Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0028738 (nystagmus)
 7,431 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Despite the well-recognized association between maternal varicella infection and a specific pattern of malformation in the offspring referred to as fetal varicella syndrome, the full spectrum of this disorder as well as its frequency in the offspring of women infected with varicella during pregnancy is unknown. In order to resolve these issues, we have evaluated pregnancy outcome in 194 women who contacted us during pregnancy, soon after the onset of their infection: 132 (68%) during the 1st trimester and 171 (88%) prior to the onset of the 20th week, the most critical period relative to fetal susceptibility. Of those 171 women, there were 146 liveborn babies, 6 spontaneous abortions, 7 therapeutic abortions (one because of an anencephalic fetus), 2 stillborn infants, and nine pregnancies lost to follow-up. Of the 146 liveborn births, there were 2 with features consistent with the fetal varicella syndrome: one a child with mild cutaneous scars on her face, arms, and legs, Horner syndrome involving her left eye and a scar on her retina, leading to blindness in that eye; and the other, a child with horizontal nystagmus who is otherwise normal at 1 year of age. An additional four liveborn infants had a single major malformation which included unilateral microtia, bowel obstruction secondary to an ileal band, bilateral double ureters, and a thyroglossal duct cyst. This study suggests that prenatal varicella infection is associated with a wider spectrum of severity than has been previously appreciated and indicates that maternal varicella infection prior to the 20th week of pregnancy is associated with only a small risk for the developing fetus.
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PMID:Offspring of women infected with varicella during pregnancy: a prospective study. 817 94

Background. Wernicke's Encephalopathy (WE) is a clinical diagnosis with serious neurological consequences. Its occurrence is underestimated in nonalcoholics and is uncommon in adolescents. We aim to draw the attention to a rare case, which had additional clinical and radiological features. Case. A 16-year-old girl presented with three-week history of vomiting secondary to intestinal obstruction. She developed diplopia soon after hospitalization. Neurological evaluation revealed restriction of bilateral lateral recti with horizontal nystagmus, and bilateral limb dysmetria. Brain MRI was normal. She had prompt improvement to thiamine. Four months later, she presented with headache, bilateral severe deafness, and tinnitus. Clinically, she had severe sensorineural hearing loss, bilateral lateral recti paresis, and gait ataxia. CT head showed bilateral caudate nucleus hypodensities. MRI brain revealed gadolinium enhancement of mamillary bodies and vermis. She had significant improvement after IV thiamine. Headache completely resolved while the ocular movements, hearing, and tinnitus improved partially in 72 hours. Conclusions. Recurrent WE in adolescence is uncommon. Headache, tinnitus, and deafness are rare clinical features. Although MRI study shows typical features of WE, the presence of bilateral caudate nuclei hypodensities on CT scan is uncommon. Prompt treatment with thiamine is warranted in suspected cases to prevent permanent neurological sequelae.
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PMID:Recurrent Wernicke's Encephalopathy in a 16-Year-Old Girl with Atypical Clinical and Radiological Features. 2479 Jul 62