6 patients under corrected and two over corrected. Two of them chose secondary surgery. Mean down time was 5.5 ± 1.7 days (2–7 days). The mean late follow-up was performed after 2–10 years (mean 6.6 ± 1.7 years). Late complications included 4 recurrences 5, 6, 6, and 8 years, respectively, after the first procedure. All but one were successfully re-operated with the same technique. Overall patient satisfaction ranked high and all but one would recommend this treatment to others. Conclusion: LAB in ptosis surgery is a safe and reproducible technique particularly appreciated by patients. The procedure allows for improved intraoperative haemostasis, decreased operating time and improved appearance in the immediate postoperative periods.

Twenty-four–Year-Old Woman With a Droopy Eyelid—Quiz Case

Twenty-four–Year-Old Woman With a Droopy Eyelid—Quiz Case

Twenty-four–Year-Old Woman With a Droopy Eyelid—Diagnosis

Twenty-four–Year-Old Woman With a Droopy Eyelid—Diagnosis

Juvenile Myasthenia Gravis: A Case Report and Review of Literatures

Juvenile myasthenia gravis (JMG) is a rare autoimmune disorder of childhood. Pediatric presentation of MG is more common in Oriental than in Caucasian populations. JMG need to be differentiated from congenital myasthenia gravis which do not have haan autoimmune basis. An 11 years old girl presented with drooping of eye lids which was more marked at the later part of day and was gradually progressive . She had complained of double vision. She had no family history of myasthenia gravis. Ice pack test, repetitive nerve stimulation test, and anti acetylcholine receptor antibody test support the diagnosis. She was treated with pyridostigmine and was started as 30mg four times daily and increased to 60 mg/qds. Subsequently her symptoms improved gradually and she became stable.
 Bangladesh Journal of Neuroscience 2013; Vol. 29 (1) : 64-69

Extramedullary Hematopoietic Lesion of the Orbit Presenting as a Ring Enhancing Lesion

A 47-year-old healthy man presented with symptoms of double vision and drooping of the right upper eyelid. Examination disclosed signs of vertical diplopia and right upper-eyelid ptosis. An MRI delineated a well circumscribed, ring enhancing mass within the right orbit. The mass with peripheral enhancement located within the orbit, generated a broad differential diagnosis. After an excisional biopsy was performed, the mass was revealed to be fibrosis with extramedullary hematopoiesis. Current differential diagnoses of ring enhancing orbital lesions do not include extramedullary hematopoiesis, but we present a unique presentation of extramedullary hematopoiesis presenting as a ring enhancing orbital mass.

Swinging door lateral orbitotomy for management of anteriorly placed orbital tumours

A 70 years old female; who came to us; with drooping of right upper eyelid, gradual progressive diminution of vision with a slowly progressive swelling over right upper outer orbit of 1 year duration. Examination revealed non-axial proptosis with downward and inward deviation of the right eye. An irregular, lobulated nontender swelling was present in the superolateral orbit. CT scan revealed a well circumscribed oval encapsulated lesion with no bony destruction. The mass was removed by a modified lateral orbitotomy without removing lateral orbital rim. Histopathological evaluation showed it to be a pleomorphic adenoma of the lacrimal gland. Patient made an uneventful postoperative recovery. This presentation describes a method of lateral orbitotomy for anterior orbital lesions in which the lateral orbital rim is swung outwards without detaching it from its attachment to the temporalis muscle. This surgical approach may reduce the chance of avascular necrosis of the lateral orbital wall bone and infections of the orbit.

Presentation of conjunctival amyloidosis as bilateral ptosis and cicatricial entropion

Primary localized amyloidosis of the conjunctiva is a rare occurrence. This case report describes a 35-year-old male, who presented with drooping of both upper eyelids and a pricking sensation in the left eye. Examination of the conjunctiva showed granular swellings. The biopsy revealed amyloid deposition. Systemic amyloidosis was ruled out on further investigation. To our knowledge, this is the first report of amyloidosis of the conjunctiva and tarsus, presenting as ptosis with entropion, from India.

AN UNUSUAL CASE OF HORNER'S SYNDROME

A 71-year-old man presented with headache for a month and left eyelid droop for 2 days. He complained of recurrent sore throat, weight loss and long-standing anorexia, depression, iron deficiency...

A retrospective study: form‐deprivation myopia in unilateral congenital ptosis

Background: The aim of this retrospective study was to investigate the relationship between unilateral congenital ptosis in patients older than eight years and their refractive state and spherical equivalent refraction (SER).Methods: The study involved a review of the clinical records of 85 patients admitted to the First Affiliated Hospital, Sun Yat‐sen University between 1998 and 2010 with unilateral congenital ptosis. The average age was 16.83-years (nine to 27-years). The patients were classified into mild (27 cases), moderate (37 cases) or severe (21 cases) ptosis according to the degree of the droopy eyelid covering the cornea. The fellow eyes served as controls.Results: In 85 eyes with unilateral ptosis, the frequency of myopia (SER of ‐0.50-D or more myopia) was significantly higher than in the fellow eye (47 versus 32, p = 0.031). The frequency of myopia in eyes with severe unilateral ptosis was significantly higher than in the fellow eyes (16 versus 7, p = 0.012), whereas there were no significant differences in patients with mild (15/27 versus 13/27, p = 0.79) or moderate (16/37 versus 12/37, p = 0.47) unilateral ptosis. Similarly, the SER was significantly more myopic in eyes with severe ptosis compared with the fellow eye (‐1.37-D versus ‐0.85-D, p = 0.01), whereas no significant differences were found in patients with mild or moderate unilateral ptosis.Conclusions: The results showed a higher frequency of myopia and more myopic SER in eyes with severe unilateral ptosis compared with the fellow eye. The myopia found in eyes with unilateral ptosis might be caused by a mechanism similar to that resulting in myopia among animals subjected to form deprivation. It is important to pay attention to possible refractive error in patients with unilateral ptosis. Surgical correction of unilateral ptosis at an early age is recommended.

Udar niedokrwienny pnia mózgu bez trwałych następstw klinicznych w przebiegu samoistnego rozwarstwienia tętnicy szyjnej wewnętrznej – opis przypadku.

Internal carotid artery dissection (ICAD) is a frequent cause of a stroke in young patients. Risk factors which can lead to dissection include neck injury and diseases of the inner wall of the artery. Common symptoms in ICAD are cervical pain and headache, Horner's syndrome, paralysis of the cranial nerves and subsequently cerebral and retinal ischemia. MR angiography in TOF technique and brain MRI in T1- and T2-weighted images, FLAIR and DWI sequences are the method of choice in patients with ICAD but contrast-enhanced multislice computed tomography remains the fastest and the most available diagnostic method. A 39-year old woman, previously healthy, presented to the Hospital Emergency Department because of increasing neck pain on the right side and difficulty in swallowing. The neurological examination revealed: drooping of the right eyelid with narrow palpebral fissure, dysarthria, anisocoria (narrower pupil on the right side), unilateral hypoesthesia on the left side, weak palatal and pharyngeal reflexes on both sides, paresthesia within the left half of the body. Seven days before, the patient felt a sudden, severe neck pain radiating to the temporal apophysis. CT angiography revealed a defect in contrast filling within the left internal carotid artery and right vertebral artery. MRI of the head with MR angiography showed internal carotid artery dissection on the left side and dissection of the right vertebral artery and no ischemic changes within the brain. CT and MR angiography are methods characterized by high sensitivity in detecting dissection of the cervical arteries.

Teaching Neuro Images : A case of partial oculomotor palsy

A 68-year-old hypertensive man presented with binocular horizontal diplopia associated with drooping of right eyelid. Examination revealed medial rectus palsy, noncorrectable ptosis of the right eye (figure 1), normal pupils, and no long tract signs. Brain MRI showed a hyperintense T2 signal in the dorsal …

Internal Carotid Artery Dissection Heralded by an Oculomotor Nerve Palsy

Acute oculomotor nerve (CN III) palsies are commonly attributed to microvascular disease or compressive lesions and aneurysms, but may rarely be associated with ischemic large vessel disease. We report a case of an extracranial internal carotid artery (ICA) dissection heralded by CN III palsy with review of the relevant literature. A 24-year-old right-handed man presented with right-sided weakness preceded by vomiting 2 days earlier. The following day, the family noted his left eye to be deviated outward with enlarged pupil and droopy eyelid. On the day of admission, he had a fall owing to right-sided weakness. His neurological examination revealed significant aphasia, left third nerve palsy, right homonymous hemianopsia, and right-sided hemiplegia with hemisensory deficits. A brain magnetic resonance image showed an acute ischemic infarct in the left middle cerebral artery distribution without mass effect. Magnetic resonance angiogram showed a left extracranial internal cerebral artery (ICA) dissection with absence of flow within the distal cervical and intracranial ICA segments. He underwent a decompressive left hemicraniectomy with partial improvement in his deficits. Oculomotor nerve palsy as a result of ICA disease is a rare entity but has been reported in cases of stenosis, occlusion, and dissection. It is likely to be caused by hypoperfusion of CN III secondary to low flow or microembolism in the arteries feeding the nerve. The risk of CN III palsy in patients with ICA disease is higher in the presence of a fetal posterior cerebral artery. Acute oculomotor nerve palsies with pupillary involvement warrant thorough investigation. When routine work-up fails to elucidate an etiology, extracranial carotid pathology should be considered.

Executive functions are impaired in heterozygote patients with oculopharyngeal muscular dystrophy

Oculopharyngeal muscular dystrophy (OPMD) is an autosomal dominant disorder caused by a small expansion of a short polyalanine tract in poly(A) binding protein nuclear 1 (PABPN1). It presents with adult onset of progressive eyelid drooping, swallowing difficulties and proximal limb weakness, usually without involvement of central nervous system (CNS). However, cognitive decline with relevant behavioural and psychological symptoms has been recently described in homozygous patients. In this study, we performed for the first time an extensive neuropsychological and neuropsychiatric evaluation on 11 OPMD heterozygote patients. We found that they were less efficient than a matched control sample on several tests, particularly those tapping executive functions. Moreover, the presence of negative correlation between GCN expansion size and some neuropsychological scores raises the issue that CNS involvement might be linked to the genetic defect, being worse in patients with larger expansion. Our results might be consistent with the toxic gain-of-function theory in the pathogenesis of OPMD and hint at a possible direct role of PABPN1 in the CNS also in heterozygote patients.

OnabotulinumtoxinA for the Treatment of Chronic Migraine

OnabotulinumtoxinA for the Treatment of Chronic Migraine

Nerve Sheath Tumor found in a Patient with Horner's Syndrome

A patient with Horner's syndrome, as evidenced by a droopy eyelid, was referred to the vascular lab for a carotid duplex ultrasound exam to rule-out carotid dissection. Instead of a carotid abnormality, the duplex exam showed a mass at the carotid bifurcation, consistent with a carotid body tumor or soft tissue mass. The mass was confirmed by computed tomography (CT) and magnetic resonance imaging (MRI). Pathology, following surgical excision of the mass, determined that this was a nerve sheath tumor—a less common finding at the carotid bifurcation than is a carotid body tumor.

A History of Cigarette Smoking Is Associated With the Development of Cranial Autonomic Symptoms With Migraine Headaches

To look at the smoking history of migraine patients and to determine if a history of cigarette smoking is associated with the development of cranial autonomic symptoms with migraine headaches. It has recently been noted that a significant number of migraine patients may develop autonomic symptoms during their attacks of headache. Why some headache patients activate the trigeminal autonomic reflex and develop cranial autonomic symptoms while others do not is unknown. Cluster headache occurs more often in patients with a history of cigarette smoking, suggesting a link between tobacco exposure and cluster headache pathogenesis. Could cigarette smoking in some manner lead to activation of the trigeminal-autonomic reflex in headache patients? If cigarette smoking does lower the threshold for activation of the trigeminal autonomic reflex then do migraine patients who have a history of cigarette smoking more often develop cranial autonomic symptoms than migraineurs who have never smoked? Consecutive patients diagnosed with migraine (episodic or chronic) who were seen over a 7-month time period at a newly established headache center were asked about the presence of cranial autonomic symptoms during an attack of head pain. Patients were deemed to have positive autonomic symptoms along with headache if they experienced at least one of the following symptoms: eyelid ptosis or droop, eyelid or orbital swelling, conjunctival injection, lacrimation, or nasal congestion/rhinorrhea. A smoking history was determined for each patient including was the patient a current smoker, past smoker, or had never smoked. Patients were deemed to have a positive history of cigarette smoking if they had smoked continuously during their lifetime for at least at 1 year. A total of 117 migraine patients were included in the analysis (96 female, 21 male). Forty-six patients had a positive smoking history, while 71 patients had no smoking history. Some 70% (32/46) of migraineurs with a positive history of cigarette smoking had cranial autonomic symptoms along with their headaches, while only 42% (30/71) of the nonsmoking patients experienced at least 1 autonomic symptom along with headaches and this was a statistically significant difference (P < .005). In total, 74% of current smokers had autonomic symptoms with their headaches compared with 61% of past smokers and this was not a statistically significant difference. There was a statistically significant difference between the number of current smokers who had autonomic symptoms with their headaches compared with the number of patients who never smoked and had autonomic symptoms (P < .05). Overall, 52% of the studied migraineurs had autonomic symptoms. There was a statistically significant difference between autonomic symptom occurrence in male and female smokers vs male and female nonsmokers. Each subtype of cranial autonomic symptoms was all more frequent in smokers. A history of cigarette smoking appears to be associated with the development of cranial autonomic symptoms with migraine headaches.

Central nervous system abnormalities in two cases with neonatal Marfan syndrome with novel mutations in the fibrillin‐1 gene

Neonatal Marfan syndrome (nMFS) is the most severe form ofMarfan syndrome and affects the skeletal, respiratory, cardiovas-cular and ocular systems. Death from cardiorespiratory failuretypicallyoccursinthefirstfewyearsoflife.CentralnervoussystemabnormalitiesarenotconsideredtobepartofthenMFSphenotype.Over600mutationsintheFibrillin1(FBN1)genehavebeenfoundin Marfan syndrome patients but patients with nMFS are mostlikelytohavemissenseandframeshiftmutationsinexons24–32oftheFBN1gene[DietzandPyeritz,1995;Putnametal.,1996;Boomset al., 1999; Hennekam, 2005], with mutations involving exon25 being particularly common in nMFS [Faivre et al., 2009]. Inthe largest series’ to date, 22% of MFS patients with mutations inexons24–32presentedwithfeaturesofnMFS,comparedto0.6%ofpatients with mutations in other exons [Faivre et al., 2007, 2009].The severe phenotype usually generated by mutations in thisregionislikelytobeduetoalteredproductionofEGF-likedomains11–19codedbyexons24–32,leadingtotheformationofstructur-ally abnormal fibrillin molecules causing severe disruption ofmicrofibrils and thus the extracellular matrix formation [Liuet al., 1996].The clinical findings common to nMFS patients include arach-nodactyly, loose skin, contractures, ectopia lentis and cardio-vascular findings with characteristic atrioventricular valvulardysfunction [Morse et al., 1990]. We report on two cases of nMFSwith novel mutations in the FBN1 gene, typical severe phenotypesbut with previously unreported abnormalities in the centralnervous system.The first patient was born to a 31-year-old, G2P1 woman ofSomali descent. The couple was healthy and their family historieswere non-contributory. The pregnancy was complicated byfeeble fetal movements for the duration of the pregnancy. Earlyultrasoundswerenotdonebutafetalultrasounddoneat30weeksgestationsuggestedaright-sideddiaphragmatichernia.Fetalecho-cardiographyperformedat32weeksgestationshowednostructuralor functional cardiac abnormalities. Delivery was at 37 weeksgestation by caesarean section. The birth weight was 2,700g(25–50th centile), length 49cm (75–97th centile) and headcircumference (OFC) 35cm (90th centile). The newborn hadrespiratorydistressrequiringventilatorysupportat1hroflifeandphysical examination showed facial dysmorphism with dolicho-cephaly,highforehead,frontalbossing,deepseteyes,downslantingpalpebral fissures, droopy eyelids, a high arched palate, retrogna-thia,largeandthin/simpleearsandlooseandredundantfacialskin.

Current techniques in surgical correction of congenital ptosis

Ptosis refers to vertical narrowing of the palpebral fissure secondary to drooping of the upper eyelid to a lower than normal position. Ptosis is considered congenital if present at birth or if it is diagnosed within the first year of life. Correction of congenital ptosis is one of the most difficult challenges ophthalmologists face. Multiple surgical procedures are available including, frontalis sling, levator advancement, Whitnall sling, frontalis muscle flap, and Mullerectomy. Selection of one technique over another depends on the consideration of several factors including the surgeon experience, the degree of ptosis in the patient, as well as the degree of levator muscle function. Current recommendations for the correction of congential ptosis vary based on clinical presentation. Advantages and disadvantages of each of these procedures are presented with recommendations to avoid complications.

Loss of nuclear poly(A)-binding protein 1 causes defects in myogenesis and mRNA biogenesis

The nuclear poly(A)-binding protein 1 (PABPN1) is a ubiquitously expressed protein that plays a critical role in polyadenylation. Short expansions of the polyalanine tract in the N-terminus of PABPN1 lead to oculopharyngeal muscular dystrophy (OPMD), which is an adult onset disease characterized by eyelid drooping, difficulty in swallowing and weakness in the proximal limb muscles. Although significant data from in vitro biochemical assays define the function of PABPN1 in control of poly(A) tail length, little is known about the role of PABPN1 in mammalian cells. To assess the function of PABPN1 in mammalian cells and specifically in cells affected in OPMD, we examined the effects of PABPN1 depletion using siRNA in primary mouse myoblasts from extraocular, pharyngeal and limb muscles. PABPN1 knockdown significantly decreased cell proliferation and myoblast differentiation during myogenesis in vitro. At the molecular level, PABPN1 depletion in myoblasts led to a shortening of mRNA poly(A) tails, demonstrating the cellular function of PABPN1 in polyadenylation control in a mammalian cell. In addition, PABPN1 depletion caused nuclear accumulation of poly(A) RNA, revealing that PABPN1 is required for proper poly(A) RNA export from the nucleus. Together, these experiments demonstrate that PABPN1 plays an essential role in myoblast proliferation and differentiation, suggesting that it is required for muscle regeneration and maintenance in vivo.