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Pigmented Paravenous Retinochoroidal Atrophy

PPRCA


Presentation

  • Pigmented paravenous retinochoroidal atrophy is an uncommon fundus disorder with a striking clinical presentation.[ncbi.nlm.nih.gov]
  • In the present case the visual acuity at presentation was poor caused by the macular involvement. There was no evidence of ocular inflammation.[ojoonline.org]
Anemia
  • Manifestations of GCA 233 1332 Anterior Ischemic Optic Neuropathy 234 1333 Other Types of Ischemic Visual Loss 235 1335 Orbital Manifestations 236 1343 LargeVessel Vasculitis 237 135 Laboratory Investigations in GCA 238 1352 CReactive Protein 239 1355 Anemia[books.google.com]
Cat Scratch
  • 199 Chorioretinal Lesions in Infectious Diseases of Neuroophthalmic Interest 205 121 Introduction 206 ofToxoplasmosis in Immunocompetent Patients 207 12213 OphthalmicToxoplasmosis in AIDS Patients 209 1222 Toxocariasis 210 12233 Lyme Disease 211 1224 Cat[books.google.com]
Abnormal Eye Movement
  • Eye Movements 263 1442 NonNystagmic Abnormal Eye Movements 264 Chronic Progressive External Ophthalmoplegia A Common Ocular Manifestation of Mitochondrial Disorders 267 152 Clinical Features 268 153 Genetics 270 1533 Defects of Intergenomic Communication[books.google.com]
Suggestibility
  • Our patients, and the preponderance of males in previously reported cases of this disease, suggest the possibility of Y-chromosome mediated male-to-male transmission.[ncbi.nlm.nih.gov]
Ocular Flutter
  • Flutter 296 Rehabilitation in Neuroophthalmology 301 172 Psychophysics of Normal Reading 302 173 Diseases of the Visual Pathways and their Functional Deficits 303 Nerve Fiber Bundle Defects 305 1732 Optic Chiasmal Syndromes 307 17331 Hemianopic Reading[books.google.com]
Myokymia
  • Symptomatic Treatment 277 156 Differential Diagnosis 278 1563 Congenital Fibrosis of the Extraocular Muscles 279 Treatment of Specific Types of Nystagmus 283 161 Introduction 284 16211 Etiology 286 16212 Therapeutic Recommendations 287 1622 Superior Oblique Myokymia[books.google.com]
Encephalopathy
  • Due to Viral and NonConventional Agents 220 1243 JC Virus and Progressive Multifocal Leukoencephalopathy 221 1244 Herpetic Encephalopathy and Acute Retinal Necrosis Syndrome 222 Giant Cell Arteritis 226 1312 Triggering Event 228 1314 Macrophage Recruitment[books.google.com]
Neurologic Manifestation
  • Manifestations 215 12313 Diagnostic Tests 216 12322 HIV Retinopathy 217 12323 CMV Retinitis 218 Encephalopathies Due to Viral and NonConventional Agents 220 1243 JC Virus and Progressive Multifocal Leukoencephalopathy 221 1244 Herpetic Encephalopathy[books.google.com]
Chronic Progressive External Ophthalmoplegia
  • Progressive External Ophthalmoplegia A Common Ocular Manifestation of Mitochondrial Disorders 267 152 Clinical Features 268 153 Genetics 270 1533 Defects of Intergenomic Communication with Multiple Deletions of mtDNA 271 1534 Point Mutations of mtDNA[books.google.com]

Workup

  • Systemic workup revealed QuantiFERON-gold positive. This is the first report of unilateral PPRCA secondary to presumed ocular tuberculosis. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:345-349.].[ncbi.nlm.nih.gov]
  • Laboratory test Laboratory studies should be aimed towards narrowing the differential diagnosis depending on the presentation and given the overlap with inflammatory degenerative diseases a workup can include: comprehensive metabolic panel, complete and[eyewiki.org]
  • Results of laboratory studies, including a vasculitis workup, were within normal limits. A tuberculin skin testing result was 20 mm, erythrocyte sedimentation rate was 23 mm/hr, and a Quantiferon TB gold test (Qiagen) result was normal.[retinatoday.com]
  • We referred the patient for a medical workup and asked her to return in six to eight weeks for close observation. The patient returned two years later with complaints of vision loss OS (20/100; OD remained 20/20).[reviewofoptometry.com]
Gliosis
  • The optic nerve appears normal with no gliosis or atrophy and the retinal vessel caliber are normal.[eyewiki.org]

Treatment

  • No effective treatment is available.[ncbi.nlm.nih.gov]
  • 276 1552 Symptomatic Treatment 277 156 Differential Diagnosis 278 1563 Congenital Fibrosis of the Extraocular Muscles 279 Treatment of Specific Types of Nystagmus 283 161 Introduction 284 16211 Etiology 286 16212 Therapeutic Recommendations 287 1622[books.google.com]

Prognosis

  • The visual prognosis for patients with pigmented paravenous retinochoroidal atrophy is quite good.[ncbi.nlm.nih.gov]
  • Prognosis Patients with PPRCA have good visual prognosis with slowly progressive disease. In patients without macular involvement, visual acuity is minimally adversely affected.[eyewiki.org]
  • Ischemic Visual Loss 235 1335 Orbital Manifestations 236 1343 LargeVessel Vasculitis 237 135 Laboratory Investigations in GCA 238 1352 CReactive Protein 239 1355 Anemia 240 1361 Temporal Artery Biopsy 241 1363 Role of Ultrasound 243 137 Treatment and Prognosis[books.google.com]
  • Prognosis - this condition recurs over months and frequent foveal involvement gives rise to a relatively poor prognosis. Multiple evanescent white dot syndrome (MEWDS) Nature - uncommon, idiopathic and self-limiting condition.[patient.info]
  • WFAF may also be helpful in determining prognosis in patients with AMD, although this needs confirmation in larger trials (i.e., AREDS2).[reviewofophthalmology.com]

Etiology

  • Etiology The etiology of PPRCA remains unknown with a presumed initial inflammatory insult that leads to the slow developmental of the retinal findings.[eyewiki.org]
  • Although earlier reports have suggested a possible post-inflammatory etiology such as tuberculosis, rubeola and syphilis as possible causes, a definite etiology remains elusive. [2] Typically, bone corpuscle pigmentation is seen along the distribution[ojoonline.org]
  • The cause of the condition may be unknown or idiopathic, although a dysgenetic, degenerative, hereditary etiology or even an inflammatory cause has been hypothesized.[ingentaconnect.com]
  • We could not confirm the etiology as our patient could not undergo genetic studies. A long-term follow-up would reveal how the left eye condition progresses, particularly whether RP like degeneration develops in that eye.[ijo.in]
  • 274 1541 Myohistological Investigations 275 155 Treatment 276 1552 Symptomatic Treatment 277 156 Differential Diagnosis 278 1563 Congenital Fibrosis of the Extraocular Muscles 279 Treatment of Specific Types of Nystagmus 283 161 Introduction 284 16211 Etiology[books.google.com]

Epidemiology

  • Questions involved clinicians' perceptions of the epidemiology, diagnosis, treatment and prophylaxis of invasive candidosis. P 0.0 Rev. Soc. Bras. Med. Trop. . Publicado em: 23/07/2013 3.[academicoo.com]
  • Ocular symptoms were analyzed using a validated ocular surface... article Adenoviral keratoconjunctivitis: An update The objective of this review is to describe the clinical and epidemiological characteristics of adenoviral conjunctivitis, as well as[infona.pl]
  • Relevant External Links for CRB1 Genetic Association Database (GAD) CRB1 Human Genome Epidemiology (HuGE) Navigator CRB1 Atlas of Genetics and Cytogenetics in Oncology and Haematology: CRB1 No data available for Genatlas for CRB1 Gene Clinical and molecular[genecards.org]
  • BACKGROUND Describe clinical and epidemiological characteristics of pediatric patients diagnosed with acute diarrhea and shock, admitted to the pediatric intensive care unit, in order to compare the evolution of clinical data between the survival and[pe.anoxia.us]
Sex distribution
Age distribution

Pathophysiology

  • This case might provide preliminary information regarding the pathophysiology of PPRCA and improve our understanding of the nature of this disease.[ncbi.nlm.nih.gov]
  • Offers the most comprehensive content available on retina, balancing the latest scientific research and clinical correlations, covering everything you need to know on retinal diagnosis, treatment, development, structure, function, and pathophysiology.[books.google.com]

Prevention

  • Prevention the secondary degeneration of cones in RP represents a very promising approach. This because such a therapy could be applied in a wide range of mutations expressed in rods, and even at late stages of the disease.[institut-vision.org]
  • BACKGROUND AND OBJECTIVES The presence of adverse reactions, inherent to all treatments, justifies the necessity of deep knowledge, by the medical team of the prevention and treatment of occasional organic dysfunctions, reducing its impact.[pe.anoxia.us]

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