1.Primary angiitis of the central nervous system with myelopathy as initial clinical presentation
Cheng Yin Tan ; Ganeshwara Lingam ; Kartini Rahmat ; Suhailah Abdullah ; Ai Huey Tan ; Mei-Ling Sharon Tai ; Norlisah Ramli ; Wong Kum Thong ; Chong Tin Tan
Neurology Asia 2015;20(1):79-84
Primary angiitis of the central nervous system (PACNS) is a rare vasculitis restricted to the central
nervous system without systemic involvement. Delay in diagnosis and treatment is common due to its
non-specific symptoms and lack of non-invasive diagnostic tests. Myelopathy can occur in PACNS,
during the clinical course of the illness, with or without cerebral symptoms. We describe here a 51
year-old ethnic Chinese woman who presented initially with paraparesis without cerebral symptoms.
The diagnosis of PACNS was eventually made from brain biopsy when she subsequently developed
cerebral involvement. Despite aggressive treatment, the patient developed progressive neurological
deterioration and died. This patient demonstrates the rare occurrence of myelopathy as the sole initial
presentation of PACNS.
Central Nervous System
;
Spinal Cord Diseases
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