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Indian Pediatr 2014;51: 941

Half Expressions: A Case of Unilateral Multiple Cranial Nerves Hypoplasia


*Ajay Sharma, Naveen Bhardwaj and Sandesh Guleria

Department of Paediatrics, Dr RPGMC, Tanda,  Kangra (HP)
Email: * [email protected]

 


A 5-month-old male child with normal birth events presented with history of asymmetric face, and inability to close left eye since birth. There was history of deviation of mouth while smiling, and gurgling sound from throat along with regurgitation of feed from nose on and off. Examination was suggestive of left sided infranuclear facial nerve and 8th , 9th ,10th, 12th cranial nerve palsy (Fig. 1). Rest of central nervous system and systemic examination was normal.

Fig. 1 Photograph of infant showing LMN left sided facial nerve palsy (LMN, lower motor neuron ).

Fig. 2 MRI showing hypoplasia of multiple cranial nerves on the right side.

MRI brain showed hypoplasia of these cranial nerves on right side (Fig. 2) including that of 11th cranial nerve. In addition to syndromes like Duane syndrome, Congenital fibrosis of the extra ocular muscles (CFEOM), congenital ptosis, congenital facial palsy and Möbius syndrome, there exist a number of rare cases with congenital abnormalities of other cranial nerves. These occur due to defect in molecular signals that determine neuronal identity and axonal guiding in the brainstem.
 
 

 

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