Dilated pupils and loss of accommodation following diode panretinal photocoagulation with sub-tenon local anaesthetic in four cases.
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AIM: To describe pupillary abnormalities associated with diode laser photocoagulation with subTenon's local anaesthetic. METHODS: We describe four cases of tonic pupils following diabetic panretinal photocoagulation with subTenon's local anaesthetic. RESULTS: Six pupils of four patients became dilated and sustained loss of accommodation with denervation hypersensitivity with 0.1% pilocarpine after undergoing panretinal photocoagulation with a subTenon's local anaesthetic. The numbers of burns were not excessive and in one patient it even occurred after only 1200 laser burns. CONCLUSION: Diode laser causes histological changes deeper in the retina and the choroid than in Argon laser. Intense diode laser burns may cause damage to the short ciliary nerves traversing the choroid. In the unanaesthetised patient, pain arises when the laser burn hits these nerves and so may protect the eye from excess damage to the nerves. When the eye is anaesthetised with a subTenon's, local anaesthetic damage to the choroidal nerves may occur without the laser operator being aware that the burns are too intense. (+info)
Effect of posture on blood and intraocular pressures in multiple system atrophy, pure autonomic failure, and baroreflex failure.
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BACKGROUND: Intraocular pressure (IOP) may be partially under systemic vascular control. This study examined whether a correlation exists between blood and intraocular pressures in patients with autonomic dysfunction. METHODS AND RESULTS: Eleven patients with 3 types of confirmed autonomic dysfunction (multiple system atrophy, pure autonomic failure, and baroreflex failure) were compared with age- and gender-matched controls and had IOP, systolic blood pressure, diastolic blood pressure, heart rates, and calculated mean arterial pressures (MAP) and mean ocular perfusion pressures (MOPP) measured in the supine, sitting, and standing positions. Data were analyzed with a general linear model repeated-measures ANOVA. All pressures for patients showed a dramatic decline (P<0.001) from supine to standing (MAP -31+/-14 mm Hg; IOP -6+/-3 mm Hg; MOPP -25+/-14 mm Hg) compared with controls (MAP +4+/-7 mm Hg; IOP -1+/-2 mm Hg; MOPP +6+/-7 mm Hg). There was no significant change in heart rate from supine to standing for patients compared with controls (P=0.648). Within both the multiple system atrophy (n=5) and pure autonomic failure (n=4) groups, all initial pressures were similar to control pressures in the supine position, whereas patients with baroreflex failure (n=2) had higher mean pressures than respective controls in the supine position. CONCLUSIONS: In autonomic dysfunction, a large decrease in MAP correlated with a large decrease in IOP. These data suggest that the autonomic nervous system, perhaps through an influence on systemic blood pressure, has a significant role in IOP regulation. (+info)
Neuro-ophthalmologic and electroretinographic findings in pantothenate kinase-associated neurodegeneration (formerly Hallervorden-Spatz syndrome).
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PURPOSE: The onset of pantothenate kinase-associated neurodegeneration (PKAN) occurs in the first and second decade of life and a pigmentary retinal degeneration is a feature of the disorder. Since the neuro-ophthalmologic and electroretinographic (ERG) features have never been well delineated, we describe them in 16 patients with PKAN. DESIGN: Observational case series. METHODS: Sixteen patients with genetic and neuroimaging-confirmed PKAN were examined. Ten underwent neuro-ophthalmologic examination and all had ERGs. RESULTS: Of the 10 who underwent neuro-ophthalmologic examination, all showed saccadic pursuits and eight showed hypometric or slowed vertical saccades. Seven of eight had inability to suppress the vestibulo-ocular reflex; two patients could not cooperate. Two had square wave jerks and four had poor convergence. Vertical optokinetic responses were abnormal in five, and two patients had blepharospasm. Eight patients had sectoral iris paralysis and partial loss of the pupillary ruff consistent with Adie's pupils in both eyes. Only four of 10 examined patients showed a pigmentary retinopathy, but 11 of 16 had abnormal ERGs ranging from mild cone abnormalities to severe rod-cone dysfunction. No patient had optic atrophy. The PANK2 mutations of all of the patients were heterogeneous. CONCLUSIONS: Adie's-like pupils, abnormal vertical saccades, and saccadic pursuits were very common. These findings suggest that mid-brain degeneration occurs in PKAN more frequently than previously thought. ERG abnormalities were present in approximately 70% and no patient had optic atrophy. Although genotype-ocular phenotype correlations could not be established, allelic differences probably contributed to the variable clinical expression of retinopathy and other clinical characteristics in these patients. (+info)
Ross syndrome: a rare or a misknown disorder of thermoregulation? A skin innervation study on 12 subjects.
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Ross syndrome is described as a rare disorder of sweating associated with areflexia and tonic pupil. Since Ross's first description in 1958, approximately 40 cases have been described. We assessed the involvement of cutaneous innervation in 12 subjects with Ross syndrome using quantitative sensory testing, sweating assessment and immunohistochemical study of anhidrotic and hyperhidrotic skin. This evaluation was repeated over time in 4 out of 12 subjects. In addition, we enrolled four subjects with Holmes-Adie syndrome (areflexia and tonic pupil) to investigate similarities between the two conditions. We found in Ross patients a complex and progressive involvement of cutaneous sensory and autonomic innervation underlying the impairment of heat production and heat dissipation through both loss of sweating and loss of cutaneous blood flow regulation. In Holmes-Adie subjects we found a mild impairment of sweating without thermoregulatory problems. The persistence of a sudomotor vasoactive intestinal peptide-immunoreactive (VIP-ir) innervation, although deranged and poor, definitely differentiated Holmes-Adie from Ross patients. Ross syndrome is a progressive and complex disorder of thermoregulation difficult to differentiate from the probably pathogenetically related Holmes-Adie syndrome. Sweating assessment and skin biopsy are suitable tools to define a boundary between them. Owing to the large number of Ross patients observed in only 5 years, and to the long and complex medical history of most of them, doubts arise on the effective rarity of this condition, and we warn family doctors and other specialists, besides neurologists, to become aware of this complex disorder. (+info)
Bilateral tonic pupils: Holmes Adie syndrome or generalised neuropathy?
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AIM: To compare the pupil signs in patients with bilateral pupillotonia caused by Holmes-Adie syndrome or generalised peripheral neuropathy. METHODS: Infrared video pupillographic techniques were used to measure a number of pupil variables in patients with Holmes-Adie syndrome, generalised neuropathy (various aetiologies) and healthy age-matched control subjects. RESULTS: Regardless of aetiology, the patients generally had pupil signs typical of pupillotonia (small dark diameters, large light diameters, tonic near responses, attenuated light responses with light-near dissociation, and sector palsy). However, significant differences were found in the prevalence and magnitude of several pupil variables in the two patient groups. In particular, sector palsy and anisocoria exceeding 1 mm (in the light) were seen much more commonly in Holmes-Adie patients than patients with generalised neuropathy. The presence of both these pupil signs can be used to distinguish between these diagnoses with a sensitivity of 58% and a specificity of 90%. CONCLUSIONS: The tonic pupils of patients with Holmes-Adie syndrome are significantly different to those found in patients with generalised neuropathy; recognition of these differences may allow distinction between these diagnoses. (+info)
Larger tonic pupil size in young children with autism spectrum disorder.
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Tonic pupil following the use of dermatoscope.
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Adie's tonic pupil is a benign condition supposed to be due to a damage of the parasympathetic innervation of the eye. It may cause considerable anxiety in the patient and lead to extensive but futile investigations. Here, we describe a 34-year-old dermatologist who developed a tonic pupil just after she had started to use intensively a 6 light-emitting diode dermatoscope. We hypothesize that frequent, repetitive exposures of one eye to intense light might fatigue the pupillary reflex and trigger the appearance of a tonic pupil. (+info)
Adult-onset migraine-related ophthalmoplegia and omolateral fetal-type posterior cerebral artery.
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