Background: The unique neurovascular structure of the retina has provided an opportunity to observe brain pathology in many neurological disorders. However, such studies on neurodegeneration with brain iron accumulation (NBIA) disorders are lacking.
Objectives: To investigate NBIA's neurological and ophthalmological manifestations.
Objective: To describe a case of optic neuropathy after prolonged sirolimus therapy in the setting of cardiac transplant.
Background: Sirolimus is an immunosuppressant that inhibits Mechanistic Target of Rapamycin (mTOR) and blocks T-cell activation and B-cell differentiation by preventing response to Interleukin-2 (IL-2). Tacrolimus is another immunosuppressive agent, one of the known but uncommon side effects of which is bilateral optic neuropathy years after taking the medication.
Am J Ophthalmol Case Rep
September 2022
Purpose: We report a case of a 10-year-old with Moring glory disc anomaly (MGDA) associated with Moyamoya disease and pituitary stalk duplication.
Observations: A 10-year-old Asian child presented with decreased vision in the right eye and bilateral nystagmus. Both dilated fundus exam and magnetic resonance imaging (MRI) of the orbit confirmed MGDA of the right eye.
Background: Retinal biomarkers in neurodegenerative disorders have attracted much attention in recent years. Recent studies have reported visual dysfunction in Huntington's disease (HD). However, little is known about retinal structural changes in HD.
View Article and Find Full Text PDFIdiopathic intracranial hypertension (IIH) has an increasing incidence worldwide over the past decade, with a high economic burden on patients and society. Up to 10% of patients with IIH have progressive visual decline requiring an invasive intervention (including cerebrospinal fluid shunting, cerebral dural sinus stenting, or optic nerve sheath fenestration [ONSF]). IIH patients with visual decline usually undergo evaluation and initial management through the emergency department (ED) and commonly have a long hospital stay due to the lack of a dedicated methodology for evaluation and management, particularly in patients who present with visual loss (i.
View Article and Find Full Text PDFMult Scler Relat Disord
November 2021
Objective: To determine the longitudinal changes in retinal microstructure, microvasculature, microcirculation, and axonal and neuronal functions in patients with relapsing-remitting multiple sclerosis (RRMS) over the time course of about two years.
Methods: A total of 30 patients (60 eyes) with RRMS were followed for a period of 27 ± 6 months and evaluated with a battery of clinical tests including low contrast letter acuity (LCLA), intraretinal layer thicknesses by optical coherence tomography (OCT), ganglion cell function by steady-state pattern electroretinography (PERG), axonal function by polarization-sensitive OCT, volumetric vessel density (VVD) by OCT angiography, and retinal tissue perfusion (RTP) by retinal function imager.
Results: Axonal function measured as retinal nerve fiber layer birefringence in the temporal quadrant and vessel density in the deep vascular plexus were significantly decreased at 2-year follow-up (P < 0.
During the COVID-19 pandemic, elderly individuals (with the chronological age of 65 years and above) are more susceptible to the SARS-CoV-2 infection complications due to altered immune system response and the higher rate of underlying comorbidities. A vast majority of mortalities are reported in elderly patients; thus, this study aimed to evaluate complications of COVID-19 in elderly patients. A systematic review was conducted according to MOOSE guidelines.
View Article and Find Full Text PDFBackground: Impaired vision and remodeled foveal pit have been demonstrated in Parkinson's disease (PD) patients using different techniques.
Methods: Ten PD (20 eyes) and eight healthy controls (HC) subjects (16 eyes) were enrolled. Subjects were evaluated for N70 and P100 latencies using two-channel VEP with pattern reversal and on/off pattern; Contrast sensitivity (CS) using Pelli-Robson chart; macular thickness measured using Zeiss-HD optical coherence tomography (OCT).
Background: Optical coherence tomography offers a potential biomarker tool in Parkinson's disease (PD). A mathematical model quantifying symmetry, breadth, and depth of the fovea was applied.
Methods: Nintey-six subjects (72 PD and 24 healthy controls) were included in the study.
Ann Clin Transl Neurol
February 2015
Inner foveal thinning and intracellular alpha-synuclein were demonstrated in the retina in Parkinson disease. While pathognomonic alpha-synuclein is associated with embryonic dopaminergic (DA) neurons, postmortem studies in the nervous system and retina show prominent effect also in non-DA neurons. We evaluated foveal capillaries and foveal thickness in 23 Parkinson disease subjects and 13 healthy controls using retinal fluorescein angiography and optical coherence tomography.
View Article and Find Full Text PDFBackground: Restless legs syndrome (RLS) is the most common movement disorder in pregnancy, which can be idiopathic or secondary. There are limited comparative data regarding these two forms of RLS. The aim of this study was to compare clinical features of idiopathic and secondary RLS in pregnant women.
View Article and Find Full Text PDFRestless legs syndrome (RLS) is a common sleep disorder that can present secondary to medical conditions such as renal failure. This study aimed to evaluate RLS frequency and its related factors in chronic renal failure patients treated with hemodialysis. In a cross-sectional design, 163 patients with chronic renal failure were consecutively enrolled from hemodialysis center at Rasool-Akram hospital.
View Article and Find Full Text PDFLittle is known about the results of pallidal deep brain stimulation (DBS) in DYT6 dystonia. This will be the first report of DYT6 dystonia treated with pallidal DBS from Iran. A 21 years old male patient with DYT6 dystonia underwent bilateral deep brain stimulation.
View Article and Find Full Text PDFBackground: Deep brain stimulation (DBS) of the globus pallidus internus (GPi) is recommended as a promising technique for the management of the primary generalized dystonia (PGD) with DYT1 gene mutation. We present the first report of DBS results in Iranian patients with DYT1 positive PGD.
Methods: Nine patients who suffered from severely disabling DYT1 positive PGD consecutively were recruited for the study between 2008 and 2010.
Behavioral, electrophysiological, and imaging data reveal impaired visual processing and altered retinal morphology in Parkinson disease. Are visual changes epiphenomena? We report the presence of misfolded α-synuclein in the retina, not hitherto shown, in discrete retinal neurons within the inner retina. They demonstrate the histopathology that may underlie impaired vision and retinal remodeling in Parkinson disease.
View Article and Find Full Text PDFJ Neural Transm (Vienna)
November 2014
Spectral-domain Optical coherence tomography (OCT) has shown remarkable utility in the study of retinal disease and has helped to characterize the fovea in Parkinson disease (PD) patients. We developed a detailed mathematical model based on raw OCT data to allow differentiation of foveae of PD patients from healthy controls. Of the various models we tested, a difference of a Gaussian and a polynomial was found to have "the best fit".
View Article and Find Full Text PDFThe development of optical coherence tomography (OCT) has led to increasing interest in the retina in Parkinson's disease (PD). The retina is a multilayered tissue: looking into the eye from the outside, these layers comprise the nerve fiber layer (NFL); the ganglion cell layer (GCL); the inner plexiform layer (IPL), which contains the interconnecting plexus, including tyrosine hydroxylase-positive (dopaminergic) fibers of amacrine cells; the inner nuclear layer; and several outer retinal layers. Commercial spectral-domain OCT has a specific program for detecting peripapillary NFL defects and a different macular program for diabetic retinopathy.
View Article and Find Full Text PDFPseudoaneurysms of superficial temporal artery are very rare and commonly occur as a result of blunt trauma. There are several methods for the diagnosis, but the diagnosis can be accurately made only from a history and physical examination. A 27-year-old man presented with a painless pulsatile mass over his right temporal region after a blunt head trauma.
View Article and Find Full Text PDFObjective: To investigate the prevalence of restless legs syndrome (RLS) during pregnancy and to evaluate factors associated with RLS in a population of Iranian pregnant women.
Methods: In the present cross-sectional study, 443 consecutive pregnant women admitted for delivery underwent an interview within 2 days of parturition. The diagnosis of RLS was established by the 4 criteria of the International Restless Legs Syndrome Study Group (IRLSSG).
Restless legs syndrome (RLS) is a common neurological movement disorder that is often seen in multiple sclerosis patients. However, the association between RLS and multiple sclerosis (MS) is still unclear and RLS is not considered as one of the MS symptoms. This study was conducted to assess the frequency of RLS and its associated factors in patients with MS.
View Article and Find Full Text PDFBackground: There is no documented demographical study on Iranian Parkinson's disease (PD) patients, so this study was conducted to identify demographic information about patients with PD in Iran, and to explore demographical differences between PD patients in Iran and other countries.
Methods: We reviewed medical records of 1656 patients diagnosed with PD, who referred from all parts of Iran to a referral Parkinson's disease clinic in Tehran. We collected data about their age, gender, age of onset, side of motor symptoms' onset, and drug history.