Low CSF pressure headache - PowerPoint Presentation

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Low CSF pressure headache

A. GHORBANI .MD

Neurology department of Esfahan university of medical sciences

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INTRODUCTION 

Production

absorptionFlow of cerebrospinal fluid(CSF)

play key roles in the dynamics of intracranial pressure. Alterations in CSF pressure can lead to neurologic symptoms the most common being headache.

CSF hypovolemia syndrome

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Intracranial hypotension (IH)- CSF hypovolemia syndrome

is an uncommon, benign, and usually self-limiting condition caused by low cerebrospinal fluid (CSF) pressure,

primary (

spontaneous intracranial hypotension – SIH) secondary e.g. iatrogenic or traumatic. 2-Secondary intracranial hypotension injury of the dura mater

spinal surgery, lumbar puncture,

classified into two main types:

cranial

leaks

and spinal leaks

The vast majority of leaks are spinal

CSF can be seen dripping out of the nose

, or ear

1-Spontaneous

intracranial hypotension

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Spontaneous intracranial hypotension (SIH)

spontaneous dural dehiscence

Dural tears caused by degenerative causesmay result in dural weakness and play a role in the development of spontaneous low CSF pressure,Meningeal diverticula, often seen in patients with CSF leaks,

spinal CSF venous

fistula

 

The location of CSF leaks associated with spontaneous intracranial hypotension is almost exclusively spinal

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1-the loss of buoyant force of CSF, venous engorgement

, and sagging of the brain, which lead to traction on cerebral and cerebellar veins, meninges, and cervical nerves, cause headaches.

2-A stalk effect hypothesis puts forward that sagging of the brain, which develops due to the loss of buoyant force of CSF, results in stretching of the pituitary stalk and/or hypothalamic region,

It should be stressed also that pituitary hyperaemia or pituitary haematomas can lead to the above-mentioned endocrine disorders 3-Traction of the bridging veins may cause their rupture and bleeding into CSF, which explains the presence of erythrocytes, high protein level, and pleocytosis in CSF

PATHOPHYSIOLOGY

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Diagnostic criteria Headache attributed to spontaneous( or idiopathic) low CSF pressures

A-diffuse or dull headache that worsen within 15-minutes after sitting or standing ,with at least one of the

Following and fulfilling criteria D1-neck stiffness2-tinnitus3-hypacusia4-photophobia5-nauseaB. At least one of the following:1. evidence of low CSF pressure on MRI (

eg, pachymeningeal enhancement)2. evidence of CSF leakage on conventional myelography, CT myelography, orcisternography3. CSF opening pressure < 60mmH2O in sitting positionC. No history of dural puncture or other cause of CSF fistulaD. Headache resolves within 72 hours after epidural blood patching

Variable pattern

Disappear orthostatic headache if leak no treated

Exertional headache

Occur at the end of day

Paradoxical headacheIntermittent headache

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hyperprolactinemia in patients with IH is not associated with pituitary adenomaBladowska

J, Sokolska V, Sozanski

T et al: Comparison of post- surgical MRI presentation of the pituitary gland and its hormonal function. Pol J Radiol, 2010; 75(1): 29–36 Clinical

FeaturesThe headache may improve at high attitude with caffeine and great occipital nerve blockadeOnabutalinumtoxin A injections Pituitary enlargement co- occurs with IH in approximately 43%Neurologic examination is usually normal or may reaveal abnormalities referable to the nonheadache symptoms

SIH IS MORE COMMON IN WOMEN-SLIM AND ELONGATED NECK

Improvement of symptoms in the

tredelenburg

position

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The key to diagnosis is a high level of clinical suspicion+careful historyTrauma (trivial)

CAUSES AND PREDISPOSING FACTORS1- connective tissue matrix disorders2-ehlers- danlos

syndrome type 2 - marfan disease3-autosomal dominant poly cystic kidney disease4- joint hypermobility5-retinal detachment at a young ageSPINAL DISORDERS1- calcified herniated disks2-osteophytes and spondylotic spur

The diagnosis may be delayed for many years

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Hypermobile joints sometimes seen in those with SIH

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LABORATORY STUDIESLUMBAR PUNCTURE(< 60MM CSF)34% had a CSF pressure of 60mm CSF or less45% had opening pressure between 60mm and 120mmCSF16% between 120 AND 200 mmCSF

5% had greater than 200 mm CSFThus, most patients have normal CSF pressure

Rarely aseptic meningitiselevated prolactin levels

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Diagnostic Imaging-1 CSF leakage is broadly divided into three commonly observed patterns

: fast leaks, slow leaks

, and cases in which no leak is visible despite the presence of other clinical and imaging signs of IH

The majority of leakages are detected in the thoracic region 1-radioisotope cisternography2- CT myelogram3-conventional MRI4- MR myelogram5- digital subtraction myelography

 

For most patients, spinal imaging to localize their spinal CSF leak may not be necessary, because 1-2 epidural blood patch procedures will be curative

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Diagnostic Imaging-2 1-When suspected, it is important that a magnetic resonance imaging (MRI) study of the brain be done with contrast to look for several specific findings.

2-These findings may be absent in up to 20% of cases, more often when this imaging is done weeks or months after onset.

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Diagnostic Imaging-3

Conventional MRI

Brain diffuse pachymeningeal enhancementSagging of the brain

subdural fluid collections (usually hygromas, less commonly haematomas)Poterior lobe pituitary haematoma

Spinal

diffuse

dural

enhancement of the spinal canal, spinal epidural fluid collection

Meningeal diverticula

distension of the spinal epidural venous plexus, and abnormal intensity around the root sleeves

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Diagnostic Imaging-41-brain MRI with gadolinium and MRI of the spine without gadolinium to assess for the typical imaging features of the syndrome

3-digital

subtraction myelography 

detection of direct spinal venous fistulae2-One rategy is to inject the contrast material for the CT and MRI simultaneouslyAfter injecting a bolus (approximately 15 mL) of intrathecal saline or artificial CSFTo increase the CSF volume. The patient is immediately imaged with CT, followed by delayed imaging using MRI. Both techniques are invasive, and The use of gadolinium for intrathecal injection is

off-label.

Continuum,volume24,august2018

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Current imaging techniques are not sensitive enough to identify a spinal CSF leak in a significant percentage of cases, so it is important for physicians to recognize that negative imaging does not rule out the disorder. This also impacts treatment options.Diagnostic Imaging-5

Mokri B. Spontaneous CSF leaks: low CSF volume syndromes.

Neurol Clin. 2014 May;32(2):397–422.

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Delayed postcontrast FLAIR image shows diffuse pachymeningeal enhancement and opacification of the subdural space with gadolinium (

arrowheads

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Engorgement

of

Dural Venous SinusesOn T1W the middle 1/3 of dominant transverse sinus, shows convex bordersAll venous sinuses become engorged

The falx & tentorium show marked enhancement

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Sagging of the brain

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CSF epidural leak

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sagittal T2 images of the thoracic spine, predominantly posterior to the thecal sac

Conventional CTM shows a collection of contrast outside of the thecal sac (

C, D),

extends from C3 to L4 and the leak cannot be localized.extraarachnoid extravasation of contrast on the right at T6-T7American Journal of Neuroradiology April 2012, 33 (4) 690-694; DOI: https://doi.org/10.3174/ajnr.A2849

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Axial CT myelogram shows extradural leakage into the ventral

epidural space

in the midthoracic spine

in this patient with a fast thoracic CSF leak.

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Complication of SIH Subdural fluid collection

Subdural hematoma Cranial nerve palsy (eg. abducens palsy)

Cerebral sinus venous thrombosis The diagnosis of SIH should be considered in young patientspresenting with SDH in the absence of trauma and withnormal clotting.

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Differential diagnosis1-Symptoms of meningitis2- pachymeningeal enhancement3- pituitary adenoma

4- chiari malformation

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conservative therapy:

avoidance of the upright position,

2-strict bed rest and the possible addition of analgesics

oral or intravenous hydration,4-and high salt intake5-abdominal binderApproximately 200 to 300 mg of caffeine, given two to three times daily,

TREATMENT-1

3-Blocking

the

adenosine

receptors with high oral caffeine intake

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TREATMENT-2Epidural blood patching is the mainstay of treatment for

SIH

present with headache that is severe or longstanding (ie, two weeks or more since onset) should be treated initially with an epidural blood patch (EBP),

additional options include continuous epidural saline infusion, epidural fibrin glue, or surgical repair of the defect. Both epidural fibrin glue and surgical repair require definitive localization of the cerebrospinal fluid (CSF) leak or leaks

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Conclusions 1-Although IH is a benign and usually self-limiting condition, it may mimic other, even life-threatening, diseases. Knowledge of the typical clinical symptoms of IH and careful MRI assessment may lead to a

correct diagnosis

3-clinicians and radiologists should be aware of characteristic MRI features of IH

4-However, it is important to be aware of the low prevalence of diffuse intracranial pachymeningeal enhancement in patients with prolonged atypical headaches due to IH 5-Absence of dural enhancement may exacerbate the problem of

underdiagnosis

of chronic

IH

 

2-

Even

in the modern era of MRI availability, misdiagnosis

is common