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Vaccines Feb 2022Due to the COVID-19 pandemic, numerous vaccines have been developed for the disease. However, with large-scale vaccination has come the gradual emergence of...
Due to the COVID-19 pandemic, numerous vaccines have been developed for the disease. However, with large-scale vaccination has come the gradual emergence of immunological phenomena caused by these new vaccines. Herein, we report a 48-year-old female with a sudden onset of inferior visual field defects in the left eye following her first dose of the ChAdOx1 vaccine. Dilated fundus examination combined with optical coherence tomography and fluorescein angiography confirmed the diagnosis of branch retinal artery occlusion. Within 4 weeks following vaccination, symptoms associated with hearing impairment developed, and magnetic resonance imaging revealed leptomeningeal enhancement. The diagnosis of Susac syndrome (SS) was confirmed. The development of SS may be caused by endotheliopathy resulting from the molecular mimicry of the ChAdOx1 vaccine. Clinicians should be aware of the symptoms of SS, which may develop after COVID-19 vaccination. Further experimental surveillance and case-control studies are required to confirm this relationship.
PubMed: 35334994
DOI: 10.3390/vaccines10030363 -
Lakartidningen Feb 2020Susac syndrome is an autoimmune disease characterized by the clinical triad of encephalopathy, branch retinal artery occlusions and sensorineural hearing loss. It most... (Review)
Review
Susac syndrome is an autoimmune disease characterized by the clinical triad of encephalopathy, branch retinal artery occlusions and sensorineural hearing loss. It most commonly affects young women. Susac syndrome is most likely underdiagnosed, not the least since only 13% have the clinical triad upon presentation. Many are misdiagnosed with multiple sclerosis or another neuroinflammatory entity. Susac syndrome is a microangiopathy affecting the precapillary arterioles causing infarcts of the brain, retina and inner ear. Beside the clinical symptoms, Susac syndrome is diagnosed by typical radiological features on magnetic resonance imaging and branch retinal artery occlusions, which are best evaluated using fluorescein angiography. Early diagnosis and correct immunosuppressive therapy are of utmost importance for clinical improvement and prevention of permanent disability. Diagnosis and treatment of Susac syndrome requires close cooperation between neurologists, radiologists, ophthalmologists and otorhinologists. Here, we present three cases and a review of the literature.
Topics: Brain; Female; Humans; Magnetic Resonance Imaging; Multiple Sclerosis; Retinal Artery Occlusion; Susac Syndrome
PubMed: 32045006
DOI: No ID Found -
Indian Journal of Otolaryngology and... Jun 2022Susac Syndrome (SS) is a rare unique autoimmune underdiagnosed disorder characterized by the clinical triad of central nervous system (CNS) dysfunction, sensorineural...
Susac Syndrome (SS) is a rare unique autoimmune underdiagnosed disorder characterized by the clinical triad of central nervous system (CNS) dysfunction, sensorineural hearing impairment, and branch retinal artery occlusion (BRAO). The purpose of reporting the case is to succinctly highlight this enigmatic condition with focus on otological and audiological aspect of SS and also to augment awareness of this disease among otolaryngologists neurologists, psychiatrists and ophthalmologists. Additionally we reviewed the otological menifestations and audiological findings related to SS. A case of 38 year old female is presented with bilateral SNHL as the initial symptom of SS. The review of otological and audiological findings of previous 10 year case reports was executed using the PRISMA protocol on 20 cases. It was seen that inspite of the ear being one of the main organs involved in the SS very few had undergone detailed audiological assessment. Our review of literature showed that only 40% cases had done audiological assessment other than PTA, out of which only 5% had undergone complete audiological workup. 30% of the patients were having auditory symptoms as presenting chief complaint. Otolyryngologists should keep SS in differentials when a case of SNHL of unknown etiology is encountered.We emphasize the use newer noninvasive Powered by Editorial Manager® and ProduXion Manager® from Aries Systems Corporation diagnostic tools like Optical coherence tomography (OCT), MRI and detailed audiological tests for timely diagnosis of SS. Further multidisciplinary assessment protocols should be used to prevent the development of severe sequelae.
PubMed: 35813770
DOI: 10.1007/s12070-021-02853-w -
Cureus May 2022Susac's syndrome (SS) is a rare, autoimmune-mediated vasculitis involving central nervous system (CNS) microvasculature, which typically targets the brain, retina, and...
Susac's syndrome (SS) is a rare, autoimmune-mediated vasculitis involving central nervous system (CNS) microvasculature, which typically targets the brain, retina, and cochlea. The disease pathology in these regions produces the characteristic triad of encephalopathy, visual loss, and hearing loss. Unfortunately, less than 20% of cases present as the full triad, often making diagnosis challenging. Diagnosis is also confounded by the similarity in the clinical presentation of multiple sclerosis (MS), with an overlap also seen in laboratory studies and radiographic imaging. In this report, we present a case of SS in a young and previously healthy adult male that was initially mistaken as MS. We review the characteristics of SS and highlight the key differences between the two diseases that can be used by diagnosing physicians. Lastly, we describe the treatment strategies involved in SS compared to MS.
PubMed: 35765402
DOI: 10.7759/cureus.25366 -
Journal of Clinical Neurology (Seoul,... Sep 2023
PubMed: 37635426
DOI: 10.3988/jcn.2022.0477 -
BMC Neurology Apr 2022In this case, we reported the pseudobulbar affect (PBA) in a patient with Susac's syndrome-a rare condition that was caused by a rare syndrome. Previous case reports of...
BACKGROUND
In this case, we reported the pseudobulbar affect (PBA) in a patient with Susac's syndrome-a rare condition that was caused by a rare syndrome. Previous case reports of Susac syndrome described psychiatric symptoms such as emotional disturbances or personality changes. Only a few case reports have reported psychiatric disorders in patients with Susac's syndrome. There were no reported cases of Susac syndrome with PBA as an initial presentation.
CASE PRESENTATION
Our patient was 56 years old and presented with involuntary crying, left-sided headache, left-sided hearing loss, and tinnitus. Brain MRI showed numerous areas of restricted diffusion and enhancement involving the corpus callosum, bilateral hemispheres, and brainstem. Ophthalmological evaluation showed bilateral branch retinal artery occlusion. She was diagnosed with Susac's syndrome and PBA. She was treated with cyclophosphamide and dextromethorphan hydrobromide/quinidine sulfate with excellent recovery. This is a 2-year clinical course.
DISCUSSION AND CONCLUSIONS
Recognition of the clinical presentation of Susac's syndrome and PBA with early diagnosis and treatment are the keys to preventing further disability and impact on patients and their families.
Topics: Corpus Callosum; Crying; Female; Humans; Middle Aged; Rare Diseases; Retinal Artery Occlusion; Susac Syndrome
PubMed: 35468771
DOI: 10.1186/s12883-022-02639-9 -
Chinese Medical Journal Jul 2020
Topics: Fluorescein Angiography; Humans; Magnetic Resonance Imaging; Retinal Artery Occlusion; Susac Syndrome
PubMed: 32649514
DOI: 10.1097/CM9.0000000000000909 -
The Pan African Medical Journal 2019The association between deafness and visual disorders is frequent. These disorders range from simple refractive disorder to severe disease that can lead to disability....
The association between deafness and visual disorders is frequent. These disorders range from simple refractive disorder to severe disease that can lead to disability. Hence the interest of early screening. This study aims to highlight the importance of multidisciplinary management and of the need for ophthalmological examination in each deaf child. We conducted a prospective monocentric data collection from medical records of 200 children followed for hypoacousia from January 2014 to January 2015. Each child underwent complete ophthalmological examination, ENT examination and clinical examination. Data from 155 medical records were collected. Ocular involvement was found in 47 patients, reflecting a rate of 30.4%. Bilateral involvement was found in 45 patients. The main syndromic causes were: Usher syndrome (8 casess), Waardenbourg syndrome (5 cases), Alport syndrome (3 cases), Wolfram syndrome (2 cases), Goldenhar syndrome (3 cases), Cogan syndrome (3 cases), Franceschetti-Kleinsyndrome (1 case), Charge syndrome(1 case), otomandibular syndrome (1 case), Stickler syndrome(1 case), Alström syndrome (1 case), Refsum disease (1 case), Susac syndrome (1 case) and KID Syndrome (1 case). Screening for ocular involvement allowed to shorten the average length of cochlear implantation from 9 months to 3 months. There are numerous ocular and auditory involvements because of the embryological and cellular similarities of these two organs, including the retina and the inner ear. The diagnosis of these involvements is facilitated by the presence of facial dysmorphism; on the other hand, diagnosis is difficult when there are visual and auditory sensorineural involvements. Early diagnosis of ocular and auditory involvements allows for best psychomotor development and optimal social inclusion. Therefore multidisciplinary management is necessary to allow for the best psychomotor, orthophonic and visual rehabilitation.
Topics: Adolescent; Child; Child, Preschool; Cochlear Implantation; Deafness; Early Diagnosis; Female; Humans; Infant; Male; Mass Screening; Prospective Studies; Syndrome; Time Factors; Vision Disorders
PubMed: 31565135
DOI: 10.11604/pamj.2019.33.174.17771 -
Journal of Surgical Case Reports Oct 2023Susac syndrome (SS) is a rare microangiopathy affecting the precapillary arterioles of the brain, inner ear, and retina. We present a novel case of SS, presenting as...
Susac syndrome (SS) is a rare microangiopathy affecting the precapillary arterioles of the brain, inner ear, and retina. We present a novel case of SS, presenting as acute incomplete bitemporal field loss in addition to temporally spaced neurological and vestibulocochlear symptoms. A 39-year-old female was referred to the ophthalmology clinic with acute incomplete bitemporal hemianopia and worsening hemicrania. History revealed progressive hearing loss, subjective short-term memory impairment, and vertigo temporally spaced over the preceding 12 months. Magnetic resonance brain revealed multiple small colosal lesions and liner 'spoke' lesions. Fundus fluorescein angiography revealed multiple branch retinal artery occlusions in the right eye. Audiometry confirmed bilateral sensorineural hearing loss. Treatment included intravenous corticosteroids and rituximab. This case highlights the importance of early consideration and evaluation of SS in individuals presenting with atypical ocular disturbances, where no clear cause can be elicited, in order to limit the sequelae of disease.
PubMed: 37867919
DOI: 10.1093/jscr/rjad541 -
Neurology(R) Neuroimmunology &... May 2024
PubMed: 38593388
DOI: 10.1212/NXI.0000000000200247