Stroke at a young age

Strokes are always a somatoneurological problem. This applies not only to strokes in adulthood, but also, above all, to strokes in young people (according to the WHO classification, in the range from 15 to 45 years). Ischemic strokes in young people are characterized by etiologic heterogeneity; they can be caused by vascular disease, cardiogenic embolism, hematological disorders, drug abuse, oral contraceptives, migraine, and many other rarer causes. Hemorrhagic strokes are manifested by subarachnoid, parenchymatous, and intraventricular hemorrhage and are caused by arterial hypertension, aneurysm, hemangioma, atherosclerosis, hemiphilia, systemic lupus erythematosus, eclampsia, hemorrhagic vasculitis, venous thrombosis, Werlhof's disease, and cocaine abuse. In some cases, the cause of the stroke remains unknown even after a comprehensive examination of the patient.

This article does not cover the causes of stroke in childhood.

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The Main Causes of Stroke in Young Adults

• Hypertension (lacunar infarction)
• Cardiogenic embolism (endocarditis, atrial fibrillation, myocardial infarction, artificial valve, aortic valve stenosis, etc.)
• Vascular malformations
• Carotid artery dissection (pseudoaneurysm in trauma)
• Hypercoagulable states (systemic thrombosis in antiphospholipid syndrome, Snedon syndrome, use of oral contraceptives, etc.)
• Fibromuscular dysplasia (non-inflammatory segmental angiopathy of unknown etiology)
• Moyamoya disease (non-inflammatory occlusive intracranial vasculopathy of unknown etiology)
• Migraine infarction (migraine with aura)
• Vasculitis
• Hematological disorders (polycythemia, dysglobulinemia, DIC syndrome, etc.)
• Inflammatory diseases (rheumatoid arthritis, systemic lupus erythematosus, scleroderma, Sjogren's syndrome, periarteritis nodosa, Wegener's granulomatosis, sarcoidosis, etc.)
• Infectious diseases (neuroborreliosis, neurocysticercosis, Herpes zoster, bacterial meningitis, chlamydial pneumonia, hepatitis C, HIV infection)
• Tumor cell embolism
• Hereditary diseases (neurofibromatosis, epidermal nevus syndrome, autosomal dominant leukoencephalopathy with multiple deep small infarcts, Williams syndrome)
• Iatrogenic (treatment with high doses of female sex hormones, administration of L-asparagenase, high doses of intravenous immunoglobulin, interferon, etc.)

Hypertension and symptomatic arterial hypertension are the most common causes of ischemic (lacunar infarction) and hemorrhagic strokes. The latter also develop with vascular anomalies such as aneurysms and vascular malformations. Less frequently, hemorrhagic strokes develop as a complication of coagulopathy, arteritis, amyloid angiopathy, moyamoya disease, traumatic brain injury, migraine, and the use of certain drugs (cocaine, fenfluramine, phentermine). Hereditary intracerebral hemorrhage (Dutch and Icelandic types) has been described.

Differential diagnosis of causes of stroke in young people

Differential diagnosis of the causes of stroke at a young age requires a particularly thorough study of the patient's medical history, targeted somatic examination and the use of special methods for studying cerebral circulation and the cardiovascular system as a whole.

Currently, lacunar infarctions are diagnosed during life using computed tomography (but they may not be detected in the first 24 hours). Their size ranges from 1 mm to 2 cm. They develop due to destructive changes in the walls of penetrating (intracerebral) arteries in hypertension and are either asymptomatic or have characteristic syndromes: "pure motor hemiplegia" ("isolated hemiplegia or hemiparesis"), "pure sensory stroke" ("isolated hemihypesthesia"), "homolateral ataxia and crural paresis" ("ataxic hemiparesis"), "dysarthria and awkwardness in the hand". Less often, lacunar infarction can manifest itself in other syndromes.

Cerebral infarction may develop as a result of prolonged vasospasm during subarachnoid hemorrhage from an aneurysm. Cerebral infarctions developing at the height of a migraine attack (migraine infarction) are periodically described.

The following may be sources of cardiogenic embolism: endocarditis, atrial fibrillation, recent myocardial infarction, akinetic myocardial segment, dilated cardiomyopathy, intracardiac thrombus or tumor, changes in the heart valves in nonbacterial thrombotic endocarditis, prosthetic heart valves, right-to-left shunt, cardiac aneurysm. Possible sources of embolism may also be: mitral valve prolapse, distant (past) myocardial infarction, left ventricular hypertrophy, hypokinetic myocardial segment, atrial septal defect, calcifying stenosis of the aorta or mitral valve, aneurysm of the sinus of Valsalva.

Traumatic dissection of the carotid artery can be a cause of stroke in trauma (including mild trauma and hyperextension) and unsuccessful manual therapy. It has also been described as a spontaneous phenomenon in fibromuscular dysplasia, Marfan syndrome, Ehlers-Danlos syndrome type IV, migraine, and some other rare diseases.

A rare cause of stroke is moyamoya disease, which has a characteristic neuroimaging pattern.

When vasculitis is detected, it is necessary to clarify whether the process is limited to the central nervous system (isolated CNS angiitis) or whether there is a systemic disease such as Takayasu's disease, periarteritis nodosa, etc.

Hypercoagulable states (various variants of antiphospholipid syndrome, Sneddon syndrome, use of oral contraceptives, malignancy, antithrombin III deficiency, protein C deficiency, protein S deficiency, afibrinogenemia, pregnancy, malignancy, nephrotic syndrome, paroxysmal nocturnal hemoglobulinemia, diabetes mellitus, homocystinuria) and hematological disorders (polycythemia, dysglobulinemia, sickle cell anemia, disseminated intravascular coagulation syndrome, leukoagglutination, thrombocytosis, thrombocytopenic purpura, protein C deficiency, protein S deficiency, fibrinolytic disorders) are a well-known cause of strokes in young age. Hematological (and immunological) studies are of decisive importance in the diagnosis of this range of diseases.

Inflammatory systemic diseases (rheumatism, rheumatoid arthritis, systemic lupus erythematosus, scleroderma, Sjogren's syndrome, polymyositis, Henoch-Schonlein purpura, periarteritis nodosa, Churg-Strauss syndrome, Wegener's granulomatosis, sarcoidosis) as well as isolated angiitis of the central nervous system can lead to stroke as a complication of the underlying disease. Diagnosis is facilitated by the presence of symptoms of the current systemic disease, against the background of which neurological cerebral symptoms develop acutely.

Strokes against the background of infectious lesions of the nervous system (neurocysticercosis, neuroborreliosis, bacterial meningitis, Herpes zoster, chlamydial pneumonia, hepatitis C, HIV infection) also develop against the background of already existing clinical manifestations of a somatic or neurological disease, the diagnosis of which is crucial for identifying the nature of the stroke.

Tumor cell embolism is a rare cause of stroke (like fat embolism and air embolism) and remains unrecognized in a significant percentage of cases.

Hereditary diseases (homocystinuria, Fabry disease, Marfan syndrome, Ehlers-Danlos syndrome, pseudoxanthoma elasticum, Rendu-Osler-Weber syndrome, neurofibromatosis, epidermal nevus syndrome, CADASIL syndrome, Williams syndrome, Sneddon syndrome, mitochondrial encephalopathy with lactic acidosis and strokes - the so-called MELAS syndrome), capable of leading to stroke, are confirmed by clinical and genetic analysis, characteristic neurological, skin and other somatic manifestations.

Iatrogenic forms of stroke develop acutely in response to the administration of certain medications (high doses of female sex hormones, L-asparginase, immunoglobulin, interferon and some others), which serves as the basis for suspecting the iatrogenic origin of the stroke.

When examining the medical history of patients with stroke at a young age, it is important to pay attention to the presence of risk factors for certain diseases or characteristic somatic symptoms that occurred in the past or were detected during examination.

Certain eye and skin symptoms should not be underestimated. The likelihood of atherosclerosis as a possible cause of stroke increases in the presence of risk factors such as smoking, hypertension, hyperlipidemia, diabetes mellitus, radiation therapy; carotid artery dissection may be suspected in the presence of a history of trauma or manual manipulations in the neck area.

A cardiac cause of stroke may be suspected if frequent intravenous drug use is detected, or if stroke is associated with physical activity, deep vein thrombosis, heart murmur, heart valve surgery, or bone marrow transplantation are detected.

The hematological cause of stroke may be indicated by: sickle cell anemia, deep vein thrombosis, livedo reticularis, bone marrow transplant. Sometimes the key to unraveling the nature of the stroke is information about the use of oral contraceptives, alcohol abuse, a recent (within one week) febrile illness, pregnancy, HIV infection, a previous myocardial infarction, indications of stroke in the family history.

The presence of a "corneal arc" around the iris indicates hypercholesterolemia; corneal opacity may reflect Fabry disease; detection of Lisch nodules allows us to suspect neurofibromatosis; subluxation of the lens - Marfan disease, homocystinuria; retinal perivasculitis - gray cell anemia, syphilis, connective tissue disease, sarcoidosis, inflammatory bowel disease, Behcet's disease, Eales disease. Retinal artery occlusion may accompany cerebral embolism and multiple infarctions; retinal angioma - cavernous malformation, von Hippel-Lindau disease; optic nerve atrophy - neurofibromatosis; retinal hamartoma - tuberous sclerosis.

A simple examination of the skin sometimes suggests or directly indicates a particular somatic or neurological pathology. Osler nodes and traces of hemorrhage sometimes accompany endocarditis; xanthoma indicates hyperlipidemia; coffee-colored spots and neurofibromas - neurofibromatosis; fragile skin with easy bruising and blue sclera - Ehlers-Danlos syndrome (type IV); telangiectasias force us to exclude Osler-Weber-Rendu disease (hereditary hemorrhagic telangiectasia) and scleroderma; purple hemorrhages - coagulopathy, Henoch-Schonlein disease, cryoglobulinopathy; aphthous ulcers - Behcet's disease; angiokeratosis - Fabry disease; livedo reticularis - Sneddon syndrome; Facial angiofibromas - tuberous sclerosis.

NB: Cerebral vein thrombosis is a rare complication of various diseases. Cerebral vein thrombosis can be aseptic and septic (infections of the frontal, paranasal and other sinuses; otitis; pregnancy; carcinoma; dehydration; marasmus; therapy with androgens, cisplatin, aminocaproic acid; intravenous catheterization; nodular periarteritis; systemic lupus erythematosus; Wegener's granulomatosis; Behcet's disease; Degos disease; sarcoidosis; nephrotic syndrome; chronic lung diseases; diabetes mellitus; TBI; some hematological diseases; disseminated intravascular coagulation; arteriovenous malformation; Sturge-Weber disease; idiopathic cerebral vein thrombosis).

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Diagnostic studies in young people with stroke

Clinical blood test (platelet count, hemoglobin, red and white blood cell count, ESR), urinalysis, blood chemistry (including electrolytes potassium and sodium, glucose, creatinine, urea, bilirubin, liver function tests, AST and ALT, cholesterol, triglycerides, etc.), plasma osmolarity, blood gas composition, acid-base balance, pregnancy test, HIV infection, antibodies to phospholipids, lupus anticoagulant, cryoglobulins; A coagulogram is examined to determine fibrinogen, fibrinolytic activity, thrombin time, prothrombin, hematocrit, blood clotting time, antithrombin III, as well as the aggregation capacity of erythrocytes, blood viscosity, toxicological examination of blood and urine, the Wasserman reaction, a blood test for HB antigen, CT or MRI, ECG (sometimes Holter ECG monitoring), EEG, radioisotope scanning of the brain and blood flow study, ophthalmoscopy, various Doppler sonography methods, lumbar puncture, blood culture, if indicated - carotid or vertebral angiography, immunological blood test, chest X-ray. A consultation with a therapist is indicated.

When making a differential diagnosis, it is important to remember that some other diseases with a stroke-like course can also occur under the guise of a stroke: multiple sclerosis, partial (“hemiparetic”) epileptic seizures, brain tumor, brain abscess, subdural hematoma, brain contusion, migraine with aura, and dysmetabolic disorders in diabetes mellitus.

Chronic progressive hemiplegia syndrome is not considered here.

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