Malignant angioendotheliomatosis (intravascular lymphomatosis) clinically simulating primary angiitis of the central nervous system

primary angiitis of the central nervous system

primary angiitis of the central nervous system (pacns) is an idiopathic disorder (vasculitis) restricted to the central nervous system (cns). it often presents with focal neurological deficits suggesting stroke or a combination of confusion and headache. we herein report three cases with various combinations of fever, partial seizure, encephalopathy, paresis, headache and ataxia. one of them wa...

Intravascular lymphomatosis (malignant angioendotheliomatosis) presenting as pulmonary hypertension.

Intravascular lymphomatosis is a rare lymphoma characterized by proliferation of malignant cells within the lumen of small blood vessels. We describe a case of intravascular lymphomatosis resulting in pulmonary hypertension, hypoxemia, and dyspnea. This lymphoma occasionally responds to combination chemotherapy, suggesting that pulmonary hypertension secondary to intravascular lymphomatosis may...

Primary Angiitis of the Central Nervous System

Primary Angiitis of the Central Nervous System.

Primary angiitis of the central nervous system (PACNS) is rare but often included in the differential diagnosis for unusual or unexplained neurologic symptoms owing to its array of clinical manifestations. PACNS is not a single entity, but rather an umbrella term for distinct subtypes of disease affecting different parts of the CNS, different sized vessels, and with different histologic and pat...

Primary Angiitis of the Central Nervous System

Primary angiitis of the central nervous system (PACNS) is an idiopathic disorder (vasculitis) restricted to the central nervous system (CNS). It often presents with focal neurological deficits suggesting stroke or a combination of confusion and headache. We herein report three cases with various combinations of fever, partial seizure, encephalopathy, paresis, headache and ataxia. One of them wa...