CSF circulation and
Low pressure headaches
-Dr. Sachin Adukia
Cerebral ventricles: Anatomy
Ventricular System two lateral ventricles interventricular foramina (of Monro) third ventricle. cerebral aqueduct (aqueduct of Sylvius Fourth ventricle
central canal of the spinal cord through the three foramina in its roof, with the
subarachnoid space. terminal ventricle
Choroid plexus of second and third ventricles
Aqueduct Forth ventricle 2 foramens
Medial : Magendie Lateral : Luschka
Subarachnoid Space
Brain Mucoperiosteum of nose. Along cerebral blood vessels as they enter and leave Stops at arteriole or venule. Subarachnoid space expands to form subarachnoid
cisterns Cerebellomedullary cistern, Pontine cistern Interpeduncular cistern
Inferiorly, extends spinal cord ---------cauda equina Surrounds CN and spinal nerves to where they leave skull
and vertebral canal-------preineurium
Formation of CSF: Microscopy
Lateral View of CSF Circulation
CSF hypotension
And
Low CSF Pressure headaches
Other terms
Spontaneous (or idiopathic) low CSF pressure headache
Low CSF volume headache Hypoliquorrhoeic headache Aliquorrhea CSF leak headache CSF hypovolemia CSF volume depletion
Epidemiology
Annual incidence is 5 per 100,000 Peak incidence - age 40, but children and older
adults are also affected F:M = 2:1
Cause of CSF hypovolemia or CSF leaks
1. True hypovolemic state (reduced total body water)2. CSF shunt overdrainage3. Traumatic CSF leaks
a. Overt injuries (MVAs, sports injuries, brachial plexus avulsions)b. Iatrogenic (postdural puncture, postepidural catheterization)c. Postsurgical (cranial or spinal surgeries, ENT surgeries)
4. Spontaneous CSF leaksa. Unknown causeb. Preexisting dural sac weaknessc. Meningeal diverticulad. Evidences disorders of connective tissue matrix
Marfan syndrome or marfanoid features Joint hypermobility Retinal detachment at young age Abnormalities of elastin and fibrillin in dermal fibroblast cultures Familial occurrence of spontaneous CSF leaks
5. Trivial trauma (perhaps in the setting of preexisting dural weakness)6. Herniated disks, spondylotic spurs
Varieties of headaches Orthostatic Neck or interscapular pain or a lingering nonorthostatic
headache preceding the orthostatic headache (by days or weeks) Orthostatic evolving into lingering non-orthostatic chronic daily
headaches (transformed orthostatic headaches) Nonorthostatic chronic daily headaches from start Exertional headaches Acute thunderclaplike onset of orthostatic headaches Second-half-of-the-day headaches Paradoxic orthostatic headaches Intermittent headaches of intermittent leaks Acephalgic form
•Stupor, diencephalic compression Coma• Parkinsonism bulbar weakness• Frontotemporal dementia Encephalopathy• Gait unsteadiness Trouble with sphincter control• Bibrachial amyotrophy Chorea
•Rarely. PRES
“Frontotemporal brain sagging syndrome" (FBSS)
progressive behavioral symptoms and cognitive dysfunction suggestive of behavioral variant frontotemporal dementia
Atypical C/F: headache, daytime somnolence. MRI : typical of SIH Treatement: Rx of cause, and CSF leak
CSF in Low CSF PressurePressure often low, occasionally atmospheric, or rarely even negative
Colour Clear , Xanthocromia , Blood tinged
Protein Normal or high (upto 1000mg/dL)
Sugar Normal but NEVER LOW
Cells Normal upto 50 (highest = 222)
RBC May be high
Cytology Negative
Radioisotope Cisternography
MRI in Low CSF Pressure
Acronym : SEEPS for MRI Features
SEEPS S- Subdural fluid collections E- Enhancement of the pachymeninges E- Engorgement of the venous structures P- Pituitary enlargement S- Sagging of the posterior structures
Diagnostic criteria - ICHD-3
A) Any headache fulfilling criteria B through D
B) Low CSF pressure (<60 mmH20) and/or evidence of CSF leakage on imaging
C) Headache has developed in temporal relation to the low CSF pressure or CSF leakage, or has led to its discovery
D) Not better accounted for by another ICHD-3 diagnosis
Treatment with potential drawbacksConservative Rest, Coffee, Hydration, time
Medications Analgesics, caffiene, theophylline
?Corticosteroids (efficacy, durablility, longterm adv effects)
Binders Binders Corsets
Patch EBPFibrin glue (fibrin sealant) single level, Bi or MultilevelFibrin glue and blood
Surgery Disadvantage is……………Surgical closure (not always possible)Reinforcement with muscle and/or fibrin sealant
Others Epidural saline or dextanIntrathecal fluidsIV Saline solitions
Complications of CSF leaks
Subdural Hematomas Rebound IC HTN Superficial Siderosis Papilloedema Bibrachial amyotrophy CVT
ORTHOSTATIC HEADACHES WITHOUT CSF LEAK
Postural orthostatic tachycardia syndrome After surgery for Chiari malformation syndrome of the trephined Increased compliance of dural sac Colloid cyst of the third ventricle
References
Snell RS. The Ventricular System, the Cerebrospinal Fluid, and the Blood-Brain and Blood–
Cerebrospinal Fluid Barriers; In: Clinical Neuroanatomy. Philadelphia: Lippincott Williams &
Wilkins ; 2010; page 446-481.
Mokri B. Spontaneous CSF Leaks. Neurologic Clinics. 2014 May 1;32(2):397-422.
Schievink WI. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. JAMA
2006; 295:2286.
Hammad T, DeDent A, Algahtani R, et al. Posterior Reversible Encephalopathy Syndrome
Secondary to CSF Leak and Intracranial Hypotension: A Case Report and Literature Review. Case
Rep Neurol Med 2015; 2015:538523.
Wicklund MR, Mokri B, Drubach DA, et al. Frontotemporal brain sagging syndrome: an SIH like
Presentation mimicking FTD. Neurology 2011; 76:1377.
Headache Classification Committee of the International Headache Society (IHS). The International
Classification of Headache Disorders, 3rd edition (beta version). Cephalalgia 2013; 33:629.
Thank You