Below you will find information on four world-renowned physicians who
have done extensive work related to intracranial hypotension and
cerebrospinal fluid leakage. I have also selected excerpts from some
medical articles that I believe will be of interest to you.
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Dr. Wouter I. Schievink, of the Maxine Dunitz Neurosurgical Institute
(part of the Cedars-Sinai Health System in Los Angeles):
"Wouter I. Schievink, MD, is Co-Director of the Neurovascular Surgery
Program at the Maxine Dunitz Neurosurgical Institute at Cedars-Sinai.
Dr. Schievink specializes in sophisticated neurovascular procedures
and is an expert in the areas of familial aneurysms and strokes.
Dr. Schievink's research has focused on the genetic, etiologic and
epidemiologic aspects of intracranial aneurysms and cervicocephalic
arterial dissections. He also has a special interest in cerebrospinal
fluid leaks and intracranial hypotension."
Cedars-Sinai Medical Center
http://www.csmc.edu/2414.html
"Misdiagnosis of Spontaneous Intracranial Hypotension
Wouter I. Schievink, MD
Spontaneous intracranial hypotension is an important cause of "new
daily persistent headaches" but is not a well-recognized entity. The
misdiagnosis of spontaneous intracranial hypotension can have serious
consequences... The clinical course in 18 consecutive patients with
spontaneous intracranial hypotension who were evaluated for definitive
surgical treatment of the underlying spontaneous spinal cerebrospinal
fluid leak from January 1, 2001, through June 30, 2002, was
investigated by correspondence with the patients and physicians...
Seventeen patients (94%) initially received an incorrect diagnosis,
and the diagnostic delay ranged from 4 days to 13 years (median, 5
weeks; mean, 13 months). Migraine, meningitis, and psychogenic
disorder were the most commonly entertained diagnoses. Diagnostic or
therapeutic procedures for disorders that mimicked spontaneous
intracranial hypotension included cerebral arteriography in 2
patients, craniotomies for Chiari malformation in 2 patients,
craniotomy for evacuation of subdural hematomas in 1 patient, and
brain biopsy in 1 patient.
...Patients with spontaneous intracranial hypotension are commonly
misdiagnosed, causing a significant delay in the initiation of
effective treatments and exposing patients to the risks associated
with treatment for disorders that mimic intracranial hypotension.
Increasing the awareness of this spontaneous type of intracranial
hypotension is required to decrease the high rate of misdiagnosis."
Archives of Neurology
http://archneur.ama-assn.org/cgi/content/abstract/60/12/1713
"Spontaneous intracranial hypotension has become a well-recognized
clinical entity, but it remains an uncommonly, and probably
underdiagnosed, cause of headache; its estimated prevalence is only
one in 50,000 individuals. The clinical spectrum of spontaneous
intracranial hypotension is quite variable and includes headache, neck
stiffness, cranial nerve dysfunction, radicular arm pain, and symptoms
of diencephalic or hindbrain herniation. Leakage of the spinal
cerebrospinal fluid (CSF) is the most common cause of spontaneous
intracranial hypotension. A combination of an underlying weakness of
the spinal meninges and a more or less trivial traumatic event is
often found to cause this event in these patients.
...The initial treatment of choice is a lumbar epidural blood patch,
regardless of the location of the CSF leak. If the epidural blood
patch fails, the blood patch procedure can be repeated at the lumbar
level, or a blood patch can be directed at the exact site of the leak.
Surgical repair of the CSF leak is safe and generally successful,
although a distinct structural cause of the leak often is not found."
Neurosurgery On-Call
http://www.neurosurgery.org/focus/july00/9-1-8.pdf
Dr Schevink is associated with the Maxine Dunitz Neurosurgical
Institute, which offers a service called the Scan Review Program,
which enables your physician to submit your MRI scans for review by
the experts at the Institute:
"The Maxine Dunitz Neurosurgical Institute's Scan Review Program is a
consultation service offered to your primary care physician in order
to help guide your treatment. The program is designed to give a basic
review of your scans, and offer information and resources on the
newest methods of treatment for brain, pediatric, blood vessel and
spinal abnormalities...
Maxine Dunitz Neurosurgical Institute
8631 W. Third St., Suite 800E
Los Angeles, CA 90048
Phone: (310) 423-7900
Fax: (310) 423-0777
Cedars-Sinai Medical Center
http://www.csmc.edu/2233.html
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Dr. Bahram Mokri, of the Department of Neurology at the Mayo Clinic:
"Bahram Mokri MD
Department of Neurology, 200 First Street SW, Mayo Clinic, Rochester,
MN, 55905, USA
Spontaneous intracranial hypotension (SIH) is typically manifested by
orthostatic headaches that may be associated with one or more of
several other symptoms, including pain or stiffness of the neck,
nausea, emesis, horizontal diplopia, dizziness, change in hearing,
visual blurring or visual field cuts, photophobia, interscapular pain,
and occasionally face numbness or weakness or radicular upper-limb
symptoms. Cerebrospinal fluid (CSF) pressures, by definition, are
quite low. SIH almost invariably results from a spontaneous CSF
leak... Although various treatment modalities have been implemented,
epidural blood patch is probably the treatment of choice in patients
who have failed an initial trial of conservative management. When
adequate trials of epidural blood patches fail, surgery can offer
encouraging results in selected cases in which the site of the leak
has been identified. Some of the spontaneous CSF leaks are related to
weakness of the meningeal sac, likely in connection with a connective
tissue abnormality."
BioMed Central
http://www.biomedcentral.com/1528-4042/1/109/abstract
More on Dr. Mokri:
Mayo Clinic College of Medicine
http://mayoresearch.mayo.edu/mayo/research/staff/mokri_b.cfm
"Phone:
Appointment Information Desk: (507) 284-2111
TDD service for the hearing impaired: (507) 281-9786
Between 8 a.m. and 5 p.m. Central time, Monday through Friday.
Write to:
Mayo Clinic
Appointment Information
200 First Street SW
Rochester, MN 55905"
Mayo Clinic
http://www.mayoclinic.org/becomingpat-rst/appointments.html
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Dr. Jerome Posner, of Sloan-Kettering Institute:
"SC Pannullo, JB Reich, G Krol, MD Deck and JB Posner
Department of Neurology, Memorial Sloan-Kettering Cancer Center, New York, NY.
We report seven patients with the syndrome of intracranial hypotension
who were referred to Memorial Sloan-Kettering, primarily because of
suspicion of meningeal tumor or infection raised by the finding of
meningeal enhancement on MRI. In three patients, symptoms occurred
after lumbar puncture; in four, there was no clear precipitating
event. Lumbar puncture after MRI in six patients revealed low CSF
pressure (six patients) and pleocytosis or high protein, or both (four
patients). Three patients had subdural effusions. Six patients had
measurable descent of the brain on midsagittal images. Postural
headache resolved in all seven patients, six of whom had follow-up
MRIs. Meningeal enhancement resolved or diminished in all six.
Subdural effusions resolved spontaneously in two and were evacuated
(but were not under pressure) in one. Downward brain displacement
improved or resolved in all patients. The clinical syndrome and MRI
abnormalities generally resolve on their own. An extensive workup is
not helpful and may be misleading. Patients should be treated
symptomatically."
American Academy of Neurology
http://www.neurology.org/cgi/content/abstract/43/5/919
"Jerome Posner
George C. Cotzias Laboratory of Neuro-Oncology
Office Phone : (212) 639-7047
Office Email : posnerj@mskcc.org"
Memorial Sloan-Kettering Cancer Center
http://www.mskcc.org/mskcc/html/11404.cfm
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Dr. Julián Benito León, of the Servicio de Neurología at the Hospital
General de Móstoles in Madrid, Spain:
Diario Medico
http://www.diariomedico.com/neurologia/n271200.html
"Dr. Julián Benito-León, Avda de la Constitución 73, portal 3, 7°
Izquierda, E-28820 Coslada, Madrid, Spain; e-mail: jbenitol@meditex.es
The authors analyzed whether the pituitary gland enlarges in
intracranial hypotension syndrome by studying 11 consecutive patients.
Initial MRI scans showed pituitary gland enlargement with a convex
superior margin. Follow-up MRI studies revealed that the size of the
gland invariably diminished in all patients. The authors hypothesize
that pituitary gland enlargement in intracranial hypotension syndrome
is due to hyperemia of dural and epidural venous sinuses."
American Academy of Neurology
http://www.neurology.org/cgi/content/abstract/55/12/1895
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More info on intracranial hypotension:
"Abstract: Background: Spontaneous intracranial hypotension (SIH) is a
neurologic syndrome of unknown etiology, characterized by features of
low cerebral spinal fluid (CSF) pressure, postural headache and
magnetic resonance imaging (MRI) abnormalities. Methods: Four
symptomatic cases of SIH presented to our institution over a six-month
period. Magnetic resonance imaging studies were performed in all four
cases. Diagnostic lumbar puncture was done in all except one case.
Results: All of the patients on whom lumbar punctures were performed
demonstrated low CSF pressure and CSF protein elevation with negative
cultures and cytology. Three out of the four patients exhibited MRI
findings of diffuse spinal and intracranial pachymeningeal gadolinium
enhancement and extradural or subdural fluid collections. One patient
had no MRI abnormalities despite prominent postural headache and
reduced CSF pressure at lumbar puncture. All patients recovered with
intravenous fluids and conservative treatment. Conclusions: Magnetic
resonance imaging abnormalities are found in most, but not all
patients, with SIH. Cerebral spinal fluid abnormalities can be
detected even in patients with normal MRI studies. It is important to
recognize the variability of imaging results in this usually benign
disorder."
Canadian Journal of Neurological Sciences
http://www.cjns.org/29augtoc/spontaneous.html
http://pub.ucsf.edu/newsservices/releases/2004010945/
"Spontaneous intracranial hypotension is a syndrome due to a
Cerebrospinal Fluid CSF leak followed by decreased CSF volume and
hydrostatic CSF pressure changes. Clinical-radiological presentation
are characterised by orthostatic headaches related with low CSF
pressure and diffuse dural-meningeal (pachymeningeal) contrast
enhancement on magnetic resonance imaging with or without subdural
fluid collections or imaging evidence of descent of the brain. MRI may
shows abnormal dural venous sinus enhancement. Nausea and abducens
palsy may represent additional symptoms. Therapy includes: increased
fluid intake, steroids or epidural blood patch, continuous epidural
saline infusion (CESI) and bed rest. Spontaneous intracranial
hypotension is due to a CSF leak and hydrostatic CSF pressure changes
associated with decreased CSF volume. Spontaneous intracranial
hypotension should be considered in the differential diagnosis of
chronic postural headache."
European Congress of Radiology
http://www.ecr.org/T/ECR01/sciprg/abs/pc0597.htm
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"Headaches that may at first seem to be caused by a brain tumor can
actually stem from a leak of spinal cord fluid, according to a study
in the December 25 issue of Neurology, the scientific journal of the
American Academy of Neurology...
'This is a disorder that was recently believed to be rare. New imaging
technology has told us quite the opposite,' said Jerome Posner, MD,
Memorial Sloan-Kettering Cancer Center neurologist and co-author of an
editorial accompanying the study.
Primarily diagnosed by a low spinal cord fluid pressure reading, the
intracranial hypotension experienced by study participants had varied
causes including spontaneous occurrence, unexplained fluid loss,
lumbar puncture (spinal tap) and cervical spine surgery. Neck pain,
nausea, hearing and vision problems and facial numbness accompanied
the headaches... Many patients respond to conventional treatments such
as bed rest, hydration, caffeine and corticosteroids. Surgical repair
of the leak is performed in only the most severe cases."
Eurekalert
http://www.eurekalert.org/pub_releases/2000-12/AAoN-Nabt-2512100.php
"Spontaneous Intracranial Hypotension
- Spontaneous intracranial hypotension may present quite suddenly or
more gradually.
- Headache may involve the whole head, or be frontal or occipital.
- It worsens in the erect position with improvement on lying down.
- It is worsened by jugular venous compression or the Valsava manoeuvre.
Other features include:
- Neck stiffness
- tinnitus
- faintness
- photophobia
- nausea and vomiting
Causes
The cause is often unclear, although cases have been attributed to
tears in nerve root sleeves resulting from a sneeze or strain. A
similar headache may occur after lumbar puncture
Diagnosis
Lumbar puncture should be performed. The pressure is usually less than
60 mm of CSF. The protein may be elevated, and red cells or
xanthochromia may be present.
Isotope cisternography shows a rapid decrease of activity in the
subarachnoid space, and a specific site of CSF leak may be
demonstrated.
Treatment
- bed rest
- epidural blood patch
- intrathecal saline infusion
- oral caffeine
- surgical repair of any meningeal defect that is found."
The Resident Medical Officer's Online Handbook
http://mentor.wnmeds.ac.nz/groups/rmo/headache/headache16.html
"The syndrome of spontaneous intracranial hypotension is being seen
"...with increasing frequency in clinical practice," Drs. C. Philip
O'Carroll and M Brant-Zawadzki of Newport Beach, California, note in
the March issue of Cephalalgia... Drs. O'Carroll and Brant-Zawadzki
have seen four patients with spontaneous intracranial hypotension
during a three year period. All presented with orthostatic headache.
...The Newport Beach clinicians say a high index of suspicion is
required for prompt and early diagnosis... A lumbar epidural patch is
a 'reasonable approach' in confirmed cases of spontaneous intracranial
hypotension, Drs. O'Carroll and Brant-Zawadzki say. If this fails,
radioisotope cisternography or computed tomography myelography may
yield useful information. Prognosis is generally 'excellent."
Brain.com
http://www.brain.com/Merchant2/merchant.mv?Screen=PROD&Store_Code=W&Product_Code=body_14833&Product_Count=0&Category_Code=headache
"Q: I need information about spontaneous intercranial hypotension...
A: The condition you mention is quite rare. It relates to reduced
spinal fluid pressure and generally causes headaches when the person
is upright; the headache clears rapidly upon lying down, in the
absence of preceding trauma or lumbar puncture (spinal tap). Diagnosis
is usually made by a combination of tests that may include lumbar
puncture (spinal tap) to measure the pressure, MRI scanning, and RISSA
scanning (isotope scan of spinal fluid flow). Treatment depends upon
the cause and can include bed rest, intravenous caffeine, abdominal
binder, steroids, continuous epidural saline infusion, and epidural
blood patch. You should discuss any concern about this diagnosis with
a neurologist."
PeaceHealth
http://www.peacehealth.org/apps/Expert/qa.asp?SubjectID=50
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Google search strategy:
Google Web Search: "intracranial hypotension" + "research"
://www.google.com/search?hl=en&ie=UTF-8&oe=UTF-8&q=%22intracranial+hypotension%22+research
Google Web Search: "csf leak OR leakage" + "research"
://www.google.com/search?hl=en&ie=UTF-8&oe=UTF-8&q=%22csf+leak+OR+leakage%22+research
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I hope this helps. Please keep in mind that Google Answers is not a
source of authoritative medical advice; the material presented above
is for informational purposes, and should not be taken as a diagnosis
nor as a substitute for the services of a qualified medical
professional.
Best regards,
pinkfreud |
