ICD-11

Specified other abnormalities of intracranial or extracranial arteries or veins. Entities in this section may be used in combination with other diagnostic codes in this block. Several of the entities may each cause different types of cerebrovascular disease such as TIA, cerebral ischemic stroke or intracerebral haemorrhage; may be associated with other clinical syndromes; or may be asymptomatic (not having caused acute focal dysfunction of the brain).

Section on Intracranial vascular malformations has been much revised compared to ICD-10 based on major scientific progress in this field.

Cerebral vasoconstriction syndromes and Posterior reversible encephalopathy are considered to be separate entities (as vasoconstriction is not always present in the latter).

”Progressive vascular leukoencephalopathy (Binswanger’s disease)” has been removed as a separate entity.

Cerebral cavernomas, still called cavernous angiomas, angioma cavernosum or cavernous hemangiomas, are vascular malformations in the brain that are asymptomatic or lead to seizures and/or cerebral haemorrhages. These are often found in an inheritable disorder with autosomal dominant inheritance.

Cerebral cavernous malformation (CCM) is composed of blood vessels devoid of muscular and elastic tissue that are lined with endothelial cells that do not have intervening tight junctions. CCMs can be sporadic or inherited as an autosomal dominant trait, in which multiple CCMs occur, and appear de novo.CCMs are prone to haemorrhage, focal neurological symptoms, and epileptic seizures. They can also occur withot symptoms. CCM have a characteristic MRI appearance.