Analysis of various factors affecting pupil size in patients with glaucoma.

BACKGROUND: Pupil size is an important factor in predicting post-operative satisfaction. We assessed the correlation between pupil size, measured by Humphrey static perimetry, and various affecting factors in patients with glaucoma. METHODS: In total, 825 eyes of 415 patients were evaluated retrospectively. Pupil size was measured with Humphrey static perimetry. Comparisons of pupil size according to the presence of glaucoma were evaluated, as were correlations between pupil size and various factors, including age, logMAR best corrected visual acuity (BCVA), retinal nerve fiber layer (RNFL) thickness, spherical equivalent, intraocular pressure, axial length, central corneal thickness, white-to-white, and the kappa angle. RESULTS: Pupil size was significantly smaller in glaucoma patients than in glaucoma suspects (p < 0.001) or the normal group (p < 0.001). Pupil size decreased significantly as age (p < 0.001) and central cornea thickness (p = 0.007) increased, and increased significantly as logMAR BCVA (p = 0.02) became worse and spherical equivalent (p = 0.007) and RNFL thickness (p = 0.042) increased. In patients older than 50 years, pupil size was significantly larger in eyes with a history of cataract surgery. CONCLUSIONS: Humphrey static perimetry can be useful in measuring pupil size. Pupil size was significantly smaller in eyes with glaucoma. Other factors affecting pupil size can be used in a preoperative evaluation when considering cataract surgery or laser refractive surgery.

Factors associated with the effectiveness of plasma exchange for the treatment of NMO-IgG-positive neuromyelitis optica spectrum disorders.

To identify factors associated with plasma exchange response in neuromyelitis optica (NMO) spectrum disorders, the clinical and magnetic resonance imaging (MRI) features of 31 NMO-IgG-positive patients receiving plasma exchange for steroid-resistant exacerbations were analyzed. Functional improvement was observed in 65% of the patients. A lower baseline Expanded Disability Status Scale score was associated with favorable response (p = 0.040). Patients without cord atrophy had a higher success rate than patients with atrophy (p = 0.016). Levels of NMO-IgG did not differ between responders and non-responders before and after plasma exchange. In conclusion, a minimal pre-existing disability is the primary determinant of the effectiveness of plasma exchange.

Photobiomodulation improves the synapses and cognitive function and ameliorates epileptic seizure by inhibiting downregulation of Nlgn3.

BACKGROUND: Temporal lobe epilepsy (TLE) remains one of the most drug-resistant focal epilepsies. Glutamate excitotoxicity and neuroinflammation which leads to loss of synaptic proteins and neuronal death appear to represent a pathogen that characterizes the neurobiology of TLE. Photobiomodulation (PBM) is a rapidly growing therapy for the attenuation of neuronal degeneration harboring non-invasiveness benefits. However, the detailed effects of PBM on excitotoxicity or neuroinflammation remain unclear. We investigated whether tPBM exerts neuroprotective effects on hippocampal neurons in epilepsy mouse model by regulating synapse and synapse-related genes. METHODS: In an in vitro study, we performed imaging analysis and western blot in primary hippocampal neurons from embryonic (E17) rat pups. In an in vivo study, RNA sequencing was performed to identify the gene regulatory by PBM. Histological stain and immunohistochemistry analyses were used to assess synaptic connections, neuroinflammation and neuronal survival. Behavioral tests were used to evaluate the effects of PBM on cognitive functions. RESULTS: PBM was upregulated synaptic connections in an in vitro. In addition, it was confirmed that transcranial PBM reduced synaptic degeneration, neuronal apoptosis, and neuroinflammation in an in vivo. These effects of PBM were supported by RNA sequencing results showing the relation of PBM with gene regulatory networks of neuronal functions. Specifically, Nlgn3 showed increase after PBM and silencing the Nlgn3 reversed the positive effect of PBM in in vitro. Lastly, behavioral alterations including hypoactivity, anxiety and impaired memory were recovered along with the reduction of seizure score in PBM-treated mice. CONCLUSIONS: Our findings demonstrate that PBM attenuates epileptic excitotoxicity, neurodegeneration and cognitive decline induced by TLE through inhibition of the Nlgn3 gene decrease induced by excitotoxicity.

Decreased retinal thickness in patients with Alzheimer's disease is correlated with disease severity.

BACKGROUND AND PURPOSE: The loss of retinal ganglion cells observed in Alzheimer's disease (AD) may be attributable to a neurodegeneration of the neuro-retinal structure. Amnestic mild cognitive impairment (aMCI) has been considered a prodromal stage of AD. We evaluated retinal thicknesses in patients with aMCI and AD compared to healthy controls using spectral-domain optical coherence tomography (OCT) to investigate whether changes in retinal thickness are correlated with the clinical severity of dementia. METHODS: Patients with aMCI (n = 14), mild to moderate AD (n = 7), severe AD (n = 9), and age-matched controls (n = 17) underwent neuro-ophthalmologic examinations. Global deterioration scale (GDS), clinical dementia rating (CDR), and mini-mental status examination (MMSE) were used to evaluate the clinical overall severity of dementia. The thicknesses of the peripapillary retinal nerve fiber layer (RNFL), total macula, and macular ganglion cell-inner plexiform layer (GC-IPL) were measured using Cirrus HD-OCT. RESULTS: The severe AD group had overall significantly thinner GC-IPL, total macula, and peripapillary RNFL compared to the controls (p<0.05). In the mild to moderate AD group, the total macula, average RNFL, and superior RNFL thickness were each significantly reduced compared to controls (p<0.05). The aMCI group had reduced total macula, average RNFL, and inferior RNFL thickness, but there were no significant differences compared to the controls. The GDS and CDR scores had a negative correlation with the thickness of the GC-IPL and the total macula. The MMSE scores had a positive correlation with both the total macular and average RNFL thickness, when adjusted for age (p<0.05). CONCLUSIONS: This study confirmed that retinal thickness is decreased in AD patients. There is a correlation between reduced retinal thickness and the clinical severity of dementia.

Abnormal Oculomotor Functions in Amyotrophic Lateral Sclerosis.

BACKGROUND AND PURPOSE: Although traditionally regarded as spared, a range of oculomotor dysfunction has been recognized in amyotrophic lateral sclerosis (ALS) patients. ALS is nowadays considered as a neurodegenerative disorder of a third compartment comprising widespread areas of extra-motor brain including cerebellum. Our objective was to perform an observational study to examine for ocular motor dysfunction in patients with ALS and for any differences between bulbar-onset and spinal-onset patients. METHODS: Thirty two ALS patients (bulbar onset: 10, spinal onset: 22) underwent the standardized systemic evaluations using video-oculography. RESULTS: Oculomotor dysfunctions such as square wave jerks, saccadic dysmetria, abnormal cogwheeling smooth pursuits and head shaking and positional nystagmus of central origin have been observed in the ALS patients at a relatively early stage. Abnormal smooth pursuits and saccadic dysmetria were increased in the bulbar-onset compared to the spinal-onset (p<0.05). CONCLUSIONS: These oculomotor abnormalities may be a marker of neuro-degeneration beyond motor neurons in ALS, especially in bulbar-onset disease. Future longitudinal studies of eye movement abnormalities have provided insights into the distribution and nature of the disease process.

Opsoclonus-myoclonus syndrome associated with mumps virus infection.

BACKGROUND: Opsoclonus-myoclonus syndrome (OMS) is a rare neurological disorder that is characterized by involuntary eye movements and myoclonus. OMS exhibits various etiologies, including paraneoplastic, parainfectious, toxic-metabolic, and idiopathic causes. The exact immunopathogenesis and pathophysiology of OMS are uncertain. CASE REPORT: We report the case of a 19-year-old male who developed opsoclonus and myoclonus several days after a flu-like illness. Serological tests revealed acute mumps infection. The findings of cerebrospinal fluid examinations and brain magnetic resonance imaging were normal. During the early phase of the illness, he suffered from opsoclonus and myoclonus that was so severe as to cause acute renal failure due to rhabdomyolysis. After therapies including intravenous immunoglobulin, the patient gradually improved and had fully recovered 2 months later. CONCLUSIONS: This is the first report of OMS associated with mumps infection in Korea. Mumps infection should be considered in patients with OMS.

Small fiber function in drug naïve patients with idiopathic restless legs syndrome.

To assess whether idiopathic restless legs syndrome (RLS) is associated with small fiber abnormalities, we evaluated somatic and autonomic small fiber function in 56 patients with idiopathic RLS and 36 age-matched and sex-matched healthy controls using quantitative sensory testing and quantitative sudomotor axon reflex tests. The warm detection thresholds (WDT) of the hands (p=0.017) and feet (p=0.008) were higher in patients with RLS than in controls. Cooling detection thresholds (CDT) were higher in the feet of patients with RLS than controls (p<0.001), but CDT in the hands did not differ between groups (p=0.161). There were no significant between-group differences in total sweat volume and sweat production in the forearms, proximal legs, distal legs, and feet (p>0.1 each). These findings suggest that abnormal sensory perception in patients with idiopathic RLS may result from impairment of central somatosensory processing rather than small fiber neuropathy.

Fulminant guillain-barré syndrome mimicking cerebral death following acute viral hepatitis a.

A 32-year-old man was transferred to an intensive care unit due to respiratory difficulties with a 4-day history of progressive areflexic quadriparesis following acute hepatitis A. A nerve-conduction study revealed inexcitability of most nerves. The cerebrospinal fluid showed albuminocytologic dissociation, suggesting Guillain-Barré syndrome (GBS). The patient appeared brain dead on day 4, showing absent brainstem reflexes, respiratory failure, and fully dilated and fixed pupils. This case is an example of how GBS can evolve and simulate a brain-dead state from fulminant deafferentation following acute hepatitis A.