Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0017638 (glioma)
 30,880 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 5-year-old boy with monocular vertical nystagmus, initially believed to have spasmus nutans, subsequently developed optic atrophy and visual loss. Neuroradiologic investigation indicated probable chiasmal glioma. The case of this patient re-emphasizes the necessity of careful clinical and radiologic assessment before assuming acquired monocular nystagmus to be a benign and self-limited disorder.
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PMID:Monocular vertical nystagmus as an initial sign of chiasmal glioma. 43 58

This study is a multicenter, retrospective report of 10 infants in whom acquired nystagmus was the initial sign of chiasmal/ parachiasmal glioma. Nine patients presented before the age of 10 months. The nystagmus, primarily described as pendular and asymmetric, was difficult to differentiate from and therefore most often diagnosed as spasmus nutans . On average in the ten patients, the intracranial glioma was not recognized for 8.6 months after the onset of nystagmus. In the five diagnosed as spasmus nutans , the mean delay in recognizing the tumor was 14.5 months. Three associated clinical findings were present or developed in these patients to distinguish this entity from spasmus nutans : optic atrophy in all ten patients, poor feeding due to diencephalic syndrome in 5 of 10, and increased intracranial pressure with hydrocephalus in 3 of 10. The acquired nystagmus in these infants was evidence of a life-threatening chiasmal/ parachiasmal glioma.
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PMID:Acquired nystagmus in early childhood: a presenting sign of intracranial tumor. 673 45

A small number of children who develop disconjugate nystagmus, torticollis, and head titubation (spasmus nutans) have been found to have optic chiasm or third ventricle gliomas. However, the prevalence of glioma or other developmental abnormalities in this disorder is unknown because no large series of spasmus nutans cases has previously been reported. A reviewer of the records of 67 consecutive children initially diagnosed with spasmus nutans and followed for an average of 3.3 years at the St Louis Children's Hospital revealed the following: 61% had a history of prematurity, developmental delay, or other systemic abnormality; strabismus, most commonly infantile esotropia, developed in 55%; 43% had neuroimaging studies; and 0% had evidence of a glioma or showed signs of tumor on follow-up examinations. From this consecutive patient series, we estimate the prevalence of tumor in spasmus nutans to be less than 1.4%. Without other evidence of an intracranial mass lesion, neuroimaging of infants initially diagnosed with spasmus nutans may not be immediately warranted.
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PMID:Prevalence of intracranial lesions in children initially diagnosed with disconjugate nystagmus (spasmus nutans) 896 30

: We diagnosed chiasmal glioma in an 8-month-old infant who had spasmus nutans that spontaneously resolved. Magnetic resonance imaging showed no interval change in tumor size over the next 8 months. Clinical resolution of spasmus nutans does not preclude chiasmal glioma as the underlying cause.
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PMID:Chiasmal glioma in spasmus nutans: a cautionary note. 2469 42

We report the case of a 9-year-old girl with chiasmal glioma and longstanding monocular nystagmus, in whom video-oculography showed a disconjugate binocular nystagmus indistinguishable from spasmus nutans. The "acquired monocular nystagmus" associated with chiasmal glioma is actually a binocular nystagmus that is indistinguishable from spasmus nutans, except that it lacks the associated head nodding and torticollis. Superimposed visual loss in one eye predisposes to acquired monocular nystagmus and explains the absence of the other components of the spasmus nutans triad.
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PMID:Acquired monocular nystagmus in chiasmal glioma-a video-oculographic study. 3073 Nov 26