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Segmental facial anhidrosis and tonic pupils with preserved deep tendon reflexes: a novel autonomic neuropathy.

A 31-year-old woman had exertional right-sided hemifacial flushing and sweating. Examination demonstrated slightly dilated pupils with absent constriction to light and a tonic near response and redilatation, features consistent with Adie syndrome. Neurological examination was otherwise normal, including preservation of deep tendon reflexes. Magnetic resonance imaging of brain and spine were normal. The combination of unilateral loss of sudomotor and vasomotor activity without loss of ocular sympathetic innervation fulfills the diagnosis of Harlequin syndrome. The combination of Harlequin and Adie syndromes has been called Ross syndrome, but the preservation of deep tendon reflexes precludes a diagnosis of Ross syndrome in our patient. This previously undescribed variant adds further complexity to the spectrum of autonomic neuropathies.

Adie Syndrome↗

Accommodative fluctuations in Adie's syndrome.

Recent reports suggest that the higher frequency components (around 2 Hz) of accommodative fluctuations may arise from the accommodative 'plant' and may have no role in the control of the accommodation system. The dynamic responses of accommodation in two cases with Adie's syndrome were recorded. The results showed that the velocity of accommodative responses was reduced, and the accommodative fluctuations in the higher frequency components were completely diminished. It can be concluded that the origin of fast fluctuations is not located at a more peripheral site than the lesion of tonic accommodation. Since the possible lesion exists at the ciliary ganglion, the origin of the fast oscillation may not be the accommodative 'plant', but the neural one.

Accommodation, Ocular↗