INTRODUCTION Granulomatous inflammation is a distinctive ...

identified within the CNS (Calabrese and Duna, 1995).
Granulomatous angiitis of the nervous system, is a name that draws attention to a pathological
feature of the disease seen in some, but not all, cases. More recently, the preferred name for this
form of vasculitis has been primary angiitis of the central nervous system (PACNS). Finally,
recognition that a subgroup of patients appear to have milder disease courses has led to the
designation of benign angiopathy (as opposed to angiitis) of the CNS, or “BACNS” (Calabrese et
al., 1993).
Pathology
The cause of granulomatous angiitis is not known. It is very possible that viral infections (which
remain difficult to diagnose) initiate the inflammatory process that somehow becomes self–
sustaining. Although anecdotal experience and case series associate BACNS with heavy nicotine or
caffeine use as well as with oral contraceptive and cold remedy use, the true associations between
these exposures and the development of BACNS remain uncertain (Calabrese, 1995).
Pathologically there are monocytes, lymphocytes and plasma cells infiltrating the walls of small
arteries, particularly in the leptomeninges, although occasional involvement of the internal carotid
and vertebral arteries is seen. Numerous small foci of infarction are common, but there maybe
large areas of ischemia, sometimes with superimposed hemorrhage (George et al., 1995).
Epidemiology
The distribution of PACNS is nearly equal between the sexes, with perhaps a slight male
predominance (4 3). The mean age of people affected by the disease is approximately 42 years, but
the range is wide. In contrast, patients with BACNS tend to be young women, often those with
previous histories of headaches (such as migraines). These patients often have histories of heavy
nicotine or caffeine use, over–the–counter cold remedy use (e.g., ephedrine), and oral
contraceptive or estrogen replacement therapy (Hankkey, 1991).
Clinical picture
The clinical manifestations of PACNS and BACNS may be identical, and include many neurologic
symptoms and signs such as headache, focal weakness (like that with a stroke), seizures, bleeding
within the CNS, confusion, disorders of memory, and altered consciousness. All of these symptoms
and signs are non-specific, and can be mimicked by a variety of other conditions (Arora and
white, 1994).
There are two main differences in the clinical presentations of PACNS and BACNS. First,
whereas PACNS patients are more likely to develop symptoms subacutely and remain
undiagnosed for months, those with BACNS are more likely to have relatively acute presentations
and be diagnosed within weeks of onset (in either case, however, making the diagnosis is
challenging). Second, without treatment, patients with PACNS tend to have progressively
downhill courses that often lead to death. In contrast, BACNS patients may require less aggressive
treatment than PACNS (Bettoni et al., 1984).
Investigations
Because many diseases may mimic granulomatous angiitis and because its treatment is potentially
dangerous, it is essential to confirm the diagnosis before starting treatment. Nearly all patients
therefore require either an angiogram or biopsy of the brain. Tests such as MRI studies and
lumbar punctures (spinal taps) are also helpful in the work–up of a patient with possible