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Case Reports
. 2019 Aug 6:6:2329048X19866617.
doi: 10.1177/2329048X19866617. eCollection 2019.

Autonomic Cross-Innervation in Patients With Neurofibromatosis Type 2: Frey Syndrome and Unilateral Epiphora With Rhinorrhea

Erica Evans  1 , David Nascene  2 , Katherine Sommer  3 , Christopher Moertel  3
Affiliations
Case Reports

Autonomic Cross-Innervation in Patients With Neurofibromatosis Type 2: Frey Syndrome and Unilateral Epiphora With Rhinorrhea

Erica Evans et al. Child Neurol Open. .

Abstract

The authors present 2 cases of cross-innervation in patients with neurofibromatosis type 2. In the first case, an iodine test was performed to demonstrate Frey syndrome in a 28-year-old female with neurofibromatosis type 2 who developed symptoms at age 10 years. The second patient is an 18-year-old female with neurofibromatosis type 2, 2 years status post left vestibular schwannoma subtotal resection who presented with paradoxical unilateral lacrimation and rhinorrhea triggered by heat stress and exercise. The pathophysiology of these cases is discussed.

Keywords: auriculotemporal nerve syndrome; nervus intermedius; neurofibromatosis type 2; paradoxical lacrimation and rhinorrhea; vestibular schwannoma.

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Conflict of interest statement

Declaration of Conflicting Interests: The authors declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Dr. Moertel is a consultant for Recombinetics, Inc.

Figures

Figure 1.
Figure 1.
Arrow denotes a vestibular schwannoma in the left cerebellopontine angle cistern. Arrowhead denotes a meningioma inferior to the schwannoma that extends slightly into the pars nervosa region of the jugular foramen.
Figure 2.
Figure 2.
Magnetic resonance imaging (MRI) after left vestibular schwannoma resection. Arrows denote bilateral internal auditory canal schwannomas. The 3 arrowheads denote a presumed meningioma in the left Meckel cave.
See this image and copyright information in PMC

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