Letters to the Editor / Clinical Neurology and Neurosurgery 121 (2014) 76–78
Dale Ding ∗ University of Virginia, Department of Neurosurgery, Charlottesville 22908, USA ∗ Correspondence to: University of Virginia, Department of Neurosurgery, P.O. Box 800212, Charlottesville 22908, USA. Tel.: +1 434 924 2203; fax: +1 434 982 5753. E-mail address: [email protected]
Available online 1 March 2014 http://dx.doi.org/10.1016/j.clineuro.2014.01.028
Superficial siderosis of the central nervous system presenting with dysuria initially To the Editor, Superficial siderosis of the central nervous system (SSCNS) is a rare disorder characterized by a triad of symptoms consisting of hypacusia, cerebellar ataxia, and myelopathy. Kumar et al. has also reported its less frequent manifestation of headache, dementia [1]. We treated a 50-year-old male patient with an initial presentation of dysuria. He developed the symptom of dysuria 3 years ago and has hearing loss for 2 years and ataxia for 1 year. Routine blood tests, biochemistry, hepatic and renal function, and trace elements were normal. Brain and spinal MRI showed (Fig. 1) cord-like abnormal signal on the surface of bilateral temporal lobe, corpus callosum, cerebellar hemispheres, brain stem and spinal cord; broadening of the sulcus of cerebellum; L2–3, L4–5 disk bulge; L3–4, L5–S1 disk
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herniation. He was given vitamin B1, vitamin B12, lipoic acid for two weeks after admission, but with no evident improvements. Hypacusia and cerebellar ataxia are typically common symptoms in SSCNS. Additionally, anosmia, vision impairment, ageusia, headaches, bladder dysfunction, spinal cord disease, seizures, and cognitive dysfunction have also been reported in previous literatures [2,3]. However, symptoms of SSCNS often gradually manifest a few years later following the appearance of the disease. This patient had an initial complaint of dysuria, the reasonable cause for which could be an early involvement of spinal cord. Urinary tract ultrasonography shows slight enlargement and calculi formation of the prostatic portion with accompanying symptom of nocturia; which are mainly because of spinal cord involvement, but the possibility of an enlarged prostate contributing to dysuria could not be entirely ruled out. Other symptoms of our patient, such as hearing loss, tinnitus and cerebellar ataxia, are consistent with previous reports. MRI images present typical pathologic findings of deposition of iron beneath the surfaces of CNS tissues. Cerebellar atrophy is evident. MRI imaging of this case showed a typical SSCNS features consistent with literatures which report the widening of cerebellar sulci. Untreated SSCNS often has poor prognosis. Surgical removal of hemorrhage may be an effective way to treat SSCNS [4,5], applying to nerve root lesion tear, arteriovenous malformations, dural rupture. The efficacy of iron chelator is still uncertain [2]. Levy [2] has reported one case treated with iron chelator, hemosiderin reducing in brainstem and cerebellar vermis. However, there have been few failures too, which might be due to its difficulty in passing through the blood–brain barrier. In this case, rehabilitation treatment could be beneficial to some extent in order to improve the syndrome which may attribute to the above reason.
Fig. 1. (A) T1-weighted imaging: slightly higher signal on the surface of pons. (B) T2-weighted imaging: hypointensive-rim on the surface of pons. (C) T2 FLIAR imaging: slightly high signal or isointensity on the surface of pons. (D) Sagittal T2 weighted imaging: low signal on the surface of the corpus callosum, spinal cord thinning. (E) Sagittal T2-weighted imaging: the spinal cord thinning; linear low signal on the surface of spinal cord. (F) Cross-sectional T2-weighted imaging: low signal change on the surface of cervical spinal cord.
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Letters to the Editor / Clinical Neurology and Neurosurgery 121 (2014) 76–78
In conclusion, SSCNS is a rare clinical chronic progressive neurological disease. MRI examination is the most valuable method for the diagnosis of this disease. As for the treatment, there is long way to explore. References [1] Kumar N, Cohen-Gadol AA, Wright RA, et al. Superficial siderosis. Neurology 2006;66(8):1144–52. [2] Levy M, Turtzo C, Llinas RH. Superficial siderosis: a case report and review of the literature. Nat Clin Pract Neurol 2007;3(1):54–8. [3] Wang K, Xu Z, Xiong G, et al. Superficial siderosis of the central nervous system manifested with seizures. Clin Neurosci 2010;17(2):277–8. [4] Cohen-Gadol A, Krauss W, Spinner R. Delayed central nervous system superficial siderosis following brachial plexus avulsion injury. Report of three cases. Neurosurg Focus 2000;16(5):E10. [5] Offenbacher H, Fazekas F, Schmidt R, et al. Superficial siderosis of the central nervous system: MRI findings and clinical significance. Neuroradiology 1996;38(Suppl. 1):S51–6.
Wen-Na Li Shan Qiao Tao Han Xiu-He Zhao Sheng-Jun Wang Ai-Qin Wang Ming-Zhu Meng Zhao-Fu Chi Department of Neurology, Qilu Hospital of Shandong University, Jinan, Shandong 250012, PR China ∗ Corresponding
author. Tel.: +86 531 82166166; fax: +86 531 82169022. E-mail address: [email protected] (X.-W. Liu) 22 November 2013 Available online 13 March 2014
Kun-Kun Wei Xue-Wu Liu ∗
http://dx.doi.org/10.1016/j.clineuro.2014.02.026
Dale Ding ∗ University of Virginia, Department of Neurosurgery, Charlottesville 22908, USA ∗ Correspondence to: University of Virginia, Department of Neurosurgery, P.O. Box 800212, Charlottesville 22908, USA. Tel.: +1 434 924 2203; fax: +1 434 982 5753. E-mail address: [email protected]
Available online 1 March 2014 http://dx.doi.org/10.1016/j.clineuro.2014.01.028
Superficial siderosis of the central nervous system presenting with dysuria initially To the Editor, Superficial siderosis of the central nervous system (SSCNS) is a rare disorder characterized by a triad of symptoms consisting of hypacusia, cerebellar ataxia, and myelopathy. Kumar et al. has also reported its less frequent manifestation of headache, dementia [1]. We treated a 50-year-old male patient with an initial presentation of dysuria. He developed the symptom of dysuria 3 years ago and has hearing loss for 2 years and ataxia for 1 year. Routine blood tests, biochemistry, hepatic and renal function, and trace elements were normal. Brain and spinal MRI showed (Fig. 1) cord-like abnormal signal on the surface of bilateral temporal lobe, corpus callosum, cerebellar hemispheres, brain stem and spinal cord; broadening of the sulcus of cerebellum; L2–3, L4–5 disk bulge; L3–4, L5–S1 disk
77
herniation. He was given vitamin B1, vitamin B12, lipoic acid for two weeks after admission, but with no evident improvements. Hypacusia and cerebellar ataxia are typically common symptoms in SSCNS. Additionally, anosmia, vision impairment, ageusia, headaches, bladder dysfunction, spinal cord disease, seizures, and cognitive dysfunction have also been reported in previous literatures [2,3]. However, symptoms of SSCNS often gradually manifest a few years later following the appearance of the disease. This patient had an initial complaint of dysuria, the reasonable cause for which could be an early involvement of spinal cord. Urinary tract ultrasonography shows slight enlargement and calculi formation of the prostatic portion with accompanying symptom of nocturia; which are mainly because of spinal cord involvement, but the possibility of an enlarged prostate contributing to dysuria could not be entirely ruled out. Other symptoms of our patient, such as hearing loss, tinnitus and cerebellar ataxia, are consistent with previous reports. MRI images present typical pathologic findings of deposition of iron beneath the surfaces of CNS tissues. Cerebellar atrophy is evident. MRI imaging of this case showed a typical SSCNS features consistent with literatures which report the widening of cerebellar sulci. Untreated SSCNS often has poor prognosis. Surgical removal of hemorrhage may be an effective way to treat SSCNS [4,5], applying to nerve root lesion tear, arteriovenous malformations, dural rupture. The efficacy of iron chelator is still uncertain [2]. Levy [2] has reported one case treated with iron chelator, hemosiderin reducing in brainstem and cerebellar vermis. However, there have been few failures too, which might be due to its difficulty in passing through the blood–brain barrier. In this case, rehabilitation treatment could be beneficial to some extent in order to improve the syndrome which may attribute to the above reason.
Fig. 1. (A) T1-weighted imaging: slightly higher signal on the surface of pons. (B) T2-weighted imaging: hypointensive-rim on the surface of pons. (C) T2 FLIAR imaging: slightly high signal or isointensity on the surface of pons. (D) Sagittal T2 weighted imaging: low signal on the surface of the corpus callosum, spinal cord thinning. (E) Sagittal T2-weighted imaging: the spinal cord thinning; linear low signal on the surface of spinal cord. (F) Cross-sectional T2-weighted imaging: low signal change on the surface of cervical spinal cord.
78
Letters to the Editor / Clinical Neurology and Neurosurgery 121 (2014) 76–78
In conclusion, SSCNS is a rare clinical chronic progressive neurological disease. MRI examination is the most valuable method for the diagnosis of this disease. As for the treatment, there is long way to explore. References [1] Kumar N, Cohen-Gadol AA, Wright RA, et al. Superficial siderosis. Neurology 2006;66(8):1144–52. [2] Levy M, Turtzo C, Llinas RH. Superficial siderosis: a case report and review of the literature. Nat Clin Pract Neurol 2007;3(1):54–8. [3] Wang K, Xu Z, Xiong G, et al. Superficial siderosis of the central nervous system manifested with seizures. Clin Neurosci 2010;17(2):277–8. [4] Cohen-Gadol A, Krauss W, Spinner R. Delayed central nervous system superficial siderosis following brachial plexus avulsion injury. Report of three cases. Neurosurg Focus 2000;16(5):E10. [5] Offenbacher H, Fazekas F, Schmidt R, et al. Superficial siderosis of the central nervous system: MRI findings and clinical significance. Neuroradiology 1996;38(Suppl. 1):S51–6.
Wen-Na Li Shan Qiao Tao Han Xiu-He Zhao Sheng-Jun Wang Ai-Qin Wang Ming-Zhu Meng Zhao-Fu Chi Department of Neurology, Qilu Hospital of Shandong University, Jinan, Shandong 250012, PR China ∗ Corresponding
author. Tel.: +86 531 82166166; fax: +86 531 82169022. E-mail address: [email protected] (X.-W. Liu) 22 November 2013 Available online 13 March 2014
Kun-Kun Wei Xue-Wu Liu ∗
http://dx.doi.org/10.1016/j.clineuro.2014.02.026
