Malformations vascular CNS

vascular CNS

The term CNS vascular malformations encompasses several non-neoplastic vascular lesions of the CNS. McCormick in 1966 identified 4 types of vascular malformations:

    • Arteriovenous malformation
    • Cavernous angioma
    • Venous angioma
    • capillary telangiectasia.

Genetic aspects. There are several types of CNS vascular malformations, for example type 1 (*116860, 7qll.2-q21, damage to the CCM1, CAM, 9i genes). Clinically: cavernous angioma of the brain, intracranial hemorrhage, focal neurological symptoms, migraine, acute chiasmal syndrome, angiomas of the retina, skin, liver, cavernous angiomas of soft tissues. X-ray: cavernous malformations on MRI, intracranial calcification. Synonyms: cavernous familial angioma, hemangioma of cavernous malformations of the central nervous system and retina, cavernous angiomatous malformations.

Clinical picture

    • Seizures or headache (most common)
    • Progressive neurological deficit (usually as a result of spontaneous intracranial hemorrhage).

Diagnostics

    • Angiography. Sometimes, vascular malformations of the CNS are unbelievable to be detected angiographically due to obliteration of vessels as a result of bleeding, slow blood flow and small size of pathological vessels.
    • CT and MRI.

Surgical treatment (indicated mainly for hematoma evacuation and repeated bleeding). See also Aneurysm intracranial, Aneurysm arteriovenous. ICD. D1S.O Hemangioma of any localization of SS. 116860 Vascular malformations of the CNS, type 1 Literature. Gunel M et al: A founder mutation as a cause of cerebral cavernous malformation in Hispanic Americans. NewEng. J. Med. 334: 946-951.1996

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