痛性眼肌麻痹综合征5例临床分析

目的 :探讨痛性眼肌麻痹综合征 (TolosaHuntSyn drome)的临床特点。方法 :对 5例痛性眼肌麻痹综合征患者的临床资料进行分析。结果 :5例痛性眼肌麻痹综合征患者均符合诊断标准 ,对激素治疗效果佳 ,4例痊愈 ,1例并发化脓性脑膜炎。结论 :痛性眼肌麻痹病因多样 ,临床表现复杂 ,诊断时应慎重。头颅CT、MRI及动脉造影对其诊断有一定的价值。大部分患者对激素疗效好 ,但需警惕炎症播散     

Angiographic Findings in the Tolosa–Hunt Syndrome and Resolution after Corticosteroid Treatment

The Tolosa–Hunt syndrome is a rare clinical condition characterized by painful opthalmoparesis associated with idiopathic granulomatous inflammation of the orbital apex and cavernous sinus. Historically, this condition was thought to result from arteritic changes in the internal carotid artery and cavernous sinus. Modern digital angiographic techniques were unavailable when THS was initially described, and few reports exist on its high-resolution angiographic findings. Painful ophthalmoparesis, especially of the oculomotor nerve, warrants vascular imaging because of the concern for an underlying aneurysm. Here, we describe angiographic findings of THS which may be useful for clinicians when encountering patients presenting with painful ophthalmoplegia.     

Optic perineuritis as a rare initial presentation of sarcoidosis

Sarcoidosis is a multisystem granulomatous disorder of unknown aetiology and establishing the correct diagnosis can be challenging. Although dysfunction of the anterior visual pathways is uncommon, it is the most common neuro-ophthalmological manifestation of this condition and given the potential for irreversible, severe visual loss, prompt diagnosis and treatment are essential. We describe a patient with optic perineuritis as a rare initial presentation of sarcoidosis and discuss the underlying pathophysiology and management.     

Malignant lymphoma in the cavernous sinus with bilateral total ophthalmoplegia and tonic pupils

A 58-year-old man presents with bilateral total ophthalmoplegia caused by malignant lymphoma in the cavernous sinuses. He reported right retrobulbar pain and diplopia; however, corticosteroid therapy was not effective. After admission, right total ophthalmoplegia progressed to bilateral involvement within a few days. From biopsy of the ethmoidal sinus and axillary lymph nodes, non-Hodgkin's lymphoma was diagnosed. Chemotherapy was started immediately, and non-Hodgkin's lymphoma was completely remitted within 3 months. Bilateral total ophthalmoplegia also improved; however, tonic pupils became prominent in both eyes. Paraneoplastic effects of non-Hodgkin's lymphoma may be responsible for the tonic pupils.     

A rare case of eyelid sarcoidosis presenting as an orbital mass

Intraorbital sarcoidosis presenting externally as a solitary eyelid mass has been described in the literature as isolated case reports. We describe a rare case of asymptomatic sarcoidosis with orbital mass as the presenting feature in a young woman. The lesion was excised with the clinical possibility of a thrombosed varix. On histology, the lesion was characterized by numerous nonnecrotizing epithelioid cell granulomas with several multinucleated giant cells containing abundant asteroid bodies and oxalate crystals. No tubercular bacilli were detected. A diagnosis of sarcoidosis was rendered and on further clinical work-up, she was detected to have hilar lymphadenopathy. Sarcoidosis should be considered in the differential diagnosis of orbital mass as it could be the initial manifestation of the disease process.     

Nonhealing orbital floor fracture in a pediatric patient: A unique presentation of pseudo-silent sinus syndrome

Silent sinus syndrome was first described as spontaneous enophthalmos and hypoglobus associated with subclinical maxillary sinusitis without prior trauma or surgery. This clinical entity has later been described after trauma in which damage to the ostiomeatal complex leads to atelectasis of the maxillary sinus. We report a case of a 14-year-old boy who presented 4 years after sustaining a non-operative orbital floor fracture with enophthalmos and transient diplopia. Computed tomography (CT) demonstrated enlargement in size of the original orbital floor fracture and bilateral maxillary sinus disease. Bilateral chronic sinusitis suggested an anatomical predisposition to sinusitis unrelated to the prior trauma. The authors propose that, in this case, negative pressure in the maxillary sinus and chronic inflammation led to bone resorption and failure of the orbital fracture to heal. This differs from prior reports of silent sinus syndrome in that there was complete resorption of bone of the orbital floor and no decrease in volume of the maxillary sinus given the open communication of the sinus and the orbit, making this a unique presentation of pseudo-silent sinus syndrome in a pediatric patient.     

Conjunctival deposits as an initial manifestation of sarcoidosis.

PURPOSE: To report conjunctival deposits as an initial manifestation of sarcoidosis. METHODS: Case reports. RESULTS: Two patients with bilateral bulbar conjunctival white deposits underwent conjunctival biopsy. The biopsy specimens showed noncaseating granulomas with prominent Schaumann bodies. Serum angiotensin-converting enzyme levels were increased in both patients. The first patient had abnormal results of pulmonary function tests. Sarcoidosis was diagnosed in both patients. CONCLUSIONS: Conjunctival deposits may represent the initial clinical manifestation of sarcoidosis.     

Choroidal granuloma as an initial manifestation of systemic sarcoidosis

Systemic sarcoidosis presenting as a choroidal nodule is a rare phenomenon, with limited reports in the past. We present a case of systemic sarcoidosis in a young adult male presenting as a solitary choroidal mass. The diagnosis was made based on elevated serum lysozyme, serum angiotensin-converting enzyme, negative Mantoux, and computed tomography (CT) scan of the chest. Ultrasound examination and fundus fluorescein angiography helped us to differentiate this lesion from choroidal melanoma. Magnetic resonance imaging (MRI) of brain was done to rule out neurosarcoidosis. Patient responded well to systemic steroid therapy, with marked resolution of the choroidal granuloma, and reduced size of mediastinal lymph nodes on repeat CT scan of the chest.     

Uveitis and internuclear ophthalmoplegia as ocular manifestations of sarcoidosis

Sarcoidosis is a multisystemic granulomatous chronic disease of unknown etiology with a wide range of clinical presentations. Diagnosis of sarcoidosis in patients with ocular manifestations can be challenging.We first describe a case of sarcoidosis presented with pulmonary involvement and both uveitis and internuclear ophthalmoplegia as ocular manifestations.A 55-year-old caucasian woman with non-productive cough and weakness presented with bilateral granulomatous anterior uveitis. Few days later, the patient presented again complaining of horizontal diplopia due to internuclear ophthalmoplegia. The diagnosis of sarcoidosis was made as a result of clinical examination and systemic investigations. Particularly, high-resolution computed tomography scanning of the chest was able to identify bilateral hilar lymphadenopathy not previously detected by chest X-ray. Biopsy confirmed diagnosis showing classic non-caseating granulomas.     

Tolosa-Hunt综合征的临床分析

Tolosa-Hunt综合征(THS)在临床上属于少见病,是由于海绵窦区(有时病变范围波及眶上裂甚至眶尖)非特异性炎性肉芽肿所致的痛性眼肌麻痹。THS临床特征为眶周或眼眶后疼痛同时伴有单根或多根眼外肌麻痹以及同侧三叉神经感觉区障碍。临床上表现为痛性眼肌麻痹的疾病有很多(眼肌麻痹性偏头痛、海绵窦段颈内动脉瘤、脑膜炎、糖尿病致颅神经损伤及鼻咽癌所致的痛性眼肌麻痹等),容易造成诊断上的困难,本文对于THS病因、临床特点、诊断标准、鉴别诊断以及治疗原则做了综合性的论述,同时着重介绍了MRI对于诊断THS的重要性。     

Metastatic B-cell lymphoma of the cecum masquerading as Tolosa-Hunt syndrome

CASE REPORT: Imaging studies of a 41-year-old man who had developed a painful unilateral ophthalmoplegia showed a mass involving the right cavernous sinus region. The systemic workup disclosed a B-cell lymphoma in the cecum. COMMENTS: We report a case of metastatic cecum lymphoma masquerading as Tolosa-Hunt syndrome.     

Semicircular tumor of the iris and uveitis as unilocal manifestation of sarcoidosis

Purpose:To describe an unusual manifestation of sarcoidosis as a large tumor of the iris and ciliary body without any other involvement of the body. Methods:We describe a 20-year-old female presenting with a granulomatous tumor of the right iris and ciliary body and concomitant uveitis. Results:Extensive ocular and systemic workup revealed the tumor to be a large solitary sarcoid granuloma. As systemic steroids were not able to control the activity of the uveitis and granuloma, only the initiation of immunosuppressive therapy with cyclosporine A achieved a lasting remission. Conclusion:The possibility of an exclusively ocular sarcoidosis should always be kept in mind despite negative regular screening tests. In these cases, a biopsy should be considered and immunosuppressive agents like cyclosporine A should be evaluated in cases not responding to first-line treatment with systemic steroids.     

Bilateral exudative retinal detachments as initial presentation of sympathetic ophthalmia in Turner syndrome

CASE REPORT: Sympathetic ophthalmia typically presents as bilateral granulomatous panuveitis that occurs after penetrating ocular injury. We describe a case of bilateral exudative retinal detachments in a patient with Turner syndrome and sympathetic ophthalmia. COMMENTS: To our knowledge, this is the first report of sympathetic ophthalmia in Turner syndrome.     

A case of multiple cranial nerve palsies as the initial ophthalmic presentation of antiphospholipid syndrome

PURPOSE: To report a case of third, fourth, and six cranial nerve palsies with antiphospholipid syndrome (APS). METHODS: Medical records of a 16-year-old female diagnosed with idiopathic intracranial hypertension (IIH) in primary APS were reviewed. RESULTS: A 16-year-old female presented with headache and diplopia. Ocular examinations revealed marked bilateral disc edema. She was unable to depress, adduct, and abduct in left eye and had limited abduction in the right eye. Cerebrospinal fluid had a normal composition and a pressure of 400 mmH20. Lupus anticoagulant and IgG anticardiolipin antibody were positive. There was no clinical evidence of other autoimmune disease. Brain magnetic resonance (MR) imaging, MR angiography, and conventional angiogram with venous phase were normal. She was diagnosed with bilateral sixth, and left third and fourth cranial nerve palsies secondary to idiopathic intracranial hypertension in primary APS. CONCLUSIONS: To our knowledge this is the first reported case of concurrent third, fourth, and sixth cranial nerve palsies in a patient with primary APS.     

Septic thrombosis of cavernous sinus extended to the ipsilateral internal jugular vein and transversal sinus with favorable outcome: Clinical and radiological features of a Lemierre syndrome

An 86-year-old patient was hospitalized for fever and left acute orbital syndrome (red eye with moderate visual impairment, chemosis, ophthalmoplegia without proptosis, or any fundus involvement). CT scan showed superior ophthalmic vein and cavernous sinus enlargement complicating ipsilateral sphenoidal sinusitis. Magnetic resonance imaging (MRI) demonstrated the left cavernous sinus thrombosis extended to the ipsilateral jugular vein and transversal sinus (Lemierre syndrome). Intravenous broad-spectrum antibiotics and curative anticoagulation were prescribed. Blood cultures allowed the identification of Streptococcus intermedius and Staphylococcus warneri species. Evolution was favorable and the patient was discharged 3 weeks after. Antibiotics and anticoagulation were carried out for a total duration of 4 and 12 weeks, respectively. Lemierre syndrome is a potentially life-threatening emergency rarely encountered; thus, ophthalmologists should be cognizant of clinical and radiological features. Broad-spectrum antibiotics are the mainstay of treatment. Curative anticoagulation may be added if no blood coagulation disorder nor bleeding on neuroimaging has been identified.     

Bilateral panophthalmitis as the initial presentation of meningococcal meningitis in an infant.

Endophthalmitis is a well-recognized, frequently devastating ophthalmic disease. The colonization of the eye and the subsequent development of endophthalmitis may be exogenous (including postsurgical and post-traumatic infections) or it may be of endogenous origin, representing a metastasis from a focus of infection elsewhere in the body associated with bacteremia (such as meningitis or cellulitis).