Patients suffering from Urbach-Wiethe syndrome (UWS), also known as lipoid proteinosis or hyalinosis cutis et mucosae, may have an ophthalmologist involved in the diagnosis and management of their disease. Along with moniliform blepharosis as a pathognomonic feature of the disease, an ophthalmologist may encounter other manifestations of UWS in any part of the eye such as cornea; conjunctiva; sclera; trabecular meshwork; iris/pupil; lens and zonular fibers; retina; nasolacrimal duct. This paper provides a review on the pathogenesis and the diverse ocular manifestations seen in UWS patients. Uncommon complications are discussed in this paper (glaucoma; dry eye and epiphora; complications of lens, retina, cornea; iris/pupil and conjunctiva). Moreover, a 27-year-old male UWS patient is described with bilateral diffuse anterior stromal iris atrophy, diffuse keratic precipitates; posterior subcapsular cataract; 1 + vitreous cell in anterior vitreous examination. This case was thought to be the first instance of bilateral uveitis associated with UWS. Overall, ophthalmologists may encounter diverse ocular complications accompanying this syndrome. They should be familiar with well-established ophthalmologic manifestations leading them to cooperate with other specialists in diagnosis and management of the disease.

To investigate any correlation between lower lid retraction and proptosis and also between lower lid retraction and lamellar length, as measured by fornix depth, in patients with thyroid eye disease (TED). One hundred and sixty-six eyes of 83 patients with TED were enrolled. The inferior fornix depth, Hertel exophthalmometry measurement, clinical activity score, and lower lid position were the main outcome variables. The correlation between lower lid position measurement and Hertel measurements and also between the lower lid position measurement and inferior fornix depth were evaluated using ANOVA and Pearson's tests. The mean age of subjects in patients with and without lid retraction was 42.8 ± 1.5 and 47.7 ± 1.6 years, respectively (P = 0.4). The inferior fornix depth in patients with and without lower lid retraction was 11.8 ± 1.5 and 11.8 ± 1.3 mm, respectively (P = 0.960). Pearson's analysis showed a significant correlation between the degree of proptosis and lower lid retraction in TED patients (P = 0.01). However, no significant correlation was found between the level of lower lid retraction and the fornix depth (P = 0.87). The main cause of lower lid retraction in TED is proptosis. The beneficial effect of orbital decompression on improvement of lower lid retraction must be considered during a stepwise surgical approach in TED patients.

The function of subthalamic nucleus (STN) which is a part of the basal ganglia system is not clear, but it is hypothesized that this component might be involved in action selection. Unilateral damage to STN, which can commonly occur due to the small vessel stroke mainly, causes hemiballismus and sometimes hemichorea-hemiballismus. This paper deals with a 60-year-old patient with sudden onset of abnormal movements in his right limbs. He had increased appetite and hyperphagia and also developed mood and behavioral changes (aggressiveness, irritability, anxiety, and sometimes obscene speech). The magnetic resonance imaging revealed infarct area in left subthalamus. In our case, hemiballismus is caused by infarction in left subthalamic area. Occurrence of irritability, anxiety, and some behavioral changes such as aggressiveness and obscene speech can be explained by impairment of STN role in nonmotor behavior and cognitive function as a result of infarct.

PurposeAmeloblastoma is a non-encapsulated and slow-growing tumor with high recurrence rate. Orbital involvement by this neoplasm is an extremely rare entity. In this study, we present a systematic review on this situation along with clinical and paraclinical features of a case.MethodsAn electronic search was conducted on major medical sources. Data of the cases in the literature in addition to our own case were extracted, summarized, and statistically analyzed.ResultsA total of 36 other cases from 20 relevant studies were also reviewed. Review topics included epidemiology, clinical presentation, pathologic features, differential diagnosis, imaging, treatment, and prognosis. We provided a five-year history of a 50-year-old man with orbital/skull base invasion of plexiform maxillary ameloblastoma.ConclusionsMaxillary ameloblastoma is a locally aggressive neoplasm, and physicians must be alert to the biologic behavior of this tumor to detect any invasion to critical structures such as orbit and cranium. Orbital ameloblastoma causes significant morbidity and mortality. We advocate meticulous patient follow-up with regular clinical examinations and paraclinical work-up for timely detection of any invasion or recurrence. The best must be done to avoid extensions by aggressive removal of maxillary ameloblastoma.

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