A 🧵 about PACNS:
SPOILER: with @ESOstroke Guidelines!
▶️primary #angiitis of #central #nervous #system
▶️ a rare inflammatory disease affecting the brain and spinal cord
▶️ without any overt systemic vasculitis or underlying potential cause
acrjournals.onlinelibrary.wiley.com
/1
SPOILER: with @ESOstroke Guidelines!
▶️primary #angiitis of #central #nervous #system
▶️ a rare inflammatory disease affecting the brain and spinal cord
▶️ without any overt systemic vasculitis or underlying potential cause
acrjournals.onlinelibrary.wiley.com
/1
Typical clinical features of #PACNS include:
▶️ #Confusion and encephalopathy
▶️ #Seizures
▶️ #Headache
▶️ #Stroke
#neurotwitter #neuroscience @ESOstroke
ncbi.nlm.nih.gov
/2
▶️ #Confusion and encephalopathy
▶️ #Seizures
▶️ #Headache
▶️ #Stroke
#neurotwitter #neuroscience @ESOstroke
ncbi.nlm.nih.gov
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@ESOstroke PACNS #Pathophysiology:
🔬infiltration of immune cells within #CNS blood vessel walls
➡️ destruction and thickening of the vessel walls
➡️ stenosis of alternating segments
➡️ poor blood circulation
➡️ vessel wall weakening,
➡️ vessel ruptures and haemorrhage
4/
🔬infiltration of immune cells within #CNS blood vessel walls
➡️ destruction and thickening of the vessel walls
➡️ stenosis of alternating segments
➡️ poor blood circulation
➡️ vessel wall weakening,
➡️ vessel ruptures and haemorrhage
4/
@ESOstroke Histologically #PACNS:
🔬#inflammation affects both small and large vessels of the CNS
🔬the blood vessels supplying the brain parenchyma, spinal cord and leptomeninges, and less frequently veins and venules
link.springer.com
@ESOstroke #neurotwitter #neuroscience
🔬#inflammation affects both small and large vessels of the CNS
🔬the blood vessels supplying the brain parenchyma, spinal cord and leptomeninges, and less frequently veins and venules
link.springer.com
@ESOstroke #neurotwitter #neuroscience
@ESOstroke PACNS diagnostics:
#PACNS can only be definitively diagnosed by #histopathological examination of CNS blood vessels
@ESOstroke
#neurotwitter
5/
#PACNS can only be definitively diagnosed by #histopathological examination of CNS blood vessels
@ESOstroke
#neurotwitter
5/
@ESOstroke Non-infectious #inflammation of the vessel walls in #PACNS leads to
➡️endothelial damage
➡️necrosis
➡️hypercoagulability
➡️remodelling
➡️obstruction
➡️rupture of #CNS vessels
- and hence, to #ischemic or #hemorrhagic stroke
@ESOstroke
7/
➡️endothelial damage
➡️necrosis
➡️hypercoagulability
➡️remodelling
➡️obstruction
➡️rupture of #CNS vessels
- and hence, to #ischemic or #hemorrhagic stroke
@ESOstroke
7/
@ESOstroke @ESOstroke Recommendations:
➡️#CSF as a diagnostic tool in PACNS:
➡️CSF analyses should not be limited to determination of cell count and protein concentration.
➡️Normal CSF analyses cannot exclude the diagnosis of PACNS!
#neurotwitter #neuroscience
8/
➡️#CSF as a diagnostic tool in PACNS:
➡️CSF analyses should not be limited to determination of cell count and protein concentration.
➡️Normal CSF analyses cannot exclude the diagnosis of PACNS!
#neurotwitter #neuroscience
8/
@ESOstroke #PACNS needs to be differentiated from #RCVS
➡️in PACNS #lymphocytes and #protein may be elevated, Neuroimaging: #ischemic, T2 lesions, Vascular case, hysto: vasculitis
➡️ in RCVS - CSF normal, Neuroimaging: ischemic, edema, cSAH, ICH, initial MRI may be normal
9/
➡️in PACNS #lymphocytes and #protein may be elevated, Neuroimaging: #ischemic, T2 lesions, Vascular case, hysto: vasculitis
➡️ in RCVS - CSF normal, Neuroimaging: ischemic, edema, cSAH, ICH, initial MRI may be normal
9/
@ESOstroke #PACNS #NEUROIMAGING
#Brain #MRI :
➡️multiple acute deep and superficial #ischemic lesions (DWI)
➡️corresponding multiple hyperintensity signal on #FLAIR
➡️Post-contrast #gadolinium: leptomeningeal enhancement
@ESOstroke #neurotwitter
10/
#Brain #MRI :
➡️multiple acute deep and superficial #ischemic lesions (DWI)
➡️corresponding multiple hyperintensity signal on #FLAIR
➡️Post-contrast #gadolinium: leptomeningeal enhancement
@ESOstroke #neurotwitter
10/
@ESOstroke #PACNS #Neuroimaging
🛜 Brain MRI: #infarcts in multiple vascular territories,
🛜 Mostly medium and small intracranial arteries are affected rather than large proximal arteries.
🛜 Subcortical, peripheral, superficial, or deep lesions
@ESOstroke
11/
🛜 Brain MRI: #infarcts in multiple vascular territories,
🛜 Mostly medium and small intracranial arteries are affected rather than large proximal arteries.
🛜 Subcortical, peripheral, superficial, or deep lesions
@ESOstroke
11/
@ESOstroke @ESOstroke Guidelines
🎯#PACNS DIAGNOSTICS: #DSA OR #MRA?
➡️ESO experts suggest that DSA could be non inferior to MRA if multislice (>128) technique is employed
#neurotwitter
12/
🎯#PACNS DIAGNOSTICS: #DSA OR #MRA?
➡️ESO experts suggest that DSA could be non inferior to MRA if multislice (>128) technique is employed
#neurotwitter
12/
@ESOstroke And!
Long awaited @ESOstroke #GUIDELINES
#PACNS #Treatment:
💊consideration of adding an #immunosuppressant to glucocorticoid therapy in most patients with PACNS
💊The use of #glucocorticoids alone might be considered, in particular in milder disease phenotypes
13/
Long awaited @ESOstroke #GUIDELINES
#PACNS #Treatment:
💊consideration of adding an #immunosuppressant to glucocorticoid therapy in most patients with PACNS
💊The use of #glucocorticoids alone might be considered, in particular in milder disease phenotypes
13/
@ESOstroke @ESOstroke Guidelines on #PACNS:
Treatment with #cytostatics?
💊Cyclophosphamide per os or iv or mycophenolate mofetil ➡️ in induction phase in conjunction with glucocorticoids
💊Mycophenolate mofetil ➡️maintenance therapy
14/
Treatment with #cytostatics?
💊Cyclophosphamide per os or iv or mycophenolate mofetil ➡️ in induction phase in conjunction with glucocorticoids
💊Mycophenolate mofetil ➡️maintenance therapy
14/
@ESOstroke @ESOstroke Guidelines on #PACNS:
#Aspirin may have a beneficial effect in PACNS, which may be due to a combined antithrombotic and anti-inflammatory effect and its possible complementary action with glucocorticoid therapy
15/
#Aspirin may have a beneficial effect in PACNS, which may be due to a combined antithrombotic and anti-inflammatory effect and its possible complementary action with glucocorticoid therapy
15/
@ESOstroke @ESOstroke Guidelines on #PACNS:
#THROMBOLYSIS in #PACNS: considering thrombolysis in patients with a history of PACNS presenting with symptoms of acute ischemic #stroke
16/
#THROMBOLYSIS in #PACNS: considering thrombolysis in patients with a history of PACNS presenting with symptoms of acute ischemic #stroke
16/
@ESOstroke @ESOstroke Guidelines on #PACNS:
#Thrombectomy is reasonable in #ischemic #stroke in #PACNS presenting within the early or extended time windows for EVT
17/
#Thrombectomy is reasonable in #ischemic #stroke in #PACNS presenting within the early or extended time windows for EVT
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