Biomaterial Database

[Neurofibromatosis with occlusion of the internal carotid artery: case report]. 1985

N Kuwayama, and S Takahashi, and M Sonobe, and S Kagawa, and H Ikeda

Vascular lesions of the central nervous system in neurofibromatosis (NF) are uncommon. Cerebral arterial occlusive diseases in NF have been documented in only 29 patients. This paper reports a case of a 16-year-old male having occlusion of the right internal carotid artery with NF. He was admitted with an episode of clonic convulsion and left hemiparesis at the age of five years. On admission, he had multiple café-au-lait spots, depigmentation and fibroma molle throughout his body. Neurological examination revealed left hemiparesis with hypereflexia and pathological reflex on the left side. CT scan showed brain atrophy of the right hemisphere and low density area in the white matter of the right frontal lobe. Cerebral angiography showed occlusion at the extracranial portion of the right internal carotid artery. He underwent STA-MCA bypass on the right side. On histological examination of a section of STA, the intimal layer was thickened by elastofibrosis. The mechanisms of arterial occlusion in patients with NF are discussed with review of the literature.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D009072	Moyamoya Disease	A noninflammatory, progressive occlusion of the intracranial CAROTID ARTERIES and the formation of netlike collateral arteries arising from the CIRCLE OF WILLIS. Cerebral angiogram shows the puff-of-smoke (moyamoya) collaterals at the base of the brain. It is characterized by endothelial HYPERPLASIA and FIBROSIS with thickening of arterial walls. This disease primarily affects children but can also occur in adults.	Cerebrovascular Moyamoya Disease,Progressive Intracranial Occlusive Arteropathy (Moyamoya),Moya-Moya Disease,Moyamoya Disease, Classic,Moyamoya Disease, Primary,Moyamoya Disease, Secondary,Moyamoya Syndrome,Classic Moyamoya Disease,Disease, Classic Moyamoya,Disease, Moya-Moya,Disease, Primary Moyamoya,Moya Moya Disease,Moyamoya Diseases, Primary,Primary Moyamoya Disease,Primary Moyamoya Diseases,Secondary Moyamoya Disease
D009456	Neurofibromatosis 1	An autosomal dominant inherited disorder (with a high frequency of spontaneous mutations) that features developmental changes in the nervous system, muscles, bones, and skin, most notably in tissue derived from the embryonic NEURAL CREST. Multiple hyperpigmented skin lesions and subcutaneous tumors are the hallmark of this disease. Peripheral and central nervous system neoplasms occur frequently, especially OPTIC NERVE GLIOMA and NEUROFIBROSARCOMA. NF1 is caused by mutations which inactivate the NF1 gene (GENES, NEUROFIBROMATOSIS 1) on chromosome 17q. The incidence of learning disabilities is also elevated in this condition. (From Adams et al., Principles of Neurology, 6th ed, pp1014-18) There is overlap of clinical features with NOONAN SYNDROME in a syndrome called neurofibromatosis-Noonan syndrome. Both the PTPN11 and NF1 gene products are involved in the SIGNAL TRANSDUCTION pathway of Ras (RAS PROTEINS).	Peripheral Neurofibromatosis,Recklinghausen Disease of Nerve,von Recklinghausen Disease,Cafe-au-Lait Spots with Pulmonic Stenosis,Molluscum Fibrosum,NF1 (Neurofibromatosis 1),Neurofibromatosis I,Neurofibromatosis Type 1,Neurofibromatosis Type I,Neurofibromatosis, Peripheral Type,Neurofibromatosis, Peripheral, NF 1,Neurofibromatosis, Peripheral, NF1,Neurofibromatosis, Type 1,Neurofibromatosis, Type I,Pulmonic Stenosis with Cafe-au-Lait Spots,Recklinghausen Disease, Nerve,Recklinghausen's Disease of Nerve,Recklinghausens Disease of Nerve,Watson Syndrome,von Recklinghausen's Disease,Cafe au Lait Spots with Pulmonic Stenosis,Neurofibromatoses, Peripheral,Neurofibromatoses, Type I,Neurofibromatosis, Peripheral,Peripheral Neurofibromatoses,Pulmonic Stenosis with Cafe au Lait Spots,Syndrome, Watson,Type 1 Neurofibromatosis,Type 1, Neurofibromatosis,Type I Neurofibromatoses,Type I, Neurofibromatosis,von Recklinghausens Disease
D011859	Radiography	Examination of any part of the body for diagnostic purposes by means of X-RAYS or GAMMA RAYS, recording the image on a sensitized surface (such as photographic film).	Radiology, Diagnostic X-Ray,Roentgenography,X-Ray, Diagnostic,Diagnostic X-Ray,Diagnostic X-Ray Radiology,X-Ray Radiology, Diagnostic,Diagnostic X Ray,Diagnostic X Ray Radiology,Diagnostic X-Rays,Radiology, Diagnostic X Ray,X Ray Radiology, Diagnostic,X Ray, Diagnostic,X-Rays, Diagnostic
D012021	Reflex, Abnormal	An abnormal response to a stimulus applied to the sensory components of the nervous system. This may take the form of increased, decreased, or absent reflexes.	Hyperreflexia,Hyporeflexia,Abnormal Deep Tendon Reflex,Abnormal Reflex,Abnormal Reflexes,Bulbocavernosus Reflex, Decreased,Bulbocavernousus Reflex Absent,Hoffman's Reflex,Palmo-Mental Reflex,Reflex, Absent,Reflex, Acoustic, Abnormal,Reflex, Anal, Absent,Reflex, Anal, Decreased,Reflex, Ankle, Abnormal,Reflex, Ankle, Absent,Reflex, Ankle, Decreased,Reflex, Biceps, Abnormal,Reflex, Biceps, Absent,Reflex, Biceps, Decreased,Reflex, Corneal, Absent,Reflex, Corneal, Decreased,Reflex, Decreased,Reflex, Deep Tendon, Abnormal,Reflex, Deep Tendon, Absent,Reflex, Gag, Absent,Reflex, Gag, Decreased,Reflex, Knee, Abnormal,Reflex, Knee, Decreased,Reflex, Moro, Asymmetric,Reflex, Pendular,Reflex, Triceps, Abnormal,Reflex, Triceps, Absent,Reflex, Triceps, Decreased,Reflexes, Abnormal,Absent Reflex,Decreased Bulbocavernosus Reflex,Decreased Reflex,Palmo Mental Reflex,Pendular Reflex,Reflex Absent, Bulbocavernousus,Reflex, Decreased Bulbocavernosus,Reflex, Hoffman's,Reflex, Palmo-Mental
D002340	Carotid Artery Diseases	Pathological conditions involving the CAROTID ARTERIES, including the common, internal, and external carotid arteries. ATHEROSCLEROSIS and TRAUMA are relatively frequent causes of carotid artery pathology.	Carotid Atherosclerosis,Common Carotid Artery Disease,Internal Carotid Artery Disease,Arterial Diseases, Carotid,Arterial Diseases, Common Carotid,Arterial Diseases, External Carotid,Arterial Diseases, Internal Carotid,Atherosclerotic Disease, Carotid,Carotid Artery Disorders,Carotid Atherosclerotic Disease,Common Carotid Artery Diseases,External Carotid Artery Diseases,Internal Carotid Artery Diseases,Arterial Disease, Carotid,Artery Disease, Carotid,Artery Diseases, Carotid,Artery Disorder, Carotid,Artery Disorders, Carotid,Atherosclerotic Diseases, Carotid,Carotid Arterial Disease,Carotid Arterial Diseases,Carotid Artery Disease,Carotid Artery Disorder,Carotid Atheroscleroses,Carotid Atherosclerotic Diseases,Disorders, Carotid Artery
D002343	Carotid Artery, Internal	Branch of the common carotid artery which supplies the anterior part of the brain, the eye and its appendages, the forehead and nose.	Arteries, Internal Carotid,Artery, Internal Carotid,Carotid Arteries, Internal,Internal Carotid Arteries,Internal Carotid Artery
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293	Adolescent	A person 13 to 18 years of age.	Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D001157	Arterial Occlusive Diseases	Pathological processes which result in the partial or complete obstruction of ARTERIES. They are characterized by greatly reduced or absence of blood flow through these vessels. They are also known as arterial insufficiency.	Arterial Obstructive Diseases,Arterial Occlusion,Arterial Obstructive Disease,Arterial Occlusions,Arterial Occlusive Disease,Disease, Arterial Obstructive,Disease, Arterial Occlusive,Obstructive Disease, Arterial,Occlusion, Arterial,Occlusive Disease, Arterial