However, less than 15%-20% of cases have all three symptoms at presentation [2-4]. Our patient presented with only 2 features (visual and neurological), this can make the diagnosis quite difficult. [clinmedjournals.org]

However, all three features may not be present concurrently upon initial presentation. [eyewiki.aao.org]

This is the first reported case of Susac Syndrome in Peru, presented with the classic triad, which is an infrequent presentation. [ncbi.nlm.nih.gov]

• Constitutional Symptom

  Crossref PubMed Google Scholar Some patients with Susac syndrome have constitutional symptoms, arthralgias, and myalgias, as well as pathologic microvascular changes on muscle biopsy, without frank arthritis or myopathy, suggesting that the disease may [mayoclinicproceedings.org]

  Some patients, however, have constitutional symptoms and microvascular changes on muscle biopsy, which suggests a possible systemic disease with predilection for the triad of symptoms of Susac syndrome either because microvascular occlusions in the brain [ajnr.org]

• Retinal Damage

  The sectorial pattern of retinal damage supports the hypothesis of a vascular origin with patchy lesions. [journals.plos.org]

  Patterns of retinal damage facilitate differential diag-nosis between Susac's syndrome and MS. Plos One. 2012;7(6):e38741. 2 Avino JA, Espana E, Peris-Martínez C, Menezo JL. [scielo.br]

  Patterns of retinal damage facilitate differential diagnosis between Susac syndrome and MS. PLoS ONE, 7(6):e38741. [zora.uzh.ch]

  Brandt AU, Zimmermann H, Kaufhold F, Promesberger J, Schippling S, Finis D et al (2012) Patterns of retinal damage facilitate differential diagnosis between Susac syndrome and MS. PLoS ONE 7:e38741 CrossRef PubMed Google Scholar 13. [doi.org]

  The anti-endothelial response leads to retinal damage ranging from arteriolar plaques to occlusions. [retina-specialist.com]

• Visual Impairment

  At one-year follow-up, bilateral hearing loss, mild visual impairment, and mood changes were still apparent. Frontal intermittent rhythmic delta activity can be seen in Susac syndrome. [ncbi.nlm.nih.gov]

  Case Report: The case is presented of a 28-year-old patient hospitalized due to visual impairment of the left eye, and whose hearing and neuropsychiatric disorders had appeared two years earlier. [aaem.pl]

  Case Description A healthy 38-year-old woman presented initially to the ophthalmologist on account of sudden visual impairment in her left eye. She had no known illnesses and no known risk factors for visual loss. No complaints of hearing loss. [clinmedjournals.org]

  A high index of suspicion leading to early recognition of this syndrome is important because immunosuppressive treatment may decrease permanent neuropsychological or visual impairment. [synapse.koreamed.org]

• Central Scotoma

  This, in turn, can delay the diagnosis and subsequent treatment of the disease. 74.1.1 Symptoms Possible loss of peripheral vision and/or central scotomas is often described. [entokey.com]

  Perimetry showed a central scotoma with a macular problem [Figure 5]. With the confirmation of cerebral, cochlear, and retinal involvement, a diagnosis of Susac's syndrome was made. [ncbi.nlm.nih.gov]

  Conclusions An important differential diagnosis of Susac syndrome is multiple sclerosis, which typically shows decreased VA and contrast vision combined with central scotoma, an overall reduced RNFL thickness but normal FA. [iovs.arvojournals.org]

• Anterior Uveitis

  […] a uveitis referral centre. [joii-journal.springeropen.com]

• Tinnitus

  She also complained of decreased hearing with tinnitus of the right ear and mild headache. She was previously healthy and had no remarkable medical history. Best-corrected visual acuity was 20 / 50 in the left eye and 20 / 20 in the right eye. [ncbi.nlm.nih.gov]

  […] into Ophthalmology department of Mymensingh Medical College Hospital, Mymensingh, Bangladesh on 18 December 2017 presented with the complains of gross dimness of vision of left eye for 10 years, vitiligo over the whole body for 5years,hearing loss with tinnitus [pesquisa.bvsalud.org]

  The patient was a 32-year-old woman, complaining of diplopia, right blurred vision, progressive gait disturbance, tinnitus, attention deficit, and slight memory loss. [idibgi.org]

• Hearing Impairment

  In Susac syndrome, occlusions of pre-capillary arterioles of the brain, retina, and cochlea lead to the classical clinical triad of subacute encephalopathy, visual disturbances due to branch retinal artery occlusions and sensorineural hearing impairment [ncbi.nlm.nih.gov]

  Later, he developed bilateral hearing impairment but had no visual symptoms. [research.regionh.dk]

• Fluctuating Hearing Loss

  Susac syndrome is a rare disorder of unknown origin characterized by the triad of encephalopathy, fluctuating hearing loss, and visual loss resulting from microangiopathy of the brain, cochlea, and retina. [ncbi.nlm.nih.gov]

  Fluctuating hearing loss, episodic headache, and stroke with platelet hyperaggregability: coexistence of auditory neuropathy and cochlear hearing loss. Brain Dev 2006;28(1):55-9. [medlink.com]

• Suggestibility

  Development of symptoms during pregnancy and oral contraceptive use suggest a possible contribution of coagulation abnormalities in such patients. [wikidoc.org]

  Impedance testing suggested normal pressure and good compliance in both ears. These findings were highly suggestive of Susac syndrome, and interferon beta-1a treatment was discontinued. [jamanetwork.com]

  Treatment with IVIg and corticosteroids provides a therapeutic option that avoids the toxicities of chemotherapy and suggests the possible importance of pathologic antibodies in the pathogenesis of Susac syndrome. [ncbi.nlm.nih.gov]

  She underwent a cerebral Magnetic Resonance (MRI) showing T2-weighted lesions, that were suggestive of multiple sclerosis (Figure 1). [clinmedjournals.org]

  Patient was noted to have focal retinal whitening at the proximal trunk of the inferotemporal vascular arcade with several faint peripapillary cotton wool spots suggestive of retinal vasculitis. [sma.org]

• Headache

  Dawe JA, Green AL (2021) Headache in susac’s syndrome. Curr Pain Headache Rep 25: 25. Wilf-Yarkoni A, Elkayam O, Aizenstein O, Oron Y, Furer V, et al. (2020) Increased incidence of susac syndrome: A case series study. BMC Neurol 20: 332. [clinmedjournals.org]

  Headache is estimated to affect up to 80% of Susac syndrome patients, but the relevance of headache characteristics and profile is not yet clear. [mdpi.com]

  I have two patients who presented to headache clinics with migraine headaches and branch retinal artery occlusions and were misdiagnosed as "complicated migraine". The migraine headaches respond to triptans and Topamax. [web.archive.org]

  Symptoms include headaches, slurred speech, and trouble focusing. Treatment includes drugs that suppress the immune system, such as steroids. Susac’s Syndrome Menu What is Susac's syndrome? Susac's syndrome is a rare, autoimmune disease. [my.clevelandclinic.org]

  Migraine-like headaches are also common and are present in 80% of reported cases. These headaches may precede presenting symptoms by several months. [eyewiki.aao.org]

• Confusion

  At 1-month follow-up, patient's confusion had resolved. The second patient was a 32-year-old man who had acute onset of confusion. CSF demonstrated pleocytosis and elevated protein. [ncbi.nlm.nih.gov]

  He received IVIG and demonstrated resolution of confusion at one-month follow-up. [medicalxpress.com]

  During the hospital course, she became confused and disoriented to time and place. She eventually developed transient blurring of vision. [sma.org]

• Ataxia

  The encephalopathy, ataxia, and urinary incontinence resolved, and the hearing loss stabilized. Cognitive dysfunction, a result of multifocal, micro‐ischemic injury, improved considerably but has not fully resolved. [onlinelibrary.wiley.com]

  A 24-year-old female patient suffering from hearing loss of the right ear, bilateral visual field defects, memory loss, and ataxia was examined using kinetic and static threshold perimetry, fluorescein angiography, indocyanine green angiography, and electrophysiological [tandfonline.com]

  The patient's condition was ultimately characterized as a Susac-like syndrome manifesting as current spastic ataxia and previous episodes of retinal vein occlusion and vertigo. [neuro.psychiatryonline.org]

  All cases manifested varied neurological symptoms including pyramidal and/or sensory tract symptoms, amnesic disorders, ataxia and vertigo. [ncbi.nlm.nih.gov]

  Headache is often the initial symptom followed by cognitive changes, ataxia, confusion, and memory and psychiatric disturbance due to involvement of the brain (8). [sboportal.org.br]

• Personality Change

  A 43-yr-old man presented with headaches, forgetfulness, and personality changes. Based on imaging, there was concern for acute disseminated encephalomyelitis. He improved with steroids and immunoglobulin. [ncbi.nlm.nih.gov]

  Encephalopathy is characterized by memory loss, confusion, disorientation, behavioral disturbances, personality changes and psychosis. Focal CNS signs include ataxia, motor and sensory impairment, dysarthria and oculomotor dysfunction. [orpha.net]

  Often sufferers experience a personality change and develop bizarre and paranoid behavior. [aarda.org]

  This is a case report of a 29-year-old man, who presented with progressive confusion, memory problems and personality changes during 1.5 months. Later, he developed bilateral hearing impairment but had no visual symptoms. [research.regionh.dk]

• Dysarthria

  Discussion This case summary provides an example of a woman diagnosed with possible Susac's syndrome manifested as ataxic gait, dysarthria, and vision and hearing changes, with associated cognitive decline. [neuro.psychiatryonline.org]

  She had dysarthria and mild dysnomia. Ophthalmoscopic examination demonstrated pallor of the optic disks and evidence of past retinal arteriolar occlusions. [mayoclinicproceedings.org]

  Focal CNS signs include ataxia, motor and sensory impairment, dysarthria and oculomotor dysfunction. Optic involvement includes scotoma and visual distortion. BRAOs may also occur at the periphery and not result in clinical deficits. [orpha.net]

  This was accompanied by nausea, vertigo, left facial numbness, and dysarthria that lasted 3 hours. The visual illusion recurred twice, the latter two episodes accompanied by the illusion of looking through a kaleidoscope. There was no headache. [n.neurology.org]

  Susac syndrome classically consists of a clinical triad of: acute or subacute encephalopathy which could manifest in a broad spectrum of symptoms, e.g. memory impairment, confusion, behavioral disturbances, ataxia, dysarthria, paranoid psychosis, and [radiopaedia.org]

We surmise that more patients could have been diagnosed with the recommended diagnostic workup. [ncbi.nlm.nih.gov]

• Central Scotoma

  This, in turn, can delay the diagnosis and subsequent treatment of the disease. 74.1.1 Symptoms Possible loss of peripheral vision and/or central scotomas is often described. [entokey.com]

  Perimetry showed a central scotoma with a macular problem [Figure 5]. With the confirmation of cerebral, cochlear, and retinal involvement, a diagnosis of Susac's syndrome was made. [ncbi.nlm.nih.gov]

  Conclusions An important differential diagnosis of Susac syndrome is multiple sclerosis, which typically shows decreased VA and contrast vision combined with central scotoma, an overall reduced RNFL thickness but normal FA. [iovs.arvojournals.org]

• White Matter Lesions

  Conclusions: The MR scans in SS show a rather distinctive pattern of supratentorial white matter lesions that always involve the corpus callosum. [n.neurology.org]

  When symptoms recurred, magnetic resonance imaging showed new scattered gray and white matter lesions, many within the corpus callosum. Angiogram showed multiple branch retinal artery occlusions, pathognomonic for Susac syndrome. [ncbi.nlm.nih.gov]

Vodopivec I, Prasad S (2016) Treatment of susac syndrome. [clinmedjournals.org]

There are no controlled studies on the treatment of Susac syndrome. [karger.com]

Visual fields showed improvement immediately after treatment. This is the first report of hyperbaric oxygen treatment for Susac syndrome. Dramatic post-treatment visual field and acuity changes suggest benefit of treatment. [ncbi.nlm.nih.gov]

The etiology of this syndrome is still unknown, but the prognosis is good in most cases. Spontaneous resolution usually occurs, but early treatment minimizes the risk of sequelae. [ncbi.nlm.nih.gov]

Susac syndrome: clinical characteristics, clinical classification, and long-term prognosis. Medicine (Baltimore). 2016; 95(43): e5223. https://doi.org/10.1097/MD.000.... [aaem.pl]

Free PMC article Erratum in Erratum: Susac syndrome: clinical characteristics, clinical classification, and long-term prognosis: Erratum. [pubmed.ncbi.nlm.nih.gov]

Contents 1 Disease Entity 2 Ocular manifestations 3 Etiology 4 Diagnostic procedures 5 References Disease Entity Susac syndrome is a triad of Encephalopathy, sensorineural hearing loss, and branch retinal artery occlusions. [eyewiki.aao.org]

The objective of this study is to characterize the epidemiological, clinical, and etiological features of SUSAC's Syndrome. [clinicaltrials.gov]

These features are consistent with Susac syndrome, a multifocal central nervous system disorder of uncertain etiology. This is the first reported case of Susac syndrome in a patient with hepatitis C. [ncbi.nlm.nih.gov]

Etiology The etiology is unknown, although it is thought to be an autoimmune process leading to inflammation and occlusion of the pre-capillary arterioles of the brain, retina, and inner ear. [orpha.net]

The objective of this study is to characterize the epidemiological, clinical, and etiological features of SUSAC's Syndrome. [clinicaltrials.gov]

In this review, we have demonstrated the epidemiology and pathology of Susac syndrome with detailed description of clinical signs, diagnostic procedures and therapeutic possibilities. [ncbi.nlm.nih.gov]

Topics discussed include epidemiology, pathophysiology, clinical manifestations, differential diagnoses, classification schema, laboratory investigations, characteristic audiometric findings, high-yield radiographic imaging, temporal bone histopathology [pennstate.pure.elsevier.com]

The underlying pathophysiology is likely an autoimmune mediated occlusion of microvessels resulting in variable degrees of central nervous system (CNS) dysfunction, visual disturbances, and hearing loss. [ncbi.nlm.nih.gov]

Topics discussed include epidemiology, pathophysiology, clinical manifestations, differential diagnoses, classification schema, laboratory investigations, characteristic audiometric findings, high-yield radiographic imaging, temporal bone histopathology [pennstate.pure.elsevier.com]

Awareness of the condition and suspicion in selected patients will provide early diagnosis and treatment, which are both important to prevent the development of severe sequelae. [ncbi.nlm.nih.gov]

Early diagnosis and treatment can halt the progression and prevent permanent disability. Methods: Here, we describe a Hungarian case and review the clinical characteristics, diagnostic procedures and current concepts of therapy. [hungary.pure.elsevier.com]