Retinal Function in Long-Term Type 1 Diabetes without Retinopathy: Insights from Pattern Electroretinogram and Pattern Visual Evoked Potentials Assessments.

BACKGROUND: To evaluate changes in pattern electroretinogram (pERG) and pattern visual evoked potentials (pVEP) in patients with long-lasting type 1 diabetes without diabetic retinopathy (DR). METHODS: Prospective study involving 92 eyes divided into two groups. The diabetic group included 46 eyes of 23 patients with type 1 diabetes (T1DM); the control group included 23 age-matched healthy subjects. pERG and pVEP were assessed using the RETI-port/scan21 recording software (version 1021.3.0.0). RESULTS: Mean age was 48 ± 9.77 years for the diabetic group and 51.7 ± 4.75 years for the control group. The mean duration of diabetes was 28.88 ± 8.04 years. The mean HbA1c value was 7.29 ± 0.89%. There were no differences in the age or sex distribution. Regarding the pERG, T1DM patients exhibited a significant decrease in the amplitude of the P50 and N95 waves compared to the control group (p = 0.018 and p = 0.035, respectively), with no differences in the peak time of each component. pVEP showed no significant changes in either peak time or amplitude of the different components. CONCLUSIONS: Long-term T1DM patients without DR showed changes in the amplitude of pERG waves with preserved peak times. We did not observe modifications in pVEP. pERG may serve as a subclinical marker of ganglion cell damage in long-term T1DM patients.

Electrophysiological findings in long-term type 1 diabetes patients without diabetic retinopathy using different ERG recording systems.

To assess full-field electroretinogram findings in long-term type 1 diabetes patients without diabetic retinopathy. Prospective study including 46 eyes of 23 patients with type 1 diabetes and 46 age-matched healthy eyes evaluated by the RETI-port/scan21 and the portable system RETeval following ISCEV guidelines. The average duration of diabetes was 28.88 ± 8.04 years. In scotopic conditions, using the RETI-port/scan21, diabetic patients showed an increase in b-wave implicit time (IT) (p = 0.017) with the lowest stimuli; a diminished b-wave amplitude (p = 0.005) in the mixed response, an increased IT (p = 0.004) with the high-intensity stimuli and an OP2 increased IT (p = 0.008) and decreased amplitude (p = 0.002). Under photopic conditions, b-wave amplitude was lower (p < 0.001) and 30-Hz flicker response was diminished (p = 0.021). Using the RETeval, in scotopic conditions, diabetic patients showed a reduction in the rod b-wave amplitude (p = 0.009), an increase in a-wave IT with the 280 Td.s stimulus (p = 0.005). OP2 had an increased IT and diminished amplitude (p = 0.003 and p = 0.002 respectively). 16 Td.s flicker showed an increased IT (p = 0.008) and diminished amplitude (p = 0.048). Despite variations in values between both systems, nearly all results displayed positive correlations. Long-term type 1 diabetes patients without diabetic retinopathy exhibit alterations in scotopic conditions, as evidenced by both conventional and portable electroretinogram devices. These findings suggest a modified retinal function, particularly in rod-driven pathways, even in the absence of vascular signs.

Diagnostic accuracy of the upper limb neurodynamic test 1 using neurodynamic sequencing in diagnosis of carpal tunnel syndrome.

BACKGROUND: The upper limb neurodynamic test 1 is used in the diagnosis of median nerve neuropathies such as carpal tunnel syndrome but its diagnostic validity remains limited. Neurodynamic sequencing has been suggested to increase the specificity of the neurodynamic tests, however, to date, information on the diagnostic accuracy of this variation in neurodynamic testing is required. OBJECTIVES: The aim of this study was to analyze the diagnostic validity of the local sequence of ULNT1 (LS-ULNT1) (i.e. a sequence that begins at the joint where the problem is (wrist) and progressively moves joints further away from it), in the diagnosis of CTS. A secondary aim was to describe the location of sensory responses to this modified neurodynamic test sequence. DESIGN: A prospective diagnostic accuracy study was designed. METHOD: Nerve conduction studies were used as the gold standard. The LS-ULNT1 was performed in 58 consecutive patients (17 men, 44 women) with suspected CTS. RESULTS: Sensitivity of the LS-ULNT1 was 65.7% (CI 48.0-80.9%) and the specificity was 95.7% (CI 78.1-99.9%). The positive and negative likelihood ratios were >5 and < 0.5, respectively, indicating the ability of the test to generate small but sometimes important changes in post-test probability. CONCLUSIONS: The overall results of this study showed that the LS-ULNT1 could be useful in confirming the diagnosis of CTS. The test demonstrated high specificity and the +LR indicated the ability of the test to generate changes in posttest probability, especially with a positive LS-ULNT1 result.

Is the Straight Leg Raise Suitable for the Diagnosis of Radiculopathy? Analysis of Diagnostic Accuracy in a Phase III Study.

The straight leg raise test (SLR) has been proposed to detect increased nerve mechanosensitivity of the lower limbs in individuals with low back pain. However, its validity in the diagnosis of lumbosacral radiculopathy shows very variable results. The aim of this study was to analyse the diagnostic validity of the SLR including well-defined diagnostic criteria (a change in symptoms with the structural differentiation manoeuvre and the reproduction of the patient's symptoms during the test or the asymmetries in the range of motion or symptoms location between limbs) in a sample of participants in phase III with suspicion of lumbar radiculopathy using the electrodiagnostic studies (EDX) as the reference standard. A phase III diagnostic accuracy study was designed. In total, 142 individuals with suspected lumbosacral radiculopathy referred for EDX participated in the study. Each participant was tested with EDX and SLR. SLR was considered positive using three diagnostic criteria. The sensitivity of the SLR for Criterion 3 was 89.02% (CI 81.65-96.40), the specificity was 25.00% (CI 13.21-36.79), and the positive and negative likelihood ratios were 1.19 (CI 1.01-1.40) and 0.44 (0.21-0.94), respectively. SLR showed limited validity in the diagnosis of lumbosacral radiculopathy. The incorporation of more objective diagnostic criteria (asymmetry in range of motion or localisation of symptoms) improved the diagnostic validity but the imprecision of the confidence intervals limited the interpretation of the results.

Electrophysiology and optical coherence tomography to evaluate Parkinson disease severity.

PURPOSE: To evaluate correlations between visual evoked potentials (VEP), pattern electroretinogram (PERG), and macular and retinal nerve fiber layer (RNFL) thickness measured by optical coherence tomography (OCT) and the severity of Parkinson disease (PD). METHODS: Forty-six PD patients and 33 age and sex-matched healthy controls were enrolled, and underwent VEP, PERG, and OCT measurements of macular and RNFL thicknesses, and evaluation of PD severity using the Hoehn and Yahr scale to measure PD symptom progression, the Schwab and England Activities of Daily Living Scale (SE-ADL) to evaluate patient quality of life (QOL), and disease duration. Logistical regression was performed to analyze which measures, if any, could predict PD symptom progression or effect on QOL. RESULTS: Visual functional parameters (best corrected visual acuity, mean deviation of visual field, PERG positive (P) component at 50 ms -P50- and negative (N) component at 95 ms -N95- component amplitude, and PERG P50 component latency) and structural parameters (OCT measurements of RNFL and retinal thickness) were decreased in PD patients compared with healthy controls. OCT measurements were significantly negatively correlated with the Hoehn and Yahr scale, and significantly positively correlated with the SE-ADL scale. Based on logistical regression analysis, fovea thickness provided by OCT equipment predicted PD severity, and QOL and amplitude of the PERG N95 component predicted a lower SE-ADL score. CONCLUSIONS: Patients with greater damage in the RNFL tend to have lower QOL and more severe PD symptoms. Foveal thicknesses and the PERG N95 component provide good biomarkers for predicting QOL and disease severity.

Electropysiologic evaluation of the visual pathway in patients with multiple sclerosis.

PURPOSE: To evaluate the ability of visual evoked potentials and pattern electroretinograms (PERG) to detect subclinical axonal damage in patients during the early diagnostic stage of multiple sclerosis (MS). The authors also compared the ability of optical coherence tomography (OCT), PERG, and visual evoked potentials to detect axonal loss in MS patients and correlated the functional and structural properties of the retinal nerve fiber layer. METHODS: Two hundred twenty-eight eyes of 114 subjects (57 MS patients and 57 age- and sex-matched healthy controls) were included. The visual pathway was evaluated based on functional and structural assessments. All patients underwent a complete ophthalmic examination that included assessment of visual acuity, ocular motility, intraocular pressure, visual field, papillary morphology, OCT, visual evoked potentials, and PERG. RESULTS: Visual evoked potentials (P100 latency and amplitude), PERG (N95 amplitude and N95/P50 ratio), and OCT parameters differed significantly between MS patients and healthy subjects. Moderate significant correlations were found between visual evoked potentials or PERG parameters and OCT measurements. CONCLUSIONS: Axonal damage in ganglion cells of the visual pathway can be detected based on structural measures provided by OCT in MS patients and by the N95 component and N95/P50 index of PERG, thus providing good correlation between function and structure.

Influence of cataract surgery on optical coherence tomography and neurophysiology measurements in patients with retinitis pigmentosa.

PURPOSE: To evaluate the effect of uncomplicated cataract phacoemulsification on the measurements of visual evoked potentials (VEP), pattern electroretinogram (PERG), and macular and retinal nerve fiber layer (RNFL) using 2 spectral-domain optical coherence tomography (OCT) instruments, the Cirrus OCT (Carl Zeiss Meditech) and Spectralis OCT (Heidelberg Engineering), in patients with retinitis pigmentosa (RP), and to assess the reliability of the OCT measurements before and after cataract surgery. DESIGN: Observational cross-sectional study. METHODS: Thirty-five eyes of 35 patients with RP (20 men and 15 women, 45-66 years) who underwent cataract phacoemulsification were studied. At 1 month before and 1 month after surgery, visual acuity, VEP, PERG, and 3 repetitions of scans using the RNFL and macular analysis protocols of the Cirrus and Spectralis OCT instruments were performed. The differences in measurements between the 2 visits were analyzed. Repeatability of OCT measurements was evaluated by calculating the coefficients of variation. RESULTS: VEP amplitude, RNFL thicknesses provided by Cirrus and Spectralis, and macular measurements provided by Cirrus OCT differed between the 2 visits. VEP latency, PERG measurements, and macular thicknesses provided by the Spectralis OCT before surgery did not differ significantly from those after surgery. The OCT repeatability was better after surgery, with lower coefficients of variation for scans performed after surgical removal of the cataract. The nuclear, cortical, and posterior subcapsular types of cataracts did not show different repeatability. CONCLUSIONS: The presence of cataracts affects VEP amplitude, RNFL, and macular measurements performed with OCT in eyes with RP. Image repeatability significantly improves after cataract phacoemulsification.

Neuro-ophthalmologic evaluation, quality of life, and functional disability in patients with MS.

OBJECTIVE: To evaluate correlations between longitudinal changes in neuro-ophthalmologic measures and quality of life (QOL) and disability in patients with multiple sclerosis (MS), using optical coherence tomography (OCT), visual evoked potentials (VEP), and visual field examination. METHODS: Fifty-four patients with relapsing-remitting MS were enrolled in this study and underwent Multiple Sclerosis Quality of Life questionnaire (54 items) (MSQOL-54) and Expanded Disability Status Scale (EDSS) evaluation, as well as complete neuro-ophthalmologic examination including visual field testing and retinal nerve fiber layer (RNFL) measurements using Cirrus and Spectralis OCT and VEP. All patients were re-evaluated at 12, 24, and 36 months. Logistical regression was performed to analyze which measures, if any, could predict QOL. RESULTS: Overall, RNFL thickness results at the baseline evaluation were significantly different from those at 3 years (p ≤ 0.05), but there were no differences in functional measures (visual acuity, contrast sensitivity, color vision, visual field, and VEP). A reduced MSQOL-54 score was associated with an increase in EDSS score and a decrease in both functional and structural parameters. Patients with longer MS duration presented with a lower MSQOL-54 score (reduction in QOL). CONCLUSIONS: Patients with progressive axonal loss as seen in RNFL results had a lower QOL and more functional disability.

Progressive degeneration of the retinal nerve fiber layer in patients with multiple sclerosis.

PURPOSE: To quantify changes in the retinal nerve fiber layer (RNFL) of patients with multiple sclerosis (MS) over 3 years and to evaluate whether treatment protects against RNFL degeneration. METHODS: Ninety-four MS patients and 50 healthy subjects were followed-up over 3 years. All subjects underwent a complete ophthalmic examination, which included assessment of visual acuity (Snellen chart), color vision (Ishihara pseudoisochromatic plates), visual field examination, optical coherence tomography (OCT), and visual evoked potentials (VEPs). All patients were reevaluated at 12, 24, and 36 months to quantify changes in the RNFL. RESULTS: Changes were detected in RNFL thickness at the 36-month follow-up. Significant decreases (P < 0.05, t-test) were observed in the mean, superior, inferior, and temporal RNFL thicknesses, and macular volume provided by OCT, and in the P100 latency of VEP of the MS group, but only in the mean and inferior RNFL thicknesses of the healthy control group. Greater changes in the superior and inferior RNFL thicknesses during follow-up were detected in the MS group. Differences between treatments were not detected, but untreated patients had higher degeneration in the mean and superior RNFL thicknesses during the follow-up (P = 0.040 and P = 0.19, respectively). CONCLUSIONS: Progressive axonal loss can be detected in the optic nerve fiber layer of MS patients. Analysis of the RNFL by OCT can be useful for evaluating MS progression and efficacy of treatment as a neuroprotective factor against axonal degeneration.

Optical coherence tomography in retinitis pigmentosa: reproducibility and capacity to detect macular and retinal nerve fiber layer thickness alterations.

PURPOSE: To evaluate the ability of time-domain and Fourier-domain optical coherence tomographies (OCTs) to detect macular and retinal nerve fiber layer atrophies in retinitis pigmentosa (RP). To test the intrasession reproducibility using three OCT instruments (Stratus, Cirrus, and Spectralis). METHODS: Eighty eyes of 80 subjects (40 RP patients and 40 healthy subjects) underwent a visual field examination, together with 3 macular scans and 3 optic disk evaluations by the same experienced examiner using 3 OCT instruments. Differences between healthy and RP eyes were compared. The relationship between measurements with each OCT instrument was evaluated. Repeatability was studied by intraclass correlation coefficients and coefficients of variation. RESULTS: Macular and retinal nerve fiber layer atrophies were detected in RP patients for all OCT parameters. Macular and retinal nerve fiber layer thicknesses, as determined by the different OCTs, were correlated but significantly different (P < 0.05). Reproducibility was moderately high using Stratus, good using Cirrus and Spectralis, and excellent using the Tru-track technology of Spectralis. In RP eyes, measurements showed higher variability compared with healthy eyes. CONCLUSION: Differences in thickness measurements existed between OCT instruments, despite there being a high degree of correlation. Fourier-domain OCT can be considered a valid and repeatability technique to detect retinal nerve fiber layer atrophy in RP patients.

[Expressive language disorder and focal paroxysmal activity]. / Trastorno del lenguaje expresivo y actividad paroxistica focal.

INTRODUCTION: In cases of expressive language disorder (ELD), the child is unable to put his or her thoughts into words. Comorbidity is present with difficulties in repeating, imitating or naming. There are no problems with pronunciation, as occurs in phonological disorder, it may present before the age of three years and is crucial between four and seven years of age. Electroencephalogram (EEG) studies have been carried out not only in ELD, but also in clinical pictures where the language disorder was the main symptom or was associated to another neurodevelopmental pathology. PATIENTS AND METHODS: We conducted a retrospective study involving a review of 100 patient records, with patients (25 girls and 75 boys) aged between two and six years old who had been diagnosed with ELD (according to the Diagnostic and Statistical Manual of Mental Disorders, fourth edition, text revised) and were free of seizures and not receiving treatment. They were submitted to an EEG and received treatment with valproic acid if EEG findings were positive. RESULTS: Only six patients (males) presented localised spike-wave paroxysmal EEG activity in the frontotemporal region. This 6% is a percentage that is higher than the one found in the normal children's population (2%), but lower than the value indicated in the literature for language disorders, which ranges between 20% and 50%. These patients responded positively to the treatment and both expressive language and EEG findings improved. CONCLUSIONS: It is possible that in ELD without paroxysms there may be a dysfunction in the circuit made up of the motor cortex-neostriatum prior to grammatical learning, whereas if there are paroxysms then this would point to neuronal hyperactivity, perhaps associated to this dysfunction or not, in cortical areas. In our cases valproic acid, together with speech therapy, helped the children to recover their language abilities.

Trastorno del lenguaje expresivo y actividad paroxística focal / Expressive language disorder and focal paroxysmal activity

Introducción. En el trastorno del lenguaje expresivo (TLE), el niño no puede plasmar su pensamiento en palabras. Se presenta comorbilidad con dificultades en la repetición, imitación o nominación. No se dan problemas con la pronunciación, como ocurre en el trastorno fonológico, puede presentarse antes de los 3 años y es clave entre 4 y 7 años. El electroencefalograma (EEG) se ha venido practicando no sólo en el TLE, sino también en cuadros donde el trastorno del lenguaje era el síntoma principal o asociado a otra patología del neurodesarrollo. Pacientes y métodos. Estudio retrospectivo con revisión de 100 historias clínicas, con pacientes (25 niñas y 75 niños) en edades entre 2 y 6 años, a los cuales se les diagnosticó TLE (según el Manual diagnóstico y estadístico de los trastornos mentales, cuarta edición, texto revisado), libres de crisis convulsivas y sin tratamiento. Se les realizó EEG y recibieron tratamiento con ácido valproico, si aquél era positivo. Resultados. Sólo 6 pacientes (varones) presentaron en el EEG actividad paroxística de punta-onda localizada en la región frontotemporal. Este 6% es un porcentaje superior al encontrado en la población infantil normal (2%), pero inferior al señalado en la bibliografía para trastornos del lenguaje, que oscila entre el 20 y el 50%. Éstos mejoraron el lenguaje expresivo y el EEG en respuesta positiva al tratamiento. Conclusiones. Posiblemente en el TLE sin paroxismos puede existir una disfunción del circuito córtex motor-neoestriado previo al aprendizaje gramatical, mientras que con paroxismos señala una hiperactividad neuronal, asociada o no a dicha disfunción, de zonas corticales. En nuestros casos el ácido valproico, junto con la intervención logopédica, contribuyó a la recuperación del lenguaje (AU)

Introduction. In cases of expressive language disorder (ELD), the child is unable to put his or her thoughts into words. Comorbidity is present with difficulties in repeating, imitating or naming. There are no problems with pronunciation, as occurs in phonological disorder, it may present before the age of three years and is crucial between four and seven years of age. Electroencephalogram (EEG) studies have been carried out not only in ELD, but also in clinical pictures where thelanguage disorder was the main symptom or was associated to another neurodevelopmental pathology. Patients and methods. We conducted a retrospective study involving a review of 100 patient records, with patients (25 girls and 75 boys) aged between two and six years old who had been diagnosed with ELD (according to the Diagnostic and Statistical Manual of Mental Disorders, fourth edition, text revised) and were free of seizures and not receiving treatment. They were submitted to an EEG and received treatment with valproic acid if EEG findings were positive. Results. Only six patients (males) presented localised spike-wave paroxysmal EEG activity in the frontotemporal region. This 6% is a percentage that is higher than the one found in the normal children’s population (2%), but lower than the value indicated in the literature for language disorders, which ranges between 20% and 50%. These patients responded positively to the treatment and both expressive language and EEG findings improved. Conclusions. It is possible that in ELD without paroxysms there may be a dysfunction in the circuit made up of the motor cortex-neostriatum prior to grammatical learning, whereas if there are paroxysms then this would point to neuronal hyperactivity, perhaps associated to this dysfunction or not, in cortical areas. In our cases valproic acid, together with speech therapy, helped the children to recover their language abilities (AU)