- Predictors of retinal atrophy in multiple sclerosis: A longitudinal study using spectral domain optical coherence tomography with segmentation analysis. [Journal Article]
- MSMult Scler Relat Disord 2018 Feb 11; 21:56-62
- CONCLUSIONS: MS is associated with longitudinal thinning affecting AMT inner retinal layers (MRNFL, GCIPL, PRNFL). Early disease duration, female gender, MRI activity, and prior optic neuritis were predictive of faster rate of neuro-axonal loss. This may have implications in the design of future therapeutic trials.
- Incidence of Autoimmune and Related Disorders After Resolution of Endogenous Cushing Syndrome in Children. [Journal Article]
- HMHorm Metab Res 2018 Feb 19
- Glucocorticoids are widely used for immunosuppression in autoimmune diseases. After the resolution of hypercortisolemia, the immune system recovers allowing for autoimmune diseases to manifest. Here ...
Glucocorticoids are widely used for immunosuppression in autoimmune diseases. After the resolution of hypercortisolemia, the immune system recovers allowing for autoimmune diseases to manifest. Here we investigated the presence of autoimmune and related diseases that developed after cure of endogenous Cushing syndrome (CS) in children. We identified 129 children who were diagnosed and successfully treated for endogenous CS at the National Institutes of Health from 1997 until 2017, and who were followed for at least 6 months after treatment. We performed a retrospective chart review analysis to identify the presence of autoimmune or related diseases after cure. Ten children were diagnosed with a new autoimmune or related disorder after resolution of hypercortisolemia. This results in a frequency of 7.8% of our pediatric CS population. The identified patients had a shorter duration of hypercortisolemia prior to diagnosis, but did not otherwise differ from the remaining patients. The various identified diseases were: celiac disease (n=1), psoriasis (n=1), Hashimoto thyroiditis (n=1), Graves disease (n=1), optic neuritis (n=2), skin hypopigmented lesions/vitiligo (n=2), allergic rhinitis/asthma (n=1), and neuropathy responding to glucocorticoid treatment (n=1). The reported time between the treatment of CS and diagnosis of autoimmune disorder ranged from 6 to 19 months. The presence of autoimmune or related diseases might be masked by the hypercortisolemic state in endogenous CS. After resolution of hypercortisolemia, the presentation of new autoimmune diseases or recurrence of previously known autoimmune conditions should be considered when concerning symptoms arise.
- Clinical correlation of biopsy results in patients with temporal arteritis. [Journal Article]
- RARev Assoc Med Bras (1992) 2017; 63(11):953-956
- CONCLUSIONS: We were not able to find a correlation between the analysis of biopsy results and clinical evaluation of patients with temporal arteritis. We suggest that diagnosis of temporal arteritis depends on clinical suspicion. Laboratory examination results may not be helpful in accurate diagnosis of tempoal arteritis.
- Diagnosis and treatment of anti-myelin oligodendrocyte glycoprotein antibody positive optic neuritis. [Review]
- JJJpn J Ophthalmol 2018 Feb 14
- Anti-myelin-oligodendrocyte glycoprotein (MOG) antibody positive optic neuritis has been established as a new subset of optic neuropathy. Anti-MOG antibodies are usually measured by cell-based assay....
Anti-myelin-oligodendrocyte glycoprotein (MOG) antibody positive optic neuritis has been established as a new subset of optic neuropathy. Anti-MOG antibodies are usually measured by cell-based assay. Patients with anti-MOG antibody positive optic neuritis respond well to steroid therapy, and, while visual acuity outcomes are favorable, significant visual field defects remain. Furthermore, patients who are anti-MOG antibody positive have higher rates of recurrence compared to antibody negative patients. Based on these findings, anti-MOG antibody positive patients with optic neuritis have the characteristics of good visual outcomes, residual visual field defects, and high risk of recurrence. Tests for anti-MOG antibody are useful for the diagnosis and treatment of optic neuritis.
- Early Nodal and Paranodal Disruption in Autoimmune Optic Neuritis. [Journal Article]
- JNJ Neuropathol Exp Neurol 2018 Feb 10
- Disturbances in the nodes of Ranvier are an early phenomenon in many CNS disorders, including the autoimmune demyelinating disease multiple sclerosis (MS). Using an animal model of optic neuritis, a ...
Disturbances in the nodes of Ranvier are an early phenomenon in many CNS disorders, including the autoimmune demyelinating disease multiple sclerosis (MS). Using an animal model of optic neuritis, a common early symptom of MS, we have investigated nodal and paranodal compartments in the optic nerve during disease progression. Both nodes and paranodes, as identified by immunohistochemistry against sodium channels (Nav) and Caspr, respectively, were observed to increase in length during the late induction phase of the disease, prior to onset of the demyelination and immune cell infiltration characteristic of optic neuritis. These changes were correlated with both axonal stress and microglial/macrophage activation, and were most apparent in the vicinity of the retrobulbar optic nerve head, the unmyelinated region of the optic nerve where retinal ganglion cell axons exit the retina. Using intravitreal glutamate injection as a model of a primary retinal insult, we demonstrate that this can induce similar nodal and paranodal changes. This may suggest that onset of neurodegeneration in the absence of demyelination, as reported in several studies into the nonaffected eyes of MS patients, may give rise to subtle disturbances in the axo-glial junction.
- Paediatric ADEM followed by optic neuritis: disease course, treatment response and outcome. [Journal Article]
- EJEur J Neurol 2018 Feb 14
- CONCLUSIONS: ADEM-ON is a MOG-Ab associated relapsing disorder and can have a heterogeneous disease course. Patients were refractory for maintenance immunosuppression and appeared to be corticosteroid-dependent. Further international collaborations are now required to unify guidelines in this difficult to manage group of patients. This article is protected by copyright. All rights reserved.
- Guillain-Barré syndrome and optic neuritis after Mycoplasma pneumoniae infection. [Journal Article]
- BDBrain Dev 2018 Feb 09
- We report the case of a 12-year-old girl who developed Guillain-Barré syndrome (GBS) and optic neuritis (ON) following Mycoplasma pneumoniae infection. Her symptoms, including bilateral vision impair...
We report the case of a 12-year-old girl who developed Guillain-Barré syndrome (GBS) and optic neuritis (ON) following Mycoplasma pneumoniae infection. Her symptoms, including bilateral vision impairment and tingling in her hands and right foot, were resolved after methylprednisolone pulse therapy. Serum anti-galactocerebroside (Gal-C) IgM antibodies were detected in our patient. This is the first report of a child with GBS and ON associated with M. pneumoniae infection.
- [Clinical observation on the evolution process of macular ganglion cell complex and peripapillary retinal nerve fiber layer of neuritis patients]. [Journal Article]
- ZYZhonghua Yan Ke Za Zhi 2018 Jan 11; 54(1):62-68
- Objective: To discuss the dynamic changes and correlation of macular ganglion cell (mGCC) and peripapillary retinal nerve fiber layer (pRNFL) of neuritis patients through optical ...
Objective: To discuss the dynamic changes and correlation of macular ganglion cell (mGCC) and peripapillary retinal nerve fiber layer (pRNFL) of neuritis patients through optical coherence tomography (OCT).Methods:A retrospective case series study. Eleven eyes from 11 patients who have been diagnosed with neuritis and received regular follow-up during June 2013 through June 2015 were included. The dynamic characteristic changes of mGCC and pRNFL over the course of disease, as well as their correlations between best visual acuity and visual field have been analyzed based on OCT measurements.Results:According to the observation on the 11 eyes of the 11 patients, patients showed symptoms of decreased vision, abnormal visual field, swollen mGCC and pRNFL with normal or increased thickness during the subnormal period. During the advance-separation period (around 3 weeks), the thickness of mGCC decreased resulting from atrophy while pRNFL was still swollen. Druing the late period (usually 6-8 weeks after onset of the disease), both mGCC and pRNFL were getting thinner due to atrophy. Vision acuity and visual field of the patients improved after treatment, however, they were still not comparable with the normal level before the disease.Conclusions:The thickness changes of mGCC and pRNFL differs in neuritis patients over time. OCT can help us in detecting these changes, thus provide a foundation for us to further explore the treatment and anatomical changes of neuritis patients.(Chin J Ophthalmol, 2018, 54: 62-68).
- [Clinical features of chronic relapsing inflammatory optic neuropathy]. [Journal Article]
- ZYZhonghua Yi Xue Za Zhi 2018 Feb 06; 98(6):450-453
- Objective: To explore the clinical characteristics and prognosis of chronic relapsing inflammatory optic neuropathy (CRION).Methods:A retrospective analysis was conducted. Clinica...
Objective: To explore the clinical characteristics and prognosis of chronic relapsing inflammatory optic neuropathy (CRION).Methods:A retrospective analysis was conducted. Clinical features, disease course, prognosis and magnetic resonance imaging (MRI) of patients with CRION who were admitted to Department of Neurology between 2014 and 2016 were reviewed and analyzed.Results:Totally, there were 27 patients (10 males and 17 females), with an age range of 17-59 years. The disease duration was between 40 days and 8 years. There were 2 to 9 CRION episodes. The mean frequency of CRION episodes was 3.30±1.56. The outcome of visual acuity showed that the more episodes frequency was, the worse outcome became. There was 25 abnormal optic nerve signals in MRI. And in 22 cases, the abnormal signals were in intraorbital segment or inner pipe section. The antinuclear antibodies (ANA) titers were elevated in 5 patients. Five cases relapsed during reduction or withdrawal of steroids, but steroids was not added in time. The final outcome of these 5 patients was poor.Conclusions:CRION was more common in female than male patients. Most patients were companied by pain, and the lesions were more common in the intraorbital segment of optic nerve. The more episode frequency was, the worse prognosis became. If a relapse happened, steroids or other immunosuppressive agents should be used.
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- MRI of the first event in pediatric acquired demyelinating syndromes with antibodies to myelin oligodendrocyte glycoprotein. [Journal Article]
- JNJ Neurol 2018 Feb 08
- Antibodies against the myelin oligodendrocyte glycoprotein (MOG-Ab) can be detected in various pediatric acquired demyelinating syndromes (ADS). Here, we analyze the spectrum of neuroradiologic findi...
Antibodies against the myelin oligodendrocyte glycoprotein (MOG-Ab) can be detected in various pediatric acquired demyelinating syndromes (ADS). Here, we analyze the spectrum of neuroradiologic findings in children with MOG-Ab and a first demyelinating event. The cerebral and spinal MRI of 69 children with different ADS was assessed in regard to the distribution and characteristics of lesions. Children with acute disseminated encephalomyelitis (n = 36) or neuromyelitis optica spectrum disorder (n = 5) presented an imaging pattern characterized predominantly by poorly demarcated lesions with a wide supra- and infratentorial distribution. Younger children also tended to have poorly defined and widespread lesions. The majority of patients with an isolated optic neuritis (n = 16) only presented small non-specific brain lesions or none at all. A longitudinally extensive transverse myelitis mainly affecting the cervical, and less often so the thoracic, lumbar, and conus regions, was detected in 31 children. The three children of our cohort who were then finally diagnosed with multiple sclerosis had at onset already demarcated white matter lesions as well as transverse myelitis. In conclusion, children with MOG seropositive ADS present disparate, yet characteristic imaging patterns. These patterns have been seen to correlate to the disease entity as well as to age of symptom onset.