AN INTERESTING CASE OF

HEADACHE
• 40 Year old female with no previous known comorbidities presented with complaints
of severe headache holocranial 1-2 episodes per day for 1 week received with
paracetamol and sleep , no vomiting , blurring of vision , no fever

• MRI brain and MRV were done which were normal.

• Ophthal examination showed ?peripheral disc blurring

• In suspicion of IIH guarded LP was done

• CSF pressures of 160mm of water with no other significant findings.

• Neurology review was obtained and patient was started on T.Tryptomer and
topiramate

• Patient symptomatically improved

• TSH was 8.020 and patient was started on T. Thyronorm 25 mcg.

• Psychiatry opinion was obtained and their impression was that there is a functional
overlay.

• Due to family history of migraine and recent stressor migraine with functional overlay
was suspected
• Patient was started on Tryptomer, Topiramarte and was discharged
• 2nd admission

• 2 days later patient presented with complaints of left upper limb weakness
predominantly proximal

• Patient also gave history of swaying and difficulty walking since one day and was
walking with support which improved

• No complaints of facial asymmetry , double vision , loss of sensation, decrease in

intensity of headache
• On arrival
• PR- 90/min
• BP- 140/100

• CVS- S1S2 heard , no murmurs

• CNS - Power on left side was 4+/5

• Left hand grip - 90%
• Left sided reflexes were exaggerated
• MRI brain showed

• Acute non hemorrhagic lacunar infarct in watershed areas of right frontal and
occipital lobe . Moderate narrowing of Right MCA (with paucity of cortical
branches ) right ACA and PCA. Patient was started on DAPT and statin

• Young stroke work up was done and homocysteine was 14.06. Patient was started on
T.Homochek
• CT angiogram showed diffuse area of moderate thinning with beading and pruning of vessels is seen
in ACA, MCA, PCA, Basilar and V4 segment of vertebral arteries distal flow was maintained . Based
on the symptoms and CT angio RCVS was suspected

• ANA was 1:100 , LIA was not significant. P-ANCA and C-ANCA were done to rule out vasculitis
which were negative.
• Interventional radiology opinion was obtained and DSA showed diffuse narrowing of ICA ,MCA and
alternating dilation and narrowing of PCA suggestive of RCVS and B/L ICA para ophthalmic
aneurysm

• Patient was started on Nimodipine 60mg BD

• Patient at discharge had power of 5/5 in all limbs and was walking without support
• REVERSIBLE CEREBRAL VASOCONSTRICTION SYNDROME
• It is transient cerebral vascular spasms which usually present with thunderclap
headaches and recover within 3 months

• The mean age of onset is 43 years

• It affects women more than men

• Triggers- Sex hormones , vasoactive drugs , head trauma and tumours

• Pathophysiology
• Exact mechanism is unknown

• Hightened sympathetic drive there is sudden release of vasoconstrictors ,

catecholamines, neuropeptide Y or endothelin 1 may cause dysregulation of cerebral
vascular tone. This impaired auto regulation might manifest as dilation of distal
arteriole partially due to an exaggerated trigeminovascular reflex.

• The dilation of distal arteriorles , capillaries and meningeal collaterals may abruptly
stretch the nociceptive neurofibres to cause thunderclap headache
• Following which large and medium cerebral arteries constrict to counteract distal
dilation of arterioles

• This manifests the propagation of vasoconstriction during the disease course

• The presence of multiple thunderclap headaches recurring over few days has nearly
100 percent sensitivity and specificity for RCVS
• Presentations
• Can present as ischaemic stroke

• Convexity subarachnoid haemorrhage

• Intracranial haemorrhage

• PRES
• Differential diagnosis
• Aneurysmal subarachnoid haemorrhage
• Migraine
• Cerebral artery dissection
• CVT
• Ischaemic stroke
• Intracranial hypertension
• Reversible posterior leukoencephalopathy
• Pituitary apoplexy
• Angiographic differentials
• Intracranial arteriopathies
• Primary angitis of central nervous system
Management
• Blood pressure
• Broad range is allowed 90-180 mmhg
• BP greater than 180 mmhg can be treated with labetolol or nicardipine

• Pain
• Often requires opioids along with NSAIDs.Triptans and ergot derivatives are contra
indicators as they are vasoconstrictive
• Seizures
• Anti seizure medications are given in acute seizures

• As seizures are usually present only upon presentation and do not recur long term
seizure prophylaxis is not required

• Avoid empirical steroids for possible PACNS a condition that shares certain features
with RCVS
• However glucocorticoids are often administered to minimise the risk of delaying
treatment in patients who may actually have PACNS and is believed to be fatal without
therapy
• Vasoconstriction
• Nimodipine does not affect time course of cerebral vasoconstriction but might relieve
the number and intensity of headaches
• Intra arterial dilation
• This is reserved for patient exhibiting clear signs of clinical progression