A 39-year old woman with asymmetric facial flushing

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Presentation transcript:

A 39-year old woman with asymmetric facial flushing Teaching NeuroImages Neurology Resident and Fellow Section © 2016 American Academy of Neurology

Vignette A 39-year old woman with asymmetric facial flushing developed: Episodic deformation of her left pupil Left cheek pain at mealtime 2 months later, left pupil became permanently miotic © 2016 American Academy of Neurology Ladaique et al.

Imaging Left. Selfie of left pupillary deformation known as tadpole pupil. This is presumably sympathetic overactivity causing segmental spasm of the iris dilator. Some tadpole pupils evolve to permanent Horner syndrome (sympathetic deficit). Right. Absence of facial flushing and sweating on the left side during sport activities indicating sudomotor fiber dysfunction. Ladaique et al. © 2016 American Academy of Neurology

Imaging Top. Baseline anisocoria (pre-apraclonidine) in roomlight. Bottom. After instillation of one drop of topical apraclonidine in each eye, the miotic left pupil became mydriatic; the right pupil remained unchanged. This reversal of anisocoria is due to adrenergic denervation hypersensitivity of the left iris dilator and confirms a Horner syndrome. © 2016 American Academy of Neurology Ladaique et al.

Multiple clinical manifestations of a ganglionic sympathetic defect Patient had harlequin sign, a tadpole pupil which evolved to a Horner syndrome and cheek pain at mealtime. The pain is “First bite syndrome” , due to parasympathetic overactivation of myoepithelial cells in the parotid gland. These findings localize the site of dysfunction to the superior cervical ganglion. The cause remains unknown. References: 1.Drummond PD, Lance JW. Facial flushing and sweating mediated by the sympathetic nervous system. Brain. 1987;110:793-803. 2.Laccourreye O, Werner A, Garcia D, Malinvaud D, Tran Ba Huy P, Bonfils P. First bite syndrome. Eur Ann Otorhinolaryngol Head Neck Dis. 2013;130:269-273. © 2016 American Academy of Neurology Ladaique et al.