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Superficial siderosis following trauma to the cervical spine: Case series and review of literature

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Superficial siderosis following trauma to the cervical spine:
Case series and review of literature
Pranab Sinha, Sophie Jane Camp, Harith Akram, Robin Bhatia,
Adrian Thomas Carlos Hickman Casey
ABSTRACT
Supercial siderosis is a rare progressive disease associated with chronic hemosiderin
deposition on the surfaces of the central nervous system (CNS). It typically manifests clinically
in sensorineural hearing loss, cerebellar ataxia, and pyramidal signs. Recurrent or continuous
bleeding into the cerebrospinal uid is implicated in the disease process. The magnetic resonance
imaging gradient-echo T2-weighted images have high sensitivity for hemosiderin deposits that
bathe the CNS, giving the characteristic black rimmed area of hypointensity apparent on these
images. The natural history and its treatments are still not clearly dened in literature. Our
report details the clinical course and management of three cases of supercial siderosis following
either cervical spine or brachial plexus injury. All of them underwent surgical intervention. In
two of the cases, positive cessation of the intradural bleeding was achieved through surgery
but clinical and radiological improvement occurred in only one of the cases. One patient had
a negative intradural exploration. To date, 30 cases of supercial siderosis reported in the
literature have undergone surgical intervention. Cessation of disease progression or neurological
improvement has been documented in 18 of these cases. Our cases reveal that patients with
supercial siderosis often develop severe functional impairment due to the progressive nature
of the disease. On balance, we are of the opinion that early craniospinal imaging and surgical
exploration should be undertaken, at least to attempt to halt neurological deterioration.
(This page in not part of the published article.)
International Journal of Case Reports and Images, Vol. 8 No. 1, January 2017. ISSN – [0976-3198]
Int J Case Rep Images 2017;8(1):11–16.
www.ijcasereportsandimages.com
Sinha et al. 11
CASE SERIES PEER REVIEWED | OPEN ACCESS
Superficial siderosis following trauma to the cervical spine:
Case series and review of literature
Pranab Sinha, Sophie Jane Camp, Harith Akram, Robin Bhatia,
Adrian Thomas Carlos Hickman Casey
ABSTRACT
Superficial siderosis is a rare progressive
disease associated with chronic hemosiderin
deposition on the surfaces of the central nervous
system (CNS). It typically manifests clinically
in sensorineural hearing loss, cerebellar
ataxia, and pyramidal signs. Recurrent or
continuous bleeding into the cerebrospinal
fluid is implicated in the disease process. The
magnetic resonance imaging gradient-echo
T2-weighted images have high sensitivity for
hemosiderin deposits that bathe the CNS,
giving the characteristic black rimmed area of
hypointensity apparent on these images. The
natural history and its treatments are still not
clearly defined in literature. Our report details
the clinical course and management of three
cases of superficial siderosis following either
Pranab Sinha1, Sophie Jane Camp2, Harith Akram3, Robin
Bhatia4, Adrian Thomas Carlos Hickman Casey5
Affiliations: 1MRCS, BSc, MBBS, Flat 1, 28-32 London Rd,
Tunbridge Wells, Kent, TN1 1BX; 2PhD, MRCS, MBBS, MA
(Oxon), the National Hospital for Neurology and Neuro-
surgery, Queen Square, London, United Kingdom; 3FRCS
(Neuro.Surg), MB ChB, the National Hospital for Neurology
and Neurosurgery, Queen Square, London, United Kingdom
4FRCS (SN), PhD, MA, MBBS, the National Hospital for
Neurology and Neurosurgery, Queen Square, London, Unit-
ed Kingdom; 5FRCS, FRCS SW, MBBS, the National Hospi-
tal for Neurology and Neurosurgery, Queen Square, London,
United Kingdom.
Corresponding Author: Sinha, Pranab, MRCS, BSc, MBBS,
MRCS, BSc, MBBS, Address: Flat 1, 28-32 London Rd, Tun-
bridge Wells, Kent, TN1 1BX; E-mail: pranab@doctors.org.
uk
Received: 31 August 2016
Accepted: 28 September 2016
Published: 01 January 2017
cervical spine or brachial plexus injury. All of
them underwent surgical intervention. In two
of the cases, positive cessation of the intradural
bleeding was achieved through surgery but
clinical and radiological improvement occurred
in only one of the cases. One patient had a
negative intradural exploration. To date, 30
cases of superficial siderosis reported in the
literature have undergone surgical intervention.
Cessation of disease progression or neurological
improvement has been documented in 18 of
these cases. Our cases reveal that patients
with superficial siderosis often develop severe
functional impairment due to the progressive
nature of the disease. On balance, we are of the
opinion that early craniospinal imaging and
surgical exploration should be undertaken,
at least to attempt to halt neurological
deterioration.
Keywords: Cervical Spine, Review, Superficial Si-
derosis, Trauma
How to cite this article
Sinha P, Camp SJ, Akram H, Bhatia R, Casey ATCH.
Superficial siderosis following trauma to the cervical
spine: Case series and review of literature. Int J Case
Rep Images 2017;8(1):11–16.
Article ID: Z01201703CS10082PS
*********
doi:10.5348/ijcri-201703-CS-10082
International Journal of Case Reports and Images, Vol. 8 No. 1, January 2017. ISSN – [0976-3198]
Int J Case Rep Images 2017;8(1):11–16.
www.ijcasereportsandimages.com
Sinha et al. 12
INTRODUCTION
Superficial siderosis of the central nervous system
(CNS) is a rare progressive disease entity associated with
chronic hemosiderin deposition in the leptomeninges
and sub-pial parenchyma [1]. It typically presents
with sensorineural hearing loss, cerebellar ataxia, and
pyramidal signs [1–3]. Other features include cognitive
impairment, bladder dysfunction, anosmia, myelopathy,
back pain, bilateral sciatica, and lower motor neuron
signs [4–6].
Recurrent or continuous bleeding into the
cerebrospinal fluid is implicated in the aetiology of
superficial siderosis. This may be as a result of intracranial
or spinal neoplasm, subarachnoid hemorrhage, subdural
hematoma, hemorrhagic meningitis, head injury, and
spinal trauma with nerve root avulsion [3, 6]. Iatrogenic
damage during surgery is a further possible cause [1, 5].
The natural history and treatment paradigms for
superficial siderosis are not clearly defined. It is unknown
why only a proportion of patients with hemosiderin
deposition after subarachnoid hemorrhage develop the
condition. Heme metabolism within the blood-brain
barrier underpins the aetiology.
The following report details the clinical course and
subsequent management of three cases of superficial
siderosis following cervical spine or brachial plexus
injury.
CASE 1
A left handed patient was referred with a seven year
history of progressive deterioration in gait and balance,
and a two-year history of left sided hearing loss with
associated tinnitus and dysarthria. On further questioning,
the patient had been involved in a motorcycle accident at
the age of 23, sustaining a partial right C7 and complete
right C8 and T1 nerve root avulsions.
The patient had a broad based ataxic gait, and exhibited
a cerebellar dysarthria. Examination of the cranial nerves
revealed hypometric saccades, a mild left facial weakness,
and a sensorineural hearing loss on the left. The intrinsic
muscles of the right hand were atrophied, with clawing of
the digits. Sensation was absent in the right C7, C8, and
T1 dermatomes. The right triceps reflex was absent, but
all other upper limb reflexes were brisk. Reflexes were
slightly reduced in the lower limbs, with bilaterally down
going plantars.
Magnetic resonance imaging scan of brain
demonstrated superficial siderosis, most markedly over
the superior cerebellum, which was grossly atrophic
(Figure 1A). These findings were also noted over
the posterior aspect of the midbrain and around the
calcarine sulci. Imaging of the cervical spine showed
only degenerative changes, with a right-sided ventral
longitudinal intraspinal fluid filled collections (VLISFC)
at the C6/7 level. A CT myelogram to identify the
dural defect was carried out and it confirmed a well-
defined 20x15 mm lesion expanding into the right C7/
T1 intervertebral foramen, consistent with a VLISFC.
Routine blood tests revealed mild iron deficiency anemia,
whilst examination of the cerebrospinal fluid (CSF)
showed an increased ferritin concentration (100 IU/L).
Microtrauma to the internal venous plexus in the epidural
space by CSF leak may be the a reason for red blood cells
seen in the cervical spine.
The patient underwent a C6 to T1 laminectomies and
exploration of the VLISFC in March 2008. Following a
durotomy, the right C8 nerve root was inspected, and the
VLISFC was also explored. There was obvious staining on
inspection of the cord. A small intradural bleeding vessel
was identified, this was in close proximity to the site of
dural aberration but separate from dissection site. This
was coagulated. The defect in the dura was then repaired.
Postoperative imaging taken three months after the
surgery showed that the intramedullary signal change
had become less conspicuous (Figure 1B). The patient’s
radiological changes postoperatively were mirrored by
clinical improvement in his postoperative symptoms over
the course of the two years following the surgical repair.
CASE 2
A right handed patient was referred with a six year
history of a worsening left hemiparesis, progressive
gait ataxia, dysarthria, increasing urinary urgency,
complex double vision in all directions, and declining
cognitive function. The patient’s symptoms followed
recurrent thunderclap headaches eighteen months
previously, which had been investigated at his local
hospital, without a conclusive diagnosis. The patient’s
background history revealed that they had sustained a
C1/2 hangman fracture following a cycling accident 24
years earlier. As a consequence the patient was left with
a Brown-Sequard syndrome, with left pyramidal signs
and a right spinothalamic sensory level from C3 down.
During the accident the patient also sustained a right C5
and C6 brachial plexus avulsion injury, with resultant
right upper limb weakness. Cranial nerve examination
showed disconjugate eye movements with convergence
nystagmus, scanning dysarthria, and cerebellar signs.
Brain MRI scan revealed susceptibility artefacts
along the surface of the cerebellum bilaterally, which
were thought to represent superficial siderosis (Figure
2). There was an appearance of a VLISFC at the level
of C6 and C7. There was also an incidental finding of
tonsillar ectopia in keeping with a Chiari I formation, and
general non-significant cervical degenerative changes.
Intracerebral and spinal angiography did not show any
obvious vascular pathology.
The patient’s symptoms displayed signs of intracranial
hypotension. There is a close association with superficial
siderosis with the underlying mechanism being a dural
tear [7]. Given the patient’s progressive neurological
deterioration, and the imaging findings consistent with
superficial siderosis, the patient agreed to a surgical
International Journal of Case Reports and Images, Vol. 8 No. 1, January 2017. ISSN – [0976-3198]
Int J Case Rep Images 2017;8(1):11–16.
www.ijcasereportsandimages.com
Sinha et al. 13
exploration of the VLISFC. This was undertaken in
August 2008.
Through a posterior spinal approach, a unilateral
partial facetectomy at C6/C7 revealed the capsule of the
VLISFC. The capsule was opened and the cavity fully
explored using the microscope. Staining of the spinal
cord was observed. No bleeding point was identified.
Artificial dura and tissue glue were applied, followed by
layered closure.
Over the two weeks following surgery, the patient’s
cognitive function deteriorated. However, the patient
then slowly recovered to their preoperative baseline.
Subsequently, the patient experienced worsening
episodes of postural headache, and their preoperative
symptoms failed to improve. Postoperative imaging
did not reveal a surgical complication as a cause of the
headaches. Local occupational and speech and language
therapists were enlisted to facilitate his activities of daily
living. In addition, a local hematologist commenced
deferiprone in place of trientine, to aid improvement in
the patient’s functional state.
CASE 3
A right-handed patient was referred with a 10 year
history of deteriorating gait and an eight-year history
of slurring of speech, dysphagia, and diplopia, with
worsening bilateral sensorineural hearing loss worse on
the left side. The patient had sustained a C2 and a C3
vertebral body and left forearm fractures following a road
traffic accident 33 years earlier. At this time, the patient
had undergone and open reduction and internal fixation
of the forearm fractures, and posterior cervical fixation.
The patient had no other significant past medical history.
The patient’s medications comprised baclofen and
detrusitol for bladder dysfunction.
On examination the patient had anosmia, horizontal
nystagmus on lateral gaze, and diplopia on right and left
gaze. The patient had a complete sensorineural hearing
loss on the left and partial loss on the right. The patient
exhibited poor co-ordination of the right upper limb, with
past pointing and dysdiadochokinesia. The tone, power,
and reflexes were normal throughout all four limbs, with
down going plantars. The patient walked with a wide
based gait and their higher mental functions were intact.
A cervical spine x-ray revealed fusion of the spinous
process of C1 and C2, with interspinous wires. A cervical
spine CT scan showed the wiring through the posterior
elements of C1 and C2, with bony fusion. There was fusion
of the peg anteriorly at C1 and fusion of C2 and C3.
An MRI scan of the head revealed evidence of
superficial deposition of blood degradation products
primarily affecting the posterior fossa (Figure 3A).
These were predominantly surrounding the midbrain,
extending down the cervicomedullary junction to the
superior cervical cord and lining the posterior lateral,
and the fourth ventricles. Similar findings, but to a
lesser degree, were seen on the surfaces of both cerebral
hemispheres. There was some signal attenuation
affecting the left lateral semicircular canal which was
most obvious on the axial high resolution images. An
MRI scan of the spine showed that the odontoid peg was
malformed and posteriorly inclined. However, no thecal
sac compression was reported. Metallic artefact was seen
in the soft tissues posteriorly. The visualized spinal cord
was normal in contour. The cervical cord also showed
siderosis on its surface but otherwise was of normal
signal intensity. Intracerebral and spinal angiography
did not show obvious vascular pathology. This could
be due to craniospinal hypovolemia, which may lead to
prominent vasculature on the MRI but negative results
on angiography.
The patient underwent C1-C3 laminectomy through a
posterior spinal approach in March, 2009. A microscopic
intradural exploration revealed a bleeding point, which
was separate from the dissection site. This was cauterized.
The dura was then repaired.
In the postoperative period, the patient noted
worsening headaches, numbness of the right side of his
body, and he became doubly incontinent. A subsequent
MRI scan revealed a large irregular VLISFC in the
Figure 1: Showing black rimmed hypointensity in the superior
cerebellum due to hemosiderin deposits on the (A) T2 coronal
and (B) T1 sagittal MRI views, (C) Showing postoperative T2,
sagittal MRI views three months later with less conspicuous
signal change in the superior cerebellum.
International Journal of Case Reports and Images, Vol. 8 No. 1, January 2017. ISSN – [0976-3198]
Int J Case Rep Images 2017;8(1):11–16.
www.ijcasereportsandimages.com
Sinha et al. 14
posterior extraspinal tissues. The patient underwent a
re-exploration of the surgical site and the VLISFC was
repaired.
With intense neurorehabilitation the patient regained
fecal continence and the patient’s urinary sphincter
control improved. However, the patient’s right-sided
sensory loss persisted. The patient’s most recent MRI
showed focal cord atrophy at the level of C2. The
previously noted VLISFC was no longer visualized (Figure
3B). The patient was subsequently transferred to his local
neurorehabilitation unit.
DISCUSSION
Superficial siderosis is a rare condition [3, 8], due
to chronic hemorrhage into the subarachnoid space [1].
Historically, the diagnosis of superficial siderosis was
made by histological examination of biopsied tissue, or
at autopsy. However, in the modern era, MRI scan can
facilitate the diagnosis, especially the gradient-echo
T2-weighted images which have a high sensitivity for
hemosiderin deposition [8].
Macroscopically, superficial siderosis is apparent by
the brownish discoloration of the leptomeninges and
adjacent parenchyma, with a predilection for the superior
vermis, crest of the cerebellar folia, basal frontal lobe,
temporal cortex, brainstem, spinal cord, nerve roots, and
cranial nerves I, II, V, VIII, and X [6]. At a microscopic
level, hemosiderin deposition occurs within macrophages
in the perivascular spaces, and along the pial vessel walls.
This may lead to neuronal loss, gliosis, and demyelination
[6]. The hemosiderin is derived from the breakdown of
heme within the glia and the microglia. These cells will
synthesize ferritin, however, once their biosynthesis
capabilities have been exceeded, hemosiderin is produced.
Unbound ferric ions mediate apoptosis by free radical
mechanisms [8]. The iron deposits on the CNS surfaces
bathed by cerebrospinal fluid have a paramagnetic
effect on gradient echo sequences on MRI (T2-weighted
images), giving a characteristic black rimmed area of
hypointensity [9, 10].
The aim of treatment is to prevent progression of the
neurological deficit. Medical and surgical options have
been proposed. Medical management includes the use of
iron chelators, with variable success [3, 8, 11]. Trientine
has been associated with iron and copper chelation,
but may cause increased iron levels within the liver,
presumably due to its interference with ceruloplasmin
[3].
Surgical management of superficial siderosis involves
identifying and terminating the source of chronic bleeding
[3]. Posti et al. reported that of 27 cases undergoing
surgical intervention, disease progression was halted
in 13 patients, four patients clinically improved, five
patients had further clinical deterioration, whilst five
patients sustained other complications [12]. Kumar et al.
reported a case of superficial siderosis where intradural
Figure 2: Showing area of black rimmed hypointensity along
the surface of the cerebellum bilaterally T2, axial, coronal and
sagittal MRI views respectively
Figure 3: Showing black rimmed hypointensity of the
cerebellum on T2 (A) axial and (B) Coronal MRI views. Showing
no significant change in the area of black rimmed hypointensity
postoperatively on T2 (C) Axial and (D) Sagittal views.
International Journal of Case Reports and Images, Vol. 8 No. 1, January 2017. ISSN – [0976-3198]
Int J Case Rep Images 2017;8(1):11–16.
www.ijcasereportsandimages.com
Sinha et al. 15
exploration did not identified a bleeding source [7, 9,
10, 13, 14]. Egawa et al. reported two cases of superficial
siderosis where the patients had dural defects with
fluid-filled collections in the spinal canals, which were
successfully closed [2]. One of the patients had cessation
of further neurological deterioration, whilst the other
partially deteriorated after surgery.
To date, 30 cases of superficial siderosis reported in
the literature have undergone surgical intervention, and
in five individuals no source of bleeding was identified
intraoperatively. Disease progression has either been
halted or there has been neurological improvement in 18
of these cases thus far.
All our three cases were referred late to our
quaternary centre from other hospitals, this was perhaps
due to delay in presentation together with difficulty in
diagnosing this rare and obscure disease. All three cases
had negative angiography. Previous studies of patients
with cranial cerebellar superficial siderosis have shown
to have negative angiography [3, 15]. The authors
recognize that angiography seems to have limited
sensitivity.
Post-traumatic VLISFC was deemed a potential
target for a bleeding vessel and therefore explored.
Intraoperatively all three patients had obvious staining
of the spinal cord. Authors considered biopsy of the
pia arachnoid but believed it to be hazardous and of no
additional benefit to the patients. The two cases which
revealed the intradural microscopic bleeding vessel were
entirely separate from the dissection site. The intradural
spinal artery after the arachnoid layer opened is usually
bloodless and were not damaged intraoperatively in
these cases. The intradural microscopic bleeding vessels
were likely bleeding over a period of years. This is clearly
unusual and the authors cannot explain this as the normal
coagulation cascade should have stopped this process.
However, Tapscott el al. have previously described of a
case where superficial siderosis developed more than a
decade after the traumatic brachial nerve root avulsion
[16]. Angiography was not able to locate a specific
bleeding source but surgical repair of the meningeal
diverticulum and venous cauterization helped reduce
the overall central nervous system bleeding.
Our cases reveal that patients with superficial
siderosis often develop severe functional impairment
due to the progressive nature of the disease. This may
significantly compromise their quality of life. The first
case showed a positive outcome at two years. However,
the second case highlights the possibility of a negative
intradural exploration. The third case demonstrates that
quality of life is not necessarily improved even if there
is confirmed cessation of an intradural bleeding point.
On balance, we are of the opinion that early craniospinal
imaging and surgical exploration should be undertaken,
at least to attempt to halt neurological deterioration.
CONCLUSION
Patients with superficial siderosis often develop
myriad of neurological symptoms and signs that lead
to severe functional impairment due to its progressive
nature. On balance, we are of the opinion that early
craniospinal imaging and surgical exploration should
be undertaken, at least to attempt to halt neurological
deterioration.
*********
Author Contributions
Pranab Sinha – Substantial contributions to concept
and design, Drafting the article, Revising it critically
for important intellectual content, Final approval of the
paper to be published
Sophie Jane Camp – Substantial contributions to concept
and design, Drafting the article, Revising it critically for
important intellectual content, Final approval of the
paper to be published
Harith Akram – Substantial contributions to concept
and design, Drafting the article, Revising it critically
for important intellectual content, Final approval of the
paper to be published
Robin Bhatia – Substantial contributions to concept
and design, Drafting the article, Revising it critically
for important intellectual content, Final approval of the
paper to be published
Adrian Thomas Carlos Hickman Casey – Substantial
contributions to concept and design, Drafting the article,
Revising it critically for important intellectual content,
Final approval of the paper to be published
Guarantor
The corresponding author is the guarantor of submission.
Conflict of Interest
Authors declare no conflict of interest.
Copyright
© 2017 Pranab Sinha et al. This article is distributed
under the terms of Creative Commons Attribution
License which permits unrestricted use, distribution
and reproduction in any medium provided the original
author(s) and original publisher are properly credited.
Please see the copyright policy on the journal website for
more information.
REFERENCES
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2. Egawa S, Yoshii T, Sakaki K, et al. Dural closure for
the treatment of superficial siderosis. J Neurosurg
Spine 2013 Apr;18(4):388–93.
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www.ijcasereportsandimages.com
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3. Fearnley JM, Stevens JM, Rudge P. Superficial
siderosis of the central nervous system. Brain 1995
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6. Koeppen AH, Dentinger MP. Brain hemosiderin and
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Context: Cervical root avulsion secondary to traumatic plexus injury is a rare etiology of superficial siderosis (SS) of the central nervous system (CNS). We describe the case of an isolated progressive compressive myelopathy revealing this complication and discuss the pathogenesis of such a presentation, its clinical and imaging peculiarities with a literature review. Findings: We report on the case of a 48-year-old man with history of left brachial plexus injury at the age of 2 years. Since the age of 38 years, he had presented with a progressive paraplegia, bladder and erectile dysfunction, neuropathic pain and sensory level. The diagnosis was made by spinal cord and brain magnetic resonance follow-up imaging revealing hypointensity T2-weighted gradient echo linear dark rim around the entire neuraxis and cervical dural pseudomeningoceles. These MRI findings were suggestive of extensive hemosiderin deposition consolidating the diagnosis of SS of CNS. Conclusion/Clinical relevance: Our case report illustrates diagnosis difficulties in unusual or paucisymptomatic presentations of SS. A history of brachial plexus trauma with nerve root avulsion should prompt gradient-echo T2-weighted imaging to bring out such a complication. Superficial siderosis of the CNS should be included in the panel of differential diagnosis of the parethospastic syndromes and compressive myelopathy.
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Superficial siderosis of the central nervous system results from chronic or intermittent hemorrhage into the subarachnoid space that causes hemosiderin deposition in subpial layers of the brain and the spinal cord leading to neuronal damage. Patients present with progressive and debilitating symptoms that typically include adult-onset slowly progressive cerebellar gait ataxia and sensorineural hearing impairment. Regardless of extensive investigations, the origin of the hemorrhage is often not clear. Because of the good availability of magnetic resonance imaging, asymptomatic cases of superficial siderosis of the central nervous system are increasingly discovered. SS cases are increasingly reported in the literature. We present three new cases. The etiology, pathogenesis, clinical features, and treatment options of SS are reviewed.
Article
Superficial siderosis (SS) of the CNS is a rare disease caused by repeated hemorrhages in the subarachnoid space. The subsequent deposition of hemosiderin in the brain and spinal cord leads to the progression of neurological deficits. The causes of bleeding include prior intradural surgery, carcinoma, arteriovenous malformation, nerve root avulsion, and dural abnormality. Recently, surgical treatment of SS associated with dural defect has been reported. The authors of the present report describe 2 surgically treated SS cases and review the literature on surgically treated SS. The patients had dural defects with fluid-filled collections in the spinal canal. In both cases, the dural defects were successfully closed, and the fluid collection was resolved postoperatively. In one case, the neurological symptoms did not progress postoperatively. In the other case, the patient had long history of SS, and the clinical manifestations partially deteriorated after surgery, despite the successful dural closure. In previously reported surgically treated cases, the dural defects were closed by sutures, patches, fibrin glue, or muscle/fat grafting. Regardless of the closing method, dural defect closure has been shown to stop CSF leakage and subarachnoid hemorrhaging. Successfully repairing the defect can halt the disease progression in most cases and may improve the symptoms that are associated with CSF hypovolemia. However, the effect of the dural closure may be limited in patients with long histories of SS because of the irreversibility of the neural tissue damage caused by hemosiderin deposition. In patients with SS, it is important to diagnose and repair the dural defect early to minimize the neurological impairments that are associated with dural defects.
Article
Recent studies have revealed that some patients with superficial siderosis (SS) show evidence of an intraspinal fluid-filled collection on imaging. Some of these patients also show clinical and/or imaging features of craniospinal hypovolemia related to dural defects. We report a patient with SS whose clinical presentation was suggestive of motor neuron disease and whose history was remarkable for cerebrospinal fluid (CSF) hypovolemia. This report also reviews the literature on the relationship between SS, dural defects, and CSF hypovolemia. A 58-year-old left-handed man was evaluated for an 18-month history of progressive imbalance with limb muscle weakness, wasting, and fasciculations. Brain magnetic resonance imaging (MRI) studies were remarkable for evidence of SS and diffuse pachymeningeal enhancement similar to that seen in craniospinal hypovolemia. Spine MRI showed a longitudinal intraspinal fluid-filled collection. A dynamic computed tomographic myelogram of the spine showed a CSF leak adjacent to a peripherally calcified disk at the T2-3 level. Following repair of the dural defect the patient noted an improvement in balance and strength and resolution of the fasciculations. A cervical and thoracic spine MRI showed resolution of the intraspinal fluid-filled collection, and a CSF study showed no red blood cells or xanthochromia. The clinical spectrum of disorders related to dural defects includes craniospinal hypovolemia, SS-related ataxia and impaired hearing, segmental weakness and atrophy with or without hyperreflexia, and spinal cord herniation. The clinical features of these conditions may overlap. Longitudinally extensive ventral dissecting meningoceles can be seen in all these conditions. A dynamic computed tomographic myelogram can identify a dural defect. In some cases the dural defect may result from an osteophyte.
Article
A source of bleeding is often not evident during the evaluation of patients with superficial siderosis of the CNS despite extensive imaging. An intraspinal fluid-filled collection of variable dimensions is frequently observed on spine MR imaging in patients with idiopathic superficial siderosis. A similar finding has also been reported in patients with idiopathic intracranial hypotension. The authors report on a patient with superficial siderosis and a longitudinally extensive intraspinal fluid-filled collection secondary to a dural tear. The patient had a history of low-pressure headaches. His spine MR imaging and spine CT suggested the possibility of an underlying vascular malformation, but none was found on angiography. Repair of the dural tear resulted in resolution of the intraspinal fluid collection and CSF abnormalities. The significance of the association between superficial siderosis and idiopathic intracranial hypotension, and potential therapeutic and pathophysiological implications, are the subject of this report.
Article
Despite extensive imaging, a source of bleeding is often not evident during the evaluation of patients with superficial siderosis (SS) of the central nervous system. An intraspinal fluid-filled collection of variable dimensions is frequently seen on spine MR imaging in patients with idiopathic SS. A similar finding has also been reported in patients with craniospinal hypotension. This review discusses the role of multitechnique imaging in the work-up of patients with SS and focuses on recent developments.
Article
Superficial siderosis is a rare condition resulting from the presence of chronic bleeding into the subarachnoid space usually causing gait instability and deafness. The surgical management of superficial siderosis depends on localizing the source of hemorrhage. The surgical treatment of this rare condition has not been well described in the literature. We present a case illustrating the surgical treatment for superficial siderosis. Case report. The authors describe the case report of a 70-year-old gentleman with gait instability and deafness found to have an abnormal communication between the spinal epidural venous plexus and the subarachnoid space. The source of hemorrhage into the subarachnoid space was identified to be a fistula in the ventral thoracic dural. A costotransversectomy approach was undertaken at the T4-T5 level to expose the fistula. The abnormal communication was patched and sealed. The patient's symptoms remained stable on follow-up at 15 months with no worsening of his symptoms. Superficial siderosis is a neurologic disorder that arises from chronic hemosiderin deposition into the subarachnoid space. The progressive nature of the disease can be halted if a source of hemorrhage can be found and treated surgically.
Article
Chronic or intermittent extravasations of blood into the subarachnoid space, and dissemination of heme by circulating cerebrospinal fluid, are the only established causes of superficial siderosis of the central nervous system (CNS). We studied the autopsy tissues of nine patients by iron histochemistry, immunocytochemistry, single- and double-label immunofluorescence, electron microscopy of ferritin, and high-definition X-ray fluorescence. In one case, frozen brain tissue was available for quantitative assay of total iron and ferritin. Siderotic tissues showed extensive deposits of iron and ferritin, and infiltration of the cerebellar cortex was especially severe. In addition to perivascular collections of hemosiderin-laden macrophages, affected tissues displayed iron-positive anuclear foamy structures in the neuropil that resembled axonal spheroids. They were especially abundant in eighth cranial nerves and spinal cord. Double-label immunofluorescence of the foamy structures showed co-localization of neurofilament protein and ferritin but comparable merged images of myelin-basic protein and ferritin, and ultrastructural visualization of ferritin, did not allow the conclusion that axonopathy was simply due to dilatation and rupture of fibers. Heme-oxygenase-1 (HO-1) immunoreactivity persisted in macrophages of siderotic cerebellar folia. Siderosis caused a large increase in total CNS iron but high-definition X-ray fluorescence of embedded tissue blocks excluded the accumulation of other metals. Holoferritin levels greatly exceeded the degree of iron accumulation. The susceptibility of the cerebellar cortex is likely due to Bergmann glia that serve as conduits for heme; and the abundance of microglia. Both cell types biosynthesize HO-1 and ferritin in response to heme. The eighth cranial nerves are susceptible because they consist of CNS axons, myelin, and neuroglial tissue along their subarachnoid course. The persistence of HO-1 protein implies continuous exposure of CNS to free heme or an excessively sensitive transcriptional response of the HO-1 gene. The conversion of heme iron to hemosiderin probably involves both translational and transcriptional activation of ferritin biosynthesis.
Article
Brain tissue from five patients with superficial siderosis of the central nervous system was examined by immunocytochemistry for ferritin, glial fibrillary acidic protein (GFAP), alpha 1-antitrypsin, and alpha 1-antichymotrypsin, and by lectin affinity cytochemistry with biotinylated Ricinus communis agglutinin-1 (RCA-1). The sections were pretreated with 2,2'-dipyridyl and sodium hydrosulfite to remove iron and to reveal the antigenic sites. In siderotic cerebellar cortex, ferritin reaction product occurred in the hemosiderin matrix, the cell bodies and processes of Bergmann glia, and in microglia. Astrocytes other than Bergmann glia did not contain ferritin reaction product. RCA-1 stained microglia and hemosiderin whereas antisera to alpha 1-antitrypsin and alpha 1-antichymotrypsin only reacted with iron-depleted granules. The selective vulnerability of the eighth cranial nerve was explained by the presence of ferritin-reactive and lectin-positive microglia. Hemosiderin isolated from frozen cerebellum contained ferritin, GFAP, and vimentin. The presence of the intermediate filament proteins was likely due to co-localization with hemosiderin granules in Bergmann glia. The ability of the brain to biosynthesize ferritin in response to prolonged contact with hemoglobin iron is thought to be the most important factor in the pathogenesis of superficial siderosis. The great severity of the lesion in the exposed cerebellar cortex is readily explained by accelerated ferritin biosynthesis in Bergmann glia.
Article
There have been 87 cases of superficial siderosis of the CNS reported in the world literature and 63 cases had developed the clinical syndrome with sufficient details to be reviewed. It is a distinct clinical syndrome characterized by sensorineural deafness (95%), cerebellar ataxia (88%) and pyramidal signs (76%). Other features include dementia (24%), bladder disturbance (24%), anosmia (at least 17%), aniscoria (at least 10%) and sensory signs (13%). Less frequent features are extra-ocular motor palsies, neck or backache, bilateral sciatica and lower motor neuron signs (5-10% each). Males are more often affected than females (3:1). The age of onset ranged from 14 to 77 years, age at death from 29 to 78 years and duration until death from 1 to 38 years excluding premature death due to the underlying cause or as a result of surgery. Up to 27% become bed bound at 1-37 years from the first symptom due to either cerebellar ataxia, a myelopathic syndrome or both. Symptomatic subarachnoid haemorrhage occurred in 37% and the CSF was haemorrhagic and/or xanthochromic in 75%. It is now accepted that superficial siderosis is due to chronic subarachnoid haemorrhage and a source of bleeding has been reported in 54% of cases; it was either due to dural pathology (47%) including a CSF cavity lesion or cervical root lesion, a vascular tumour (35%) or a vascular abnormality (18%). Arguments are presented that the remaining cases were also due to chronic haemorrhage and that there is no evidence for a non-haemorrhagic form of superficial siderosis. There have been 14 incidental cases diagnosed by MRI or at post-mortem with no symptoms attributable to superficial siderosis during life, supporting the notion of a pre-symptomatic phase to the illness. In 22 patients who had developed the syndrome, the duration of this pre-symptomatic phase could be calculated and ranged from 4 months to 30 years with an average of 15 years. At present the most promising treatment for superficial siderosis is surgical ablation of the bleeding sources.