Journal of Child Neurology

 

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Journal of Child Neurology, Vol. 21, No. 6, 523-525 (2006)
DOI: 10.1177/08830738060210060501

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

Roshan Koul, MD, DM, FRCPCH, FAAN

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

Rajeev Jain, MD

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

Alexander Chacko, MD, DCH

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

Amna Alfutaisi, MD, FRCPC

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

Javad Hashim, MD, MRCPCH

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

Jacob Chacko, MD, DM

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

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).


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