Title : A case of vile vindictive primary CNS vasculitis
Abstract:
Introduction/Objective: Primary Angiitis of the Central Nervous System (PACNS) is rare and causes inflammation of the small and medium vessels of the brain, leptomeninges and spinal cord. We present a case of PACNS, demonstrate the diagnostic challenges, and appreciate outcomes with and without treatment in a young patient.
Case: A 29-year-old male with no past medical history presented with a week of non-migrainous headaches, vertigo, slurred speech, and unsteady gait. Neurological exam was significant for dysarthria, mild dysmetria, wide-based ataxic gait, and inability to walk tandem. A brain CT revealed a hemorrhagic cerebellar lesion with mass effect. A brain MRI demonstrated numerous foci of hemosiderin. He was treated with pulsed steroids and improved, was transitioned to an oral steroid taper, and discharged.
A few months later the patient was admitted for recurrent headaches, dysarthria, and gait ataxia. An interval CT brain showed resolution of the cerebellar hematoma; however, brain MRI indicated an increase in small vessel infarcts. The patient underwent a frontal lobe biopsy compatible with CNS vasculitis. He was acutely treated with pulse steroids with dramatic improvement and bridged to cyclophosphamide.
His condition was controlled for about five years when he felt well and self-discontinued cyclophosphamide. He then presented with altered mental status, aphasia, increased ataxia, and right sided weakness. He, again, received pulse steroids followed by a steroid taper and mycophenolate was initiated. The patient was followed in the clinic and transitioned to rituximab. His aphasia and weakness have resolved.
Discussion: Primary Angiitis of the Central Nervous System (PACNS) results in vascular inflammation of the small and medium vessels isolated to the brain, leptomeninges and spinal cord. A rare disorder, it is present in 2.4 cases per 1 million person-years, disproportionately affects males compared to females (2:1), and remains of unclear etiology. Given the non-specific and variable symptomatology, mimicking pathologies, and imperfection of investigative modalities, PACNS poses a diagnostic challenge.
Conclusion:
Primary Angiitis of the Central Nervous System (PACNS) is rare and causes inflammation of the small and medium vessels of the brain, leptomeninges and spinal cord. Prognosis appears to be favorable with treatment, but without treatment, PACNS has a vile and vindictive progressive course, as occurred in this case.
