spontaneous intracranial hypotension.pptx

Csf pressure less than 60mm water

1. SPONTANEOUS INTRACRANIAL HYPOTENSION

2. lower-than-normal volume of cerebrospinal fluid (CSF) because of leakage of CSF through the dural membrane at one or multiple sites EXCLUDE CSF leaks of known cause, such as craniospinal trauma, spinal surgery, or most commonly, lumbar puncture or spinal anesthesia. treatable cause of headache

3. Pathophysiological Features CSF leak within the spinal column downward displacement of cerebral structures and traction or distortion of pain-sensitive nerve endings in the cranial dura and its vasculature

4. 3 TYPES linear tear in the dura located ventral to or posterolateral to the spinal cord leakage at sites of simple meningeal diverticula or with diffuse dilatations of the dural sac ( dural ectasia ) a spinal CSF– venous fistula .

5. Predisposing factors An underlying connective-tissue disorder - Marfan’s and Ehlers– Danlos syndromes Spinal osteophytes or calcified disk herniations

6. EPIDEMIOLOGY 4 to 5 cases per 100,000

7. Clinical Manifestations ORTHOSTATIC HEADACHE ► Absent or only mild (1–3/10 on verbal rating scale (VRS)) on waking or after prolonged lying flat. ► The onset of the headache occurs within 2 hours of becoming upright. ► After lying flat, the headache should have a ‘good’ improvement in severity (>50% on VRS) within 2 hours. ► The timing of headache onset and offset is consistent.

8. The interval between standing and the onset of the headache is typically a minute or several minutes, but the headache can develop instantaneously, after a number of hours, or after a delay that extends into the afternoon -second-half-of-the-day” headache. thunderclap headache which is followed by orthostatic headache new daily persistent headache with an initial orthostatic quality. Headache mimicking benign exertional headache

9. holocephalic or bilaterally suboccipital but may be unilateral occasionally - throbbing component that simulates migraine. Valsalva maneuver–induced worsening of the headache -CSF–venous fistulas

10. DIFFERENTIAL DIAGNOSIS postural tachycardia syndrome ( PoTS ), orthostatic hypotension cervicogenic headache migraine.

11. PoTS and orthostatic hypotension postural tachycardia orthostatic hypotension autonomic testing.

12. Cervicogenic headache headache is provoked by cervical movement rather than posture, Migraine provoked by movement rather than posture,

13. COMPLICATIONS Subdural haematoma The presumed mechanism - tearing of bridging veins caused by brain sagging MRI of the brain+ WSS with contrast - to investigate the possibility of spinal CSF leak in patients with subdural haematoma / hygromas frontal-lobe and temporal-lobe downward sagging- similar to the symptoms associated with behavioral-variant frontotemporal dementia

14. Cerebral venous thrombosis CT or MR venography should be considered in any sudden change in headache pattern or neurological examination in the context of SIH EBP should be prioritised as initial treatment of SIH with cerebral venous thrombosis. *anticoagulation Coma-extreme downward displacement of the midbrain and brain stem (brain sagging).

15. Superficial siderosis MRI of the brain and spine with blood-sensitive sequences which can detect superficial siderosis ataxia, hearing loss or myelopathic features. Symptomatic patients with superficial siderosis should be offered non-targeted EBP, or targeted treatment of the CSF leak site if detected on imaging

16. INVESTIGATIONS MRI brain + spine screening and contrast - IOC MRI of the brain with contrast- First line MRI of the whole spine is not always necessary for the diagnosis and is unlikely to locate the site of the CSF leak 20%- NORMAL MRI

17. IMAGING Gadolinium-enhanced MRI Pachymeningeal enhancement the most common and recognizable feature on imaging attributed to secondary dilatation of the venous system in the cranial dura as a result of the lowered volume of CSF within the intracranial space and prolonged transit time. Usually homogenous

18. MENINGEAL ENHANCEMENT

19. SUBDURAL COLLECTIONS

20. Venous distension sign Distension of dominant transverse sinus- convex rather than concave shape

21. Pituitary Enlargement Sagging of the Brain

22. Reduction of Optic-Nerve Sheath Subarachnoid Space

23. ventral spinal longitudinal epidural collection (SLEC) extending from the upper cervical to thoracic regions

24. Lumbar puncture should not routinely be performed for the sole purpose of confirming the diagnosis of SIH Only 30-80 % - low csf pressure Additional dural defects may exacerbate leakage LP may be technically challenging

25. INDICATIONS OF MYELOGRAPHY Detect extradural spinal CSF collections, signifying CSF leakage in the spinal column to locate the site of a spinal CSF leak in order to plan the targeted treatment Patients who have at least one brain or spine MRI finding of SIH and have derived no benefit or only temporary benefit from non-targeted EBPs. .gold standard – CT myelography

26. Extradural spinal CSF collections -50% patients. digital-subtraction myelography /dynamic CT myelography may detect CSF– venous fistulas in patients without an extradural spinal CSF collection

27. MANAGEMENT CONSERVATIVE-a few days or weeks NON PHARMACOLOGICAL bed rest hydration (2.0–2.5 L daily). use of abdominal binders avoidance of Valsalva manoeuvres PHARMACOLOGICAL oral caffeine or intravenous caffeine

28. NON TARGETTED EBP autologous blood obtained by venipuncture is injected into the lumbar spinal epidural space administered up to 40 mL , ideally at a minimum total volume of 20 Ml should cease when the patient experiences back pain/pressure, headaches or radicular symptoms that they cannot tolerate

29. Immediate relief is sometimes obtained-immediate increase in CSF pressure caused by the epidural hematoma. formation of a fibrin clot at the site of the dural tear-resolution of leak The recommended time interval between EBPs (or following symptom recurrence in those with a transient response) should be 2–4 weeks.

30. persistent symptoms after blood patching?? digital-subtraction myelography or dynamic CT myelography may be necessary to localize the site of the CSF leak. treatment with directed epidural blood patching or glue injections to seal the site of the CSF leak or for microsurgical repair of the leak. For CSF– venous fistulas, endovascular glue embolization has been used

31. targeted patches patients who remain symptomatic following appropriate conservative management and/or non-targeted EBPs, in whom a causative lesion has been identified on DSM or CTM which is safely accessible via an image-guided transcutaneous approach.

32. Asymptomatic patients with imaging signs of SIH potential significant long-term sequelae (particularly superficial siderosis ) from persistent ventral spinal CSF leaks discuss with patients and offer to investigate and treat asymptomatic spinal CSF leak with SLEC clinical review and repeat neuroimaging every 1–2 years .

33. In patients not responding to initial management of SIH, look for comorbid primary headache and treat . For management of associated primary headache, drugs that potentially lower CSF pressure such as topiramate and indomethacin , and migraine preventives that can reduce blood pressure such as beta blockers should be used with caution

34. THANK YOU