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]

  Case Reports Case 1 A 36-year-old woman presented in January 1996 with vertigo, ataxia, vomiting, visual impairment of the left eye, hearing loss in the left ear, and weakness on the left side of her body. Routine laboratory studies were normal. [ajnr.org]

  2016 Staelens C,Audenaert K,Tandt H,Van Driessche V,Lemmens G, diagnosis,epidemiology,diagnostic imaging,diagnosis,epidemiology, Visual impairment. [allmedx.com]

• Photopsia

  BRAO also can cause photopsia, black spots, and scintillating scotomas. [2] A good clue for the rapid diagnosis of SS in our patient was Gass plaques. [ijo.in]

  Case 3 A 39-year-old woman presented to the emergency ward of another hospital in August 2012 with vertigo, gait ataxia, headaches with photopsia, nausea and vomiting. [njmonline.nl]

  A 39-year-old male patient presented with severe headache, photopsias, and a sudden onset of hearing loss in the right ear. Fluorescein angiography of the right eye revealed multiple branch retinal artery occlusions. [allmedx.com]

  Involvement of the posterior pole of the retina leads to profound blurred vision, but peripheral involvement can cause no symptom.4,18 BRAO also can cause photopsia, black spots, and scintillating scotomas.10 Therefore, detailed fundoscopic examination [ncbi.nlm.nih.gov]

  Open table in a new tab Visual disturbances were described as blurring, partial visual field deficits or blind spots, and flashing lights (photopsias). Inner ear dysfunction manifested as tinnitus and hearing loss in all patients. [jns-journal.com]

• 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]

  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]

  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]

  impairment, and branch retinal artery occlusion (BRAO). [insights.ovid.com]

• 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]

• Psychiatric Manifestation

  With respect to therapeutic intervention, the main clinical outcome measures were return of vision, improvement of neurologic and psychiatric manifestations, and recovery of auditory function. [ncbi.nlm.nih.gov]

  Main outcome measures With respect to therapeutic intervention, the main clinical outcome measures were return of vision, improvement of neurologic and psychiatric manifestations, and recovery of auditory function. [aaojournal.org]

• 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

  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]

  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]

  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]

  Brain symptoms: Severe headache, often with vomiting Problems with thinking, such as short-term memory loss, confusion, slow thought processing, and reduced ability to solve problems Inability to remain alert or focused Slurred speech Changes in personality [my.clevelandclinic.org]

• 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]

  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]

  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]

• 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]

  It is characterized by three main symptoms: encephalopathy (which can include headache, mild memory losses, personality changes, and confusion); sensorineural hearing loss; and vision loss. [susacssyndromesupportnetwork.wordpress.com]

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

  Public Health Relevance Susac's syndrome is a disorder characterized by visual defects, hearing loss, and a variety of brain dysfunctions (headaches, memory loss, personality changes, dementia). [grantome.com]

• 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]

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

  (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]

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]

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]

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]

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]