Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0029089 (ophthalmoplegia)
 3,338 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

One-and-a-half syndrome is a clinical disorder featuring extraocular movements characterized by horizontal conjugate gaze palsy with internuclear ophthalmoplegia. It usually results from a unilateral lesion of the midbrain, and the most common cause of this syndrome in young women is multiple sclerosis. We report the case of a 38-year-old woman diagnosed as having acute myeloblastic leukemia presenting with characteristic neurologic and imaging features of one-and-a-half syndrome. Hyperleukocytosis, cancer procoagulants, tissue factor expression, and the increased proteolysis of coagulation factors by leukemic cells may all contribute to the propensity for thrombotic vascular occlusion. The optimal treatment of acute brain infarction in acute leukemia patients with hyperleukocytosis remains unclear. However, this patient illustrates that leukapheresis alone can provide rapid and effective relief of visual symptoms without neurologic sequela. To achieve better outcomes and survival, clinicians must maintain a heightened awareness of this distinctly unusual manifestation.
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PMID:Acute myeloid leukemia presenting as one-and-a-half syndrome. 1841 Aug 25

Central nervous system manifestations of acute myeloid leukemia are rare at presentation. Acute cranial nerve findings on neurologic examination can be indications for brain imaging. Magnetic resonance imaging can highlight cranial nerves emerging from the brainstem, particularly if they are gadolinium-enhanced or thickened. We describe a 15-month-old girl with acute unilateral ophthalmoplegia as the presenting sign of acute myeloid leukemia. Her presentation emphasizes the importance of appropriate laboratory and radiographic evaluation in a toddler with new-onset strabismus, which may be discounted as a previously unrecognized or benign finding.
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PMID:Acute unilateral ophthalmoplegia as the presenting sign of acute myeloid leukemia in a 15-month-old girl. 2304 20

A 64-year-old woman with relapsed acute myelogenous leukemia (AML) undergoing salvage chemotherapy developed rapid onset of right-sided ophthalmoplegia, proptosis, optic neuropathy, and vision loss from 20/30 to hand motions over a 3-hour period on day 4 of her treatment. CT scan of her orbits revealed a superolateral orbital mass and periocular edema. She underwent immediate canthotomy and cantholysis, and lateral orbitotomy with debulking of the mass later the same day. The histopathology was consistent with aggregates of myeloid blasts. Her vision recovered to 20/20 on postoperative day 1. Orbital granulocytic sarcoma is a rare condition often concurrent with AML, typically in the pediatric population and rarely in adults. Presentation as a fulminant orbitopathy with rapidly progressive optic neuropathy and vision loss over several hours has not been previously reported.
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PMID:Granulocytic Sarcoma of the Orbit Presenting as a Fulminant Orbitopathy in an Adult With Acute Myeloid Leukemia. 2585 4

Epstein-Barr virus (EBV)-associated smooth muscle tumors (SMTs) have recently been associated with primary and secondary immunodeficiencies. They are broadly divided into 3 subgroups: HIV-related, posttransplant, and congenital immunodeficiency. Subsequent to organ transplantation and acquired immunosuppression, a few cases of EBV-associated SMTs have been described in the liver, respiratory tract, and gastrointestinal system. To the authors' knowledge, intracranial involvement after peripheral blood stem cell transplantation has never been reported previously. The authors describe the case of a 65-year-old woman who presented with recent-onset painful ophthalmoplegia. She had a prior history of acute myelogenous leukemia requiring allogenic peripheral blood stem cell transplantation 2 years earlier, but she was in a remission phase. Imaging revealed a T1/T2 isointense, homogeneously enhancing lesion of the left cavernous sinus. A presumptive diagnosis of Tolosa-Hunt syndrome was made, and she was treated with steroids; however, her symptoms progressed quickly and repeat imaging revealed that the lesion was growing. To rule out leukemic deposits, a minimally invasive lateral orbitotomy extradural transcavernous approach was performed for biopsy sampling and debulking of the lesion. The biopsied tumor tissue was found to be infiltrative, grayish, firm, and moderately vascular. The final pathology results indicated an EBV-associated SMT of the cavernous sinus. Subsequently, the patient's steroid treatment was stopped and she had obtained partial symptomatic relief at her last follow-up visit, 3 months after surgery. EBV-associated SMT should be included in the differential diagnosis for intracranial and dural-based central nervous system lesions, especially in immunocompromised patients. Paradoxical response to steroids with worsening of symptoms is a hallmark of EBV-associated SMTs.
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PMID:Epstein-Barr virus-associated smooth muscle tumor of the cavernous sinus: a delayed complication of allogenic peripheral blood stem cell transplantation: case report. 2734 Oct 41