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Hypoplasia-Hyperplasia

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44•<br />

Pathophysiology<br />

Remains Unknown<br />

Brainstem Nuclei; Nerves or muscle Aplasia<br />

Involved CN: 6-12 except 8<br />

6 (Abducens) – 75%<br />

7 (Facial) – 100%<br />

8 (Vestibulocochlear) – Never<br />

9,10,11 – Variable<br />

12 (Hypoglossal) – Rare<br />

3,4 (Oculomotor, Trochlear)– Very rare<br />

Toxin induced (Moebius)<br />

Vascular etiology:<br />

Theories<br />

- Basilar artery insufficiency<br />

- Premature regression of trigeminal a.<br />

- Subclavian a. disruption sequence<br />

Mesodermal dysplasia of muscles from 1 0 & 2 0 Branchial<br />

arches<br />

CN Nuclei changes are 2 0 to retrograde atrophy<br />

Disruption sequence of normal morphology @ 4-7 wks<br />

(Explains simultaneous limb anomalies)

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