The effectiveness of treatment of partial optic nerve atrophy in children

2021 ◽  
pp. 128-131
Author(s):  
V.A. Goncharenko ◽  
◽  
E.V. Gromakina ◽  
G.G. Basova ◽  
◽  
...  
Keyword(s):  
Visual Acuity ◽  
Optic Nerve ◽  
Key Words ◽  
Early Treatment ◽  
Complex Treatment ◽  
Case Histories ◽  
Optic Nerve Atrophy ◽  
Initiation Of Therapy ◽  
Partial Atrophy

Purpose. To evaluate the effectiveness of early treatment in patients with partial optic nerve atrophy, nystagmus and albinism. Material and methods. A study of case histories and outpatient records of 265 patients with partial optic nerve atrophy was carried out. Among them, 219 children received a single course of treatment and 46 children received two or more courses of treatment at different periods of initiation of therapy. Results. After a single course of treatment, 54% of children had no dynamics of visual acuity. After regular complex treatment, visual acuity increased by 0.2 and higher in 58.3% of children at the beginning of treatment under 3 years of age. In cases of isolated lesions of the optic nerve and with a combination of partial atrophy of the optic nerve with nystagmus, an increase in visual acuity by 0.2 and higher was noted in 50% and 60%, respectively, at the start of treatment for children under 3 years of age. Conclusion. Treatment of partial atrophy of the optic nerve is effective in children with regular courses of therapy. An increase in visual acuity by 0.2 and higher was observed twice as often at the start of complex treatment up to 3 years. Key words: optic nerve atrophy, children, visual acuity

scholarly journals CMT2A Harboring Mitofusin 2 Mutation with Optic Nerve Atrophy and Normal Visual Acuity

2020 ◽  
Vol Volume 13 ◽  
pp. 41-45
Author(s):  
Silvana Guerriero ◽  
Francesco D'Oria ◽  
Giacomo Rossetti ◽  
Rosa Anna Favale ◽  
Stefano Zoccolella ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Optic Nerve ◽  
Optic Nerve Atrophy ◽  
Mitofusin 2 ◽  
Normal Visual Acuity

scholarly journals Bilateral Syphilitic Optic Neuropathy with Secondary Autoimmune Optic Neuropathy and Poor Visual Outcome

2019 ◽  
Vol 10 (1) ◽  
pp. 81-88 ◽  
Author(s):  
Tanya Kowalski ◽  
Dujon  Fuzzard ◽  
Isla Williams ◽  
Jonathan Darby ◽  
Heather Gwen Mack
Keyword(s):  
Visual Acuity ◽  
Optic Nerve ◽  
Optic Neuropathy ◽  
Nerve Palsy ◽  
Visual Loss ◽  
Visual Outcome ◽  
High Dose ◽  
Optic Nerve Atrophy ◽  
Poor Visual Outcome ◽  
Appropriate Treatment

We describe the case of a 65-year-old man who suffered progressive visual loss despite appropriate treatment of ocular syphilis. Our patient initially presented with a unilateral 6th nerve palsy and associated double vision, which self-resolved over 6 months. His ophthalmic examination was otherwise normal. 12 months after the initial complaint, he represented with dyschromatopsia, reduced visual acuity, tonic pupils, and optic nerve atrophy. He tested positive for syphilis and was admitted for treatment of neurosyphilis with high-dose benzylpenicillin. Despite treatment, at a 4-month review his visual acuity remained poor and progression of optic nerve atrophy was noted alongside the development of bilateral central scotomas. Further testing was congruent with a diagnosis of autoimmune optic retinopathy. We propose this to be secondary to his syphilitic infection. Syphilis is known as the “great mimicker,” and despite being quite treatable, this case highlights ongoing complexity in the diagnosis and management of syphilis, unfortunately with a poor visual outcome.

scholarly journals Diseases of the eye in chronic alcoholism

2013 ◽  
Vol 94 (1) ◽  
pp. 101-105
Author(s):  
R R Iskhakova ◽  
F R Saifullina
Keyword(s):  
Visual Acuity ◽  
Optic Nerve ◽  
Peripheral Vision ◽  
Clinical Manifestations ◽  
Chronic Alcoholism ◽  
Low Grade ◽  
Optic Nerve Atrophy ◽  
Eye Disorders ◽  
Temporal Parts ◽  
Late Stages

Chronic alcoholism is a disease affecting all the vital organs, including development of functional and organic eye disorders in 2-70% of cases. Alcoholic (ethanol) amblyopia with such features as slow gradient visual acuity decrease in both eyes (although visual acuity can decrease down do a very low grade, the complete blindness is rare) is among the disorders in patients with chronic alcoholism. Fundus of the eye at the beginning of the disease is normal in most of the cases, sometimes an optic nerve congestion and mild features of optic neuritis can be observed. Sometimes optic nerve hyperemia or anemia can be observed. Simple optic nerve atrophy seen as the temporal parts or the entire disc blanching can be seen at the late stages. Alcohol toxicity can also result as peripheral vision decrease, with degree of it increasing depending on the clinical manifestations of the alcoholism. Generally, eye disorders in patients with chronic alcoholism in most of the cases manifest as central retinal area damage and combination of retinal and optic nerve involvement.

scholarly journals Modern Technologies in the Examination of Patients with Optic Nerve Atrophy Due to Multiple Sclerosis

2018 ◽  
Vol 15 (2S) ◽  
pp. 246-253 ◽  
Author(s):  
E. E. Ioyleva ◽  
M. S. Krivosheeva ◽  
E. Yu. Markova
Keyword(s):  
Visual Acuity ◽  
Optic Nerve ◽  
Visual Field ◽  
Visual Field Defects ◽  
Functional Disorders ◽  
Optic Nerve Atrophy ◽  
Central Visual Field ◽  
Mean Sensitivity ◽  
Field Defects ◽  
Central Fixation

Purpose: to develop an algorithm for testing patients with optic nerve atrophy due to MS using microperimetry with the different functional disorders.Patients and Methods. There were 20 patients (40 eyes) with the correct diagnosis of MS at the age of 33 ± 1.88 years in the study. The BCVA was from 0.1 to 1.0. Microperimetry was done on MP-1 (Nidek technologies, Vigonza, Italy) with the definition of mean sensitivity, stability and type of fixation. We chose the parameters of microperimetry: the research program, the size of the stimulus, the fixation mark and the test mode.Results. The best program for diagnosing central visual field defects was the program macula 12° 10 dB, for paracentral visual field defects – retina 40° 20 dB. The testing was conducted by using a standard stimulus Goldmann III, mark and survey mode is selected individually, according at the various types of functional disorders. The visual acuity was from 0.1 to 0.4 in 11 eyes with optic nerve atrophy. There were revealed absolute or relative defects of the central field of vision and the decreasing of mean sensitivity to 8.21 ± 2.3 dB, unstable central fixation — 71.18 ± 10, 3%. In 29 eyes with optic nerve atrophy and visual acuity 0.5–1.0 have been identified relative small central visual field defects and paracentral visual field defects in the inferior side with a decrease in sensitivity to 15,32 ± 0,84 dB in the area of 6° from the fixing point, stable central fixation 88,96 ± 2,9%.Conclusion. The authors developed an algorithm for testing patients with optic nerve atrophy due to MS. Using the algorithm identified the central and paracentral visual field defects with the decrease in the mean sensitivity. 

scholarly journals Аналіз доступності до кваліфікованої медичної допомоги хворим з атрофіями зорових нервів

2015 ◽  
Author(s):  
В. А Васюта
Keyword(s):  
Optic Nerve ◽  
Medical Care ◽  
Macular Degeneration ◽  
Key Words ◽  
Optic Nerve Atrophy

АНАЛІЗ ДОСТУПНОСТІ ДО КВАЛІФІКОВАНОЇ МЕДИЧНОЇ ДОПОМОГИ ХВОРИМ З АТРОФІЯМИ ЗОРОВИХ НЕРВІВ - Метою роботи було провести аналіз надання медичної допомоги хворим з атрофіями зорових нервів (АЗН). Виявлено помилки у встановленні діагнозу та призначенні неправильного лікування. Частіше хворих лікують з діагнозами глаукоми, маку- лодистрофії, ретинопатії, ангіопатії сітківки. Порівняно частоту встановлення діагнозу АЗН у різних офтальмологічних закладах. Проаналізовано надання медичної допомоги у відділі нейроофтальмології (кратність консультацій до встановлення діагнозу). Виявлені недоліки потребують подальшого вивчення та оптимізації допомоги хворим з АЗН різного генезу.<br />АНАЛИЗ ДОСТУПНОСТИ КВАЛИФИЦИРОВАННОЙ МЕДИЦИНСКОЙ ПОМОЩИ БОЛЬНЫМ С АТРОФИЯМИ ЗРИТЕЛЬНЫХ НЕРВОВ - Целью работы было провести анализ предоставления медицинской помощи больным с атрофиями зрительных нервов (АЗН). Выявлены ошибки в постановке диагноза и назначении неправильного лечения. Чаше всего больных лечат с диагнозами глаукомы, макулодистрофии, ретинопатии, ангиопатии сетчатки. Сравнены частота установки диагноза АЗН в различных офтальмологических учреждениях. Проанализировано оказание медицинской помощи в отделе нейроофтальмологии (кратность консультаций до установления диагноза). Выявленные недостатки требуют последующего изучения и оптимизации помощи больным с АЗН различного генеза.<br />ANALYSIS OF SKILLED HEALTHCARE FOR PATIENTS WITH OPTIC NERVE ATROPHY - An analysis of medical care in patients with optic nerve atrophy (ONA) is conducted. Еру purpose was to conduct the analysis of medical care a patient with ONA. There were errors in diagnosis and wrong treatment. The most patients were treated with a diagnosis of «glaucoma, macular degeneration, retinopathy, retinal angiopathy».Frequency of setting of diagnosis is compared in various institutions. Analyzed the medical care in neuroophtalmological department (multipleconsultations prior to diagnosis). The exposed failings require a subsequent study and optimization of medical help a patient with ONA of different genesis.<br />Ключові слова: атрофія, зоровий нерв, медична допомога, помилки, діагноз.<br />Ключевые слова: атрофия, зрительный нерв, медицинская помощь, ошибки, диагноз.<br />Key words: atrophy, optic nerve, medical care, mistakes, diagnosis.

scholarly journals Early intervention and lifelong treatment with GLP1 receptor agonist liraglutide in a Wolfram Syndrome rat model with an emphasis on visual neurodegeneration, sensorineural hearing loss and diabetic phenotype

2021 ◽  
Author(s):  
Toomas Jagomäe ◽  
Kadri Seppa ◽  
Riin Reimets ◽  
Marko Pastak ◽  
Mihkel Plaas ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Hearing Loss ◽  
Optic Nerve ◽  
Early Diagnosis ◽  
Rat Model ◽  
Early Onset ◽  
Receptor Agonist ◽  
Wolfram Syndrome ◽  
Retinal Ganglion ◽  
Optic Nerve Atrophy

Abstract BackgroundWolfram syndrome (WS), also known as a DIDMOAD (Diabetes Insipidus, early-onset Diabetes Mellitus, Optic nerve Atrophy and Deafness) is a rare autosomal disorder caused by mutations in the Wolframin1 ( WFS1 ) gene. Previous studies revealed that glucagonlike peptide-1 receptor agonist (GLP1 RA) anti-diabetic drugs are effective in delaying and restoring glucose control in WS animal models and patients. The GLP1 RA liraglutide has also been shown to have neuroprotective properties in aged WS rats, reducing neuroinflammation, retinal ganglion cell death and optic nerve degeneration. WS is an early-onset, chronical condition and, therefore, early diagnosis and lifelong pharmacological treatment is the best solution to control disease progression in WS patients. Hence, the aim of this study was to evaluate the efficacy of the long-term liraglutide treatment on progression of WS symptoms. For this purpose, 2-month-old WS rats were treated with liraglutide (0.4mg/kg/day) up to the age of 18 months and changes in diabetes markers, visual acuity, hearing sensitivity were monitored in vivo over the course of the 16-month treatment period. ResultsEarly and chronic (16-month) intervention with the GLP-1 RA liraglutide delayed the development of glucose intolerance in WS rats. At the end of the experiment, 91% of saline- and 55% of liraglutide-treated WS rats needed daily insulin supplementation. Liraglutide administration was effective in maintaining visual acuity in WS rats by stalling the progression of cataract, degeneration of retinal ganglion cells and of optic nerve atrophy. Prolonged liraglutide therapy could not prevent sensorineural hearing loss at low frequencies. ConclusionThe rat model of WS used in this study is an excellent predictive model for preclinical trials as it closely recapitulates the relative onset and severity of the main symptoms of WS observed in human patients. We found that a 16-month treatment with GLP1 receptor agonist liraglutide delays or prevents the onset of diabetes and protects against vision loss in a rat model of Wolfram syndrome. Therefore, early diagnosis and prophylactic treatment with the GLP-1R agonist liraglutide may also prove to be a promising treatment option for Wolfram syndrome patients by increasing the quality of life of WS patients.

scholarly journals Panhypopituitarism Presenting as Progressive Visual Acuity Deterioration Secondary to Suprasellar Germinoma

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A596-A596
Author(s):  
Rainer Wandrew Young Po ◽  
Monica Therese B Cating-Cabral
Keyword(s):  
Visual Acuity ◽  
Optic Nerve ◽  
Optic Disc ◽  
Clinical Sign ◽  
External Beam Radiation ◽  
Optic Nerve Atrophy ◽  
Beam Radiation ◽  
Intracranial Mass ◽  
The Right ◽  
The Brain

Abstract Background: Overall the incidence of germ cell tumors worldwide is 0.1% with 60% comprising of germinomas. These may present in the suprasellar region a third of the time and thus impart variability in its presenting characteristics. Clinical Case: A 27-year male presented with progressive blurring of vision with incongruent visual acuity and refraction grade with optic disc pallor assessed with normal angiography. Patient also presented with increasing docility, sluggishness and more withdrawn behavior paired with polyuria, polydipsia and cold intolerance. Progressive worsening of vision led to cranial CT revealing an enhancing hyper density in the right caudate head later elucidated via MRI with several interspersed non-enhancing foci in the right caudate nucleus, both internal capsules with associated mild mass effect. The largest component located in the right capsular region with measurement of 21 x 21 x 16 mm with enhancing lesions in the hypothalamus and pituitary stalk. Endocrine evaluation revealed low serum cortisol at 91.03nmol/L (NV 138-685 nmol/L), responsive to ACTH stimulation test (65.94nmol/L to 387nmol/L), high prolactin at 856.9 mIU/L (42.4-296.8mIU/L) and low FT4 8.62pmol/l (9.01-19.05 pmol/L) despite normal TSH 1.528uIU/ml (0.35-4.94 uIU/mL). Optic nerve atrophy was now attributed to compressive intracranial mass. Secondary adrenal insufficiency was managed with hydrocortisone 50mgIV every 8 hours, and central hypothyroidism was managed with levothyroxine 75mcgtab 1tab once a day. Increased urinary output assessed as diabetes insipidus was given desmopressin 60mcg/tab ½ tab 2x a day at this time. Stereotactic biopsy of the brain revealed a germinoma of the right caudate lobe. Given the nature of the intracranial mass with high sensitivity to chemotherapeutic and radiotherapy, a multidisciplinary approach to treatment was taken with radiotherapy, hormonal and steroid replacement. Patient underwent external beam radiation therapy of the brain and spine for a total of 51 treatments allowing for improvement of visual acuity to counting, with polyuria less than 2-3 diaper changes per day. Conclusion: Optic disc pallor is a clinical sign that indicates optic nerve atrophy reflective of the optic tract. This may be an important clinical sign to increase clinical suspicion for intracranial mass lesion especially with correlation to manifestation of pituitary hormone deficiencies.

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