Cases reported "Blepharoptosis"

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1/13. The jaw-winking syndrome.

    The jaw-winking syndrome involves synkinesis of the pterygoid muscles and levator palpebrae superioris, where the eyelid retracts with mandibular movements. Its appearance in the adult may be associated with other pathology, and recognition in the neonate may allow for earlier recognition of any ophthalmological problems. As it is elicited by jaw movements, the oral health care provider may be the first to recognise the condition. This report describes the incidental finding of jaw-winking syndrome in a 22-year-old Asian man. Although he suffered no physical or psychological effects from the condition, he did have an associated, corrected, ophthalmological defect.
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2/13. facial nerve palsy secondary to internal carotid artery dissection.

    We report facial palsy as the sole cranial neuropathy complicating an ipsilateral internal carotid artery dissection. A previously healthy 44-year-old man developed retro-orbital and temporal headache with associated nausea while engaged in modest physical exercise. On the following morning he noticed a left ptosis and miotic pupil. One week later he woke with a left facial weakness. On the same day he had a 90-minute episode of expressive dysphasia. magnetic resonance imaging and angiography demonstrated left internal carotid artery dissection. The temporal association between our patient's facial nerve palsy and typical features of spontaneous internal carotid artery dissection suggests a common aetiology. We suggest that involvement of the VII cranial nerve in isolation followed disruption of an anomalous nutrient artery. The delay in clinical manifestation may imply extension of the dissection.
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3/13. Superior orbital fissure syndrome: current management concepts.

    The superior orbital fissure syndrome is an uncommon complication of craniofacial fractures: middle-third facial fractures and lesions of the retrobulbar space. This article reviews the anatomy and etiology of the superior orbital fissure as it relates to pathophysiology and physical findings. Cases reported in the literature are reviewed, emphasizing diagnosis and established treatment options. Two cases are presented and their management discussed, including the use of pre- and postoperative steroids as an adjunct to standard fracture reduction and stabilization therapy.
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4/13. Isolated left oculomotor nerve palsy following measles.

    A 10-month-old boy was admitted with ptosis on the left eyelid, which rapidly occurred following a disease with rash about 20 days before admission to our hospital. By history, none of the vaccinations had been performed. On physical examination, his vital signs were stable, and he had marasmus. Isolated left oculomotor nerve palsy was diagnosed. Cranial magnetic resonance imaging was normal. serum IgM antibody to measles virus was positive. oculomotor nerve palsy markedly improved on the 15th day of follow-up, and complete improvement was noted on the second month of follow-up. To our knowledge, this is the first case of oculomotor nerve palsy following measles.
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5/13. vincristine-induced neuropathy as the initial presentation of charcot-marie-tooth disease in acute lymphoblastic leukemia: a Pediatric Oncology Group study.

    PURPOSE: After profound peripheral neurotoxicity during induction chemotherapy for acute lymphoblastic leukemia (ALL) in the index patient with Charcot-Marie-Tooth hereditary neuropathy (CMT), study coordinators of the Pediatric Oncology Group (POG) front-line ALL protocols reviewed patient registrations to identify any other patients with possible CMT. The goal was to provide preliminary information about patients with undiagnosed CMT who develop ALL. patients AND methods: Five children with ALL who were enrolled in POG B-precursor or T-cell ALL protocols from 1994 to 1999 subsequently were determined to have CMT hereditary neuropathy. Their clinical presentations and treatment records were reviewed in detail. Records of all patients entered on POG 9201 (lesser-risk ALL) were reviewed to identify all cases of significant vincristine toxicity noted in the first 6 months of treatment. RESULTS: The five identified patients all had substantial peripheral neurotoxicity that required alteration in treatment and/or orthopedic/physical therapy evaluation and follow-up. The POG 9201 review identified 25 of 686 patients (3.6%) with significant peripheral neuropathy. Three of 25 were diagnosed with CMT; the others have had no testing reported. CONCLUSIONS: A family history of CMT or other peripheral neuropathy should be sought at the time of diagnosis of ALL. Testing for CMT should be considered in any child with substantial vincristine-induced peripheral neurotoxicity. Treatment of such patients must be individualized. Testing of all patients with significant peripheral neuropathy would be necessary to determine the percentage of such neuropathy explained by underlying CMT.
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6/13. Oculopharyngeal muscular dystrophy in an Irish family.

    Victor and his associates coined the term oculopharyngeal muscular dystrophy to describe a clinical syndrome characterised by dysphagia and ptosis. Subsequent authors have traced a large series in French Canadians to a single quebec isolate and have emphasized that the condition is usually inherited as a dominant trait. In 1974 Fried reported two isolated cases in an Ashkenazi Jewish family of Hungarian extract in whom the condition was recessively inherited. In this paper we report an Irish family from North Cork with typical features of oculopharyngeal muscular dystrophy in whom the inheritance pattern is dominant with incomplete expression. In reporting such cases the ethnic and genetic heterogeneity of oculopharyngeal muscular dystrophy is highlighted. Case Report 1. A 68 year old farmer was admitted for investigation of recurrent chest infections. He complained of a 20 year history of progressive difficulty swallowing, productive cough and impaired vision. On examination he had a gaunt expressionless appearance with bilateral ptosis to mid pupillary level. Both right and left gag reflexes were noted to be reduced and sluggish, and on swallowing fluids were noted to regurgitate through his nostrils. Chest examination revealed the presence of bibasal coarse crepitations suggestive of aspiration. No further physical abnormalities were noted. On investigation, full blood count, urea, and electrolytes, liver function tests, sedimentation rate, thyroid function tests, muscle enzymes (PK, LDH) and electrocardiogram were normal. Chest radiograph confirmed aspiration. Tensilon test was negative. Oesophagoscopy outruled organic obstruction, and confirmed sluggish pharyngeal muscular activity. A clinical diagnosis of oculopharyngeal dystrophy was made on the basis of associated ptosis and dysphagia occurring in the presence of a supportive family history.(ABSTRACT TRUNCATED AT 250 WORDS)
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7/13. Blepharochalasis.

    The condition of blepharochalasis and its treatment are discussed. Four cases are presented which show a physical sign that may help in its diagnosis.
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8/13. Graves' disease presenting with bilateral acute painful proptosis, ptosis, ophthalmoplegia, and visual loss.

    Two middle-aged women presented with bilateral acute painful proptosis, ptosis, ophthalmoplegia, and visual loss. In both an initial diagnosis of orbital cellulitis was made, but they did not respond to systemic antibiotics. Orbital computerised tomographic (CT) scans were thus done within 36 h of admission and they showed grossly enlarged extraocular muscles in each case suggestive of dysthyroid eye disease. Clinical examination was otherwise normal. When high doses of systemic steroids were substituted for the antibiotics the physical signs resolved rapidly, accompanied by a dramatic reduction in the size of extraocular muscles on CT scanning. One patient subsequently became clinically hypothyroid, while the other showed clinical and biochemical evidence of thyroid overactivity. These case-reports suggest that patients with bilateral acute painful proptosis should have an early CT scan to exclude atypical dysthyroid disease. Delay in giving systemic steroids may allow the development of unnecessary visual loss due to optic nerve damage.
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9/13. Traumatic bilateral carotid-cavernous fistulas treated with detachable balloon. A case report.

    A 29-year-old male developed ptosis and progressive pulsating protrusion of the right eye, accompanied by conjunctival injection and tinnitus following a bicycle accident. MR angiography revealed dilation of both superior ophthalmic veins and facial veins. Right internal carotid angiography demonstrated right carotid-cavernous fistula (CCF) at the C3 portion of the right internal carotid artery with abnormal venous drainage. After right carotid balloon occlusion test had been performed, a detachable balloon was introduced into the right CCF while preserving the lumen of the right internal carotid artery. A left CCF, which was detected after closure of the right CCF, was also closed with a detachable balloon. Follow-up carotid angiographies showed complete closure of both CCFs and no abnormal venous drainage. After 1 year no abnormal physical manifestations, or abnormal neurologic signs or symptoms were present.
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10/13. Unusual penetrating faciocranial injury caused by a knife: a case report.

    Penetrating head and neck trauma in children is uncommon and are potentially life-threatening injuries. Penetrating trauma to the head in children is a challenging problem for both the initial evaluating physicians and surgeons. We report upon a patient who had fallen from a tree while cutting vegetables and sustained a penetrating faciocranial injury caused by his knife. Clinical examination showed a knife which had entered his face in the right preauricular, pre-temporomandibular joint area below the zygomatic arch. His left bulbus oculi was exophthalmic and a complete ptosis was present. He was fully conscious. The only abnormal finding was complete left visual loss. The other neurological ophthalmological and systemic physical evaluations were normal. The Glasgow coma scale score was 14. The modalities of treatment and the outcome of the operation are described and the management of similar injuries is discussed.
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