Babinski’s sign was positive in both feet, although there were no signs of spastic or ataxic movement in his limbs and in his gait. The distal portion of the upper limbs was slightly weak and the deep tendon reflexes in the limbs were slightly accentuated. Although the light reflex was absent, the near reflex was normal. On examination, he presented pupillary abnormalities which were similar to those of patient 1 (mydriasis 6.5 mm, light-near dissociation). He received dialysis three times a week because he had renal failure due to pyelonephritis. He consulted our clinic for examination, although he had not experienced any neurological problems. Patient 2 was a 20 year old man, the brother of patient 1. 99mTechnetium-hexamethylpropyleneamine oxime ( 99mTc-HMPAO) SPECT disclosed a hypoperfusion of the cerebellar vermis, pons, and basal ganglia. Brain MRI showed remarkable atrophy of the cerebellum and a slight atrophy of the pontine tegmentum. Her pupils reacted to 1% pilocarpine, but not to 0.2% pilocarpine. Blood and urine laboratory findings were normal. Her speech was ataxic slight limb ataxia was detected in the limbs and her gait was wide based and ataxic. Babinski’s and Chaddock’s signs were positive on both sides. The deep tendon reflexes were augmented in her upper and lower limbs.
The distal muscles of the limbs were slightly weak, although muscle tone was normal. Her tongue showed atrophy and fasciculation. The extraocular movements were saccadic and the upper gaze of both eyes was slightly limited.
Neurological examination showed bilateral mydriasis (7.0 mm in diameter) and light-near dissociation (figure). The condition of our patient deteriorated gradually, and she was admitted to our hospital in April, 1997. Her mother had had gait disturbance since her 20s and died of pneumonia at the age of 35.
Thereafter, she noticed difficulties in speech and in the fine movement of her hands. Patient 1 was a 21 year old woman who complained of gait instability in 1996.
We present a patient with spinocerebellar ataxia type 1 (SCA1) and her asymptomatic younger brother who both exhibited pseudo-Argyll Robertson pupil. Dilute pilocarpine constricts the pupils of patients with Holmes-Adie pupil, but it is not effective in patients with pseudo-Argyll Robertson pupil. The responsible lesion in pseudo-Argyll Robertson pupil is in the central region, whereas that of Holmes-Adie pupil is peripheral. 1 Although the appearance of pseudo-Argyll Robertson pupil is very similar to Holmes-Adie pupil, the first is distinguishable from the second by the location of lesions and pharmacological response. It has been reported in patients with diabetes mellitus, multiple sclerosis, Wernicke’s encephalopathy, sarcoidosis, tumours, and haemorrhage. Conveniently, a popular mnemonic to remember Argyll Robertson pupils is that, just like prostitutes, they "accommodate but do not react".A pseudo-Argyll Robertson pupil is a neurological sign indicating a normal near reflex but the absence of a light reflex (light-near dissociation), a lack of miosis, and pupil irregularity. Named after Douglas Argyll Robertson (1837–1909), a Scottish surgeon and ophthalmologist, who first described this condition in mid-1860s in patients with neurosyphilis.Īrgyll Robertson pupils are also sometimes called "prostitute's pupils" because of their association with late neurosyphilis. The exact anatomical lesion behind this phenomenon is unknown but is thought to be caused by bilateral damage of the pretectal nuclei in the midbrain. When seen in these non-syphilitic etiologies, the pupil is termed ' pseudo-Argyll Robertson pupil' 3. It is a highly specific sign of late neurosyphilis, however can also occur in diabetic neuropathy, multiple sclerosis, alcoholic midbrain degeneration, and stroke 2,3. Argyll Robertson pupil is usually bilateral and presents as bilaterally miotic and irregular pupils, which constrict briskly with accommodation but do not react to bright light therefore displaying light-near dissociation 1.
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