Presentation
Up to 4-10% of patients with untreated syphilis may develop NS. [1] These days NS rarely presents in its classical forms, tabes dorsalis or general paresis, and often presents with atypical features. [2] The clinical and laboratory features of NS have [annalsofian.org]
An abnormal fundus examination on initial presentation is a significant predictor of visual impairment at 3 months after presentation. [omicsonline.org]
Two cases of the disease with other central nervous system involvement are presented and briefly discussed as to nosology and relationship to the heredofamilial group of diseases. [link.springer.com]
Less than five percent of patients only present with sole ocular involvement at diagnosis. [japi.org]
Strabismus and latent nystagmus are often present. Up slanting palpebral fissures and epicanthal folds have been noted. Visual acuity levels have not been reported. [disorders.eyes.arizona.edu]
Entire Body System
- Disability
Applying for SSD benefits on behalf of a minor child is somewhat different than it is when submitting an adult disability application for yourself. [disabilitybenefitscenter.org]
NR2F1 mutations cause optic atrophy with intellectual disability. Am J Hum Genet. 2014 Feb 6;94(2):303-9. [disorders.eyes.arizona.edu]
Please enable JS and disable any ad blocker [ophthalmologytimes.modernmedicine.com]
SUPPORT Below are the links to the organizations providing disability care and support services to people with impaired vision. FACEBOOK SUPPORT LINKS Support groups bring together people facing similar issues. [rawa.com.au]
- Epilepsy
Associated optic disc dysplasia, microphthalmia, cortical dysgenesis, mental retardation, and epilepsy may be seen. [18] Hereditary optic neuropathies include dominant optic atrophy and Leber hereditary optic neuropathy. [ijri.org]
Gastrointestinal
- Choking
Major points in favour of diagnosis of pseudo-papilloedema as opposed to pathological "choked-disc" are (1) the type of eye (hypermetropic ?) [academy.org.uk]
EYES - Optic nerve - Neuritis – choked bell. bry. dub. gels. hell. nux-v. puls. verat-v. EYES - Optic nerve - Neuritis – descending ars. cupr. merc-c. EYES - Optic nerve – Paralysis nux-v. oxyt. ph-ac. [homeopathyworldcommunity.ning.com]
Rarely, pituitary tumours may cause a choked disc which may further give rise to post-papilloedemic type of atrophy- as is the case with other intracranial tumours. [ijo.in]
See Rayleigh criterion ; point-spread function ; limit of resolution. choked d. See papilloedema. cupped disc An enlarged and deepened excavation of the physiological cup. [medical-dictionary.thefreedictionary.com]
Cardiovascular
- Vascular Disease
Most patients are elderly with other forms of vascular disease. It is managed conservatively; however, occasionally surgical intervention may be required when there is rapid progression. [72] Figure 27: Compressive optic neuropathy: Vascular. [ijri.org]
Eyes
- Progressive Loss of Vision
Bilateral, slowly progressive loss of vision; OPA1 gene on chromosome 3q28 ( 2 ) Autosomal recessive optic atrophy: OPA6 gene, rare ( 2 ) Optic atrophies associated with complex genetic disorders: Disorders of amino acid metabolism Hyperhomocysteinemia [unboundmedicine.com]
Secondary Optic Atrophy was attributed to papilloedema when there were other evidences of raised intra-cranial tension, history of slow gradual progressive loss of vision, and to papillitis when history of sudden loss of vision and no evidence of raised [ijo.in]
This may progress later in life, resulting in more severe loss of visual acuity. TREATMENT There is currently no way to reverse the vision loss associated with dominant optic atrophy. [webeye.ophth.uiowa.edu]
These cause gradual and progressive loss of vision. It is important to mention whether the optic chiasm is prefixed or postfixed. [ijri.org]
- Central Scotoma
scotoma OS Orthophoric Minimal amplitude horizontal pendular 10 CF 5′ CF 5′ 0 0 Diffuse optic atrophy OU Central scotomas OU Orthophoric None 11 20/200 20/100 Small discs with diffuse optic atrophy OU Unable OU Modest XT Moderate amplitude horizontal [iovs.arvojournals.org]
SITA Standard 30-2 Humphrey Visual Field testing from the initial visit in February 2011 shows central scotoma OS. [journal.opted.org]
(Case 6) 1 remember an experience in my early days of practice at an age when youth is synonymous with rashness, of having seen a case of optic neuritis with a central scotoma first in one eye then in the other. [ijo.in]
- Blurred Vision
The symptoms of optic atrophy relate to a change in vision, specifically: Blurred vision. Difficulties with peripheral (side) vision. Difficulties with color vision. A reduction in sharpness of vision. [my.clevelandclinic.org]
Reference: www.naturaleyecare.com Blurred vision Decrease in visual function such as a decrease in sharpness and clarity of vision (visual acuity) or decreases in side (peripheral) vision. Color vision and contrast sensitivity can also be affected. [rawa.com.au]
Blurred vision Abnormal side vision Abnormal color vision Decreased brightness in one eye relative to the other Poor constriction of the pupil in light Decreased brightness in one eye relative to the other Change in the optic disc A typical investigation [homeopathyworldcommunity.ning.com]
- Hemianopsia
Characteristic visual field patterns include papillomacular defect (cecocentral scotoma), arcuate defect (include altitudinal) or temporal wedge defect (nasal fibers) for prechiasmal, bitemporal (superior) field defects for chiasmal lesions, and hemianopsia [eyewiki.aao.org]
Sagittal and coronal T2 fat-saturated images of a 60-year-old male with bitemporal hemianopsia show a large sellar suprasellar mass with pituitary gland not seen separately. [ijri.org]
Neurologic
- Meningism
Of the established causes, meningitis topped the list involving 16 cases. [ijo.in]
Meningeal and vascular syphilis of the spinal cord. Med (Baltimore) 1944;23:181-214. 5. Wilson SA. Neurology. Ch. 17, 2 nd ed. London: Butterworths; 1954. 6. Hahn RD. Tabes dorsalis with special reference to primary optic atrophy. [annalsofian.org]
[…] surrounding the nervous system alone (termed early Neurosyphilis), there are 6 principal types: Asymptomatic Neurosyphilis Meningeal Neurosyphilis (Acute Syphilitic Meningitis) Meningovascular Neurosyphilis General Paresis of the Insane Tabes Dorsalis [dovemed.com]
Tuberculous meningitis (TBM) is the most common form of meningitis in the developing countries. [omicsonline.org]
- Papilledema
Similar Codes ICD-9 Code ICD-9 Description 377.00 Papilledema, unspecified 377.01 Papilledema associated with increased intracranial pressure 377.02 Papilledema associated with decreased ocular pressure 377.03 Papilledema associated with retinal disorder [healthprovidersdata.com]
If normal, Lumbar puncture - measure CSF exit pressure, blood, infection, etc - Any stage of papilledema is a medical emergency. [studyblue.com]
H47.10 Unspecified papilledema H47.11 Papilledema associated with increased intracranial pressure H47.12 Papilledema associated with decreased ocular pressure H47.13 Papilledema associated with retinal disorder H47.14 Foster-Kennedy syndrome H47.141 [icd10coded.com]
Optic atrophy following papilledema (secondary). [emedicine.medscape.com]
[/i] Optic atrophy following papilledema (secondary). [thehealthscience.com]
- Seizure
Many children with BOA may have other neurological problems such as seizures, developmental delays or motor problems. [secure.ssa.gov]
Seizures, problems with motor function and developmental delays are commonly seen in more severe cases. There is no cure for BOA and early treatment focuses primarily on addressing symptoms. [disabilitybenefitscenter.org]
Many children with ONA have additional neurologic problems, such as seizures, developmental delays or motor problems, and Cortical Visual Impairment (CVI). [tsbvi.edu]
[…] has been divided five major categories, that is, asymptomatic, meningeal, meningovascular, parenchymatous, and gummatous, these entities represent a continuum and frequently overlap. [3] Overlap between different forms of NS is largely restricted to seizure [annalsofian.org]
Infections, especially pneumonia and septicaemia, are a common cause of death. [7 ] Neurologic problems, such as hydrocephalus, facial paresis, seizures, and mental retardation, are also common. [5, 8 ] Dental problems are also common. [djo.org.in]
- Convulsions
Plan of study: A detailed history and examination was performed using a structured proforma and the following variables were recorded, age, gender, stage of TBM, duration of illness, convulsions, diplopia, presence of extra-neural tuberculosis, level [omicsonline.org]
- Bitemporal Hemianopsia
Sagittal and coronal T2 fat-saturated images of a 60-year-old male with bitemporal hemianopsia show a large sellar suprasellar mass with pituitary gland not seen separately. [ijri.org]
Workup
Our case report highlights the fact to incorporate serum and CSF VDRL screening in workup of noncompressive myelopathy. [annalsofian.org]
Visual Field Test
- Central Scotoma
scotoma OS Orthophoric Minimal amplitude horizontal pendular 10 CF 5′ CF 5′ 0 0 Diffuse optic atrophy OU Central scotomas OU Orthophoric None 11 20/200 20/100 Small discs with diffuse optic atrophy OU Unable OU Modest XT Moderate amplitude horizontal [iovs.arvojournals.org]
SITA Standard 30-2 Humphrey Visual Field testing from the initial visit in February 2011 shows central scotoma OS. [journal.opted.org]
(Case 6) 1 remember an experience in my early days of practice at an age when youth is synonymous with rashness, of having seen a case of optic neuritis with a central scotoma first in one eye then in the other. [ijo.in]
Treatment
Treatment Guidelines There is no local (retinal) treatment for primary optic atrophy so no referral to a retinal specialist is warranted. The key to proper treatment is determining the etiology of the optic atrophy. [decisionmakerplus.net]
One of the reasons for pessimism is that there is too often an excessive delay in starting treatment and that, as a result, optic atrophy may progress to blindness before adequate treatment is instituted. [nejm.org]
Treatment depends on which disorder that you have. With some optic nerve disorders, you may get your vision back. With others, there is no treatment, or treatment may only prevent further vision loss. [icdlist.com]
Until recently, there was no treatment indicated for LHON. [santhera.com]
However, after established optic neuropathy and visual loss there is little chance, if any, for visual recovery and no definitive treatment exist for treatment in these cases. [clinicaltrials.gov]
Prognosis
Atrophic (BR)- atrophic pallor, edema, attenuated vessels, no hemes, opacified NFL - profoundly reduced VAs, color vision, VF defects, + APD Prognosis and Management of Papilledema 1. Brain scan - rule out SOL 2. [studyblue.com]
Prognosis is variable, with moderate to severe visual loss and nystagmus. Systemic abnormalities include spastic gait, ataxia and mental handicap. 23. [slideshare.net]
It was found that the prognosis for useful vision is in general favorable in this group of cases. [pediatrics.aappublications.org]
Unfortunately, a prompt diagnosis is of extreme importance, since the sooner an appropriate treatment is initiated, the better the prognosis and the less probability of permanent visual damage. [frontiersin.org]
Visual prognosis following optic neuropathy is generally poor. Renal biopsy provided a clue for the diagnosis in the absence of clinical findings other than ocular involvement in these patients. [japi.org]
Etiology
Optic nerve damage may be reversible, so early detection and treatment of the underlying etiology is critical. Correction of an underlying nutritional deficiency and discontin... [5minuteconsult.com]
In 27 % of the cases no cause could be found while in the rest of 73% cases definite etiological factor was present. [ijo.in]
Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]
Etiology and Pathophysiology Genetic etiologies usually involve defects in mitochondrial function. Exposure of retinal nerve cells to light normally results in the generation of free radical species. [unboundmedicine.com]
Etiologic classification Regardless of etiology, optic atrophy is associated with variable degrees of visual dysfunction, which may be detected by one or all of the optic nerve function tests (see Other Tests). [thehealthscience.com]
Epidemiology
EPIDEMIOLOGY AND CAUSES OF OPTIC ATROPHY IN GENERAL OUTPATIENT DEPARTMENT OF LUMBINI EYE INSTITUTE. [nepjol.info]
The epidemiology of Leber hereditary optic neuropathy in the northeast of England. Am J Hum Genet. 2003;72:333-9. Horvath R, Abicht A, Scoubridge EA, et al. [rarediseases.org]
Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. [frontiersin.org]
Epidemiology Frequency United StatesAccording to Tielsch et al, the prevalence of blindness attributable to optic atrophy was 0.8%. [3] According to Munoz et al, the prevalence of visual impairment and blindness attributable to optic atrophy was 0.04% [thehealthscience.com]
Epidemiology Acquired ONA is more common than hereditary and congenital ONA and tends to occur later in life: Glaucoma, ischemic optic neuropathy (older), optic neuritis (younger), compressive (any age), and toxic/nutritional are leading causes in adults [unboundmedicine.com]
Pathophysiology
Pathophysiology The optic nerve comprises approximately 1.2 million axons that originate at the ganglion cell layer of the retina. [thehealthscience.com]
Pathophysiology There is a loss of axons and shrinkage of myelin, leading to widening of the optic cup. Presentation [ 1 ] This depends on the causative condition. Optic disc atrophy in isolation results in the following symptoms and signs. [patient.info]
Traumatic optic neuropathy The exact pathophysiology of traumatic optic neuropathy is poorly understood, although optic nerve avulsion and transection, optic nerve sheath hematoma, and optic nerve impingement from a penetrating foreign body or bony fragment [emedicine.medscape.com]
Etiology and Pathophysiology Genetic etiologies usually involve defects in mitochondrial function. Exposure of retinal nerve cells to light normally results in the generation of free radical species. [unboundmedicine.com]
The pathophysiology of NAION is unknown, but it is related to poor circulation in the optic nerve head. [en.wikipedia.org]
Prevention
They have to be also aware of the preventive measures and precautions to be taken, which include: Avoidance of multiple sex partners Practice of safe sex (use of condoms that prevent entry of the syphilis bacteria) Excessive craving for drugs and alcohol [dovemed.com]
Primary prevention (removal of the process causing the damage) is the goal to prevent loss of axons and optic atrophy (neuropathy). [eyewiki.aao.org]
With others, there is no treatment, or treatment may only prevent further vision loss. [icdlist.com]
Página 310 - A randomized trial comparing ticlopidine hydrochloride with aspirin for the prevention of stroke in high-risk patients. [books.google.es]
For example, early diagnosis and aggressive treatment can prevent further damage in patients with compressive and toxic neuropathies. Intravenous steroids are helpful in some cases of optic neuritis or anterior ischemic optic neuropathy. [decisionmakerplus.net]