Hypoplasia-Hyperplasia

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Imaging
CT\MRI – May demonstrate Bilateral calcifications
of Basal ganglia & CN nuclei (VI, VII)
EMG – denervation potentials (found 2-3 weeks
after perinatal trauma) may differentiate trauma
from mobius
Brain bipsy is pathognomonic – Calcified necrotic
brainstem CN nuclei
Classification
Towfighi et al
I – Simple hypoplasia atrophy of CN Nuclei
II – Primary lesions in peripheral CN
III – Focal necrosis in brainstem nuclei
IV – Primary myopathy, no CNS or CN lesions