Clinical and imaging features distinguishing Susac syndrome from primary angiitis of the central nervous system

Marrodán, Mariano; Acosta, Julián; Alessandro, Lucas; Fernández, Victoria; Contentti, Edgar Carnero; Arakaki, Naomí; Kohler, Alejandro; Fiol, Marcela; Ameriso, Sebastián F.; Correale, Jorge

doi:10.1016/j.jns.2018.09.029

Cited by 16 publications

(9 citation statements)

References 35 publications

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“…Similarly, several observational reports of MIL-like syndrome after cocaine use failed to reveal the presence of levamisole [9][10][11]. In turn, recent case reports have documented the presence of levamisole in patients with MIL or related forms of the disorder with cocaine use [4,12,13]. As in a previously reported case, the patient presented a rapidly progressive leukoencephalopathy with clinical and radiological signs consistent with SS, including numerous inflammatory white matter foci with synchronous contrast-enhancement and decreased ADC value prominently involving the corpus callosum.…”

Section: Discussionmentioning

confidence: 99%

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Acute Susac Syndrome in a Recent User of Adulterated Cocaine: Levamisole as a Triggering Factor?

et al. 2020

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Susac syndrome (SS) is a central nervous system vasculitis characterized by the clinical triad of encephalopathy, sensorineural hearing loss, and visual disturbance caused by branch retinal artery occlusion. It is considered as an inflammatory disorder, and an autoimmune etiology is suggested. A 29-year-old man with a history of recent cocaine abuse developed the clinical features of SS. Toxicological analysis including hair testing revealed that cocaine had been adulterated with levamisole. After an initial clinical improvement following corticosteroid therapy, the introduction of mycophenolate mofetil was justified a few weeks later by the progression (or relapse) of the retinal injury, followed by complete recovery. The presence of levamisole has been documented in patients with multifocal inflammatory leukoencephalopathy (MIL). Further investigations are needed to determine if levamisole as an adulterant of cocaine could also play a role in the development of rapidly progressive Case

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Section: Discussionmentioning

confidence: 99%

“…On the other hand, exposure to levamisole in monotherapy has been associated with multifocal inflammatory leukoencephalopathy (MIL). The lesions are then prominent in the centrum ovale, per-lateral ventricles, and basal ganglia bilaterally [4]. These lesions appear distinct from those of cocaine-related leukoencephalopathy.…”

Section: Introductionmentioning

confidence: 91%

Acute Susac Syndrome in a Recent User of Adulterated Cocaine: Levamisole as a Triggering Factor?

et al. 2020

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“…Distinguishing SuS from PACNS is challenging when the early manifestations are hemiparesis and epilepsy. Increased intracranial pressure and CSF protein level and lesions with restricted diffusion on DWI can be observed in both PACNS and SuS ( 5 ). Seizures seem more common in patients with PACNS than in patients with SuS (8/15 vs. 1/13, p = 0.035) according to a multicenter study ( 5 ).…”

Section: Discussionmentioning

confidence: 99%

Misdiagnosis of Susac syndrome as demyelinating disease and primary angiitis of the central nervous system: A case report

Jiang

et al. 2022

Front. Neurol.

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Susac syndrome (SuS) is a rare neuroinflammatory disease that manifests with a triad of hearing loss, branch retinal artery occlusions, and encephalopathy. Patients with SuS are frequently misdiagnosed because the clinical trial is incompletely present at disease onset. In this report, we present a case of a 29-year-old man manifesting sleepiness, epilepsy, urinary dysfunction, and hemiparesis at the initial stage. Magnetic resonance imaging (MRI) revealed multiple abnormal signals located in the lateral paraventricular, corpus callosal, and pons. In addition, the patient had sustained elevation of CSF pressure and protein. ADEM was considered according to the clinical and radiographic findings. However, symptoms were not significantly improved after methylprednisolone therapy. He showed a vision decline in the third month after the disease onset. It was considered from intracranial hypertension or optic neuritis, and therefore retinal arteriolar impairment was ignored. As the disease progresses, cognitive decline was presented. Brain MRI exhibits multiple significant hyperintensities on the DWI sequence with speck-like gadolinium enhancement. Thus, PACNS was diagnosed. The SuS was not made until the presence of hearing decline in the 4 months after the disease onset. The case will be helpful for clinicians to better recognize the atypical initial manifestation of SuS.

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“…In fact, ataxia is one of the clinical findings described in patients with SuS. It was reported in seven cases among 13 patients in a review of literature [3,5,6]. Spinal cord involvement is extremely rare [8,9].…”

Section: Case Reportmentioning

confidence: 99%

Unusual flare up of Susac syndrome

et al. 2022

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Introduction. Spinal cord involvement as a flare-up of Susac syndrome (SuS) is rarely reported in the literature. The pathogenesis of SuS includes multiple occlusions in micro vessels mediated by an autoimmune response to unknown antigens. This condition is characterized by a triad: central nervous system (CNS) dysfunction with a frequent involvement of the corpus callosum in brain MRI, visual disturbances due to branch retinal artery occlusion, and sensorineural hearing impairment. Case report. A 50-year-old man presented in December 2019 symptoms associating gait instability, diplopia and hypoacusis. A year later, he developed a vesical-sphincter disorder and a hypoesthesia in his right lower limb. Neurological examination identified a medullary syndrome, cerebellar ataxia and hypoacusis. We performed a brain and spinal MRI showing supratentorial and infratentorial white matter lesions, and gadolinium enhancing cervical lesions. Lumbar puncture with isoelectric focusing results was normal. Screening for differential diagnosis such as other inflammatory central nervous system diseases other than multiple sclerosis was performed considering this atypical presentation of the disease; workup of serological, immunological, angiotensin converting enzyme, and tumor markers was negative. A bilateral hearing deficit was confirmed with an audiogram. Retinal angiography showed ischemic retinal vasculitis. We diagnosed the patient with SuS and treated him with intravenous corticosteroids in transition to oral corticosteroids. We noticed a partial regression of his symptoms. Therefore, we highlight the importance of an early diagnosis for SuS to avoid a prognostic turnover of the disease. Spinal cord involvement should not rule out the diagnosis of SuS.

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Clinical and imaging features distinguishing Susac syndrome from primary angiitis of the central nervous system

Cited by 16 publications

References 35 publications

Acute Susac Syndrome in a Recent User of Adulterated Cocaine: Levamisole as a Triggering Factor?

Acute Susac Syndrome in a Recent User of Adulterated Cocaine: Levamisole as a Triggering Factor?

Misdiagnosis of Susac syndrome as demyelinating disease and primary angiitis of the central nervous system: A case report

Unusual flare up of Susac syndrome

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