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Symptoms ⬇

See 10.5, ISOLATED THIRD CRANIAL NERVE PALSY.

Signs ⬆ ⬇

(See Figures 10.6.1 and 10.6.2.)

The most common signs of aberrant third cranial nerve regeneration include the following:

  • Eyelid-gaze dyskinesis: Elevation of involved eyelid on downgaze (Pseudo-von Graefe sign) or adduction.
  • Pupil-gaze dyskinesis: Pupil constricts on downgaze or adduction.
  • Other signs may include limitation of elevation and depression of eye, adduction of involved eye on attempted elevation or depression, absent optokinetic response, or pupillary light-near dissociation.

10-6.2 Aberrant regeneration of right third cranial nerve showing right upper eyelid retraction on attempted left gaze.

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10-6.1 Aberrant regeneration of right third cranial nerve showing right-sided ptosis in primary gaze.

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Etiology ⬆ ⬇

Thought to result from misdirection of the third cranial nerve fibers from their original destination to alternate third cranial nerve controlled muscles (e.g., inferior rectus to the pupil).

  • Aberrancy from congenital third cranial nerve palsies: Can be seen in up to two-thirds of these patients.
  • Aberrancy from prior acquired third cranial nerve palsies: Seen most often in patients recovering from third cranial nerve damage by trauma or compression by a posterior communicating artery aneurysm.
  • Primary aberrant regeneration: A term used to describe the presence of aberrant regeneration in a patient who has no history of a third cranial nerve palsy. Usually indicates the presence of a progressively enlarging parasellar lesion such as a carotid aneurysm or meningioma within the cavernous sinus.

Work Up ⬆ ⬇

Workup
  1. Aberrancy from congenital: None. Document workup of prior congenital third cranial nerve palsy.
  2. Aberrancy from acquired: See 10.5, ISOLATED THIRD CRANIAL NERVE PALSY. Document workup of prior acquired third cranial nerve palsy if previously obtained.
  3. Primary aberrancy: All patients must undergo neuroimaging to rule out slowly compressive lesion or aneurysm.
NOTE:

Ischemic third cranial nerve palsies DO NOT produce aberrancy. If aberrant regeneration develops in a presumed ischemic palsy, neuroimaging should be performed.

Treatment ⬆ ⬇

  1. Treat the underlying disorder.
  2. Consider strabismus surgery if significant symptoms are present.

Follow Up ⬆

  1. Aberrancy from congenital: Routine.
  2. Aberrancy from acquired: As per the underlying disorder identified in the workup.
  3. Primary aberrancy: As per neuroimaging and clinical examination findings. Patients are instructed to return immediately for any changes (e.g., ptosis, diplopia, sensory abnormality).