Sudden Headache and visual disturbances in a young woman

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1 Sudden Headache and visual disturbances in a young woman A. Soupart, MD, PhD Department of Internal Medicine BSIM, December 12, 2014

2 48 years old woman with Sudden Headache 7/2014 * Admitted for Headache Two days before admission, sudden onset of intense holocranial, non-pulsating headache while patient was at her office. - Arised immediately after reaching down to pick up a pen she had dropped. - Associated symptoms: nausea, vomiting, photo- and phonophobia. Progressive worsening. - Headache disabling, patient was bedridden till admission. - No prior history of headache. 2

3 48 years old woman with Sudden Headache * Medical history - Depressive episodes (familial problems) - Iodine and nitrofurantoin allergy - Cystitis - Car crash & whiplash > 15 years - Familial: none (no migraine) * Addictions - Tobacco: no - Alcohol: no * Medications: none 3

4 48 years old woman with Sudden Headache * Physical examination - BP(supine) 120/70 (upright) 122/86 63 t 363 HR BW 72 kg BMI 28 - Patient lying curled, motionless, oriented - No neck stiffness - Normal general and neurological examination Slight obtundation - Bradypsychia -! rapid Patient unable to sit down or stand up: < (< 5 min) worsening of headache (excruciating) 4

5 48 years old woman with Sudden Headache * Complementary tests - Blood tests: normal - Brain CT Scan: normal (emergency room) - Additional tests:. Visual examination (day 1) - No papilloedema - Normal visual fields. EEG: normal - Evolution. Development of diplopia (day 2) of left lateral gaze: Partial left VI nerve palsy? Diagnosis 5

6 Brain CT Scan (admission) 6

7 48 years old woman with Sudden Headache Sudden, postural (positional orthostatic) Headache, Bilateral + Visual deficit and No other abnormalities No history of trauma No associated disease Spontaneous Intracranial Hypotension? Neuroimaging findings: = diffuse pachymeningeal enhancement - Spinal MRI Normal 7

8 Brain MRI (day 3) Diffuse pachymeningeal enhancement 8

9 Spinal MRI Normal 9

10 48 years old woman with Sudden Headache Treatment & Evolution * Conservative measures - Avoidance upright position (bed rest) - Analgesics - Hydratation, high salt intake - Oral caffeine (3 x 200 mg/d) Little improvement * Epidural blood patch (day 5) = Injection of 20 ml autologous blood into epidural space Dramatic improvement of headache after 24hrs and resolution after one week Complete resolution of VI nerve palsy after 3 weeks Patient remains well after 5 months 10

11 Spontaneous (idiopathic) Intracranial Hypotension (SIH) Unfrequent cause of new daily persistent Headache Incidence 5/ Woman/Man 2:1 Onset years 1) Mechanisms - Low CSF pressure (and volume): 0-70 mmh2o (normal opening pressure mmh2o recumbent) - Brain weight 1500 g 50 g in CSF (normal CSF vol ml, production 500 ml/d) Brain Sagging in cranial cavity Traction on anchoring and pain-sensitive supporting structures (meninges, sensory nerves, bridging veins) Headache and associated symptoms enhanced in upright position 11

12 Spontaneous (idiopathic) Intracranial Hypotension (SIH) 2) Causes of low CSF pressure (and volume) CSF leakage located mainly in the spine (cervicothoracic) Sometime skull base (into petrous, ethmoid regions, cribriform plate) Precise cause of spontaneous leaks in epidural space largely unknown * Underlying structural weakness of spinal meninges (connective tissue disorders, deficient fibrillin, elastin, ) 12

13 Spontaneous (idiopathic) Intracranial Hypotension (SIH) 2) Causes of low CSF pressure (and volume) Predisposition of wide variety of dural defects - Simple dural hole - Meningial diverticula - Spinal epidural cysts or perineural (Tarcov) - Tear in dural nerve sheath * Rarely degenerative disc disease and osseous spurs (piercing dura) 13

14 JAMA, May 17, 2006 Vol 295, No

15 JAMA, May 17, 2006 Vol 295, No

16 Spontaneous (idiopathic) Intracranial Hypotension (SIH) 3) Contributing factors History of minor trauma or incitent event preceding Headache in 50% of the cases: - Fall - Trivial trauma - Cough, sneeze, - Sports activity - Intercourse Role of mechanicals factors 16

17 Spontaneous (idiopathic) Intracranial Hypotension (SIH) 4) Clinical presentation * Postural Headache - Occurs or worsens within 15 min (sometimes 2hr) of sitting or standing - Sometimes starts as thunderclap - Improvement in 15 to 30 min after lying down Other patterns (more prominent later in the day, chronic daily with disappearance of postural component if non diagnosed for months ) * Duration: spontaneous resolution of an episode within 2 weeks but recurrences. May last months or years 17

18 Spontaneous (idiopathic) Intracranial Hypotension (SIH) 5) Associated symptoms and complications Direct results of downward displacement of the brain and tractions of nerves and brain structures. * e.g. - Visual defects (blurred vision, diplopia) distorsion of optic nerve, chiasma (visual fields), oculomotor nerves, abducens ) - Auditory syndromes (echoing ) stretching of eight nerve complex (cochlear and vestibular) - Facial numbness or pain (trigerminal) 18

19 Spontaneous (idiopathic) Intracranial Hypotension (SIH) 5) Associated symptoms and complications * Severe displacement of the brain - Decreased level of consciousness (diencephalic herniation) - Coma * Subdural hematomas (30-40%) * Stroke * Spinal symptoms - Interscapular pain - Local back pain - Radicular 19

20 Arch. Neurol., Vol 60, Dec

21 Arch. Neurol., Vol 60, Dec

22 Arch. Neurol., Vol 60, Dec

23 Arch. Neurol., Vol 60, Dec

24 Spontaneous (idiopathic) Intracranial Hypotension (SIH) 6) Diagnosis * Orthostatic Headache + Low CSF pressure + Diffuse Meningeal Enhancement = major features (see Box 2) * CSF: low opening pressure with LP (0-70 mmh2o) Avoid (LP difficult dray taps and risks to aggravate CSF leak) * Brain CT: usually normal * Brain MRI: greatly improved the diagnosis of SIH: - Enhancement of Pachymeninges (dilated vessels) - Sagging of the brain (pseudochiari, tonsilar herniation ) Normal in 20% cases 24

25 JAMA, May 17, 2006 Vol 295, No

26 Spontaneous (idiopathic) Intracranial Hypotension (SIH) 6) Diagnosis * Spinal MRI Not very effective in locating the defect (epidural fluid collections) * CT or MRI myelography Best exam to identify site of the leak 26

27 Spontaneous (idiopathic) Intracranial Hypotension (SIH) 7) Differential diagnosis * Diagnosis remains difficult: Commonly misdiagnosed (94% initial incorrect diagnosis) Diagnosis delay ++ (mean 13 months) - Unnecessary and extensive testing NB Orthostatic hypotension without CSF leak in Postural Tachycardia Syndrome or Orthostatic intolerance * Pachymeningitis Infectious (T, Cryptococcus, VIH, syphilis, ) Vasculitis (Wegener, ) Idiopathic hypertrophic cranial 27

28 JAMA, May 17, 2006 Vol 295, No

29 Arch. Neurol., Vol 60, Dec

30 Arch. Neurol., Vol 60, Dec

31 Spontaneous (idiopathic) Intracranial Hypotension (SIH) 8) Treatment and outcome No randomized clinical trials evaluating treatment strategies * Conservative treatment: Mild to moderate uncomplicated Headache - Bed rest, avoid upright position - Oral/IV hydration, salt intake - Caffeine 200 mg 3/d 31

32 Spontaneous (idiopathic) Intracranial Hypotension (SIH) 8) Treatment and outcome * Epidural Blood Patch If failed or severe symptoms Infusion of autologous blood into epidural space Initial 20 ml / Repeat if unsuccessful ( ml) (2-3 injections for 50% patients) Mechanisms (not completely understood) - Initial tamponade of dural leak Rapid (24h) improvement of Headache - Later, fibrin deposition and scar formation ( 3 weeks) 32

33 Spontaneous (idiopathic) Intracranial Hypotension (SIH) 8) Treatment and outcome * Failure of EBP or recurrence of Headache Investigate for identifying site of CSF leakage (CT myelogram) Surgical repair (meningial diverticula, dural defects or tears) (clips, suture, gelfoam, fibrin glue) 33

34 Spontaneous (idiopathic) Intracranial Hypotension (SIH) 8) Treatment and outcome * Prognosis: - Cured after EBP (n = 2 injections) 70% - Long term outcome: 10% of CSF leak regardless of treatment - Patients with abdormal MRI and focal CSF leak better prognosis than normal MRI and multiple spinal CSF leak - Persistent symptoms after treatment: residual CSF leak (chronic) undetectable 34

35 35

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