Orthostatic headaches
Alok Tyagi
West of Scotland Regional Headache service
Institute of Neurological Sciences
Southern General Hospital
Glasgow
Orthostatic headaches;
Definition
• Headache that comes on shortly after attaining the
upright posture and resolves / improves on lying
flat.
• A classic orthostatic headache is a post dural
puncture headache
• Also seen spontaneously
Overview
• Clinical presentation ;
Headache patterns
Other neurological symptoms
• Investigations ;
MRI
• Management ;
Conservative
Epidural blood patch
CSF facts
Mean CSF
volume
157+/-59 ml
Spinal CSF
volume 49 +/12.1
Sites of CSF Production;
Choroid Plexus
Extra-Choroidal Sources
Ependymal Layer
Brain Parenchyma
Spinal Subarachnoid Space
Sites of CSF absorption;
Arachnoid villi
Lymphatic drainage
Brain capillaries
Choroid plexus
CSF formed at the
rate of 0.35 ml/min
CSF flow
The volume – pressure curve
Steiner LA, Andrews PJ . Br J of Anaesth.2006. 97 (1): 26–38
Monro-Kellie hypothesis
Wolff’s Headache 2007
Headaches due to low CSF pressure
Post dural (post lumbar) puncture headache
CSF fistula headache
Headache due to spontaneous low CSF pressure
Diagnostic criteria
A. Diffuse and/or dull headache that worsens within 15 minutes after sitting or
standing, fulfilling criterion D and with 1 of the following:
1. Neck stiffness
2. Tinnitus
3. Hypacusia
4. Photophobia
5. Nausea
B. At least 1 of the following:
1. Evidence of low CSF pressure on MRI (eg, pachymeningeal enhancement)
2. Evidence of CSF leakage on conventional myelography, CT myelography,
or cisternography
3. CSF opening pressure 60 mm H2O
C. No history of dural puncture or other cause of CSF fistula
D. Headache resolves within 72 hours after epidural blood patching
Epidemiology
• Prevalance of 1 in 50,000 in the community
• In A/E 5 in 100,000
• Female:Male=1.5:1
• Peak incidence at around 40 yrs of age
Headache patterns in low CSF
pressure states
Orthostatic headaches (present when upright, relieved by recumbency)
Orthostatic headaches evolving in months to chronic lingering headaches
Exertional headaches without any orthostatic features
Acute thunder clap–like onset of orthostatic headaches
Second half of the day headaches (often with some orthostatic features)
Other clinical features of low
CSF pressure states
Common;
Pain or stiff feeling of neck—sometimes orthostatic
Nausea, sometimes emesis—often orthostatic
Horizontal diplopia (unilateral or bilateral sixth cranial nerve palsy)
Third and fourth cranial nerve palsies (much less common than sixth cranial nerve palsy)
Dizziness
Change in hearing (muffled, distant, distorted, echoed)
Visual blurring
Photophobia
Etiology of low CSF pressure state
Unknown cause (most often)
Weakness of the dural sac
A. Meningeal diverticula
B. Abnormalities of connective tissue
Dural tear from spondylosis or disc herniation
Trivial trauma
Altered distribution of cranio-spinal elasticity (increased compliance of
the lumbo-sacral CSF space)
Schievink WI. JAMA. 2006 May 17;295(19):2286-96
Head MRI abnormalities in low CSF
pressure states
Diffuse pachymeningeal enhancement
Descent (‘‘sagging’’ or ‘‘sinking’’) of the brain
Descent of cerebellar tonsils (may mimic Type I Chiari)
Obliteration of some of the subarachnoid cisterns (ie, prepontine or perichiasmatic cisterns)
Crowding of the posterior fossa
Enlargement of the pituitary
Flattening or ‘‘tenting’’ of the optic chiasm
Subdural fluid collections (typically hygromas, infrequently hematomas)
Engorged cerebral venous sinuses
Decrease in size of the ventricles (ventricular collapse)
Increase in anteroposterior diameter of the brainstem
Mokri B. Neurol Clin. 2004 Feb;22(1):55-74
MRI brain can be normal in up to 20 % of patients with a headache
due to a low CSF pressure state. Mokri B. Mayo Clin Proc.1999;74:1113-1123.
Demonstration of a spinal CSF leak
• CT myelogram
• Radionuclide cisternography
• MR imaging
• MR myelography
Myelography
• Early and delayed CT scans need to be performed
• Multiple levels may need to be scanned
• Majority of the leaks are at the cervico-thoracic junction or in the
thoracic spine
• Multiple spontaneous CSF leak are common
Mokri B. Neurol Clin. 2004 Feb;22(1):55-74
• MRI myelography can be used to identify the site of CSF leak.
Vanopdenbosch LJ et al. J Neurol Neurosurg Psychiatry. 2010 Jun 20 (epub)
Wang YF et al. Neurology. 2009 Dec 1;73(22):1892-8
Spine MRI abnormalities in low
CSF pressure states
Extra-arachnoid fluid collections (often extending across several levels)
Extradural extravasation of fluid (extending to paraspinal soft tissues)
Meningeal diverticula
Identification of level of the leak (not uncommonly)
Identification of the actual site of the leak (very uncommonly)
Spinal pachymeningeal enhancement
Engorgement of spinal epidural venous plexus
STIR
T2
Sensitivity of spinal MRI in the diagnosis of low CSF pressure
states is 94 %
Watanabe et al. Am J Neuroradiol. 2009 Jan: 30(1): 147-152
CSF findings in low CSF
pressure states
• The variability is considerable
• CSF pressures are less than 6 cm of water but can be
unmeasurable.
• The CSF pressure can be normal
• CSF cell count can be high (upto 100 cells)
• CSF protein can be high (upto 1 gm)
• CSF glucose is never abnormal
Conservative treatment
• CSF leaks can resolve spontaneously and some patients
may not require any treatment
• Bed rest
• Treatments with no evidence base;
Hydration
Abdominal binder
Caffeine (oral or iv)
Steroids
Theophylline
Epidural blood patch (EBP)
• Treatment of choice for patients who fail conservative
treatment
• The success rate with each epidural blood patch (when site
of CSF leak is unknown) is about 30%
• There is a higher likelihood of long-term (5 year)
resolution of SIH symptoms if patients are treated with
EBP rather than conservative measures (??)
Possible mechanisms of action of EBP
A replacement of CSF volume by blood
An early effect due to volume replacement, a result of dural tamponade
A reduction of CSF absorption probably by restriction of CSF flow
A latent effect probably from sealing of the leak
A reversal of the CSF-blood gradient within the epidural space
A decrease in the compliance of the lower spinal CSF space probably a
result of dural stiffening
Schievink WI. Cephalalgia. 2008 Dec;28(12):1345-56
EBP; The procedure
• 10-20 ml blood is injected after which the patient should be placed in a
Trendelenburg position for 30-60 minutes
• The injection should be terminated at the first patient complaint of
pain, back pressure, or headache
• Complications from an epidural blood patch are backache, neckache,
dizziness, auditory disturbances, signs of meningeal irritation, epidural
infection, and nerve root compression. Rarely an adhesive
arachnoiditis may result
• If the first blood patch fails a large volume patch should be given (20100 ml)
• An interval of 5 days is recommended between blood patches
Level of EBP
• If the level of the CSF leak is not known a lumbar epidural
blood patch should be given first.
• If the level of the CSF leak is not known and a lumbar
epidural blood patch has failed a thoraco-lumbar blood
patch should be given.
• If the CSF leak is localised to the thoracic level a directed
thoracic blood patch should be given
• If the CSF leak is localised to the cervical level a directed
cervical blood patch should be given
Management of refractory low
CSF pressure states
Continuous epidural saline infusion
Epidural infusion of Dextran
Epidural injection of fibrin glue
CSF shunting
Intrathecal fluid infusion
Surgical repairs of the leak
Indications for surgery for low CSF
pressure states
• Symptoms severe enough to warrant surgical
treatment
• Site of the leak has been identified
• Symptoms refractory to less invasive measures
Mokri B. Cephalalgia 2008 Dec; 28(12); 1345-56
Complications in low CSF pressure
states
• Cerebral venous sinus thrombosis
Savoiardo M. J Neurol. 2006 Sep;253(9):1197-202
• Subdural haematomas requiring intervention
de Noronha RJ. J Neurol Neurosurg Psychiatry. 2003 Jun;74(6):752-5.
• Epidural blood patch related
Prognosis
Group 1
Group2
Group 3
Group 4
Onset
gradual
acute
gradual
Severity
moderate
severe
mild
moderate
Past history
no
no
yes
yes
IHS criteria
yes
yes
yes
yes
Neurological findings
no
no
no
yes
MRI Brain
abnormal
yes (any)
yes (subdurals)
yes (any)
yes (any)
Progress
resolved
resolved
no resolution
resolved
Headaches
Other symptoms
Mea et al 2009
Conclusions 1
• Orthostatic headaches are the hallmark presenting feature
of low CSF pressure states
• Consider a low CSF pressure state as the cause of a
chronic daily headache or a new daily persistent headache
• Ask for an MRI head with contrast if headache is
orthostatic
Conclusions 2
• A significant proportion of low CSF pressure headaches do
probably resolve with conservative management / time
• Consider epidural blood patch in patients with low CSF
pressure headaches if conservative measures fail
• Milder symptoms of prolonged duration less likely to
resolve completely