Fulminant Susac syndrome—a rare cause of coma: The history of the fatal course in a young man

Andour, Hajar; Rostoum, Soufiane; Regragui, Yassine; Fikri, Muhammad; Jiddane, M.; Touarsa, Firdaous

doi:10.1177/2050313x221149826

Cited by 3 publications

(6 citation statements)

References 15 publications

(25 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…One other case of SS in a similarly aged male with previously documented unaided hearing loss is published. He presented with severe headaches, confusion, and visual disturbances; the diagnosis of SS was eventually established by MRI (11). Such cases raise the possibility of a relationship between the underlying etiology of early hearing loss and eventual development of microangiopathy, but firm conclusions cannot be drawn.…”

Section: Discussionmentioning

confidence: 99%

“…The pathophysiology of SS involves oligoclonal expansion of terminally differentiated activated CD8+ T cells, selective adhesion in CNS microvessels, and direct endothelial injury via granzyme B-cell activation. Targeted treatments are an area of ongoing research (11,12). Treatment options for patients with SNHL include cochlear implants (CIs) and hearing aids (13).…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Fatal Presentation of Susac Syndrome in a Congenitally Deaf Patient With a Cochlear Implant

Richter,

Rourk,

Stewart

et al. 2024

Otol Neurotol

View full text Add to dashboard Cite

Objective To report a fatal case of Susac syndrome in a congenitally deaf patient with a cochlear implant and a history of migraines, emphasizing the diagnostic challenges in patients with preexisting conditions. Patient A 33-year-old male with congenital hearing loss, a cochlear implant, and chronic migraines who presented with mild subacute auditory disturbance and headaches that later progressed to severe encephalopathy. Intervention Explantation of a non–magnetic resonance imaging (MRI) compatible cochlear implant followed by MRI, fundoscopy, and the administration of immunosuppressive medications. Main Outcome Measures Diagnosis was confirmed by characteristic MRI appearance and the presence of a hemi-retinal artery occlusion. Results After weeks of immunosuppressive treatment, the patient died of a global cerebral ischemic event of unknown origin. Conclusions For patients with preexisting sensorineural hearing loss and cochlear implants, Susac syndrome poses a diagnostic challenge. Auditory disturbances in the absence of cochlear implant failure should prompt further evaluation for visual disturbances and encephalopathy. MRI and fundoscopy should be performed to detect other features of the disease.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Fatal Presentation of Susac Syndrome in a Congenitally Deaf Patient With a Cochlear Implant

Richter,

Rourk,

Stewart

et al. 2024

Otol Neurotol

View full text Add to dashboard Cite

show abstract

“…In mild cases, peak disease severity lasts only for several months, and the dysfunction is reversible. On the other hand, prolonged course over several years with devastating long-term or even fatal consequences has been reported as well (1,8,10,11).…”

Section: Introductionmentioning

confidence: 99%

Case report: Susac syndrome—two ends of the spectrum, single center case reports and review of the literature

Cviková,

Štefela,

Všianský

et al. 2024

Front. Neurol.

View full text Add to dashboard Cite

Susac syndrome is a rare and enigmatic complex neurological disorder primarily affecting small blood vessels in the brain, retina, and inner ear. Diagnosing Susac syndrome may be extremely challenging not only due to its rarity, but also due to the variability of its clinical presentation. This paper describes two vastly different cases—one with mild symptoms and good response to therapy, the other with severe, complicated course, relapses and long-term sequelae despite multiple therapeutic interventions. Building upon the available guidelines, we highlight the utility of black blood MRI in this disease and provide a comprehensive review of available clinical experience in clinical presentation, diagnosis and therapy of this disease. Despite its rarity, the awareness of Susac syndrome may be of uttermost importance since it ultimately is a treatable condition. If diagnosed in a timely manner, early intervention can substantially improve the outcomes of our patients.

show abstract

“…There have been only few fatal cases described in the literature, none of which have been associated with diffuse cerebral edema or herniation. [6][7][8][9] Case Report A 33-year-old man with a pertinent past medical history of congenital sensorineural deafness status post left cochlear implant placement at the age of 3 with a strong family history of deafness in both the patient's sister and maternal aunt, though without genetic workup, presented to the Mayo Clinic Florida (MCF) as a transfer from outside facility (OSH) for acute onset encephalopathy of unknown etiology. Prior to presentation, the patient lived independently, worked at the grocery store and was able to communicate with speech and sign language.…”

Section: Introductionmentioning

confidence: 99%

“…There have been only few fatal cases described in the literature, none of which have been associated with diffuse cerebral edema or herniation. 6–9…”

Section: Introductionmentioning

confidence: 99%

Diffuse Cerebral Edema With Uncal Herniation Leading to Brain Death in Patient With Fulminant Susac syndrome: Rare Complication of Rare Disease

Tsikvadze,

Machado De Villafane,

Rose

et al. 2023

The Neurohospitalist

View full text Add to dashboard Cite

Susac Syndrome was first described as an inflammatory microangiopathy of the brain and retina. Since then, multiple articles have been published in attempts to improve the understanding of this rare disease. Clinically Susac Syndrome is known to present with triad of encephalopathy, sensorineural hearing loss and branch of retinal artery occlusion (BRAO), along with characteristic “snowball” or “spoke” appearing white matter lesions of the corpus callosum. It has been characterized by vast heterogeneity in terms of its presenting symptoms, severity, and clinical course. Although subset of patients present with severe forms of Susac Syndrome and can develop prominent residual neurologic deficits, it has been reported to be mostly non-life-threatening and only few fatal cases have been described in the literature. Based on the available case reports with fatal outcome, mortality has been related to the systemic complications either during acute disease flare or during chronic-progressive phase. We describe a case of fulminant Susac Syndrome complicated by the sudden and rapid progression of diffuse cerebral edema leading to brain herniation and ultimate brain death, in order to increase awareness of this rare and catastrophic complication.

show abstract

Fulminant Susac syndrome—a rare cause of coma: The history of the fatal course in a young man

Cited by 3 publications

References 15 publications

Fatal Presentation of Susac Syndrome in a Congenitally Deaf Patient With a Cochlear Implant

Fatal Presentation of Susac Syndrome in a Congenitally Deaf Patient With a Cochlear Implant

Case report: Susac syndrome—two ends of the spectrum, single center case reports and review of the literature

Diffuse Cerebral Edema With Uncal Herniation Leading to Brain Death in Patient With Fulminant Susac syndrome: Rare Complication of Rare Disease

Contact Info

Product

Resources

About