CAPLAN CH12: Non-atherosclerotic vasculopathies Flashcards
Major finding is a rash, often with palpable purpruic skin lesions, especially on the legs
Hypersensitivity vasculitides
Characteristic findings of FMD in arteriography
String-of-beads appearance
The most impressurve feature of carotid artery dissection in the neck?
Pain
Ipsilateral throbbing headache and sharp pain in the neck, jaw, pharynx or face
What is the most common artery affected by arterial dissections?
Extracranial ICA
- pharyngeal and distal extracranial segments
- segment above the ICA origin
Levels are higher in patients with cervical artery dissections than controls especially in patients with recurrent dissections.
Plasma levels of matrix metalloproteinase-2
In patient’s with ICA dissection, describe the Horner’s ?
INCOMPLETE. Facial sweat function is preserved because the sympathetic innervation of the sweat glands travels along the ECA.
What happens when ICA dissection extends to the carotid siphon?
Ischemic optic neuropathy can develop as a result of decreased perfusion of arteries supplyiing the optic nerves.
Characteristic ultrasound finding of a dissection?
- High amplitude signal
- With markedly reduced systolic Doppler frequencies
- Alternating flow directions over the region of the luminal narrowing
When dissections are in the extracranial ICA, what would the TCD show?
Diminished intracranial velocieties in the ICA siphon and MCA
Most common angiographic finding in patients with dissections?
String sign
Pharyngeal segment aneursym formation can lead to dysfunction of the lower cranial nerves at the skull base. Usual presentation?
- Dysgeusia
- Horner’s syndrome
- Atrophy of the tongue
Where is the usual pain of an a vertebral artery dissection?
Pain in the posterior neck or occiput and generalized headache
Most common pattern of ischemic brain damate in patients with ECVA dissections?
infarctions in the PICA distribution and lateral medullary infarction
Doppler findings of ECVA dissections?
Diminished flow in the high neck at the level of tha tlas
Decreased flow in the intracranial VA shown by TCD
Most common locations of ICA dissections in the anterior circulation?
Supraclinoid ICA
Maintem of the MCA
Usual duration of anticoagulants in patients with dissections?
6 weeks (or later until luminal stenosis improves to the point that flow is not significantly obstructed)
Indications for angioplasy in patients with dissections?
- part of intra-arterial approach to lysing MCA intra-arterial emboli from ICA dissections when ICA is occluded or nearly occluded
- in patients with continued hypoperfusion
Population wherein FMD is more common?
Middle-aged women
Finding in ICA FMD?
- bilateral (86%)is common
- usually involves the pharyngeal portion of the artery, extends from C1 proximally 7-8cm
- sparing of the carotid bifurcation and intracranial carotid artery
- 20% have coexistend FMD in the VAs of the neck
Most common form of FMD affects which layer?
Media
Possible mechanisms of stroke in patients with FMD?
- Altered contractility
- Dilatation with aneurysm formation
- Tears with intramural hematomas
Treatment modality for FMD
Antiplatelet is mainstay therapy for carotid or vertebral FMD
Surgery is rarely recommended
What to check in patients with FMD and high blood pressure?
Always check the renal arteries.
Gronblad-Starndberg disease
Pseudoxanthoma elasticum
Gene affected by Pseudoxanthoma elasticum
ABCC6 gene
- a transmembrane transporter family of proteins
Dermatologic findings of pseudoxanthoma elasticum
- First, thickened and grooved skin of the fac, neck, axilla, antecubital, inguinal and periumbilical regions
- Yellowsih papules and plaques in the mucosa
- Then, skin becomes lax and redundant
ophthalmologic findings of PSE?
- Angioid streaks
2.Peau d’ orange- speckled, yellowish mottling of the posterior pole of the retina temporal to the macula
Other complications of PSE?
- Gastrointestinal bleeding
- Cardiac manifestations
Arterial findings of PXE?
- aortic arch and intracranial arteries involved
- tortuosity and ectasia of the neck arteris and angiography
- lacunar infarcts and white matter ischemia (Biswanger type)
Most common type of Ehlers-Danlos syndrome to have cerebrovascular complications?
Type IV
(but note that 80% of EDsyndrome have Type I, II or III)
Non-neurological features of ED:
Easy bruising
Regions of translucent skin
Vascular rupture
Rupture of gravid uterus
Important and frequent cerebrovascular complications
Carotid-cavernous fistulas
Arterial dissections
Most common site of aneurysm formation in patients with Ehlers Danlos?
ICA
Inheritance pattern of Marfan’s syndrome? And gene affected
Autosomal dominant
FBN1
encodes fibrillin-1
found in chromosome 15
Phenotype of Marfan’ syndrome that is easily recognizable
Long limbs
Pectus chest deformit
Arachnodactyly
Joint laxity
Ophtha: SUBLUXATION OF THE LENS
Most of the cerebrovascular events in pateints with Marfan’s syndrome is related to ___.
cardiac and aortic manifestations of the condition
Autosomal dominant syndrome
bony and connective tissue abnormalities
aortic and arterial aneurysms and dissections
Loeys-Dietz syndrome
Gene affected in Loey Dietz syndrome?
gene encoding transforming growth factor beta receptor (TGFBR1 or 2)
Most frequent location of dilatative arteriopathy
posterior fossa where the basilar artery or one or both VAs are involved
In patients with recurrent ischemia and thrombi with dolichoectatic arteries, ___ may be used to prevent strokes
Warfarin
Lobes usually affected by amyloid angiopathy?
Parietal and occipital
Structure of affected arteries in CAA?
Distinctive double-barrel lumen with amyloid found in the outer or inner media
The Icelandic variety of CAAhas been attributed to abnormal metabloism of __.
gamma-trace protein
Mechanism of Ab-related angiitis?
Breakage of amyloid-containing vessels with release of Ab amyloid into the subarachnoid space inducing an immunological response
Brain infarction develops in about ___%of adults with bacterial meningitis, typically ___.
15-20%
typically pneumococcal meningitis
This presents a characteristic inflammatory disorder involving predominantly the medulla and pontine tegmentum. Lower cranial nerve palsies also develop.
Listeria monocytogenes
T/F In Lyme borreliosis, strokes are much less common than peripheral nerve and root sensory symptoms?
TRUE
Sensitivity and Specificity of CSF TB PCR?
48-90% Sn
100 Sp
Mucor vs Aspergillus
_ usually spread from paranasal sinuses?
_ reaches the cerebral circulation by hematogenous spread usually without meningitis?
Mucor
Aspergillus
The most common clinical vascular syndrome in VZV?
Delayed brain infarction causing hemiplegia contralateral to herpes zoster ophthalmicus
VZV uses this connection to infect the MCA, ACA, SCA, etc.
trigeminovascular connections
Most frequent parasitic infection of the CNS
Plasmodium falciparum
Angiography and TCD in children with P falciparum infection
focal stenosis of the basal intracranial arteries
Intestinal involvement (megaesophagus and megacolon) is also common in patients with this parasitic disease
Trypanosoma cruzi (Chagas disease)
Mechanism of strokes in patients with Chagas disesase?
Embolism from heart.
Note that the involvement of the ANS innervation of the heart rather than direct attack by parasites.
This syndrome is characterized by a purulent thrombophlebitis of the jugular vein caused by an amebae.
Lemierre’s syndrome
Polyarteritis nodosa affects which arteries?
small-medium sized arteries especially at branch points
Most common neurological signs of PAN relate to?
mononeuritis multiplex
Pulmonary involvement
Asthma
Eosinophilia
Churg-Strauss syndrome
Are strokes common in hypersensitivity vasculitides?
No. (Usually present with neuropathies, plexopathies, and encephalopathies)
Necrotizing, granulomatous vasculitis that affects:
lung
sinuses
upper respiratory tract
kidneys
Wegener’s granulomatosis
The presence of these two correlates to the clinical hypercoagulability of SLE.
- Lupus anticoagulant
- Antiphospholipid antibody
Abnormalities of the metabolic pathway of ___, particularly of its cleaving protease, are thought to be important in the pathogenesis of TTP?
von Willebrand Factor
