Pontobulbar Palsy and Neurosensory Deafness (Brown-Vialetto-van Laere Syndrome) With Hyperintense Brainstem Nuclei on Magnetic Resonance Imaging: New Finding in Three Siblings-Reference-Cited by-同舟云学术

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

Published:2006-06 Issue:6 Volume:21 Page:523-525
ISSN:0883-0738
Container-title:Journal of Child Neurology
language:en
Short-container-title:J Child Neurol

Author:

Koul Roshan¹,Jain Rajeev²,Chacko Alexander¹,Alfutaisi Amna¹,Hashim Javad³,Chacko Jacob⁴

Affiliation:

1. Division of Paediatric Neurology, Sultan Qaboos University Hospital Al-Khod, Sultanate of Oman

2. Department of Radiology, Sultan Qaboos University Hospital Al-Khod, Sultanate of Oman

3. Department of Child Health, Sultan Qaboos University Hospital Al-Khod, Sultanate of Oman

4. Department of Medicine Sultan Qaboos University Hospital Al-Khod, Sultanate of Oman

Abstract

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).

Publisher

SAGE Publications

Subject

Clinical Neurology,Pediatrics, Perinatology, and Child Health

Link

http://journals.sagepub.com/doi/pdf/10.1177/08830738060210060501

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