Superficial siderosis of the central nervous system: secondary progression despite successful surgical treatment, mimicking amyotrophic lateral sclerosis. Case report and review

Abstract

Superficial siderosis of the central nervous system is a rare disorder with hemosiderin deposition in the spinal and cranial leptomeninges and subpial layer, mostly from repetitive subarachnoid hemorrhage. Progressive sensorineural deafness, cerebellar ataxia, and pyramidal signs comprise the typical clinical presentation. We describe a 47-year-old patient, who showed initial 2-year improvement after successful occlusion of an intradural bleeding source at T4. Secondary progression of symptoms without further bleedings was noted thereafter, with a clinical picture of amyotrophic lateral sclerosis. This case illustrates that the disease may progress secondarily even without re-bleedings, and that secondary progression might be due to a similar pathomechanism as in amyotrophic lateral sclerosis.

Infratentorial superficial siderosis: Classification, diagnostic criteria, and rational investigation pathway

“…9 The high incidence of dural abnormalities we found in type 1 (classical) iSS is consistent with a previous case series 14 as well as a number of case reports. 15,16,22,[24][25][26][27][28] However, our findings differ from some previous reports on the causes of type 1 (classical) iSS; although arteriovenous malformations have been reported to cause 9% of cases of superficial siderosis of the CNS, 9 we found none, and only 1 very atypical aneurysm, visible on plain MRI, with a contrast-enhancing wall. 15,16,22,[24][25][26][27][28] However, our findings differ from some previous reports on the causes of type 1 (classical) iSS; although arteriovenous malformations have been reported to cause 9% of cases of superficial siderosis of the CNS, 9 we found none, and only 1 very atypical aneurysm, visible on plain MRI, with a contrast-enhancing wall.…”

Superficial Siderosis and Anticoagulation Therapy: Different Presentations, Different Outcomes

“…Epilepsy [15], psychosis [16], optic and trigeminal neuropathy, nystagmus, headache, radiculopathy, tethered cord syndrome caused by spinal venous hypertension, and pain related to arachnoiditis [6] were all associated with SS. Patients with SS presenting as a phenocopy of amyotrophic lateral sclerosis [17] or adult-onset spinocerebellar ataxia [18] have been depicted and certain authors proposed that SS should be excluded in any doubtful case. …”

“…The benchmark treatment of SS is surgical closure of the haemorrhagic source [21], which frequently, but not always [17], arrests the disease progression. Nonetheless, sporadic partial recoveries are described.…”

Cytology abnormal of cerebrospinal fluid in superficial siderosis of the central nervous system

“…[9] Surgical treatment can be used to remove the potential source of recurrent SAH in order to arrest the clinical deterioration, but a number of patients has been reported to have experienced aggravated symptoms after surgery. [10] Iron chelating drugs were identified ineffective because of the bloodbrain barrier (BBB). Recently, Schirinzi et al [11] used soluble deferiprone that could cross BBB.…”