Accepted Manuscript A poorly known CSF shunt complication: Miyazaki’s syndrome Riccardo Caruso, Venceslao Wierzbicki, colonel M.D., Luigi Marrocco, colonel M.D., Alessandro Pesce, MD, Emanuele Piccione, captain MD PII:
S1878-8750(15)00432-5
DOI:
10.1016/j.wneu.2015.04.030
Reference:
WNEU 2855
To appear in:
World Neurosurgery
Received Date: 28 January 2015 Revised Date:
9 April 2015
Accepted Date: 11 April 2015
Please cite this article as: Caruso R, Wierzbicki V, Marrocco L, Pesce A, Piccione E, A poorly known CSF shunt complication: Miyazaki’s syndrome, World Neurosurgery (2015), doi: 10.1016/ j.wneu.2015.04.030. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
A poorly known CSF shunt complication: Miyazaki’s syndrome Riccardo Caruso: Associate Professor of
Neurosurgery, Department of
Neurology and
RI PT
Psychiatry Sapienza University of Rome, Chief of the Neurosurgical Division of the Rome Army Medical Center.
Venceslao Wierzbicki: colonel M.D. Neurosurgical Division of the Rome Army Medical Center
SC
Luigi Marrocco: colonel M.D. Neurosurgical Division of the Rome Army Medical Center
Rome
M AN U
Alessandro Pesce, MD, Neurosurgical Division Sant’Andrea Hospital – Sapienza University of
Emanuele Piccione, captain MD, Neurosurgical Division and Radiological Division of the Rome Army Medical Center author:
Riccardo
Caruso
e-mail:
[email protected],
TE D
Corresponding
[email protected], tel.-fax: +39063227174; mobile phone: +393381716244; full postal address:
EP
via Pietro da Cortona 1 c.a.p. 00196 Roma Italy Keywords: intracranial CSF hypotension, myelopathy, schunt, epidural venous engorgement
AC C
Abbreviations list.
CSF: cerebrospinal fluid
ICP: intracranial pressure
MS: Miyazaki’s syndrome
ACCEPTED MANUSCRIPT
Abstract
RI PT
We studied a poorly known form of CSF hypotension characterized by cervical myelopathy, a considerable growth in volume of the venous plexus of the cervical spine and absence of headache. This form was first described by Miyazaki. We reported a case brought to our
all the various clinical aspects of this pathology
SC
attention, we reviewed the literature and formulated etiopathogenic theories that might explain
M AN U
Keywords: intracranial CSF hypotension, myelopathy, shunt, epidural venous engorgement
Introduction
TE D
The spontaneous or secondary intracranial hypotension syndrome is a well known pathophysiological entity[1]. First described by Schaltenbrand in 1938[2], the syndrome has been the subject of several publications[3-5] reported between the 1960s and early 1990s
EP
thoroughly describing most pathologies involving CSF such as volume depletion due to CSF leakage or CSF shunt over-drainage. There is however a form of CSF hypotension that has not
AC C
been studied in depth and is rarely described in the Literature. It is characterized by cervical myelopathy, a considerable growth in volume of the venous plexus of the cervical spine, absence of headache or nausea and any other symptom usually associated with intracranial hypotension. This pathology was first described by Miyazaki et al. in 1998.[6] We treated one case, which has led us to research the subject in depth. First we reviewed the Literature and then formulated
ACCEPTED MANUSCRIPT
etiopathogenic theories that might explain all the various clinical aspects of this pathology, which we shall refer to as the Miyazaki’s syndrome (MS)
RI PT
Case report In October 2012, a 35 year-old man was brought to us; he had been suffering for about 7 years from progressive spastic tetraplegia. When he was 18 months old he had undergone surgery
SC
using a medium pressure Pudenz ventriculo-atrial shunt to treat tetraventricular communicating hydrocephalus with the presence of a large supra- and infratentorial arachnoid cyst and agenesis
M AN U
of the corpus callosum. When the patient was 9 years old it had been necessary to change the catheter adding a ventriculo-peritoneal shunt with high-pressure valve without antisiphon device. After undergoing this second operation the patient, who had regular check-ups and neuroimaging scans, felt fine, had a normal social life and went to school like everyone else; he was able to
TE D
practice sports such as skiing, swimming, and tennis; went to University and even studied abroad. At 21 he began to work.
Unfortunately at the age of 28 he showed slow yet progressive difficulty in walking: it became
EP
impossible for him to take footsteps and eventually he became unable to stand on his feet without support. In the year prior to our examination, the patient had also observed loss of strength in his
AC C
arms. The Patient was seen by various neurologists, neurosurgeons and orthopaedists who performed numerous MRI scans of the brain; these did not show the presence of hydrocephalus or CSF hypotension.
Upon our advice the patient underwent a metabolic MRI of the brain and a cervical MRI scan; this last scan showed a considerable enlargement of the high cervical venous plexus with compression of the spinal cord. The Patient then underwent a CT angiography (fig.1), a venous
ACCEPTED MANUSCRIPT
Doppler ultrasound scan of the neck, which showed a right hand-side obstruction of the internal jugular vein, where the cardiac catheter of the first shunt still remained, and an angiography with study of cranial and cervical circulation, which did not reveal dural arteriovenous fistulas. At this
as the cause for the veins’ swelling. ®
-P- Tel, Raumedic AG, Helmbrechts Germany) to
SC
We applied a telemetric probe (Neurovent
RI PT
point we began to suspect, despite the absence of a headache, the presence of CSF hypotension
measure the ICP and monitored the patient for four weeks (fig.2A), the ICP values were always
M AN U
very low: -8/-10 mmHg when lying down and outside of the scale and thus non-measurable when standing. We therefore decided to close the distal catheter and we kept monitoring the ICP, which over approximately 45 days, very slowly, climbed back up to values of 8/10 mmHg when lying down (fig.2B). We monitored progress for another month and there were no further variations in ICP so we removed the probe and decided the patient no longer needed the CSF
TE D
shunt.
Follow up MRI scans and CT angiography after 3,6 and 12 months from the closing of the shunt
EP
showed a progressive reduction , which in the end was over 50%, of the cervical venous swelling (fig.3) and an unaltered cerebral context compared to before the surgery. The patient showed a
AC C
clear clinical improvement and today, despite still retaining a high level of spasticity, he can walk on his own, unaided.
Literature review. Materials, Methods and Results. Using appropriate keywords we searched various electronic databases including PubMed, ISIWeb, Scopus and Google Scholar for all articles in English from 1970 to the present date that presented a clinical situation similar to ours; in other words: CSF hypotension, swelling of the
ACCEPTED MANUSCRIPT
extra-dural cervical venous plexus, myelopathy, no headache nor any other typical symptom of intracranial hypotension syndrome. We only found 6 cases (tab.1)[6-11]. In all of them the cause of the hypotension was over drainage of CSF due to a ventricular-peritoneal shunt (VP shunt).In
didn’t include the type of malfunctioning shunt.
RI PT
5 cases the malfunctioning shunts had no antisiphon system, the case described by Howard [11]
SC
The six cases of MS comprised equally men and women across a wide age range: 17 to72 years (average 35.5); in 4 cases the first shunt had been introduced in childhood, while in the other two
M AN U
in adult age. In all cases but one there had been direct report of low CSF pressure; in the case treated by Ulrich[10] the low pressure was hypothesized on the basis of neuroimages only and then confirmed ex adjuvantibus as the symptoms resolved after substitution of the shunt valve with one that needed higher pressure to open. All patients showed a degree of improvement after
deteriorated. Discussion
TE D
the substitution or change of the type of shunt except for one who refused surgery and thus
EP
The two typical aspects that connect our case with the other 6 described in the Literature are the absence of orthostatic headache, which is typical of CSF hypotension and the presence of
AC C
cervical myelopathy caused by an abnormal swelling of the cervical venous plexus. Cases of CSF hypotension with abnormal MR findings and no headache have been described in the Literature (Mokri’s classifiction type IV)[1], but are rare (there have only ever been 11 altogether, including the MS ones) and no one has ever tried to explain the absence of a headache. The typical orthostatic headache in relation to CSF hypotension syndrome is usually explained with the traction and distortion of pain-sensitive suspending structures of the brain due
ACCEPTED MANUSCRIPT
to a sagging of the brain[12]. We must therefore hypothesize that the absence of a headache is linked to the absence of the aforementioned traction and distortion or that the same happened so slowly and became chronic in a way that did not activate the nerve endings susceptible to pain.
RI PT
Probably in the MS cases both mechanisms occur and these are somehow connected to the swelling of the cervical veins that would become a sort of supporting cushion for the structures
SC
above and also an obstacle for CSF flow.
The swelling of the peridural cervical venous plexus, as well as the cerebral one, in the presence
M AN U
of CSF hypotention has been described in various cases[12-15] and explained with the MonroKellie doctrine[16], but except in the MS
it is never associated with myelopathy. In the
Literature on the other hand there are cases of CSF hypotension with spinal compression caused by the accumulation of cervical fluid[17-19].
TE D
One could hypothesize that the presence of myelopathy in the MS may be caused not only by the compression of the spine due to the swollen plexus, but also and most importantly by the spinal circulation being hampered, with difficult blood-flow in the veins[20]. A similar, but not
EP
identical situation as that caused by dural arteriovenous fistulas would ensue; in the latter pathology the blood-flow is hindered by pressure growth in the veins caused by the direct
AC C
passage of arterial blood in the venous system. In the MS on the other hand there would be a slow yet progressive growth of venous circulation, which over time would become so substantial as to hinder flow, this would consequently lead to stagnation in the microcirculation of the spine. The few, unidentifiable symptoms at the onset of MS and the consequent considerable amount of time before a possible diagnosis can be made would certainly play an important role[15]; what could also facilitate the obstruction of the veins is the anatomical pattern of the flow in the
ACCEPTED MANUSCRIPT
cervical venous plexus[21-23] especially when, like in our case, the right internal jugular is closed and there is not an anti-siphon system .
RI PT
Hypotension in the MS is difficult to diagnose because there is no headache and the myelopathy establishes itself slowly, over time; we therefore believe that it can be misdiagnosed and it could potentially be less rare than it may appear by reviewing the Literature. We formulated a
SC
diagnosis with much difficulty and in stages. On one hand we were faced with the absence of any headache and scans that showed no change in the cerebral situation of the patient for years and
over time.
M AN U
on the other hand a progressive paralysis of all four limbs that had been inexorably happening We carried out an MRI scan only to ascertain that there was no spinal cord
compression due to other pathologies. We were actually really surprised by the substantial swelling of the venous plexus shown by the images and initially we thought that it might have been caused by an arteriovenous dural fistula. It was only when the angiography showed that
TE D
there wasn’t a fistula that we were able to diagnose CSF hypotension. In light of our experience, we believe that MS should be taken into consideration when running
EP
differential diagnostic procedures in patients who have shunts and who show signs of myelopathy, but do not suffer from headaches. We advise to immediately run an MRI of the
AC C
brain and spine with focus on the venous blood flow. Should the cervical venous plexus be swollen, even if there aren’t any other typical images of hypotension, we believe the ICP should be immediately monitored. We also believe that, possibly, in some cases this syndrome might be caused not only by secondary hypotension, but also by spontaneous hypotension[20].
ACCEPTED MANUSCRIPT
Legend fig 1 preoperative (presurgery) CT angiography showing the cervical epidural venous
RI PT
engorgement : A axial view. B coronal view. C 3D reconstruction fig 2 A: ICP before the closure of the shunt B: ICP after the closure of the shunt
SC
fig 3 CT angiography showing improvement of the cervical epidural venous engorgement :
age-sex
schunt cause
Miyazaki[6]
54y M
Wingerchuk [7]
72y W
Liu[8]
18y W
Wolfe[9]
17y M
Ulrich[10]
17y M
Howard[11]
26y W
hydrocephalus after ESA Hydrocephalus for a posterior fossa meningioma Cyst from perinatal ischemia hydrocephalus after radioterapy obstructive hydrocephalus due to a retrocerebellar cyst congenital hydrocephalus
length clinical history 7m
treatment
outcome
closing shunt
improved
4y
no
worse
replacement of the old with a programmable shunt replacement of the old with a programmable shunt replacement of the old with a programmable shunt
improved
AC C
EP
TE D
first author
M AN U
A axial view. B coronal view.C 3D reconstruction
4m ?
2y
1m
replacement of the old with a programmable shunt
tab.1 legends: M man, W woman, m month, y year, ? unknown
improved improved
improved
ACCEPTED MANUSCRIPT
References
[7] [8]
[9]
[10]
[11] [12] [13]
[14]
[15]
[16] [17] [18]
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SC
[6]
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[5]
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[3] [4]
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[2]
Mokri B. Cerebrospinal fluid volume depletion and its emerging clinical/imaging syndromes. Neurosurgical focus 2000;9(1):e6. Schaltenbrand G. Neure anschauungen zur pathophyiologie der liquorzirkulation. Zentralbl Neurochir 1938;3:290-300. Bell WE, Joynt RJ, Sahs AL. Low spinal fluid pressure syndromes. Neurology 1960;10:512–21. Lipman IJ. Primary intracranial hypotension: the syndrome of spontaneous low cerebospinal fluid pressure with traction headache. Diseases of the nervous system 1977;38(3):212-3. Marcelis J, Silverstein SD. Spontaneous low cerebrospinal fluid pressure headache. Headache 1990;30:192–6. Miyazaki T, A. C, Nishina H, Uesaka Y, Nakase H, Kanazawa I. Upper cervical myelopathy associated with low CSF pressure: A complication of ventriculoperitoneal shunt. Neurology 1998;50:1864-6. Wingerchuk DM, Patel NP, Patel AC, Dodick DW, Nelson KD. Progressive cervical myelopathy secondary to chronic ventriculoperitoneal CSF overshunting. Neurology 2005;65(1):171-2. Liu JK, Gottfried ON, Brockmeyer DL. Epidural venous engorgement resulting in progressive cervical myelopathy from shunt-related intracranial hypotension. Case report and review of the literature. Journal of neurosurgery 2006;105(6 Suppl):499-503. Wolfe SQ, Bhatia S, Green B, Ragheb J. Engorged epidural venous plexus and cervical myelopathy due to cerebrospinal fluid overdrainage: a rare complication of ventricular shunts. Case report. Journal of neurosurgery 2007;106(3 Suppl):227-31. Ulrich NH, Maier M, Bernays RL, Krayenbuhl N, Kollias S. Cervical myelopathy due to chronic overshunting in a pediatric patient: case report and review of the literature. Turkish neurosurgery 2013;23(3):410-4. Howard BM, Sribnick EA, Dhall SS. Over-shunting associated myelopathy. Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia 2014;21(12):2242-4. Paldino M, Mogilner AY, Tenner MS. Intracranial hypotension syndrome: a comprehensive review. Neurosurgical focus 2003;15(6):ECP2. Nakahara K, Iida H, Mitomi T, Osawa S, Utsuki S, Shimizu S, Fujii K. Dilation of spinal epidural veins caused by spontaneous intracranial hypotension: case report. Neurologia medicochirurgica 2005;45(8):404-6. Moayeri NN, Henson JW, Schaefer PW, Zervas NT. Spinal dural enhancement on magnetic resonance imaging associated with spontaneous intracranial hypotension. Report of three cases and review of the literature. Journal of neurosurgery 1998;88(5):912-8. Martinez-Lage JF, Alarcon F, Alfaro R, Ruiz-Espejo A, Lopez-Guerrero AL, Hernandez-Abenza J. Cervical extramedullary mass lesion due to chronic CSF overshunting: case report and literature review. Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery 2009;25(7):895-8. Mokri B. The Monro-Kellie hypothesis: applications in CSF volume depletion. Neurology 2001;56(12):1746-8. Schievink WI, Chu RM, Maya MM, Johnson JP, Cohen HCM. Spinal manifestations of spontaneous intracranial hypotension. Journal of neurosurgery. Spine 2013;18:96-101. Rabin BM, Roychowdhury S, Meyer JR, Cohen BA, LaPat KD, Russell EJ. Spontaneous intracranial hypotension: spinal MR findings. AJNR. American journal of neuroradiology 1998;19(6):1034-9.
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[1]
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[23]
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[22]
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[21]
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[20]
Yousry I, Forderreuther S, Moriggl B, Holtmannspotter M, Naidich TP, Straube A, Yousry TA. Cervical MR imaging in postural headache: MR signs and pathophysiological implications. AJNR. American journal of neuroradiology 2001;22(7):1239-50. Nowak DA, Rodiek SO, Zinner J, Guhlmann A, Topka H. Broadening the clinical spectrum: unusual presentation of spontaneous cerebrospinal fluid hypovolemia. Case report. Journal of neurosurgery 2003;98(4):903-7. Braun JP, Tournade A. Venous Drainage in the Craniocervical Region. Neuroradiology 1997;13:155-8. Chaynes P, Verdiè JC, Moscovici J, Zadeh J, Vaysse P, Becue J. Microsurgical anatomy of the internal vertebral venous plexuses. Surg Radiol Anat 1998;20(47-51). Doepp F, Schreiber SJ, von Munster T, Rademacher J, Klingebiel R, Valdueza JM. How does the blood leave the brain? A systematic ultrasound analysis of cerebral venous drainage patterns. Neuroradiology 2004;46:565–70.
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[19]
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SC
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SC
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Highlights
RI PT
SC M AN U TE D
•
EP
•
We examine a form of CSF hypotension that is characterized by cervical myelopathy, a considerable growth in volume of the venus plexus of the cervical spine, absence of headache We formulate etiopathogenic theories that might explain all the various clinical aspects of this pathology we believe that this particular form of CSF Hypotension can be misdiagnosed and it could potentially be less rare than it may appear by reviewing the literature
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•
ACCEPTED MANUSCRIPT
Conflicts of interest
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All authors have no conflicts of interest
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EP
TE D
M AN U
SC
prof. Riccardo Caruso
S1878-8750(15)00432-5
DOI:
10.1016/j.wneu.2015.04.030
Reference:
WNEU 2855
To appear in:
World Neurosurgery
Received Date: 28 January 2015 Revised Date:
9 April 2015
Accepted Date: 11 April 2015
Please cite this article as: Caruso R, Wierzbicki V, Marrocco L, Pesce A, Piccione E, A poorly known CSF shunt complication: Miyazaki’s syndrome, World Neurosurgery (2015), doi: 10.1016/ j.wneu.2015.04.030. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
A poorly known CSF shunt complication: Miyazaki’s syndrome Riccardo Caruso: Associate Professor of
Neurosurgery, Department of
Neurology and
RI PT
Psychiatry Sapienza University of Rome, Chief of the Neurosurgical Division of the Rome Army Medical Center.
Venceslao Wierzbicki: colonel M.D. Neurosurgical Division of the Rome Army Medical Center
SC
Luigi Marrocco: colonel M.D. Neurosurgical Division of the Rome Army Medical Center
Rome
M AN U
Alessandro Pesce, MD, Neurosurgical Division Sant’Andrea Hospital – Sapienza University of
Emanuele Piccione, captain MD, Neurosurgical Division and Radiological Division of the Rome Army Medical Center author:
Riccardo
Caruso
e-mail:
[email protected],
TE D
Corresponding
[email protected], tel.-fax: +39063227174; mobile phone: +393381716244; full postal address:
EP
via Pietro da Cortona 1 c.a.p. 00196 Roma Italy Keywords: intracranial CSF hypotension, myelopathy, schunt, epidural venous engorgement
AC C
Abbreviations list.
CSF: cerebrospinal fluid
ICP: intracranial pressure
MS: Miyazaki’s syndrome
ACCEPTED MANUSCRIPT
Abstract
RI PT
We studied a poorly known form of CSF hypotension characterized by cervical myelopathy, a considerable growth in volume of the venous plexus of the cervical spine and absence of headache. This form was first described by Miyazaki. We reported a case brought to our
all the various clinical aspects of this pathology
SC
attention, we reviewed the literature and formulated etiopathogenic theories that might explain
M AN U
Keywords: intracranial CSF hypotension, myelopathy, shunt, epidural venous engorgement
Introduction
TE D
The spontaneous or secondary intracranial hypotension syndrome is a well known pathophysiological entity[1]. First described by Schaltenbrand in 1938[2], the syndrome has been the subject of several publications[3-5] reported between the 1960s and early 1990s
EP
thoroughly describing most pathologies involving CSF such as volume depletion due to CSF leakage or CSF shunt over-drainage. There is however a form of CSF hypotension that has not
AC C
been studied in depth and is rarely described in the Literature. It is characterized by cervical myelopathy, a considerable growth in volume of the venous plexus of the cervical spine, absence of headache or nausea and any other symptom usually associated with intracranial hypotension. This pathology was first described by Miyazaki et al. in 1998.[6] We treated one case, which has led us to research the subject in depth. First we reviewed the Literature and then formulated
ACCEPTED MANUSCRIPT
etiopathogenic theories that might explain all the various clinical aspects of this pathology, which we shall refer to as the Miyazaki’s syndrome (MS)
RI PT
Case report In October 2012, a 35 year-old man was brought to us; he had been suffering for about 7 years from progressive spastic tetraplegia. When he was 18 months old he had undergone surgery
SC
using a medium pressure Pudenz ventriculo-atrial shunt to treat tetraventricular communicating hydrocephalus with the presence of a large supra- and infratentorial arachnoid cyst and agenesis
M AN U
of the corpus callosum. When the patient was 9 years old it had been necessary to change the catheter adding a ventriculo-peritoneal shunt with high-pressure valve without antisiphon device. After undergoing this second operation the patient, who had regular check-ups and neuroimaging scans, felt fine, had a normal social life and went to school like everyone else; he was able to
TE D
practice sports such as skiing, swimming, and tennis; went to University and even studied abroad. At 21 he began to work.
Unfortunately at the age of 28 he showed slow yet progressive difficulty in walking: it became
EP
impossible for him to take footsteps and eventually he became unable to stand on his feet without support. In the year prior to our examination, the patient had also observed loss of strength in his
AC C
arms. The Patient was seen by various neurologists, neurosurgeons and orthopaedists who performed numerous MRI scans of the brain; these did not show the presence of hydrocephalus or CSF hypotension.
Upon our advice the patient underwent a metabolic MRI of the brain and a cervical MRI scan; this last scan showed a considerable enlargement of the high cervical venous plexus with compression of the spinal cord. The Patient then underwent a CT angiography (fig.1), a venous
ACCEPTED MANUSCRIPT
Doppler ultrasound scan of the neck, which showed a right hand-side obstruction of the internal jugular vein, where the cardiac catheter of the first shunt still remained, and an angiography with study of cranial and cervical circulation, which did not reveal dural arteriovenous fistulas. At this
as the cause for the veins’ swelling. ®
-P- Tel, Raumedic AG, Helmbrechts Germany) to
SC
We applied a telemetric probe (Neurovent
RI PT
point we began to suspect, despite the absence of a headache, the presence of CSF hypotension
measure the ICP and monitored the patient for four weeks (fig.2A), the ICP values were always
M AN U
very low: -8/-10 mmHg when lying down and outside of the scale and thus non-measurable when standing. We therefore decided to close the distal catheter and we kept monitoring the ICP, which over approximately 45 days, very slowly, climbed back up to values of 8/10 mmHg when lying down (fig.2B). We monitored progress for another month and there were no further variations in ICP so we removed the probe and decided the patient no longer needed the CSF
TE D
shunt.
Follow up MRI scans and CT angiography after 3,6 and 12 months from the closing of the shunt
EP
showed a progressive reduction , which in the end was over 50%, of the cervical venous swelling (fig.3) and an unaltered cerebral context compared to before the surgery. The patient showed a
AC C
clear clinical improvement and today, despite still retaining a high level of spasticity, he can walk on his own, unaided.
Literature review. Materials, Methods and Results. Using appropriate keywords we searched various electronic databases including PubMed, ISIWeb, Scopus and Google Scholar for all articles in English from 1970 to the present date that presented a clinical situation similar to ours; in other words: CSF hypotension, swelling of the
ACCEPTED MANUSCRIPT
extra-dural cervical venous plexus, myelopathy, no headache nor any other typical symptom of intracranial hypotension syndrome. We only found 6 cases (tab.1)[6-11]. In all of them the cause of the hypotension was over drainage of CSF due to a ventricular-peritoneal shunt (VP shunt).In
didn’t include the type of malfunctioning shunt.
RI PT
5 cases the malfunctioning shunts had no antisiphon system, the case described by Howard [11]
SC
The six cases of MS comprised equally men and women across a wide age range: 17 to72 years (average 35.5); in 4 cases the first shunt had been introduced in childhood, while in the other two
M AN U
in adult age. In all cases but one there had been direct report of low CSF pressure; in the case treated by Ulrich[10] the low pressure was hypothesized on the basis of neuroimages only and then confirmed ex adjuvantibus as the symptoms resolved after substitution of the shunt valve with one that needed higher pressure to open. All patients showed a degree of improvement after
deteriorated. Discussion
TE D
the substitution or change of the type of shunt except for one who refused surgery and thus
EP
The two typical aspects that connect our case with the other 6 described in the Literature are the absence of orthostatic headache, which is typical of CSF hypotension and the presence of
AC C
cervical myelopathy caused by an abnormal swelling of the cervical venous plexus. Cases of CSF hypotension with abnormal MR findings and no headache have been described in the Literature (Mokri’s classifiction type IV)[1], but are rare (there have only ever been 11 altogether, including the MS ones) and no one has ever tried to explain the absence of a headache. The typical orthostatic headache in relation to CSF hypotension syndrome is usually explained with the traction and distortion of pain-sensitive suspending structures of the brain due
ACCEPTED MANUSCRIPT
to a sagging of the brain[12]. We must therefore hypothesize that the absence of a headache is linked to the absence of the aforementioned traction and distortion or that the same happened so slowly and became chronic in a way that did not activate the nerve endings susceptible to pain.
RI PT
Probably in the MS cases both mechanisms occur and these are somehow connected to the swelling of the cervical veins that would become a sort of supporting cushion for the structures
SC
above and also an obstacle for CSF flow.
The swelling of the peridural cervical venous plexus, as well as the cerebral one, in the presence
M AN U
of CSF hypotention has been described in various cases[12-15] and explained with the MonroKellie doctrine[16], but except in the MS
it is never associated with myelopathy. In the
Literature on the other hand there are cases of CSF hypotension with spinal compression caused by the accumulation of cervical fluid[17-19].
TE D
One could hypothesize that the presence of myelopathy in the MS may be caused not only by the compression of the spine due to the swollen plexus, but also and most importantly by the spinal circulation being hampered, with difficult blood-flow in the veins[20]. A similar, but not
EP
identical situation as that caused by dural arteriovenous fistulas would ensue; in the latter pathology the blood-flow is hindered by pressure growth in the veins caused by the direct
AC C
passage of arterial blood in the venous system. In the MS on the other hand there would be a slow yet progressive growth of venous circulation, which over time would become so substantial as to hinder flow, this would consequently lead to stagnation in the microcirculation of the spine. The few, unidentifiable symptoms at the onset of MS and the consequent considerable amount of time before a possible diagnosis can be made would certainly play an important role[15]; what could also facilitate the obstruction of the veins is the anatomical pattern of the flow in the
ACCEPTED MANUSCRIPT
cervical venous plexus[21-23] especially when, like in our case, the right internal jugular is closed and there is not an anti-siphon system .
RI PT
Hypotension in the MS is difficult to diagnose because there is no headache and the myelopathy establishes itself slowly, over time; we therefore believe that it can be misdiagnosed and it could potentially be less rare than it may appear by reviewing the Literature. We formulated a
SC
diagnosis with much difficulty and in stages. On one hand we were faced with the absence of any headache and scans that showed no change in the cerebral situation of the patient for years and
over time.
M AN U
on the other hand a progressive paralysis of all four limbs that had been inexorably happening We carried out an MRI scan only to ascertain that there was no spinal cord
compression due to other pathologies. We were actually really surprised by the substantial swelling of the venous plexus shown by the images and initially we thought that it might have been caused by an arteriovenous dural fistula. It was only when the angiography showed that
TE D
there wasn’t a fistula that we were able to diagnose CSF hypotension. In light of our experience, we believe that MS should be taken into consideration when running
EP
differential diagnostic procedures in patients who have shunts and who show signs of myelopathy, but do not suffer from headaches. We advise to immediately run an MRI of the
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brain and spine with focus on the venous blood flow. Should the cervical venous plexus be swollen, even if there aren’t any other typical images of hypotension, we believe the ICP should be immediately monitored. We also believe that, possibly, in some cases this syndrome might be caused not only by secondary hypotension, but also by spontaneous hypotension[20].
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Legend fig 1 preoperative (presurgery) CT angiography showing the cervical epidural venous
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engorgement : A axial view. B coronal view. C 3D reconstruction fig 2 A: ICP before the closure of the shunt B: ICP after the closure of the shunt
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fig 3 CT angiography showing improvement of the cervical epidural venous engorgement :
age-sex
schunt cause
Miyazaki[6]
54y M
Wingerchuk [7]
72y W
Liu[8]
18y W
Wolfe[9]
17y M
Ulrich[10]
17y M
Howard[11]
26y W
hydrocephalus after ESA Hydrocephalus for a posterior fossa meningioma Cyst from perinatal ischemia hydrocephalus after radioterapy obstructive hydrocephalus due to a retrocerebellar cyst congenital hydrocephalus
length clinical history 7m
treatment
outcome
closing shunt
improved
4y
no
worse
replacement of the old with a programmable shunt replacement of the old with a programmable shunt replacement of the old with a programmable shunt
improved
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first author
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A axial view. B coronal view.C 3D reconstruction
4m ?
2y
1m
replacement of the old with a programmable shunt
tab.1 legends: M man, W woman, m month, y year, ? unknown
improved improved
improved
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References
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Yousry I, Forderreuther S, Moriggl B, Holtmannspotter M, Naidich TP, Straube A, Yousry TA. Cervical MR imaging in postural headache: MR signs and pathophysiological implications. AJNR. American journal of neuroradiology 2001;22(7):1239-50. Nowak DA, Rodiek SO, Zinner J, Guhlmann A, Topka H. Broadening the clinical spectrum: unusual presentation of spontaneous cerebrospinal fluid hypovolemia. Case report. Journal of neurosurgery 2003;98(4):903-7. Braun JP, Tournade A. Venous Drainage in the Craniocervical Region. Neuroradiology 1997;13:155-8. Chaynes P, Verdiè JC, Moscovici J, Zadeh J, Vaysse P, Becue J. Microsurgical anatomy of the internal vertebral venous plexuses. Surg Radiol Anat 1998;20(47-51). Doepp F, Schreiber SJ, von Munster T, Rademacher J, Klingebiel R, Valdueza JM. How does the blood leave the brain? A systematic ultrasound analysis of cerebral venous drainage patterns. Neuroradiology 2004;46:565–70.
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[19]
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Highlights
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We examine a form of CSF hypotension that is characterized by cervical myelopathy, a considerable growth in volume of the venus plexus of the cervical spine, absence of headache We formulate etiopathogenic theories that might explain all the various clinical aspects of this pathology we believe that this particular form of CSF Hypotension can be misdiagnosed and it could potentially be less rare than it may appear by reviewing the literature
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Conflicts of interest
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All authors have no conflicts of interest
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prof. Riccardo Caruso
