Primary Angiitis of CNS

Primary angiitis of the CNS--an enigma that needs world-wide efforts to be solved.

Primary or isolated angiitis of the central nervous system (PACNS) is still one of the enigmas in clinical neurology. Diagnostic criteria for the diagnosis of PACNS include the symptoms of a multifocal or diffuse CNS-disorder with remitting or progressive course, cerebrospinal fluid (CSF) and magnetic resonance imaging (MRI) findings supporting the diagnosis of vasculitis, and finally either an...

Cyclophosphamide responsive primary angiitis of the CNS in a 61-year-old female

Dear Sir – A 61-year-old female with no significant past medical history presented with flu like symptoms including fever, chills and headaches after returning from a trip to Hawaii. First saw her primary care physician who advised conservative management. A couple of days later, she presented with worsening of her headaches and was admitted for meningitis work up. The neurologic exam was unrem...

Isolated angiitis of the CNS presenting as subarachnoid haemorrhage.

Isolated angiitis of the CNS (IACNS) commonly presents with recurrent ischaemic or haemorrhagic infarcts, but subarachnoid haemorrhage is rare. Three patients with IACNS and subarachnoid haemorrhage are reported. Florid granulomatous angiitis with Langhans and foreign body type giant cells was found at necropsy in a child with sudden death. In two other patients the diagnosis was made angiograp...

Hypersomnia caused by isolated angiitis of the CNS.

A 42-year-old woman presented gradual development of hypersomnia and memory disturbance. T2-weighted and FLAIR MRI scans revealed a high intensity abnormality in the hypothalamus. Stereotactic brain biopsy specimen revealed prominent infiltration of the lymphocytes in the vessel walls. Since there was no evidence of systemic angiitis, the diagnosis of isolated angiitis of the CNS (IAC) was made...

Primary Angiitis of the Central Nervous System