Superficial siderosis of the central nervous system with epilepsy originating from traumatic cervical injury: illustrative case

Liqing Xu Department of Neurosurgery, Peking University First Hospital, Xicheng District, Beijing, China

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Changwei Yuan Department of Neurosurgery, Peking University First Hospital, Xicheng District, Beijing, China

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Yingjin Wang Department of Neurosurgery, Peking University First Hospital, Xicheng District, Beijing, China

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Shengli Shen Department of Neurosurgery, Peking University First Hospital, Xicheng District, Beijing, China

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Hongzhou Duan Department of Neurosurgery, Peking University First Hospital, Xicheng District, Beijing, China

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BACKGROUND

Superficial siderosis of the central nervous system (SSCNS) is a rare condition that results from hemosiderin deposition in the brain, brainstem, cerebellum, and spinal cord as a result of chronic, repeated, and recurrent subarachnoid hemorrhage. SSCNS that originates in the spinal cord is rarely reported, and epilepsy as a manifestation of such a case has not been reported before.

OBSERVATIONS

The authors reported a rare case of SSCNS with epilepsy originating from traumatic cervical injury and presented a literature review of all reported SSCNS cases that originated in the spine. The patient was a 29-year-old man with a 16-year history of progressive headache accompanied by seizures, ataxia, and sensorineural hearing loss. He had experienced a traumatic cervical injury at age 7. Magnetic resonance imaging revealed a characteristic hypointense rim around the pons and cervical spinal cord on susceptibility-weighted imaging scans. Cerebrospinal fluid examination during a headache episode confirmed subarachnoid hemorrhage and increased intracranial pressure. Surgical exploration revealed a C6 dural defect with bone spurs inserted into the dura mater. After the patient underwent dura mater repair and shunt implantation, his symptoms disappeared completely except for hearing loss.

LESSONS

This rare case indicated that symptomatic epilepsy followed by SSCNS can be eliminated by complete repair of the cervical dura mater.

ABBREVIATIONS

CSF = cerebrospinal fluid; CT = computed tomography; ICP = intracranial pressure; MRI = magnetic resonance imaging; SSCNS = superficial siderosis of the central nervous system.

BACKGROUND

Superficial siderosis of the central nervous system (SSCNS) is a rare condition that results from hemosiderin deposition in the brain, brainstem, cerebellum, and spinal cord as a result of chronic, repeated, and recurrent subarachnoid hemorrhage. SSCNS that originates in the spinal cord is rarely reported, and epilepsy as a manifestation of such a case has not been reported before.

OBSERVATIONS

The authors reported a rare case of SSCNS with epilepsy originating from traumatic cervical injury and presented a literature review of all reported SSCNS cases that originated in the spine. The patient was a 29-year-old man with a 16-year history of progressive headache accompanied by seizures, ataxia, and sensorineural hearing loss. He had experienced a traumatic cervical injury at age 7. Magnetic resonance imaging revealed a characteristic hypointense rim around the pons and cervical spinal cord on susceptibility-weighted imaging scans. Cerebrospinal fluid examination during a headache episode confirmed subarachnoid hemorrhage and increased intracranial pressure. Surgical exploration revealed a C6 dural defect with bone spurs inserted into the dura mater. After the patient underwent dura mater repair and shunt implantation, his symptoms disappeared completely except for hearing loss.

LESSONS

This rare case indicated that symptomatic epilepsy followed by SSCNS can be eliminated by complete repair of the cervical dura mater.

ABBREVIATIONS

CSF = cerebrospinal fluid; CT = computed tomography; ICP = intracranial pressure; MRI = magnetic resonance imaging; SSCNS = superficial siderosis of the central nervous system.

Superficial siderosis of the central nervous system (SSCNS) is an uncommon and unrecognized disorder characterized by hemosiderin deposition on the surface of the brain, brainstem, cerebellum, and spinal cord as the result of chronic or intermittent bleeding into the subarachnoid space, which causes irreversible damage to the CNS and results in a series of neurological manifestations.1,2 The typical triad of SSCNS symptoms includes progressive cerebellar ataxia, central motor disability, and sensorineural hearing loss.3 Other symptoms, such as chronic increased intracranial pressure (ICP) and hydrocephalus, develop in approximately one-third of patients because of obstruction of the ventricular foramina and/or malabsorption of cerebrospinal fluid (CSF).4 Epilepsy is a rare manifestation of SSCNS as discussed in the literature, although patients with SSCNS may have a prior history of head trauma or surgical procedures.5,6 Most reported cases of SSCNS originate from traumatic brain injury and intracranial hemorrhage or surgery; relatively few cases originate from spinal injury, and in this latter group, epilepsy has not been reported.7 Medical or surgical treatments for SSCNS are often ineffective, and most reported cases progress slowly and inexorably. Although treatment with deferiprone8 and cochlear implants9 have been tried, successful therapy still depends on determining the etiology of chronic bleeding and precise treatment. We report an interesting case of SSCNS originating from a traumatic cervical injury. The patient experienced intractable epilepsy and increased ICP in addition to the typical triad. His condition was treated successfully with repair of the dura mater.

Illustrative Case

A 29-year-old male patient presented with a 16-year history of progressive headache accompanied by intractable seizures and sensorineural hearing loss. The headache consisted of sudden-onset frontal or occipital radiating pain that was often accompanied by photophobia, diplopia, nausea, and vomiting. The pain was aggravated when he lay flat, and it gradually increased in severity and frequency. In the previous year, every headache was accompanied by generalized tonic-clonic seizures, which fully subsided several minutes later. Although various antiepileptic drugs were used, the seizures were not controlled effectively. The patient also developed progressive deafness and ataxia within the previous 5 years, and the hearing loss was obvious on the left side. When he was referred to our hospital, epilepsy with headache onset was occurring approximately twice a month; furthermore, the patient was unable to walk independently, and the hearing loss in the left ear was almost complete.

When he was 7 years old, the patient had experienced a fall that resulted in a short period of disturbance in consciousness and neck pain. Neurological examination upon admission to the hospital revealed a deterioration of memory, decreased visual acuity without papilledema, nystagmus, hearing loss, positive Rinne test result, and ataxic gait. Other cranial nerve and sensory examinations produced normal results. On brain magnetic resonance imaging (MRI), axial T2-weighted images (Fig. 1A) and susceptibility-weighted imaging scans (Fig. 1B) showed a characteristic rim of hypointensity along the dorsolateral pons, which indicated the deposition of hemosiderin. CSF examination at headache onset indicated a high ICP (29 cm H2O) and blood (>1,000 red blood cells per mm3), suggesting subarachnoid hemorrhage. Except for a slight increase in protein concentration, no specific positive results were shown in biochemical or immunoelectrophoresis examinations of CSF. No abnormalities were present on routine electroencephalography except for a wide slow wave throughout the brain. To determine the reason for the subarachnoid hemorrhage, digital subtraction angiography of the brain and spinal cord was performed; however, neither aneurysms nor arteriovenous malformations were found. Based on the prior history of falling, further MRI of the cervical spine was performed, which revealed a hypointense lesion in the subdural and epidural space of C6–7 on sagittal T2-weighted images (Fig. 1C). This finding indicated the possibility of a previous hemorrhage. Cervical computed tomography (CT) examination revealed hyperosteogeny of the left C7 lamina (Fig. 1D) with a bone spur protruding into the spinal canal (Fig. 1E), which indicated a previous fracture. Because the osteophyte was believed to be the cause of recurrent subarachnoid hemorrhage, surgical exploration was indicated.

FIG. 1.
FIG. 1.

A and B: Axial T2-weighted and susceptibility-weighted brain MRI shows the characteristic hypointense rim along the dorsolateral pons. C: Sagittal T2-weighted cervical spine MRI shows a hypointense lesion in the C6–7 subdural and epidural space, indicating previous hemorrhage. D and E: CT of the cervical spine shows hyperosteogeny of the left C7 lamina, with a bone spur protruding into the spinal canal (arrow).

During the operation, we found a wide deposition of hemosiderin around the spinal cord, and a protruding osteophyte (Fig. 2) pierced the dura and the subdural space of the spinal cord, leading to an apparent dural defect. The osteophyte was removed, and the subcutaneous fascia was sutured over the dural defect in a watertight fashion. The patient recovered well after the operation; however, he experienced severe headache and vomiting after the drainage tube was pulled out. Lumbar puncture was performed, and test results indicated an extremely high ICP (>33 cm H2O) on the 15th day after the operation. However, there were no red blood cells in the CSF, and the protein concentration was normal. Although the ventricle was not obviously enlarged, increased ICP was diagnosed, and a lumbar-peritoneal shunt was placed to drain CSF and decrease ICP. The patient recovered uneventfully, and his headache disappeared immediately. His progress was followed up regularly. At the 18-month follow-up visit, the patient was free from headache and seizures, and his ataxia had improved greatly; however, his deafness had not improved.

FIG. 2.
FIG. 2.

Intraoperative photograph shows deposition of hemosiderin on the surface of the spine and a bone spur (arrow) protruding into the subdural space, leading to a dural defect in the left posterior part of the cervical spine.

Discussion

Observations

SSCNS is a rare neurodegenerative disease that results from toxic accumulation of hemosiderin on the surface of the brain and spinal cord. Although the number of reported cases is increasing, the natural history and clinical evolution of SSCNS are poorly understood. Further identification and resolution of the bleeding source do not elicit prompt clinical recovery or radiological reversal of SSCNS in most cases, leading to a major challenge in further diagnosis and treatment. Most clinical signs and symptoms of superficial siderosis are believed to be related to the anatomical distribution of hemosiderin deposits within the neural system.10,11 Hemosiderin is apt to deposit in tissues that are exposed to abundant CSF, such as the vermis, superficial sulci and gyri, basal frontal lobe, temporal lobe, brainstem, and spinal cord as well as cranial nerves I, II, and VII, which leads to the typical triad of progressive cerebellar ataxia, central motor disability, and sensorineural hearing loss. Other manifestations have been reported, such as diplopia, hyposmia, amnesia, headache, and seizures.12,13 Because most of the damage to the CNS is irreversible, it is vital to determine the etiology and intervene as early as possible. Although extensive diagnostic examinations are used to determine the causative pathologies of bleeding conditions, the etiology of more than 30% of subarachnoid hemorrhage cases remains unknown.13

Recently, attention has been drawn to the association between SSCNS and dural defects in the spinal canal. We searched all related English-language literature in PubMed, GeenMedical, and other databases and identified 41 cases of SSCNS4,12,14–42 associated with spinal dural defects (Table 1). The cases included 33 male and 8 female patients with an average age of 60.6 years (ranging from 33 to 74 years). The common definite causes were as follows: trauma (11/41), previous surgery (5/41), intervertebral disc herniation (4/41), dural ectasia (2/41), Marfan syndrome (1/41), and dural arteriovenous fistula (1/41). There were 17 cases in which the etiology was not reported. The duration from symptom onset to surgery averaged 6.81 years (ranging from 0.1 to 30 years). The most prevalent clinical manifestations were gait ataxia (31/41) and sensorineural hearing loss (28/41), followed by headache (7/41), tinnitus (6/41), dizziness (5/41), urinary incontinence (6/41), cognitive decline (4/41), limb incoordination (3/41), slurred speech (3/41), dysarthria (3/41), anosmia (2/41), neck pain (2/41), diplopia (1/41), nausea (1/41), emesis (1/41), and blurred vision (1/41). MRI indicated spinal dural defects located in the cervical spine in 5 patients and in the thoracic vertebrae in 23 patients. Most of the CSF examinations showed xanthochromia, increased red blood cells, and intracranial hypotension. Considering that SSCNS was caused by spatially defined lesions with dural defects, 34 patients were treated with reparative surgery. The repair techniques included direct suturing (8 patients), muscle grafts (4 patients), fat grafts (6 patients), fibrin glue (9 patients), patches (4 patients), gelatin sponges (3 patients), and artificial dura mater (1 patient). Postoperative MRI in most cases showed a reduction or disappearance of epidural effusion. Among the patients with reported results, the prognosis was improved in 10 patients, partially improved in 9 patients, unchanged in 9 patients, and worsened in 3 patients. The improvement rate of headache symptoms was the highest (100%, 7/7), followed by gait instability symptoms (19.4%, 6/31); sensorineural hearing loss was not likely to improve (0%, 0/28).

TABLE 1.

Reported SSCNS cases originating from the spine

Case No.Author & YearAge (yrs), GenderEtiologyMajor SymptomsTime (yrs)MRI FindingsCSF FindingsLocation of Dural DefectClosure MethodOutcome (FU)Postop MRI Findings
1Kumar et al., 20051442, MHead injury at 10 yoGait ataxia, deafness, anosmia, incontinence8Epidural fluid collection from C4 to T9Xanthochromia, ICP: NM, RBCs: 1,133T2–3Muscle graftNo change (6 mos)Reduction of fluid collection
251, FHead injury at 16 yoGait ataxia, deafness, incontinence4Epidural fluid collection from T1 to T3NMT2Reduction of fluid collection
352, MLt brachial plexus & spinal injury at 10 yoGait ataxia, deafness, tinnitus, incontinence7Epidural fluid collection from C3 to L5NMT11
4Kumar et al., 20061542, MLt brachial plexus & spinal injury at 20 yoGait ataxia, deafnessEpidural fluid collection from T1 to T5, C7–T1 pseudomeningoceleXanthochromia, RBCs: 0, ICP: NMNo change (2 yrs)
5Holle et al., 20081659, MThoracic disc herniationGait ataxia, limb incoordination, slurred speech, deafness, anosmia3Epidural fluid collection from C5 to T6, disc herniationXanthochromia, RBCs: NM, ICP: 50 cm H2OT5–6Glue-coated collagen spongeImprovement of headache, deterioration of cerebellar syndrome
6Shih et al., 20091770, MGait ataxia, deafness, tinnitus, cognitive decline2Epidural fluid collection from T2 to T8Xanthochromia, RBCs: 11, ICP: NMT4–5Dural patch, dural sealantNo change (15 mos)
7Kumar et al., 20091864, MC4–7 laminectomyGait ataxia, deafness10Epidural fluid collection from C3 to T11Xanthochromia, RBCs: 464, ICP: 4 cm H2OT7–8Free fat graft, sealantImprovement of gait (6 mos)Resolution of fluid collection
8Ikeda et al., 20101971, FGait ataxia, deafness7Epidural fluid collection from C7 to T12Xanthochromia, RBCs: >30,000, ICP: NMT2–3No change (1 yr)
9Kumar et al., 20102054, MMotor vehicle accidentGait ataxia, deafness, slurred speech5Epidural fluid collection from C2 to T7Xanthochromia, RBCs: 1,243, ICP: 175 cm H2OT3SutureImprovement of neck pain (4 mos)Resolution of fluid collection
10Cheng et al., 20112153, MArachnoid cystGait ataxia, deafness, dizziness2Epidural fluid collection from C7 to T4Xanthochromia, RBCs: 661, ICP: 115 cm H2OGlueImprovement of gait (6 mos)Resolution of fluid collection
11Boncoraglio et al., 20122269, MSurgery for L4–5 disc herniationCerebellar ataxia4Epidural fluid collection from C2 to T9, T6–7 cord herniationXanthochromia, RBCs: NM, ICP: NMT6–7Patch, fibrin glueNo change (6 mos)Resolution of fluid collection
12Egawa et al., 2013467, MHeadache, gait ataxia, deafness, dysarthria30Epidural fluid collection from C2 to T8Xanthochromia, RBCs: 1,000, ICP: 10 cm H2OT2–3Free muscle graft, fibrin glue patchImprovement of headache, deterioration of neurological symptomsResolution of fluid collection
1354, MGait ataxia, tinnitus, slurred speech, diplopia4Epidural fluid collection from C7 toT8Xanthochromia, RBCs: 1,000, ICP: 13 cm H2OT1–2Suture, muscle graftNo change (18 mos)Resolution of fluid collection
14Yokosuka et al., 20142353, MCervical laminectomy & removal of cervical schwannomaSchizophrenia26PseudomeningoceleXanthochromia, RBCs: 768–1,034, ICP: normalAutologous fatNo change (12 mos)Resolution of pseudomeningocele
15Schievink et al., 20162433, MHeadache, nausea, emesis, tinnitus, low-back pain2Extensive ventral thoracolumbar extradural CSF collection & hematoma w/in lumbar ventral CSF collectionT9–10Improvement of all symptoms (12 mos)Resolution of fluid collection
1662, FHeadache, blurred vision, aural fullness, neck pain2Intrathecal hemorrhage & extensive spinal extradural CSF collectionPatchImprovement of all symptoms (8 mos)Resolution of fluid collection
17O’Hare et al., 20162561, MExtensive dural ectasiaUrinary retention, deafness, tinnitusExtensive dural ectasiaT5–11Resolution of pseudomeningocele
18Ryu SM et al., 20162655, MGait ataxia, excretion disorder, tinnitus2Xanthochromia, RBCs: 15,250, ICP: NMC1–2
19Madkouri & Grelat, 20172758, MDural arteriovenous fistulaCerebellar ataxia, pyramidal signs, dysarthria, deafness, cognitive impairmentC3–4, C5–6, C6–7SutureImprovement of all symptoms (1 mo)Resolution of fluid collection
20Sakoda et al., 20172864, MHead injuryHeadache, dizziness, deafness2Dural defect at T2–3 level on anterior side of spinal canalXanthochromia, RBCs: 4,144, ICP: 1 cm H2OT3Autologous fascia of neck muscleImprovement of all symptoms (7 mos)Resolution of fluid collection
21Takai et al., 20172958, MGait ataxia, dysarthria, deafness5Dural defect at several spinal levels from C4 to T7Xanthochromia, RBCs: 2,800–3,300, ICP: 4 cm H2OT1
22Hiraka et al., 20183058, MGait ataxia, deafnessEpidural fluid collection from C3 to T10Colorless, RBCs: NM, ICP: 130 cm H2OT1
23Bower et al., 20183167, FMarfan syndromeGait ataxia, deafness, urinary incontinence10Thoracic & lumbar spine dural ectasia
24Hosokawa et al., 20183262, MGait ataxia, deafness, spasticity8Epidural fluid collection from T1 to T4Xanthochromia, RBCs: NM, ICP: NM
2560, MGait ataxia, deafness3Epidural fluid collection from C1 to T4Xanthochromia, RBCs: NM, ICP: NM
2649, MGait ataxia, deafness12Epidural fluid collection from T1 to T4Xanthochromia, RBCs: NM, ICP: NM
2768, FGait ataxia, deafness2Epidural fluid collection from T1 to T4Colorless, RBCs: NM, ICP: NM
2874, FGait ataxia, deafness13Epidural fluid collection from T1 to T4Colorless, RBCs: NM, ICP: NMT7–8SutureImprovement of headache, stability of other symptoms (17 mos)Resolution of fluid collection
29Arishima et al., 20183350, MSurgery for subdural hematomaGait ataxia, motor disturbance of bilat upper limbs10Epidural fluid collection from C2 to T12Xanthochromia, RBCs: NM, ICP: 20 cm H2OC7SutureImprovement of all symptoms (17 mos)Resolution of fluid collection
3059, MSurgery for subdural hematomaMotor disturbance of rt upper & lower limbs0.25Epidural fluid collection from C2 to T2Xanthochromia, RBCs: NM, ICP: 0 cm H2OT1–2, T3–4Synthetic dura materialImprovement of all symptoms (6 mos)Resolution of fluid collection
31Camlar et al., 20183458, FThoracic spinal surgeriesGait ataxia, deafness, dizziness0.75Dural defect at T1–2 levelXanthochromia, RBCs: NM, ICP: 11 cm H2OT8–9SutureImprovement of all symptoms (24 mos)Resolution of fluid collection
32Brembilla et al., 20181248, MGait ataxia, deafness3Osteophyte at T8–9 levelXanthochromia, RBCs: 6,000, ICP: 6 cm H2OStability of deafness (24 mos)Stability of meningocele
33Nasri et al., 20183548, MHead injury at 2 yoMotor disturbance of bilat upper limbs, urinary dysfunction10Dural pseudomeningoceles of lt C3 to C7 nerve rootsT1–2SutureNo changeResolution of fluid collection
34Machino et al., 20193671, MGait ataxia, giddy feeling, dizziness5Epidural fluid collection from C7 to T5T1–2Fat & fascia lataNo change (12 mos)Clivus reconstruction
35Vellutini et al., 20193735, MHead injury at 15 yoDeafnessArachnoidoceleXanthochromia, RBCs: NM, ICP: NMT3Suture, fibrin sealantDeath (8 mos)
36Nathoo et al., 20203874, MHead injuryGait ataxia, deafness, dizziness, cognitive impairment, urinary retention2Epidural fluid collection from T2 to T5T1–2, T5–6SutureImprovement of headache, stability of other symptoms (17 mos)Resolution of fluid collection
37Katoh et al., 20203974, FGait ataxia, deafness7Epidural fluid collection from C7 to T10C6–7Fibrin glueImprovement of headache (36 mos)Resolution of fluid collection
38Wiącek et al., 20204063, MRt brachial plexus & spinal injury at 35 yoGait ataxia, deafness, slurred speech, headache7Epidural fluid collection from C3 to T12Xanthochromia, RBCs: NM, ICP: 5 cm H2OT9–10Dura substitute, fibrin glue, autologous fat graft, absorbable gelatin spongeImprovement of all symptoms (16 mos)Resolution of fluid collection
39Cornips et al., 20204156, MTransdural thoracic disc herniationHeadache, cognitive dysfunction0.1T7–8Dura substitute, fibrin glue, autologous fat graft, absorbable gelatin spongeImprovement of all symptoms (4 mos)Resolution of fluid collection
4033, MTransdural thoracic disc herniationHeadache, dizziness2Epidural fluid collection from C2 to T12C7SutureImprovement of all symptomsResolution of fluid collection
41Sato et al., 20204265, MGait ataxia, deafnessEpidural fluid collection from C2 to T8Xanthochromia, RBCs: NM, ICP: NMC7SutureImprovement of headache, cerebral symptoms; stability of deafness (12 mos)
42Present case29, MTraumatic cervical injuryGait ataxia, deafness, headache, epilepsy16Bone spur at C7 level; hemosiderin circled spinal cordXanthochromia, RBCs: 1,000, ICP: 29 cm H2OC6–7SutureImprovement of gait ataxia, headache, epilepsy; stability of deafness (18 mos)Bone spur disappeared

– = not mentioned; FU = follow-up; NM = not mentioned; RBCs = red blood cell count (number of cells per mm3); Time = duration from symptom onset to the surgery; Yo = years old.

In our study, the patient with SSCNS was confirmed to have intermittent subarachnoid hemorrhage caused by a cervical osteophyte that resulted in a dural defect. The repeated activity of the osteophyte led to a small amount of bleeding, which entered the subarachnoid space through the dura defect, causing the deposition of hemosiderin on the surface of the spinal cord and brain and the generation of clinical symptoms. As a result of removal of the bone spurs and repair of the dura mater, subarachnoid hemorrhage was avoided, and the symptoms improved dramatically.

Our patient’s epileptic manifestation may be related to the increase in ICP. It has been reported that the causal relationship between intracranial hypertension and epilepsy events is evident clinically and that increased cranial pressure can induce seizures.43 Our patient experienced severe headache before epilepsy events, accompanied by increased ICP, which further confirmed the relationship. Our patient also had elevated ICP before dural closure and even higher pressure after dural closure. We speculate that malabsorption of CSF due to dysfunction of the pacchionian granulations caused by recurrent subarachnoid hemorrhage may result in chronic intracranial hypertension. Before dural closure, the dural fistula could drain some of the CSF, which is why the patient’s headache was partially relieved when he changed his position. After the dura defect was closed, the extra CSF could not be absorbed and resulted in higher ICP, which was ultimately resolved by shunt surgery.

Lessons

Our patient represents an extremely rare case of SSCNS with epilepsy originating from traumatic cervical injury. Although this situation is rare, an active search for the cause of subarachnoid hemorrhage, followed by accurate treatment, will ensure a good prognosis for such patients.

Disclosures

The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

Author Contributions

Conception and design: Duan. Acquisition of data: Yuan, Shen. Analysis and interpretation of data: Yuan, Wang. Drafting the article: Xu. Critically revising the article: Duan. Approved the final version of the manuscript on behalf of all authors: Duan.

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    Yokosuka J, Takai K, Komori T, Taniguchi M. Superficial siderosis: bleeding from the bone marrow after laminectomy for spinal tumor removal. J Neurosurg Spine. 2014;21(6):905908.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 24

    Schievink WI, Wasserstein P, Maya MM. Intraspinal hemorrhage in spontaneous intracranial hypotension: link to superficial siderosis? Report of 2 cases. J Neurosurg Spine. 2016;24(3):454456.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 25

    O’Hare M, Fearon C, Kavanagh EC, et al.. Superficial siderosis and dural ectasia: a case report. Neurology. 2016;87(16):17431744.

  • 26

    Ryu SM, Kim ES, Kim SK, et al.. Superficial siderosis of the central nervous system originating from the thoracic spine: a case report. Korean J Spine. 2016;13(2):8386.

  • 27

    Madkouri R, Grelat M. Teaching NeuroImages: superficial siderosis due to a dural cervical arteriovenous fistula. Neurology. 2017;88(8):e66.

  • 28

    Sakoda A, Yamashita KI, Hayashida M, et al.. A case of superficial siderosis ameliorated after closure of dural deficit detected by MRI-CISS (constructive interference in steady state) imaging. Rinsho Shinkeigaku. 2017;57(4):180183.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 29

    Takai K, Komori T, Niimura M, Taniguchi M. Superficial siderosis of the central nervous system associated with intraspinal hemorrhage from ventral thoracic epidural veins and a ventral spinal CSF leak: case report. J Neurosurg Spine. 2017;26(6):751753.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 30

    Hiraka T, Kanoto M, Toyoguchi Y, et al.. Superficial siderosis associated with a spinal dural defect. Magn Reson Med Sci. 2018;17(3):189190.

  • 31

    Bower M, Farag M, Phielipp N. Superficial siderosis and dural ectasia in a patient with Marfan syndrome. Clin Neuroradiol. 2018;28(4):605607.

  • 32

    Hosokawa M, Murata KY, Hironishi M, et al.. Superficial siderosis associated with duplicated dura mater detected by CISS reverse MRI. J Neurol Sci. 2018;392:3843.

  • 33

    Arishima H, Higashino Y, Yamada S, et al.. Spinal endoscopy combined with selective CT myelography for dural closure of the spinal dural defect with superficial siderosis: technical note. J Neurosurg Spine. 2018;28(1):96102.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 34

    Camlar M, Karadag A, Oztekin O, Ozer F. Superficial siderosis of the central nervous system due to recurrent surgeries of the thoracic spine: a rare case. World Neurosurg. 2018;119:384388.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 35

    Nasri A, Kacem I, Sidhom Y, et al.. Isolated spinal cord compression syndrome revealing delayed extensive superficial siderosis of the central nervous system secondary to cervical root avulsion. J Spinal Cord Med. 2018;41(4):490495.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 36

    Machino M, Imagama S, Ishiguro N. Detection of spinal dural defect in superficial siderosis by intraoperative ultrasonography. World Neurosurg. 2019;129:386388.

  • 37

    Vellutini EAS, Stamm AEC, Martins HO, et al.. Role of transnasal endoscopic surgery in the treatment of superficial siderosis of central nervous system secondary to clivus arachnoidocele: report of successful case and literature review. World Neurosurg. 2019;126:142145.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 38

    Nathoo N, Naik S, Rempel J, et al.. Superficial siderosis treated with dural tear repair and deferiprone. Pract Neurol. 2021;21:7172. doi:10.1136/practneurol-2020-002657

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 39

    Katoh H, Shibukawa S, Yamaguchi K, et al.. A combination of magnetic resonance imaging techniques to localize the dural defect in a case of superficial siderosis. A case report. Medicines (Basel). 2020;7(6):36. doi:10.3390/medicines7060036

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 40

    Wiącek M, Perenc A, Tołpa B, Bartosik-Psujek H. Superficial siderosis and intracranial hypotension syndrome following brachial plexus avulsion injury. A case of surgical treatment. Clin Neurol Neurosurg. 2020;192:105723.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 41

    Cornips E, Grouls M, Bekelaar K. Transdural thoracic disk herniation with longitudinal slitlike dural defect causing intracranial hypotension: report of 2 cases. World Neurosurg. 2020;140:e311e319.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 42

    Sato Y, Endo T, Inoue T, et al.. Successful endoscopic identification of the bleeding source in the ventral dura of the cervical spine in a case of superficial siderosis. J Neurosurg Spine. 2020;33(1):7376.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 43

    Ohira M, Takao M. Superficial siderosis. Article in Japanese. Brain Nerve. 2018;70(10):11071113.

  • Collapse
  • Expand
  • FIG. 1.

    A and B: Axial T2-weighted and susceptibility-weighted brain MRI shows the characteristic hypointense rim along the dorsolateral pons. C: Sagittal T2-weighted cervical spine MRI shows a hypointense lesion in the C6–7 subdural and epidural space, indicating previous hemorrhage. D and E: CT of the cervical spine shows hyperosteogeny of the left C7 lamina, with a bone spur protruding into the spinal canal (arrow).

  • FIG. 2.

    Intraoperative photograph shows deposition of hemosiderin on the surface of the spine and a bone spur (arrow) protruding into the subdural space, leading to a dural defect in the left posterior part of the cervical spine.

  • 1

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  • 22

    Boncoraglio GB, Ballabio E, Erbetta A, et al.. Superficial siderosis due to dural defect with thoracic spinal cord herniation. J Neurol Sci. 2012;312(1-2):170172.

  • 23

    Yokosuka J, Takai K, Komori T, Taniguchi M. Superficial siderosis: bleeding from the bone marrow after laminectomy for spinal tumor removal. J Neurosurg Spine. 2014;21(6):905908.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 24

    Schievink WI, Wasserstein P, Maya MM. Intraspinal hemorrhage in spontaneous intracranial hypotension: link to superficial siderosis? Report of 2 cases. J Neurosurg Spine. 2016;24(3):454456.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 25

    O’Hare M, Fearon C, Kavanagh EC, et al.. Superficial siderosis and dural ectasia: a case report. Neurology. 2016;87(16):17431744.

  • 26

    Ryu SM, Kim ES, Kim SK, et al.. Superficial siderosis of the central nervous system originating from the thoracic spine: a case report. Korean J Spine. 2016;13(2):8386.

  • 27

    Madkouri R, Grelat M. Teaching NeuroImages: superficial siderosis due to a dural cervical arteriovenous fistula. Neurology. 2017;88(8):e66.

  • 28

    Sakoda A, Yamashita KI, Hayashida M, et al.. A case of superficial siderosis ameliorated after closure of dural deficit detected by MRI-CISS (constructive interference in steady state) imaging. Rinsho Shinkeigaku. 2017;57(4):180183.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 29

    Takai K, Komori T, Niimura M, Taniguchi M. Superficial siderosis of the central nervous system associated with intraspinal hemorrhage from ventral thoracic epidural veins and a ventral spinal CSF leak: case report. J Neurosurg Spine. 2017;26(6):751753.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 30

    Hiraka T, Kanoto M, Toyoguchi Y, et al.. Superficial siderosis associated with a spinal dural defect. Magn Reson Med Sci. 2018;17(3):189190.

  • 31

    Bower M, Farag M, Phielipp N. Superficial siderosis and dural ectasia in a patient with Marfan syndrome. Clin Neuroradiol. 2018;28(4):605607.

  • 32

    Hosokawa M, Murata KY, Hironishi M, et al.. Superficial siderosis associated with duplicated dura mater detected by CISS reverse MRI. J Neurol Sci. 2018;392:3843.

  • 33

    Arishima H, Higashino Y, Yamada S, et al.. Spinal endoscopy combined with selective CT myelography for dural closure of the spinal dural defect with superficial siderosis: technical note. J Neurosurg Spine. 2018;28(1):96102.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 34

    Camlar M, Karadag A, Oztekin O, Ozer F. Superficial siderosis of the central nervous system due to recurrent surgeries of the thoracic spine: a rare case. World Neurosurg. 2018;119:384388.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 35

    Nasri A, Kacem I, Sidhom Y, et al.. Isolated spinal cord compression syndrome revealing delayed extensive superficial siderosis of the central nervous system secondary to cervical root avulsion. J Spinal Cord Med. 2018;41(4):490495.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 36

    Machino M, Imagama S, Ishiguro N. Detection of spinal dural defect in superficial siderosis by intraoperative ultrasonography. World Neurosurg. 2019;129:386388.

  • 37

    Vellutini EAS, Stamm AEC, Martins HO, et al.. Role of transnasal endoscopic surgery in the treatment of superficial siderosis of central nervous system secondary to clivus arachnoidocele: report of successful case and literature review. World Neurosurg. 2019;126:142145.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 38

    Nathoo N, Naik S, Rempel J, et al.. Superficial siderosis treated with dural tear repair and deferiprone. Pract Neurol. 2021;21:7172. doi:10.1136/practneurol-2020-002657

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 39

    Katoh H, Shibukawa S, Yamaguchi K, et al.. A combination of magnetic resonance imaging techniques to localize the dural defect in a case of superficial siderosis. A case report. Medicines (Basel). 2020;7(6):36. doi:10.3390/medicines7060036

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 40

    Wiącek M, Perenc A, Tołpa B, Bartosik-Psujek H. Superficial siderosis and intracranial hypotension syndrome following brachial plexus avulsion injury. A case of surgical treatment. Clin Neurol Neurosurg. 2020;192:105723.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 41

    Cornips E, Grouls M, Bekelaar K. Transdural thoracic disk herniation with longitudinal slitlike dural defect causing intracranial hypotension: report of 2 cases. World Neurosurg. 2020;140:e311e319.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 42

    Sato Y, Endo T, Inoue T, et al.. Successful endoscopic identification of the bleeding source in the ventral dura of the cervical spine in a case of superficial siderosis. J Neurosurg Spine. 2020;33(1):7376.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 43

    Ohira M, Takao M. Superficial siderosis. Article in Japanese. Brain Nerve. 2018;70(10):11071113.

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