Cerebrovascular Disease in Children and Young Adults



Cerebrovascular Disease in Children and Young Adults





Stroke is uncommon in children who are younger than 15 years. The annual incidence is approximately 2.5 cases per 100,000 children. Ischemic strokes in young adults (aged 15-40 years) constitute about 5% of all cases. Although the frequency of stroke in children and young adults is far less than that in individuals who are older than 50 years, the causes are more diverse.

The frequency of specific causes of ischemic stroke in patients who are younger than 40 years depends on age. Cerebral infarction in children (aged 1-15 years) most commonly results from cardiac diseases, head and neck trauma with dissection, migraine, hematologic disease, and other large vessel occlusive diseases (Table 21-1). In contrast to arterial thrombosis in adults, arterial thrombosis in children more commonly involves the intracranial internal carotid arterial system. Infarction is more commonly subcortical in children and particularly involves the striatum and internal capsule.


CLINICAL PRESENTATION

Because cerebrovascular ischemia in children is uncommonly caused by atherosclerotic occlusive disease, transient ischemic attack (TIA) before cerebral infarction is relatively uncommon. The clinical features of cerebral ischemia are similar to those noted in adults, but seizures are more common. Aphasias usually have some expressive component even when the lesion is posterior. A less common clinical presentation that is unique to children includes recurrent or alternating hemiplegia with or without associated headache, which may be caused by hemiplegic migraine, or, less commonly, by bilateral carotid artery thrombosis. The clinical features of craniocervical thrombosis or cerebral embolism vary according to the area involved; the anatomic and pathophysiologic principles are analogous to those described for adults in Chapters 1, 2, 3, 4, 5, 6, 7.


CAUSE

The differential diagnosis of stroke in children and young adults is outlined in Table 21-1. Many of these disorders are reviewed in the setting of cerebral infarction and intracranial hemorrhage in adults (see Chapters 12, 13, 14, 15, 16, 17). Causes of cerebral ischemia that occur much more commonly in children include congenital heart disease, head and neck trauma that leads to extracranial carotid or vertebral dissection, and distal thromboemboli or hemodynamic events. Hematologic disorders such as sickle cell disease are an important cause of infarction in children and lead to cerebrovascular disorders in 6% to 25% of patients (see Chapter 16, Hematologic Disease), and hematologic disorders can cause both ischemic and hemorrhagic stroke. Both ischemic and hemorrhagic events tend


to occur during a painful crisis and usually are seen in patients with more severe disease that includes frequent crises and decreased hematocrit value. Another cause of stroke that occurs more commonly in children than in adults is moyamoya disease.








TABLE 21-1 Differential Diagnosis of Stroke in Children and Young Adults














































































































































































































































































































Ischemia


Cardiac disease

Congenital heart disease

Rheumatic valve disease

Mitral valve prolapse

Patent foramen ovale

Bacterial or marantic endocarditis

Atrial myxoma

Pulmonary arteriovenous fistula

Rhabdomyoma

Cardiac or umbilical vein catheterization

Cardiomyopathies

Arrhythmias

Cardiac, thoracic surgery


Large vessel disease

Premature atherosclerosis

Dissection


Traumatic


Spontaneous

Inherited metabolic diseases


Homocystinuria


Fabry’s disease


Pseudoxanthoma elasticum


Sulfate oxidase deficiency


MELAS syndrome

Fibromuscular dysplasia

Infection


Bacterial


Fungal


Tuberculosis


Syphilis


Lyme disease

Vasculitis


Collagen vascular disease



Systemic lupus erythematosus, rheumatoid arthritis, Sjögren’s syndrome, polyarteritis nodosa


Takayasu’s disease


Wegener’s disease


Cryoglobulinemia


Behçet’s disease


Sarcoidosis


Churg-Strauss syndrome


Inflammatory bowel disease


Isolated CNS angiitis

Moyamoya disease

Radiation

Reversible cerebral vasoconstriction syndrome/Postpartum cerebral angiopathy

Toxic


Illicit drugs: cocaine, heroin, phencyclidine


Therapeutic drugs: L-asparaginase, cytosine arabinoside


Small vessel disease

Vasculopathy


Infectious


Noninfectious


CADASIL


Microangiopathy of brain, ear, and retina


Hematologic disease

Sickle cell disease

Leukemia

Hypercoagulable states


Antiphospholipid antibody syndromes


Protein C or protein S deficiency


Antithrombin III deficiency


Increased factor VIII


Resistance to activated protein C

Disseminated intravascular coagulation

Thrombocytosis

Polycythemia vera

Thrombotic thrombocytopenic purpura


Venous occlusion

Dehydration

Parameningeal infection (sinusitis, mastoiditis)

Meningitis

Neoplasm

Polycythemia

Leukemia

Inflammatory bowel disease


Hemorrhage


AVM


Cavernous malformation


Saccular aneurysm


Neoplasm

Primary CNS neoplasm

Metastatic neoplasm

Leukemia


Hematologic

Sickle cell disease

Hemophilia

Neoplasm


Leukemia

Thrombocytopenia


Moyamoya disease


Drug use (especially amphetamines, cocaine, phenylpropanolamine)


AVM = arteriovenous malformation; CADASIL = cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy; CNS = central nervous system; MELAS = mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes.

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Dec 14, 2019 | Posted by in NEUROLOGY | Comments Off on Cerebrovascular Disease in Children and Young Adults

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