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]

• 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]

  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]

  On follow-up, the patient’s condition had improved, but he continued to have slight cognitive impairment and left deafness persisted. There was no functional visual limitation despite the angiographic findings. [ajnr.org]

  Following by hearing loss and visual impairment due to cochlea and retinal arterial branch involvement respectively. Our patient presented with vertigo, ataxia, and hearing loss 6 months before ocular complaint. [sboportal.org.br]

• Peripheral Vision Loss

  His visual symptoms improved, but 3 months later he developed peripheral vision loss in the right eye, resolving completely after a 1-week course of prednisone. Ten months after the initial visit, he experienced bilateral sequential hearing loss. [jamanetwork.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]

  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]

  Loss of peripheral (side) vision Inner ear symptoms: Hearing loss Dizziness (vertigo) Ringing in the ears (tinnitus) All three parts of the disease may not appear at the same time. [my.clevelandclinic.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]

• Paranoid Ideation

  Psychiatric manifestations such as disinhibition, aggressive behavior, paranoid ideation and hallucinations were also present. Seven patients suffered at least one relapse. [ncbi.nlm.nih.gov]

• Headache

  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]

  A 38-year-old woman seropositive for hepatitis C developed headache, sensorineural hearing loss, encephalopathy, and retinal arteriolar occlusions. Brain MRI showed signal abnormalities in the basal ganglia and corpus callosum. [ncbi.nlm.nih.gov]

• 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]

  A diligent and discerning healthcare provider can make an accurate and prompt diagnosis, as Susac’s Syndrome is sometimes confused with inflammation of the arteries in the brain or vasculitis. [health.uconn.edu]

• 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]

  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]

  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]

  Clinical Case SG, a 49-year-old man, presented to the hospital with confusion, dysarthria, expressive aphasia, and progressive gait instability. SG’s past medical history was remarkable only for congenital left hip dysplasia. [the-rheumatologist.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]

  (Friedreich's ataxia, Ataxia telangiectasia, Hereditary spastic paraplegia) Spinal muscular atrophy: Werdnig-Hoffman - Kugelberg-Welander - Fazio Londe - MND (ALS, PMA, PBP, PP, PLS) Extrapyramidal and movement disorders Parkinson's disease - Neuroleptic [wikidoc.org]

  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]

  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]

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

• 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]

  Other neurological symptoms may develop including walking difficulties, slurred speech (dysarthria), and cognitive changes including memory loss, confusion and personality or behavioral changes. [susacssyndromesupportnetwork.wordpress.com]

  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]

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

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]

Management and treatment No evidence-based treatment strategies exist. [orpha.net]

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]

[…] pathophysiology, clinical manifestations, differential diagnoses, classification schema, laboratory investigations, characteristic audiometric findings, high-yield radiographic imaging, temporal bone histopathology, treatment strategies and overall prognosis [pennstate.pure.elsevier.com]

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]

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]

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