Low PRESSURE Headaches. What they area and what can you do? Kathleen B. Digre MD University of Utah

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1 Low PRESSURE Headaches aka Low CSF volume headache: What they area and what can you do? Kathleen B. Digre MD University of Utah

2 Disclosure: I have a part ownership on a patent for thin filmed technology ; I will discuss treatments that have not been FDA approved. There are NO FDA approved treatments for this disorder

3 32 year old car salesman Weight lifter every day before work One morning developed a new daily persistent headache: Present when he is upright and improved when he lay down. Progressed to be associated with nausea, vomiting, arm numbness, vertigo Neurological examination normal

4 MR FINDINGS

5 OTHER WORK UP Lumbar puncture: no obtainable pressure Radionucleotide scan: normal Thoracic Lumbar MR no gross abnormality

6 Dx: Spontaneous intracranial hypotension Blood patch placed at least 5 times, blindly with brief headache improvement Epidural Saline placed IV caffeine; theophylline No relief

7 CT MYELOGRAM tubular linear collections of contrast C6 7 to T7 8; worst around T6 7

8 Admitted to University Patch placed T4 7 of 18 cc lay flat for 48 hours; stool softeners Stop weight lifting for now Headache resolved

9 HISTORY First described in 1938 and later in 1953 by Schaltenbrand aliquorrhoea He was the first to observe that it is a low fluid state and not necessarily a low pressure state Mokri CSF hypovolemia even though intracranial hypotension has been used in publication Other names: spontaneous low pressure headache, low CSF volume headaches, hypoliquorrhoeic headache, orthostatic headache Intracranial hypotension

10 ICHD DEFINITION 7.2: Headache attributed to Low CSF Pressure Description: an orthostatic headache associated with low CSF pressure (secondary or spontaneous) and usually seen with neck pain, tinnitus, changes in hearing, photophobia and/or nausea. Headache resolves when there is successful sealing of the CSF leak. Diagnostic criteria: A. Any headache fulfilling criterion C B. Low CSF pressure (<60 mm CSF) and/or evidence of CSF leakage on imaging C. Headache has developed in temporal relation to low CSF pressure or CSF leakage, or led to its discovery D. Not better accounted for by another diagnosis.

11 Low Pressure Headache or CSF hypovolemia PRIMARY Spontaneous without antecedent cause SECONDARY Lumbar Puncture (7.2.1) CSF fistula (7.2.2) Intracranial shunt overdrainage Spinal and cranial surgeries Sinus surgeries Nerve root avulsion Mokri Headache 2013; 53:

12 ICHD 3 (beta) CRITERIA FOR LOW PRESSURE HEADACHE 3 types 7.2.1: Post dural puncture headache A. Any headache fulfilling C B. Dural puncture performed C. Headache within 5 days of LP D. No better diagnosis CSF fistula headache A. any headache fulfilling C B. Both of the following: Procedure or trauma that can cause a CSF leak Low CSF pressure (<60 mm CSF) and/or evidence of low CSF pressure and/or CSF leakage on MR, myelography, CT myelography, or radionuclide cisternography C. Headache developed in temporal relation to procedure or trauma D. no better diagnosis No specific criteria Headache attributed to spontaneous intracranial hypotension A. Any headache fulfilling C B. Low CSF pressure (<60 mm CSF) and or evidence of CSF leakage on imaging C. Headache developed in relation to low CSF pressure or CSF leakage or has led to its discovery D. No better diagnosis

13 Challenges with the diagnosis and treatment

14 History: Recognize Headache PLUS Typical Severe headache AND neck stiffness; Often orthostatic: better lying down; worse upright Type: throbbing +/, often intense, usually bilateral (occipital, holocephalic) often worse with coughing sneezing, straining Many other phenotypes: New Daily Persistent headache Lingering daily headache/neck pain/interscapular pain Chronic daily headaches following orthostatic headache Thunderclap onset Paradoxical headache: worse lying down; better upright Exertional headache Intermittent headache (intermittent leak) Acephalgic headache! Mokri Headache 2013; 53:

15 Other symptoms Nausea Visual symptoms: blurred, photophobia, diplopia (usually 6 th nerve, rare 3 or 4) Noises in the ear buzzing; tinnitus Characteristic postural symptoms vertigo (Meniere s like) facial numbness; limb numbness Changes in taste! Lethargy, encephalopathy, personality change FTD dementia; Parkinsonism Bowel and bladder control Gait difficulty Mokri Headache 2013; 53:

16 Recognize the SIGNS Slow pulse vagal pulse CSF fluid leakage from nose, ear No papilledema Diplopia: VI nerve palsy (1/4 of Mokri s series) Look for esotropia do cover cross cover testing!

17 23 year old woman with a chronic headache since delivery and epidural Chronic diplopia. Examination showed 20 diopter esotropia ONSET ABOUT 3 MONTHS LATER

18 RISK FACTORS are frequently daily activities! Lumbar puncture Head trauma Sports: water sports, weight lifting, golf Cough from URI Chiropractor manipulation MVA Fishing (forceful movement of casting) Yoga Genetics: Marfan s syndrome; Ehlers Danlos Look for joint hypermobility, hyperelasticity of the skin Family history of aortic aneurysm; higher incidence of intracranial aneurysm (Schievink, Maya J Neurosurg 2011; 115: 113 5) Mokri Headache 2013; 53:

19 MECHANISMS for loss of CSF Leakage of fluid: Spinal Tarlov Cyst Nose Ear Reduced production of fluid Rapid CSF absorption CSF venous fistula!

20 Recognize the MRI FINDINGS SAGGING BRAIN Tonsillar herniation Brainstem and mesencephalon slumping Descent of the iter Optic nerve/chiasmal dipping Loss of pre pontine cistern Flattened pons Flattened chiasm Crowding of posterior fossa ENLARGED PITUITARY GLAND Dilated Veins Mokri, Mayo Clin Proc 1997; 72: Mokri Headache 2013; 53:

21 MR FINDINGS

22 MR FINDINGS MENINGEAL ENHANCEMENT Small Ventricles Engorged veins

23 Imaging CHALLENGE: Is it a Chiari?? CSF hypovolemia Chiari 1 Mea et al Neurol Sci 2011; 32 suppl 3 S291 4)

24 Challenge: Finding THE LEAK SPINAL MR FINDINGS Meningeal diverticula (Tarlov Cysts) most in thoracic>lumbar>cervical Meningeal diverticula Engorged spinal epidural plexus Radionuclide scanning: See poor uptake in the brain, and early detection in the bladder

25 CT MYELOGRAPHY Most sensitive Requires myelographic material and CT scanning time (x rays) Imaging the entire spine Interpretation can be difficult ( gutter of the epidural space ) Reasons for negative scan: fast leaks and slow leaks MR gadolinium myelography 46 55% have NO CSF LEAK on myelography!!

26 Tarlov s cysts seen in 40% Look for dural sacs 4%

27 CT MYELOGRAM meningeal/dural tear anterior and posterior

28 RADIONUCLIDE SCANNING From Chung et al. Neurology 2000; 55:1321

29 CSF Venous Fistula Direct fistula of CSF and paraspinal vein Must do digital subtraction myelography and look at the time of the myelogram Focal rupture of vein Can be seen with surgery Kranz et al AJNR 2016; 37: : Schievink 2016

30 CSF FINDINGS PRESSURE 46% had pressure 40 mm or lower 25% pressure % pressure CSF FINDINGS Lymphocytosis in >50% (>5 WBC) and (20 40 cells in almost 50%) Protein elevation of > 70 mg/dl Clear, blood tinged, xanthochromia Mokri 2013

31 Challenge: TREATMENT Epidural Blood Patches Blind patches Directed patches Repeating the patch 2 large volume (20 30 ml) patches in lumbar area, followed by 10 minutes with the head lowered (Dillon) Failure only if the above tried Multiple patches can be needed Fibrin Glue

32 REASONS FOR BLOOD PATCH FAILURE Leaks too large Too many leaks all along the spine Leaks are far lateral along the nerve root sleeves Leaks have formed pseudocyst walled off from epidural space The problem is a venous CSF fistula!

33 Medical Treatment Bed rest, coffee, hydration, time IV fluids Corticosteroids Abdominal binder Vitamin A MEDICATIONS To Increase CSF (Absher) Cholinergic: carbachol, prostigmine Sympathetic agonists: albuterol, ephedrine Phosphodiesterase inhibitors: caffeine, theophylline, theobromine Promote CSF secretion: Caffeine sodium benzoate Mokri Headache 2013; 53:

34 OTHER TREATMENTS Epidural saline Infusion Quick Complications: infections, pneumocephalus Surgical correction Ligation of diverticula Packing of the site Occluding a draining vein (Schievink 2016; Krantz2016) dural reduction (Schievink Headache 2009;49: ) Other treatments Intrathecal fluid injection (replace CSF volume) IV saline infusion Epidural dextran infusion Mokri Headache 2013; 53:

35 Headache mechanism Loss of CSF volume more important than the absolute pressure. Kunkle showed that 10% loss needed to set up headache (Trans Am Acad Ophthalmol Otolaryngol Nov Dec;67:758 65) Pain due to stretching of veins, traction from descent Reduced compliance and loss of elastance (Shams, Goadsby J Neurol 2005; 252: )

36 COMPLICATIONS Subdural hematoma from traction on the Cortical veins and engorgement of the veins due to lack of CSF Stupor from central herniation even death Rebound intracranial hypertension! Venous sinus thrombosis Fronto temporal dementia syndrome (Wicklund et al Neurology 2011; 76: ) Recurrence!

37 The Final Challenge How does pressure relate to headache Little direct research into this Contribution from amount of fluid? Contribution from blood flow? CSF Venous shunts closing the vein? Other?

Intracranial hypotension secondary to spinal CSF leak: diagnosis

Intracranial hypotension secondary to spinal CSF leak: diagnosis Spinal cerebrospinal fluid (CSF) leak is an important and underdiagnosed cause of new onset headache that is treatable. Cerebrospinal fluid