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