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What is Susac syndrome? - A brief review of articles.

Nazari F, Azimi A, Abdi S - Iran J Neurol (2014)

Bottom Line: Susac's syndrome (SS) is a clinical triad of encephalopathy, branch retinal artery occlusion and sensorineural hearing loss and maybe due to an immune-mediated endotheliopathy.Because of its rarity and some similarities to other common neurological conditions such as multiple sclerosis and acute disseminated encephalomyelitis, it is often misdiagnosed and therefore mistreated.To the best of our knowledge, there is only one case report from our country with this diagnosis.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, School of Medicine, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran.

ABSTRACT
Susac's syndrome (SS) is a clinical triad of encephalopathy, branch retinal artery occlusion and sensorineural hearing loss and maybe due to an immune-mediated endotheliopathy. Because of its rarity and some similarities to other common neurological conditions such as multiple sclerosis and acute disseminated encephalomyelitis, it is often misdiagnosed and therefore mistreated. To the best of our knowledge, there is only one case report from our country with this diagnosis. Here, we have a short discussion on this issue to introduce it to our colleagues and remind it as a differential diagnosis in patients with unexplained encephalopathy.

No MeSH data available.


Related in: MedlinePlus

Periventricular and deep white matter involvement of the brain in Susac syndrome (A). Sagittal T2 showing multiple large central callosal “snowballs” (B) (courtesy of Dr. Sahraian MA)
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Figure 1: Periventricular and deep white matter involvement of the brain in Susac syndrome (A). Sagittal T2 showing multiple large central callosal “snowballs” (B) (courtesy of Dr. Sahraian MA)

Mentions: As mentioned earlier, one of the most common presentations of SS is microinfarctions in the brain that involves both white and grey matter.15 These microinfarctions can cause T2 hyperintense lesions at any area of the brain, including periventricular and juxtacortical areas, subcortical white matter (Figure 1A), cerebellum and corpus callosum;15,16 but microinfarctions in SS can also produce two specific findings on MRI: “snowball lesions” that represent microinfarctions of the central part of the corpus callosum, best seen on sagittal T2 and Fluid-attenuated inversion recovery (FLAIR) views (Figure 1B); and “string of pearls” that represents micro-infarctions of the internal capsule, best seen on diffusion weighted image sequences.17,18 Rennebohm et al. believe that the combination of typical central callosal lesions with string of pearls is unequivocally pathognomonic for SS.17 Corpus callosum involvement is always seen in encephalopathic form of SS; snowball configuration of corpus callosum lesions in acute phase of encephalopathy ultimately evolves to central callosal holes, best seen on sagittal T1 sequence views.17 In post-encephalopathic stage, MRI shows these near-pathognomonic central callosal holes and linear defects in corpus callosum called “smokes.”18,23 Another MRI characteristics of SS is leptomeningeal enhancement, seen in 30% of patients during the acute phase; in some case reports, facial and vestibule-cochlear nerve enhancement and cochlear enhancement have been reported.8,18,24 Egan et al. believe that the imaging triad of the corpus callosum involvement, deep grey matter lesions and leptomeningeal enhancement, in an encephalopathic patient may help in early diagnosis of SS and warrant early, aggressive, treatment.19 After the acute phase, generalized atrophy of the cerebrum, cerebellum and corpus callosum can be seen in severely affected individuals.10,11 Mateen et al. reported a series of 29 cases of SS with a corpus callosum involvement in 79%, punctate T2-weighted hyperintense lesions in 93%, and gadolinium enhancing lesions in 50%, of their patients.8


What is Susac syndrome? - A brief review of articles.

Nazari F, Azimi A, Abdi S - Iran J Neurol (2014)

Periventricular and deep white matter involvement of the brain in Susac syndrome (A). Sagittal T2 showing multiple large central callosal “snowballs” (B) (courtesy of Dr. Sahraian MA)
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4300795&req=5

Figure 1: Periventricular and deep white matter involvement of the brain in Susac syndrome (A). Sagittal T2 showing multiple large central callosal “snowballs” (B) (courtesy of Dr. Sahraian MA)
Mentions: As mentioned earlier, one of the most common presentations of SS is microinfarctions in the brain that involves both white and grey matter.15 These microinfarctions can cause T2 hyperintense lesions at any area of the brain, including periventricular and juxtacortical areas, subcortical white matter (Figure 1A), cerebellum and corpus callosum;15,16 but microinfarctions in SS can also produce two specific findings on MRI: “snowball lesions” that represent microinfarctions of the central part of the corpus callosum, best seen on sagittal T2 and Fluid-attenuated inversion recovery (FLAIR) views (Figure 1B); and “string of pearls” that represents micro-infarctions of the internal capsule, best seen on diffusion weighted image sequences.17,18 Rennebohm et al. believe that the combination of typical central callosal lesions with string of pearls is unequivocally pathognomonic for SS.17 Corpus callosum involvement is always seen in encephalopathic form of SS; snowball configuration of corpus callosum lesions in acute phase of encephalopathy ultimately evolves to central callosal holes, best seen on sagittal T1 sequence views.17 In post-encephalopathic stage, MRI shows these near-pathognomonic central callosal holes and linear defects in corpus callosum called “smokes.”18,23 Another MRI characteristics of SS is leptomeningeal enhancement, seen in 30% of patients during the acute phase; in some case reports, facial and vestibule-cochlear nerve enhancement and cochlear enhancement have been reported.8,18,24 Egan et al. believe that the imaging triad of the corpus callosum involvement, deep grey matter lesions and leptomeningeal enhancement, in an encephalopathic patient may help in early diagnosis of SS and warrant early, aggressive, treatment.19 After the acute phase, generalized atrophy of the cerebrum, cerebellum and corpus callosum can be seen in severely affected individuals.10,11 Mateen et al. reported a series of 29 cases of SS with a corpus callosum involvement in 79%, punctate T2-weighted hyperintense lesions in 93%, and gadolinium enhancing lesions in 50%, of their patients.8

Bottom Line: Susac's syndrome (SS) is a clinical triad of encephalopathy, branch retinal artery occlusion and sensorineural hearing loss and maybe due to an immune-mediated endotheliopathy.Because of its rarity and some similarities to other common neurological conditions such as multiple sclerosis and acute disseminated encephalomyelitis, it is often misdiagnosed and therefore mistreated.To the best of our knowledge, there is only one case report from our country with this diagnosis.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, School of Medicine, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran.

ABSTRACT
Susac's syndrome (SS) is a clinical triad of encephalopathy, branch retinal artery occlusion and sensorineural hearing loss and maybe due to an immune-mediated endotheliopathy. Because of its rarity and some similarities to other common neurological conditions such as multiple sclerosis and acute disseminated encephalomyelitis, it is often misdiagnosed and therefore mistreated. To the best of our knowledge, there is only one case report from our country with this diagnosis. Here, we have a short discussion on this issue to introduce it to our colleagues and remind it as a differential diagnosis in patients with unexplained encephalopathy.

No MeSH data available.


Related in: MedlinePlus