Contributor:
Krizia A Vinck, M.A. – William James College, Clinical Psychology Psy.D Program (with Neuropsychology and Latino Mental Health Emphasis)
Superficial siderosis (SS) is a rare condition, which results from hemosiderin deposition in the subpial layers of the brain and spinal cord, with eventual neurological dysfunction (Kumar, 2007). Patients with SS often present after the age of 40 with slowly progressive gait ataxia and an associated sensorineural hearing impairment (Fearnley, Stevens, & Rudge, 1995; Kumar, Cohen-Gadol, Wright, Miller, Piepgras, Ahlskog, 2006). While the clinical population varies, the most typical symptoms include cerebellar (ataxic) dysarthria and nystagmus (Anderson, Sheffield, & Hope, 1999). Pyramidal and sensory signs and bladder dysfunction may relate to brainstem or spinal cord involvement; however, other than hearing impairment, sensory symptoms are rarely present (Cohen-Gadol, Krauss, & Spinner, 2004). A distinct pattern of cognitive and social impairment has been observed in patients diagnosed with SS (Harskamp, Rudge, & Cipolotti, 2005), including impaired speech production, visual recall memory and executive functions. Social dysfunction, affecting the ability to represent other people's mental states (i.e., theory of mind), has also been reported. These behavioral dysfunctions are observed primarily in the context of MRI-documented lesions maximally involving the cerebellum, in particular, the superior vermis, as well as the medial and inferior frontal cortex (Harskamp, Rudge, & Cipolotti, 2005).
Patients often have a history of prior intradural surgery or trauma and can suffer subarachnoid hemorrhage before or after SS develops. Severe headaches were indicative of symptomatic subarachnoid hemorrhage in approximately one-third of the cases in one study (Fearnley, Stevens, & Rudge, 1995); however, this was relatively less frequently observed in a larger study (Kumar at al., 2006). The widespread use of MRIs is allowing presymptomatic cases to be diagnosed. Nevertheless, despite extensive investigation the cause of bleeding is often not apparent (Kumar, 2007). An early diagnosis and prompt intervention directed at removal of the bleeding source may be key in preventing the progression of SS.
Abstract
Lummel, N., Wollenweber, F. A., Demaerel, P., Bochmann, K., Malik, R., Opherk, C., & Linn, C. (2015). Clinical spectrum, underlying etiologies and radiological characteristics of cortical superficial siderosis. Journal of Neurology, (262), 1455–1462.http://doi.org/10.1007/s00415-015-7736-1
Cortical superficial siderosis (cSS) is an increasingly recognized MR-imaging marker most probably caused by focal convexity subarachnoid hemorrhage (SAH). There is accumulating evidence that cSS represents an important risk factor for subsequent intracranial hemorrhages. Here, we aimed to determine clinical symptoms, underlying etiologies, and radiological characteristics of cSS in a large patient cohort. We performed an electronic database search on all patients who presented between 2002 and 2013 to the university hospital Munich with non-traumatic and non-aneurysmal cSS. T2*-weighted gradient-echo sequences were analyzed regarding localization and extent of cSS as well as of acute SAH, intracerebral hemorrhages (ICH) and microbleeds. Besides, all available clinical, laboratory, imaging and histological data were analyzed. 113 subjects matched the inclusion criteria. The following etiologies for cSS were identified: definite (n=6; 5 %), probable (n=75; 66 %), and possible (n=28; 25 %) cerebral amyloid angiopathy (CAA); reversible cerebral vasoconstriction syndrome: 2 (2 %); central nervous system vasculitis: 1; and hyperperfusion syndrome: 1. Acute ICH was evident in 55 (49 %) cases. Other clinical manifestations were: transient focal neurological episodes (TFNE): 38 (34 %); cognitive impairment: 14 (12 %); generalized seizure: 4 (4 %); and headache: 2 (2 %). Adjusting for age and gender, cognitive impairment was more frequent in disseminated cSS, while TFNE was more often found in focal cSS (p=0.042). Our data indicate CAA to be the most common etiology of cSS. In absence of symptomatic ICH, patients with focal cSS frequently present with TFNE, while those with disseminated cSS commonly manifest with cognitive impairment.
Podcasts
Neurology® Podcast - February 25 2014 Issue. Dr. James Addington, MD is reading the e-Pearl of the week about superficial siderosis.
https://www.aan.com/rss/search/home/episodedetail/?item=2734
Webinar
“Superficial Siderosis Webinar” is an informational webinar presented by Dr. Michael Levy, Assistant Professor of Neurology at Johns Hopkins Hospital Department of Neurology in Baltimore, MD.
https://vimeo.com/135640331
References/Further Reading
Anderson, N. E., Sheffield, S., & Hope, J. K. A. (1999). Superficial siderosis of the central nervous system A late complication of cerebellar tumors. Neurology, 52(1), 163–163.http://doi.org/10.1212/WNL.52.1.163
Cohen-Gadol, A. A., Krauss, W. E., & Spinner, R. J. (2004). Delayed central nervous system superficial siderosis following brachial plexus avulsion injury. Neurosurgical Focus, 16(5), 1–6.http://doi.org/10.3171/foc.2004.16.5.11
Fearnley, J. M., Stevens, J. M., & Rudge, P. (1995). REVIEW ARTICLE: Superficial siderosis of the central nervous system.Brain, 118(4), 1051–1066. http://doi.org/10.1093/brain/118.4.1051
Kumar, N., Cohen-Gadol, A. A., Wright, R. A., Miller, G. M., Piepgras, D. G., & Ahlskog, J. E. (2006). Superficial siderosis.Neurology, 66(8), 1144–1152. http://doi.org/10.1212/01.wnl.0000208510.76323.5b
Kumar N. (2007). Superficial siderosis: Associations and therapeutic implications. Archives of Neurology, 64(4), 491–496.http://doi.org/10.1001/archneur.64.4.491
Levy, M., Turtzo, C., & Llinas, R. H. (2007). Superficial siderosis: a case report and review of the literature. Nature Clinical Practice Neurology, 3(1), 54–58. http://doi.org/10.1038/ncpneuro0356
Lummel, N., Wollenweber, F. A., Demaerel, P., Bochmann, K., Malik, R., Opherk, C., & Linn, C. (2015). Clinical spectrum, underlying etiologies and radiological characteristics of cortical superficial siderosis. Journal of Neurology, (262), 1455–1462.http://doi.org/10.1007/s00415-015-7736-1
Tari-Capone, F., Bozzao, A., Sette, G., Delfini, R., & Antonini, G. (2013). Superficial siderosis of central nervous system in patients with brachial plexus injury. Neurological Sciences,34(10), 1861-1865. http://doi.org/10.1007/s10072-013-1336-6
Wollenweber, F. A., Buerger, K., Mueller, C., Ertl-Wagner, B., Malik, R., Dichgans, M., & ... Opherk, C. (2014). Prevalence of cortical superficial siderosis in patients with cognitive impairment. Journal Of Neurology, 261(2), 277-282.http://doi.org/10.1007/s00415-013-7181-y
Zonneveld, H. I., Goos, J. D. C., Wattjes, M. P., Prins, N. D., Scheltens, P., van der Flier, W. M., … Barkhof, F. (2014). Prevalence of cortical superficial siderosis in a memory clinic population. Neurology, 82(8), 698–704.http://doi.org/10.1212/WNL.0000000000000150
Patients often have a history of prior intradural surgery or trauma and can suffer subarachnoid hemorrhage before or after SS develops. Severe headaches were indicative of symptomatic subarachnoid hemorrhage in approximately one-third of the cases in one study (Fearnley, Stevens, & Rudge, 1995); however, this was relatively less frequently observed in a larger study (Kumar at al., 2006). The widespread use of MRIs is allowing presymptomatic cases to be diagnosed. Nevertheless, despite extensive investigation the cause of bleeding is often not apparent (Kumar, 2007). An early diagnosis and prompt intervention directed at removal of the bleeding source may be key in preventing the progression of SS.
Abstract
Lummel, N., Wollenweber, F. A., Demaerel, P., Bochmann, K., Malik, R., Opherk, C., & Linn, C. (2015). Clinical spectrum, underlying etiologies and radiological characteristics of cortical superficial siderosis. Journal of Neurology, (262), 1455–1462.http://doi.org/10.1007/s00415-015-7736-1
Cortical superficial siderosis (cSS) is an increasingly recognized MR-imaging marker most probably caused by focal convexity subarachnoid hemorrhage (SAH). There is accumulating evidence that cSS represents an important risk factor for subsequent intracranial hemorrhages. Here, we aimed to determine clinical symptoms, underlying etiologies, and radiological characteristics of cSS in a large patient cohort. We performed an electronic database search on all patients who presented between 2002 and 2013 to the university hospital Munich with non-traumatic and non-aneurysmal cSS. T2*-weighted gradient-echo sequences were analyzed regarding localization and extent of cSS as well as of acute SAH, intracerebral hemorrhages (ICH) and microbleeds. Besides, all available clinical, laboratory, imaging and histological data were analyzed. 113 subjects matched the inclusion criteria. The following etiologies for cSS were identified: definite (n=6; 5 %), probable (n=75; 66 %), and possible (n=28; 25 %) cerebral amyloid angiopathy (CAA); reversible cerebral vasoconstriction syndrome: 2 (2 %); central nervous system vasculitis: 1; and hyperperfusion syndrome: 1. Acute ICH was evident in 55 (49 %) cases. Other clinical manifestations were: transient focal neurological episodes (TFNE): 38 (34 %); cognitive impairment: 14 (12 %); generalized seizure: 4 (4 %); and headache: 2 (2 %). Adjusting for age and gender, cognitive impairment was more frequent in disseminated cSS, while TFNE was more often found in focal cSS (p=0.042). Our data indicate CAA to be the most common etiology of cSS. In absence of symptomatic ICH, patients with focal cSS frequently present with TFNE, while those with disseminated cSS commonly manifest with cognitive impairment.
Podcasts
Neurology® Podcast - February 25 2014 Issue. Dr. James Addington, MD is reading the e-Pearl of the week about superficial siderosis.
https://www.aan.com/rss/search/home/episodedetail/?item=2734
Webinar
“Superficial Siderosis Webinar” is an informational webinar presented by Dr. Michael Levy, Assistant Professor of Neurology at Johns Hopkins Hospital Department of Neurology in Baltimore, MD.
https://vimeo.com/135640331
References/Further Reading
Anderson, N. E., Sheffield, S., & Hope, J. K. A. (1999). Superficial siderosis of the central nervous system A late complication of cerebellar tumors. Neurology, 52(1), 163–163.http://doi.org/10.1212/WNL.52.1.163
Cohen-Gadol, A. A., Krauss, W. E., & Spinner, R. J. (2004). Delayed central nervous system superficial siderosis following brachial plexus avulsion injury. Neurosurgical Focus, 16(5), 1–6.http://doi.org/10.3171/foc.2004.16.5.11
Fearnley, J. M., Stevens, J. M., & Rudge, P. (1995). REVIEW ARTICLE: Superficial siderosis of the central nervous system.Brain, 118(4), 1051–1066. http://doi.org/10.1093/brain/118.4.1051
Kumar, N., Cohen-Gadol, A. A., Wright, R. A., Miller, G. M., Piepgras, D. G., & Ahlskog, J. E. (2006). Superficial siderosis.Neurology, 66(8), 1144–1152. http://doi.org/10.1212/01.wnl.0000208510.76323.5b
Kumar N. (2007). Superficial siderosis: Associations and therapeutic implications. Archives of Neurology, 64(4), 491–496.http://doi.org/10.1001/archneur.64.4.491
Levy, M., Turtzo, C., & Llinas, R. H. (2007). Superficial siderosis: a case report and review of the literature. Nature Clinical Practice Neurology, 3(1), 54–58. http://doi.org/10.1038/ncpneuro0356
Lummel, N., Wollenweber, F. A., Demaerel, P., Bochmann, K., Malik, R., Opherk, C., & Linn, C. (2015). Clinical spectrum, underlying etiologies and radiological characteristics of cortical superficial siderosis. Journal of Neurology, (262), 1455–1462.http://doi.org/10.1007/s00415-015-7736-1
Tari-Capone, F., Bozzao, A., Sette, G., Delfini, R., & Antonini, G. (2013). Superficial siderosis of central nervous system in patients with brachial plexus injury. Neurological Sciences,34(10), 1861-1865. http://doi.org/10.1007/s10072-013-1336-6
Wollenweber, F. A., Buerger, K., Mueller, C., Ertl-Wagner, B., Malik, R., Dichgans, M., & ... Opherk, C. (2014). Prevalence of cortical superficial siderosis in patients with cognitive impairment. Journal Of Neurology, 261(2), 277-282.http://doi.org/10.1007/s00415-013-7181-y
Zonneveld, H. I., Goos, J. D. C., Wattjes, M. P., Prins, N. D., Scheltens, P., van der Flier, W. M., … Barkhof, F. (2014). Prevalence of cortical superficial siderosis in a memory clinic population. Neurology, 82(8), 698–704.http://doi.org/10.1212/WNL.0000000000000150
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