Stroke in young adults

Created 17/12/2021, last revision 12/05/2023

  • young adults stroke = stroke in persons aged 18 to 55 years
    • some authors use an upper age limit of 45 or 49 years
  • young adults comprise 10%–15% of all stroke patients, with a significant increase after the age of 40   [Smajlović, 2015]
  • compared to the older population (where arteriolopathy, atherothrombosis, and cardioembolism due to AFib predominate), the spectrum of risk factors and possible causes are differently distributed and are broader in the younger population  [Schöberl, 2017]
  • approximately from the age of 40, the proportion of vascular risk factors increases, together with the proportion of large and small vessel disease (TOAST 1 and 3)
  • a thorough etiologic diagnostic evaluation and individualized secondary prevention are essential
  • the acute treatment is the same for patients of all ages ( recanalization therapy if eligible)

Etiology and risk factors

  • the identification of rare causes of juvenile stroke requires a significant diagnostic effort
  • the most common etiology of stroke in young adults is a cryptogenic stroke (20-40%) and cardioembolic stroke  Etiology of juvenile strokes (Smajlović, 2015) [Smajlović, 2015]
    • TOAST classification may overestimate patients with stroke of undetermined etiology (TOAST 5), mainly because patients with two or more potential etiologies fall into this group
  • in young patients with carotid artery occlusion or stenosis, exclude dissection and vasculitis
  • modifiable vascular risk factors are similar for younger and older patients
    • hypertension, heart disease (including atrial fibrillation), dyslipidemia, and diabetes mellitus are the most common risk factors among the elderly
    • in young stroke patients, the most common vascular risk factors are dyslipidemia, smoking, and hypertension  [Putaala, 2012]
    • patients without documented risk factors have less frequent recurrent ischemic strokes and non-cerebrovascular arterial events
DDx in young stroke patients using the TOAST classification
Large-Artery Atherosclerosis (TOAST 1)
  • in young adults, large-artery pathologies are mostly caused by vasculitis or non-inflammatory vasculopathies (which belong to TOAST 4)
  • premature atherosclerosis of large vessels is rare (more likely after the age of 40)
Cardioembolism (TOAST 2) → cardioembolic stroke
  • valvular heart disease
  • patent foramen ovale (PFO)
  • intracardiac tumors (myxoma, fibroelastoma)
  • endocarditis
  • septal defects
  • cardiomyopathy
  • myocarditis
  • atrial fibrillation (rare in young people)
Small artery disease (TOAST 3)
  • typical arteriolopathy with a lacunar syndrome is uncommon in younger patients
  • hereditary cerebral microangiopathies are included in TOAST 4 (CADASIL, CARASIL, CARASAL, etc.)
Stroke of other determined etiology (TOAST 4)

→ vasculitis overview

  • bony stroke
    • rare bone or cartilage anomalies affecting arteries supplying the brain
    • may be considered in patients with recurrent ischemic stroke of unknown cause in the same vascular territory
    • in addition to conventional vascular imaging, the dynamic imaging modalities with the patient’s head rotated or reclined may confirm the diagnosis  (e.g., Bow hunter´s syndrome)
  • CADASIL   Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL)
  • CARASIL   CARASIL - arc sign (Roeben, 2016)
  • Fabry  Pulvinar sign and leukoencephalopathy in Fabry disease
  • MELAS  Typical MRI lesions in MELAS
  • HANAC (Hereditary angiopathy with nephropathy, aneurysms, and muscle cramps)
  • HERNS (Hereditary endotheliopathy with retinopathy, nephropathy, and stroke) → more here
  • Susac syndrome (retino-cochleo-cerebral vasculopathy)
    • rare microangiopathy of the cochlea, retina, and brain of unknown etiology (probably vasculitic in origin)   Susac syndrome - "string of pearls" in internal capsule
  • fat embolism
    • typically occurs after trauma (long bone fractures) and surgery (including plastic surgery with fat removal)
  • air embolism (microscopic x macroscopic) Macroscopic air embolization after endovascular surgery
    • a consequence of the incorrect insertion of a venous catheter into an artery [Riebau, 2004]
    • improper extraction of the central venous catheter (CVC) [Brockmeyer, 2009]
    • repeated IV applications in combination with pulmonary AV shunt or PFO
    • during catheterization
  • embolization of cholesterol particles from plaques should be assessed as TOAST 1 → Cholesterol Embolization Syndrome (CES)  Retinal cholesterol embolization (Hollenhorst crystals)
    • spontaneous x iatrogenic
  • diffuse lesions Diffuse cerebral edema due to hypoperfusion during surgery in ECC (Extra Corporeal Circulation)  or border zone (watershed) infarcts Border zone infarcts (BZI)
  • etiology
    • systemic hypotension
      • cardiac failure
      • extracorporeal circulation (ECC) surgery
    • hypoperfusion in carotid occlusion/stenosis (⇒ TOAST 1! )
  • various mechanisms ( e.g., vasospasm, cardioembolism in endocarditis)
  • oral contraceptives (usually in combination with a hypercoagulable state and/or smoking)
  • cocaine, crack, amphetamines, LSD, and heroin (drugs often cause IC bleeding)
  • sympathomimetics, ergotamine, sumatriptan
  • various mechanisms (most usually due to a hypercoagulable state or cardioembolism)
  • specific causes of stroke in pregnancy:
    • preeclampsia/eclampsia
    • amniotic fluid embolization (AFE)
    • choriocarcinoma
    • postpartum cerebral angiopathy
    • postpartum/peripartum cardiomyopathy (PPCM)
Cryptogenic stroke (TOAST 5)  → see here
In addition to the TOAST classification, stroke in pregnancy and puerperium of various etiopathogenesis must be mentioned

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Stroke in young adults