Strokes in Young Patients 

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Thrombotic/thromboembolic

    Normal coagulation  Head

• Cardiomyopathies: primary/secondary

• Familial atrial myxoma

• Familial dysrhythmias

• Dyslipidemias

• Endocarditis

• Fungal infections, especially aspergillus

    Hypercoagulable status Head

• Nephrotic syndrome

• Dehydration (especially in neonates and infants)

• Antithrombin III deficiency

• Protein C deficiency

• Protein S deficiency

• Resistance to activated protein C

• Prothrombin gene mutation

• Antiphospholipid syndrome

• Platelet abnormalities

• Polycythemia and hyperviscosity

Hemoglobinopathies  Head

• Sickle cell anemia [Prengler M et al., 2002]

• Beta-Thalassemia major

Vasculopathy  Head

• CADASIL (Cerebral autosomal dominant angiopathy with subcortical infarct and leukoencephalopathy)

• HERNS (Hereditary endotheliopathy, retinopathy, nephropathy, and strokes)

• Susac syndrome (retinocochleocerebral vasculpathy)

• Fabry’s disease

Vasculitis  Head

• Vasculitis, non-infectious

• Vasculitis, infectious

Infection  Head

• HIV associated arteriopathies

Metabolic and mitochondrial diseases  

    Mitochondrial Head

• MELAS syndrome (mitochondrial encephalomyopathy, lactic acidosis and stroke like episode)

• Other mitochondrial diseases

    Metabolic Head

• Fabry’s disease

• Hyperhomocysteinemia and Homocystinuria

Other systemic diseases Head

• Hypertension

• Diabetes mellitus

• Systemic lupus erythematosus (SLE)

Note: Head

Sickle cell disease: sickle cell disease is the most commonly associated with stroke and most often occurs in children and 7.8% of homozygous patients develop stroke under the age of 14. A high leukocyte count and an acute decrease of hemoglobin are risk factors for stroke in patients with homozygous sickle cell disease.[Balkaran et al., 1992]. About 15% of children with sickle cell disease expreience cerebrovascular disorders (75% strokes, 20% intracerebral hemorrhage). Major pathology is extensive intracranial arterial disease with occlusions and stenosis. [Prengler M et al., 2002]

Hyperhomocysteinemia and Homocystinuria: Homocystinuria is due to cystathionine b-synthase deficiency and hyperhomocysteinemia can be caused by rare disorders of vitamin B12 or folate metabolism and probably folate deficiency in some mild and acquired cases. Elebated levels of homocysteine in blood increases the risk of atherosclerosis and venous thrombosis. Homocystinuria is a clinical syndrome that includes abnormalities of the eye, the skeletal, central nervous and vascular systems. The pathology of the CNS is mainly due to thromboemboic disease. Intimal thickening of the arterial wall is frequently observed, even in children.

Beta-thalassemia major: usually manifest soon after birth. The patients have an increased risk of thrombotic stroke, to which the postsplenectomy thrombocytosis contributes.

Reference:

Pavlakis SG, 2000; Natowicz M, 1987

Prengler M, Pavlakis SG, Prohovnik I, Adams RJ. Sickle cell disease: The neurological complications. Ann Neurol. 2002 May;51(5):543-52.