Pontobulbar Palsy and Neurosensory Deafness (Brown-Vialetto-van Laere Syndrome) With Hyperintense Brainstem Nuclei on Magnetic Resonance Imaging: New Finding in Three Siblings

Three siblings (one boy and two girls) with Brown-Vialetto-van Laere syndrome are reported. A peculiar feature of onset with hearing loss in a patient with multiple cranial nerve palsies and a positive family history suggests this diagnosis. In our family, an autosomal recessive mode of inheritance...

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Bibliographic Details
Published in:Journal of child neurology 2006-06, Vol.21 (6), p.523-525
Main Authors: Koul, Roshan, Jain, Rajeev, Chacko, Alexander, Alfutaisi, Amna, Hashim, Javad, Chacko, Jacob
Format: Article
Language:English
Subjects:
Adolescent
Brain Stem - pathology
Bulbar Palsy, Progressive - pathology
Child
Female
Hearing Loss, Sensorineural - pathology
Humans
Magnetic Resonance Imaging
Male
Optic Nerve - pathology
Pedigree
Syndrome
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Summary:Three siblings (one boy and two girls) with Brown-Vialetto-van Laere syndrome are reported. A peculiar feature of onset with hearing loss in a patient with multiple cranial nerve palsies and a positive family history suggests this diagnosis. In our family, an autosomal recessive mode of inheritance was seen. In addition, we observed that early onset was associated with rapid deterioration and death. Optic nerve involvement and hyperintensity of the brainstem nuclei on magnetic resonance imaging (MRI) are two newfeaturesdescribedin this report.(J Child Neurol 2006;21:523—525; DOI 10.2310/7010.2006.00096).
ISSN:0883-0738
1708-8283
DOI:10.1177/08830738060210060501