A Unique Manifestation of Pupillary Fatigue in Autoimmune Autonomic Ganglionopathy.
OBJECTIVE
To demonstrate a unique abnormality of the pupillary light reflex in patients with autoimmune autonomic ganglionopathy (AAG).


METHODS
Case series.


METHODS
Autonomic clinics at 2 university hospitals (University of Texas Southwestern Medical Center and Beth Israel Deaconess Medical Center).


METHODS
Seven patients with antibody-positive AAG.


METHODS
All patients with AAG underwent either monocular or binocular infrared pupillometry using a standard 2-second light stimulus at a defined intensity. Findings were compared with those from healthy control subjects and patients with other autonomic disorders. The light stimulus used in this study was selected to eliminate the normal phenomenon of pupil escape.


METHODS
The time to onset of redilation as well as other indices of pupillary constriction to light stimulus.


RESULTS
Patients with AAG exhibited premature pupillary redilation (mean [SD], 1.02[0.20] seconds) compared with healthy control subjects (mean [SD], 2.24 [0.10] seconds) and other patients with autonomic disorders (mean [SD], 2.30 [0.12] seconds) (P.001). In healthy control subjects and patients with other autonomic disorders, pupillary redilation always followed the termination of the light stimulus; in patients with AAG, redilation consistently occurred during the light stimulus. In 1 patient, serial repetitive light stimulation further decreased the time to onset of redilation.


CONCLUSIONS
Premature redilation of the pupil is a unique physiological feature seen only in patients with AAG. This phenomenon appears to be a manifestation of pupillary fatigue, a clinical correlate of defective synaptic transmission at the level of autonomic ganglia in antibody-positive AAG.
Department of Neurology and Neurotherapeutics, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA. srikanth.muppidi@utsouthwestern.edu
Srikanth
S
Muppidi
Maggie
M
Scribner
Christopher H
CH
Gibbons
Beverley
B
Adams-Huet
Elaine B
EB
Spaeth
Steven
S
Vernino
D000328:Adult; D000704:Analysis of Variance; D000906:Antibodies / Q000378:metabolism; D001342:Autonomic Nervous System Diseases / Q000150:complications* / Q000276:immunology*; D005221:Fatigue / Q000150:complications / Q000209:etiology*; D005260:Female; D007839:Functional Laterality; D005725:Ganglia, Autonomic / Q000473:pathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011681:Pupil Disorders / Q000150:complications / Q000209:etiology*; D011950:Receptors, Cholinergic / Q000276:immunology; D012025:Reflex, Pupillary / Q000276:immunology*
D000906:Antibodies; D011950:Receptors, Cholinergic