:‫ צריכים לחשוב גם על סיבות נדירות יותר‬,‫בנוסף לסיבות הרגילות לאירוע מוחי‬
1. Procoagulation states – inherited and acquired – Factor V laiden, Protein C+S
deficiency, Activated protein C resitance, Hyperhomocystienamia, Antiphospholipid
(lupus anticoagulant) syndrome.
2. Paradoxical embolism – Patent foramen ovale, ASD.
3. Anaerysms rupture resulting in haemorrhagic strokes.
4. Vasculitis – including SLE.
5. Drugs – mainly stimulatants that cause hypertension (cocaine, amphetamines).
Table 364-2 Causes of Ischemic Stroke
Common Causes
Uncommon Causes
Thrombosis
Lacunar stroke (small vessel)
Large vessel thrombosis
Dehydration
Embolic occlusion
Artery-to-artery
Carotid bifurcation
Aortic arch
Arterial dissection
Cardioembolic
Atrial fibrillation
Mural thrombus
Myocardial infarction
Dilated cardiomyopathy
Valvular lesions
Mitral stenosis
Mechanical valve
Bacterial endocarditis
Paradoxical embolus
Atrial septal defect
Patent foramen ovale
Atrial septal aneurysm
Spontaneous echo contrast
Hypercoagulable disorders
Protein C deficiency
Protein S deficiency
Antithrombin III deficiency
Antiphospholipid syndrome
Factor V Leiden mutationa
Prothrombin G20210 mutationa
Systemic malignancy
Sickle cell anemia
β-Thalassemia
Polycythemia vera
Systemic lupus erythematosus
Homocysteinemia
Thrombotic thrombocytopenic purpura
Disseminated intravascular coagulation
Dysproteinemias
Nephrotic syndrome
Inflammatory bowel disease
Oral contraceptives
Venous sinous thrombosisb
Fibromuscular dysplasia
Vasculitis
Systemic vasculitis (PAN, Wegener's,
Takayasu's, giant cell arteritis)
Primary CNS vasculitis
Meningitis (syphilis, tuberculosis, fungal,
bacterial, zoster)
Cardiogenic
Mitral valve calcification
Atrial myxoma
Intracardiac tumor
Marantic endocarditis
Libman-Sacks endocarditis
Subarachnoid hemorrhage vasospasm
Drugs: cocaine, amphetamine
Moyamoya disease
Eclampsia
Table 364-5 Causes of Intracranial Hemorrhage
Cause
Location
Comments
Head trauma
Intraparenchymal: frontal
lobes, anterior temporal
lobes; subarachnoid
Coup and contracoup injury during
brain deceleration
Hypertensive
hemorrhage
Putamen, globus pallidus,
thalamus, cerebellar
hemisphere, pons
Chronic hypertension produces
hemorrhage from small (~100 μm)
vessels in these regions
Transformation of
prior ischemic
infarction
Basal ganglion, subcortical
regions, lobar
Occurs in 1–6% of ischemic strokes
with predilection for large
hemispheric infarctions
Metastatic brain
tumor
Lobar
Lung, choriocarcinoma, melanoma,
renal cell carcinoma, thyroid, atrial
myxoma
Coagulopathy
Any
Uncommon cause; often associated
with prior stroke or underlying
vascular anomaly
Drug
Lobar, subarachnoid
Cocaine, amphetamine,
phenylpropranolamine
Arteriovenous
malformation
Lobar, intraventricular,
subarachnoid
Risk is ~2–4% per year for bleeding
Aneurysm
Subarachnoid,
intraparenchymal, rarely
subdural
Mycotic and nonmycotic forms of
aneurysms
Amyloid angiopathy Lobar
Degenerative disease of intracranial
vessels; linkage to Alzheimer's
disease, rare in patients <60
Cavernous angioma
Intraparenchymal
Multiple cavernous angiomas linked
to mutations in KRIT1, CCM2, and
PDCD10 genes
Dural arteriovenous
fistula
Lobar, subarachnoid
Produces bleeding by venous
hypertension
Capillary
telangiectasias
Usually brainstem
Rare cause of hemorrhage
:‫אבחנה מבדלת של קרישיות יתר‬
Table 59-3 Risk Factors for Thrombosis
Venous
Venous and Arterial
Inherited
Inherited
Factor V Leiden
Homocystinuria
Prothrombin G20210A
Dysfibrinogenemia
Antithrombin deficiency
Protein C deficiency
Protein S deficiency
Mixed (Inherited and acquired)
Hyperhomocysteinemia
Elevated FVIII
Acquired
Acquired
Malignancy
Age
Antiphospholipid antibody syndrome
Previous thrombosis
Hormonal therapy
Immobilization
Polycythemia vera
Major surgery
Essential thrombocythemia
Pregnancy & puerperium
Paroxysmal nocturnal hemoglobinuria
Hospitalization
Thrombotic thrombocytopenic purpura
Obesity
Heparin-induced thrombocytopenia
Infection
Disseminated intravascular coagulation
APC resistance, nongenetic
Unknowna
Elevated factor II, IX, XI
Elevated TAFI levels
Low levels of TFPI
.‫ היו לא מעט השלמות מאינטרנט‬,‫ בהריסון‬APS ‫אין הרבה מידע על‬
Primary antiphospholipid syndrome is diagnosed in patients demonstrating the clinical
and laboratory criteria without other recognized autoimmune disease.
Secondary antiphospholipid syndrome is diagnosed in patients with other autoimmune
disorders such as SLE.
Clinical findings
Some physical findings may be associated with primary APS, but patients with secondary
APS are more likely to have findings on physical examination.
-
-
-
Venous or arterial thrombosis (DVT, stroke) - possible residual neurologic
findings.
Cutaneous manifestations:
o Digital cyanosis, livedo reticularis, digital gangrene, and leg ulcers
o Other features related to cutaneous manifestations include TIA or
amaurosis fugax, positive result from the Coombs test, hemolytic anemia,
chorea, and chorea gravidarum.
o Discoid rash is a criterion. Older lesions may be atrophic. Again, the cause
remains unexplained.
o Photosensitivity is another criterion
Heart valve disease
Hematologic abnormalities – thrombocytopenia
Pregnancy complications
o spontaneous abortions
o fetal deaths at or beyond 20 weeks' gestation
o stillbirths
o prematurity
o pre-eclampsia, which is frequently prior to 34 weeks’, and severe
preeclampsia requiring premature delivery.
Patients with APS report a higher incidence of headaches, including migraines.
The signs and symptoms of other autoimmune disorders (SLE - BRAIN SOAP MD) may
present and an appropriate evaluation should be undertaken.
Diagnosis
Diagnosis of APS requires at least 1 clinical and 1 laboratory criterion.
Clinical criteria
Vascular thrombosis
- One or more clinical episodes of arterial, venous, or small-vessel thrombosis,
occurring within any tissue or organ
Complications of pregnancy
- One or more unexplained deaths of morphologically normal fetuses at or after 10
weeks’ gestation
- One or more premature births of morphologically normal fetuses at or before 34
weeks’ gestation
- Three or more unexplained consecutive spontaneous abortions before 10 weeks’
gestation
Laboratory criteria
-
Anticardiolipin antibodies (ELISA) - Anticardiolipin IgG or IgM antibodies
present on 2 or more occasions at least 6 weeks apart
-
Lupus anticoagulant (a sensitive phospholipid-based activated prothrombin time
such as the dilute Russell venom viper test) - Lupus anticoagulant antibodies
detected in the blood on 2 or more occasions at least 6 weeks apart
.‫ שבועות‬21 ‫ לפי הריסון ההפרש צריך להיות לפחות‬:‫הערה‬
‫טיפול‬
.)LMWH( ‫) הפרין‬3(-‫) קומדין ו‬1( ,‫) אספירין‬2( :‫ תרופות עיקריות בשימוש‬3
‫ הטיפול‬,‫ כנראה‬,‫ מ"ג יהיה‬57-211 ‫ אספירון‬,)‫בחולים ללא אירוע תרומבוטי (כולל נשים עם הפלות חוזרות‬
.‫המועדף‬
,)‫ אספירין‬+/-( ‫ בנשים הרות מעדיפים הפרין‬.‫אחרי אירוע תרומבוטי ראשון הבחירה היא בין הפרין וקומדין‬
.‫מאחר ולקומדין יש אפקט טרטוגני‬
‫ אחריה ניתן‬,‫קואגולציה לשנה‬-‫ יש לתת אנטי‬,‫ מוכח‬APS-‫ ו‬DVT/PE ‫לפי הריסון אחרי אירוע ראשון של‬
.‫לשקול המשך טיפול‬
.1-3 – ‫ מטרה‬INR
Surgical Care
Patients with APS, especially secondary APS, may require surgical interventions for
long-standing complications of their autoimmune disorder.
- Cardiac valvular surgery is recommended in patients with severe aortic
regurgitation due to the noninfectious vegetations that are seen as a result of APS.
- Splenectomy is recommended in patients with the chronic form of idiopathic
thrombocytopenic purpura.