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.
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Fig. 1 Photograph of infant
showing LMN left sided facial nerve palsy (LMN,
lower motor neuron ).
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Fig. 2 MRI showing
hypoplasia of multiple cranial nerves on the right
side.
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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.