Patient history: 69 year old previously healthy male with onset of progressively worsening neurological problems after a brief

Handout case No. 5
Patient history: 69 year old previously healthy male with onset of progressively worsening
neurological problems after a brief febrile illness.
MRI of the brain showed extensive post-ischemic lesions of the white matter of both
hemispheres.
Onset of first neurological symptoms till time of death – 8 months
Postmortem examination:
Examination of the brain showed mild edema (1490g), cloudy meninges, minimal
atherosclerosis, and intraparenchymal bleeding in the region of the basal ganglia of the left
hemisphere with bleeding into the left lateral ventricle.
Apart from signs of bilateral bronchopneumonia, no significant pathological changes seen in
other internal organs.

Histology (brain):
Predominantly granulomatous vasculitis with involvement of small and medium-sized
leptomeningeal and cerebrocortical arteries
Vascular changes range from fibrinoid necrosis with intralumnial thrombosis to more chronic
changes with the presence of mural infiltration by epitheliod histiocytes, lymphocytes and
multinucleated giant cells. Multifocal ischemic lesions present with formation of glial scars.
In the region of the basal ganglia, focus of bleeding; surrounding vessels show inflammatory
changes.
Special staining for amyloid (Congo red, Saturn red), fungal organisms (Groccott
methenamine) and mycobacteria (Ziehl-Neelsen) all negative.
No histological features of vasculitis seen in other parenchymatous organs.
Diagnosis: Primary angiitis of the central nervous system (PACNS)

Differential diagnosis:
- Neuro-infection (syphilis, typhus, varicella-zoster, HIV)
- Bacterial endocarditis
- Systemic vasculitides (PAN, Wegener’s granulomatosis)
- Reversible vasoconstriction syndrome
- Demyelinating syndromes

Discussion: Primary angiitis of the CNS (PACNS) is a rare form of idiopathic vasculitis
typically involving small to medium-sized leptomeningeal, cortical and subcortical arteries.
As at 2010 only approximately 700 cases had been reported worldwide. The previously used
term ‘granulomatous angiitis’ is becoming less popular as granulomatous changes may not be
present in biopsy material.
Most reports describe male preponderance; with a reported mean age at onset of
approximately 50yrs. The principal clinical manifestations (headache, mental status changes
and focal neurological deficits) may present progressively or in an abrupt stroke-like fashion.
Brain and leptomeningeal biopsy demonstrating angiitis is the gold standard for diagnosis.
However, due to the disorder’s focal and segmental distribution, false-negative biopsies are
common. The histological features of PACNS are varied and similar to those seen in cerebral
vasculitides of other etiologies.
A combination of steroids and cyclophosphamide are recommended for treatment. Rituximab
(anti-CD20) is an alternative treatment option.
Reference:
Scolding N. Central nervous system vasculitis. Semin Immunopathol 2009;31:527-536
Birnbaum J, Hellman D. Primary angiitis of the central nervous system. Arch Neurol 2009;66:
704-709
Berlit P. Diagnosis and treatment of cerebral vasculitis. Ther Adv Nerurol Disord 2010;3:
29-42
Kuker W. Cerebral vasulitis: imaging signs revisited. Neuroradiology 2007;49:471-479

Source: http://smp.fingerland.cz/pripady/34_handout_5.pdf