Avtor/Urednik		Polenčič, Aljoša; Popović, Mara; Vodušek, David B
Naslov		Primarni vaskulitis osrednjega živčevja, omejen na možgansko deblo
Prevedeni naslov		Primary vasculitis of the CNS restricted to the brainstem
Tip		članek
Vir		Zdrav Vestn
Vol. in št.		Letnik 74, št. 7-8
Leto izdaje		2005
Obseg		str. 435-8
Jezik		slo
Abstrakt		Background. Primary or isolated vasculitis of the central nervous system (PVCNS) is a rare disease with an atypical clinical picture. It involves both small and mediumsized leptomeningeal, cortical and subcortical vessels. Etiology and pathogenesis are unknown. The first clinical sign is usually a headache and later in the disease virtually every neurological symptom and sign has been reported. The golden standard for diagnosis is a biopsy. High doses of corticosteroids and cyclophosphamide have been recommended for treatment. Patient and methods. A case of a 61 years old women with diabetes and hypertension is reported. She sudenly developed signs of focal ischemic brain stem lesion and had lesions visualised on MR in brain stem and both hemisfers. After temporary remission she developed similar signs five months later. She had died before the diagnostic procedures were performed. The diagnosis of PVCNS isolated to the brain stem was made post mortem. Conclusions. The diagnostic and therapeutic challenges of PVCNS are big even for an experienced clinician. Diagnosis depends on a combination of clinical, imaging and histological features. Often the diagnosis is confirmed post mortem.
Izvleček		Izhodišča. Primarni vaskulitis osrednjega živčevja (PVOŽ) je redka bolezen z neznačilno klinično sliko. Prizadene lahko male in srednje velike leptomeningealne, kortikalne ali subkortikalne arterije in vene. Etiologija in patogeneza bolezni nista znani. Prvi klinični znak je navadno glavobol, v nadaljevanju bolezni pa so bili v literaturi opisani praktično vsi nevrološki znaki in simptomi. Zlasti standard diagnostike je biopsija. Za zdravljenje svetujejo visoke odmerke kortikosteroidov s ciklofosfamidom. Bolnica in metode. Opisan je primer 61-letne sladkorne bolnice z zvišanim krvnim tlakom, ki je nenadno zbolela z znaki žilne okvare možganskega debla in imela z magnetnoresonančnim slikanjem ugotovljene spremembe ne samo v možganskem deblu, ampak tudi v globini obeh hemisfer velikih možganov. Po prehodnem izboljšanju so se po petih mesecih težave ponovile. Umrla je pred ponovno izpeljavo diagnostike. Posmrtno smo dokazali primarni vaskulitis osrednjega živčevja, omejen na možgansko deblo. Zaključki. Diagnosticiranje PVOŽ v času bolnikovega življenja je tudi za izkušenega klinika zelo zahtevno. Upoštevati je potrebno klinično sliko in opraviti vrsto svetovalnih preiskav. Pogosto pa se diagnoza potrdi šele nevropatološko.
Deskriptorji		CEREBRAL ARTERY DISEASES VASCULITIS CEREBRAL ANGIOGRAPHY MIDDLE AGE BIOPSY