Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0022568 (keratitis)
 5,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A consecutive series of 14 patients with trigeminal schwannoma managed surgically at the Neurological Institute of New York since 1970 is reported. Nine women and five men (mean age 40 years) were diagnosed following a mean symptom duration of 33 months. Abnormalities of trigeminal nerve function were present in 11 patients on admission examination. Facial pain was a prominent feature in eight patients. Two patients, both with schwannomas arising from the trigeminal root, presented initially with typical trigeminal neuralgia. Additional cranial nerve palsies or cerebellar or pyramidal tract signs were noted in eight patients. The surgical approach to these tumors depends on their anatomical location. Four patients had tumors confined to the middle fossa, three patients had tumors limited to the posterior fossa, and seven patients had both supratentorial and infratentorial components of their tumors. Twenty operative procedures were performed on these patients, resulting in complete extirpation in six patients, nearly complete removal in seven patients, and partial removal in one patient. Adherence of the tumor to the lateral wall of the cavernous sinus or the brain stem precluded total removal. There was one postoperative death. In the immediate postoperative period, abnormalities of cranial nerves controlling the extraocular muscles were common. In general, these deficits were transient; however, some permanent loss of trigeminal nerve function occurred in nine patients. Two patients required tarsorrhaphy for neurotropic keratitis, and two patients underwent cerebrospinal fluid (CSF) shunting procedures for hydrocephalus or for a persistent CSF leak. The follow-up period ranged from 4 to 177 months (mean 47 months). The clinical features, anatomical considerations, and surgical approach to these rare tumors are discussed. A clinical review of 106 additional cases of trigeminal schwannoma, reported in the English literature since 1935, is also presented.
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PMID:Trigeminal schwannoma. Surgical series of 14 cases with review of the literature. 305 25

A neonate presented with cloverleaf skull anomaly and severe proptosis requiring urgent cranioplasty to attain eyelid closure. Despite this, she experienced exposure keratitis and corneal perforations. A ventriculoperitoneal shunt was performed subsequently to relieve hydrocephalus, but respiratory problems eventually led to her death at 6 months. This case highlights the complexity of the problems encountered in the cloverleaf skull anomaly, and a brief review discussing its management is included. Despite improvements in treatment of this condition, the overall prognosis remains poor.
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PMID:Cloverleaf skull anomaly with extreme orbitostenosis. 1033 3

The infection of humans with the rodent-borne lymphocytic choriomeningitis virus (LCMV) can lead to central nervous system disease in adults or severe neurological disease with hydrocephalus and chorioretinitis in children infected congenitally. Although LCMV-induced meningitis and encephalitis have been studied extensively, the immunopathological mechanisms underlying LCMV infection-associated ocular disease remain elusive. We report here that the intraocular administration of the neurotropic LCMV strain Armstrong (Arm) elicited pronounced chorioretinitis and keratitis and that infection with the more viscerotropic strains WE and Docile precipitated less severe immunopathological ocular disease. Time course analyses revealed that LCMV Arm infection of the uvea and neuroretina led to monophasic chorioretinitis which peaked between days 7 and 12 after infection. Analyses of T-cell-deficient mouse strains showed that LCMV-mediated ocular disease was strictly dependent on the presence of virus-specific CD8(+) T cells and that the contribution of CD4(+) T cells was negligible. Whereas the topical application of immunosuppressive agents did not prevent the development of chorioretinitis, passive immunization with hyperimmune sera partially prevented retinal and corneal damage. Likewise, mice displaying preexisting LCMV-specific T-cell responses were protected against LCMV-induced ocular disease. Thus, antibody- and/or T-cell-based vaccination protocols could be employed as preventive strategies against LCMV-mediated chorioretinitis.
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PMID:Immunopathological basis of lymphocytic choriomeningitis virus-induced chorioretinitis and keratitis. 1894 66

Goldenhar syndrome is a rare, presumably inherited condition that has a multifactorial etiopathology that results in disturbances of blastogenesis. The syndrome is characterized by the presence of dermal epibulbar tumors, periauricular appendices, malformed ears, and vertebral anomalies, with occasional occurrence of cardiac, visceral, or central nervous system anomalies. This report describes a case of Goldenhar syndrome in a newborn who presented with all of the classic signs of Goldenhar syndrome associated with the rare occurrence of infectious keratitis and hydrocephalus.
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PMID:Infectious keratitis in a newborn with goldenhar syndrome. 2012 54