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Painful Ophthalmoplegia

Tolosa-Hunt Syndrome


Presentation

  • To present a case of peripheral T-cell lymphoma presenting as painful ophthalmoplegia. A 61-year-old woman presented with a 2-week history of headache and eyeball pain.[ncbi.nlm.nih.gov]
  • Perhaps it is only an occasional presentation of another rather poorly understood syndrome, that of "recurrent cranial neuropathies." The present patient had at least three episodes of painful ophthalmoplegia prior to this hospitalization.[ncbi.nlm.nih.gov]
  • Patients with mucormycosis rarely present with orbital apex syndrome.[ncbi.nlm.nih.gov]
  • We present a rare bacterial abscess of the cavernous sinus causing painful ophthalmoplegia.[ncbi.nlm.nih.gov]
  • A presumptive diagnosis of Tolosa-Hunt syndrome was made when he presented with left painful ophthalmoplegia.[ncbi.nlm.nih.gov]
Sulfur Granules
  • These tests demonstrated acute inflammation and sulfur granules, which responded clinically and radiologically to parenterally administered penicillin therapy.[ncbi.nlm.nih.gov]
Aspiration
  • The orbital fine-needle aspiration technique has been recommended, but experience with this method is limited, and a definitive diagnosis cannot always be reached.[ncbi.nlm.nih.gov]
Progressive Visual Loss
  • Historical review Almost 50 years ago Tolosa 2 reported a patient with left orbital pain, ipsilateral progressive visual loss, total left ophthalmoplegia, and reduced sensation over the first division of the trigeminal nerve.[jnnp.bmj.com]
Unilateral Eye Pain
  • Tolosa-Hunt is a rare condition that presents as an acute unilateral eye pain with motor dysfunction and responds to prolonged treatment with corticosteroids. Presented is a case of Tolosa-Hunt syndrome.[ncbi.nlm.nih.gov]
Unilateral Blindness
  • The ultimate evolution of our case and its atypical features of unilateral blindness, bilateral involvement, and extensive involvement of the trigeminal nerve revealed the pathogenesis of this case of alternating painful ophthalmoplegia resembling the[ncbi.nlm.nih.gov]
Facial Swelling
  • Patients with fungal infection who develop sino-orbito –cerebral syndrome often present with acute headache, fever, facial swelling, sinusitis, opthalmoplegia and/or blindness. [ 4 , 7 ] It is rare for individuals with mucormycosis to present with orbital[amhsr.org]
Aggressive Behavior
  • This case exhibited extensive and aggressive behavior simulating a malignant neoplasm.[ncbi.nlm.nih.gov]
Pyramidal Tract Signs
  • However, during his hospitalization he developed involvement of cranial nerves II, III, V, VI and VII, papilledema, pyramidal tract signs and severe psychiatric disturbances, all of which remitted.[ncbi.nlm.nih.gov]

Workup

  • A metastatic workup was performed without any evidence of extraorbital tumor. The patient was recommended to be treated with chemotherapy, however, refused to take the treatment. The patient died of progression of the disease in a month.[ncbi.nlm.nih.gov]
  • Ethan Sterk He is admitted to the neurology service, and subsequent workup includes the following tests: erythrocyte sedimentation rate, C-reactive protein, antinuclear antibody, antineutrophil cytoplasmic antibody, rapid plasma reagin, double-stranded[acepnow.com]
  • Blood and CSF workup were normal. Urgent CT angiography excluded an intracerebral aneurysm and cavernous sinus thrombosis. A repeat MRI was performed, obtaining the images below. Figure 1. MRI of the brain (A axial view. B coronal view).[nzma.org.nz]
  • However, all recruited patients had ancillary laboratory workup, cranial MR imaging, and cerebral vascular investigation.[bmcneurol.biomedcentral.com]
  • The cerebral MRI, left temporal artery biopsy and blood workup at the time were unremarkable apart from a hypercholesterolemia.[eanpages.org]
Aspergillus Niger
  • A case of painful ophthalmoplegia due to invasive aspergillosis caused by Aspergillus niger in a diabetic patient is presented.[ncbi.nlm.nih.gov]

Treatment

  • MRI and positive response to the treatment with corticosteroids were relevant for making the diagnosis.[ncbi.nlm.nih.gov]
  • While corticoids are the treatment of reference, corticodependence and corticoresistance are frequent, hence radiotherapy has been proposed as an alternative treatment.[doi.org]
  • The others have survived, but still exhibiting various neurologic abnormalities after aggressive treatment. Patients with mucormycosis rarely present with orbital apex syndrome.[ncbi.nlm.nih.gov]
  • In this study, we aimed to elucidate these causes by evaluating clinical presentations and treatment responses.[ncbi.nlm.nih.gov]

Prognosis

  • Its recognition is important since the response to steroids, although not specific, is rapid in most patients, and the prognosis for complete recovery is relatively good.[ncbi.nlm.nih.gov]
  • Individuals usually have a very good prognosis with the condition, because steroids have been proven to be an effective treatment measure Nevertheless, complications, such as the damage and abnormal repair of the cranial nerves, can worsen the prognosis[dovemed.com]
  • [ edit ] The prognosis of THS is usually considered good.[en.wikipedia.org]
  • […] involved area is hyperintense T1 C (Gd): may show contrast enhancement during active phase with resolution of enhancement following treatment 5,9 inflammatory myofibroblastic tumor (IMT) 6 idiopathic hypertrophic pachymeningitis (IHP) 6 Treatment and prognosis[radiopaedia.org]
  • Since diagnostic delays represent a life-threatening danger, early recognition of disease is important for effective treatment and good prognosis.[thejournalofheadacheandpain.springeropen.com]

Etiology

  • The etiological differential diagnosis of painful ophthalmoplegia is extensive and consists of numerous sinister etiologies including vascular (eg, aneurysm, carotid dissection, carotid-cavernous fistula), neoplasms (eg, primary intracranial tumors, local[ncbi.nlm.nih.gov]
  • The etiology is unknown.[ncbi.nlm.nih.gov]
  • This syndrome is differentiated from other causes of painful ophthalmoplegia including tumors, aneurysms, collagen disease, specific infections, mucoceles, and benign granulomas of unknown etiology.[ncbi.nlm.nih.gov]
  • The etiological differential diagnosis is extensive, including different syndromes and causes (vascular, neoplastic, infectivous, inflammatory). Current neuroimaging techniques allow visualisation of the area of the suspected pathology.[ncbi.nlm.nih.gov]
  • Painful ophthalmoplegia can be caused by various etiologies, and broad differential diagnosis is needed. Carotid-cavernous fistula (CCF) is a rare cause of painful ophthalmoplegia, and early diagnosis is quite difficult.[ncbi.nlm.nih.gov]

Epidemiology

  • Patients usually respond to corticosteroids, and spontaneous remission can occur, although movement of ocular muscles may remain damaged. [3] Roughly 30–40% of patients who are treated for THS experience a relapse. [3] Epidemiology [ edit ] THS is uncommon[en.wikipedia.org]
  • Epidemiology Frequency Tolosa-Hunt syndrome (THS) is uncommon in both the United States and internationally. The disorder is rare during the first 2 decades of life; in people older than 20 years, it appears to have an even distribution.[emedicine.com]
  • Epidemiology It is a rare condition and most of the literature is case reports rather than series. Cases of THS have been documented worldwide and at all ages. Males and females appear to be equally affected.[patient.info]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology Nonspecific inflammation (noncaseating granulomatous or nongranulomatous) within the cavernous sinus or superior orbital fissure is the cause of the constant pain, which characterizes the onset of this disorder.[emedicine.com]
  • Pathophysiology Tolosa-Hunt syndrome is idiopathic, sterile inflammation of the cavernous sinus. Its pathology is described as fibroblastic, lymphocytic, and plasmocytic infiltration of the cavernous sinus.[eyewiki.aao.org]
  • Hernia 6(2): 64-67, 2002 Pathophysiology of groin pain in sporting patients may be more complex than the standard definition of groin hernia.[eurekamag.com]

Prevention

  • Diagnosis was made by excluding the other causes, and there was a prompt response to high-dose steroid therapy, which prevents or minimizes any permanent neurologic deficit.[ncbi.nlm.nih.gov]
  • This case highlights the importance for the Emergency Physician to quickly recognize painful ophthalmoplegia and rapidly elucidate the cause to prevent further complications for the patient.[ncbi.nlm.nih.gov]
  • , prevention, prognosis, and additional useful information HERE .[dovemed.com]
  • Prevention of death, myocardial infarction, and stroke by prolonged antiplatelet therapy in various categories of patients. ‎[books.google.de]
  • Transplantation Substance Use and Addiction Surgery Surgical Innovation Surgical Pearls Teachable Moment Technology and Finance The Rational Clinical Examination Tobacco and e-Cigarettes Toxicology Trauma and Injury Treatment Adherence United States Preventive[jamanetwork.com]

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