Page 59 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Overview of Primary and Secondary Vasculitides 35

Tuberculosis

Although the different forms of bacterial purulent meningitis was possible only after
development of modern bacteriologic methods and the introduction of the lumbar puncture as
a diagnostic measure by Quinke in 1891, TB meningitis was the first type of meningitis to be
described clinically as dropsy of the brain in 1768, and subsequently shown to be
inflammatory when meningeal tubercles and visceral tubercles were found to be identical in
1830. The tuberculoma, once the commonest intracranial tumor, is now exceptionally rare.
The chief neurological signs and symptoms of tuberculous meningitis reflecting meningeal
irritation are neck stiffness and positive Kernig sign; and raised intracranial pressure notably
headache and vomiting with mental changes, seizures, and focal neurological signs.
According to Smith and Daniel [149], arteritis is the rule in the vicinity of tuberculous
lesions, wherein vessel walls are invaded by mononuclear cells, with the adventitia more
heavily involved than the media. The subintimal and intimal regions form a layer of
homogenous fibrinoid material that later involves the media, and the vessel lumen is reduced
by inflammatory cell exudation beneath the fibrinoid material, the end results of which are
reduction or complete obliteration of the lumen, proliferative endarteritis, and cerebral
infarction. The vessels most heavily involved are those at the base of the brain and others in
the Sylvian fissure.

Pathologically-proven TB-associated CNS vasculitis was described in five heterogeneous
patients [149-151]. The first patient described by Smith and Daniel [149] was an 18-year-old
girl with fever, confusion, right hemiplegia, back and neck pain for several days followed by
incontinence, complete flaccid paraplegia, delirium dementia, generalized spasticity and
death. Postmortem examination showed advanced tuberculous meningitis with dense basal
adhesions, hydrocephalus, and obliteration of the spinal subarachnoid space by adhesions,
hemorrhagic infarction, and widespread arteritis with acute fibrinoid necrosis, without
tuberculomas. The second case was Patient 4 of Greitz [150], a 4-year-old girl with fever, left
arm weakness and increasing disorientation that rapidly progressed to coma, nuchal rigidity,
and spasticity of the legs. Vertebral angiography showed local widening of a branch of the
left posterior cerebral artery and a posterior fossa mass. The patient died soon afterward and
at postmortem examination there was tuberculous meningitis with typical tuberculous
vasculitis consisting of inflammatory changes in arteries at the base of the brain, notably in
small vessels with intimal swelling leading to concentric narrowing of vessel lumina.
Headache was a presenting symptom in one of three patients described by Leher [151], none
of whom had a prior history of tuberculosis, and all of whom had diagnostic angiographic
abnormalities and histopathologic evidence of tuberculous arteritis. A third pathologically-
proven case of TB-associated CNS vasculitis was Patient 1 of Leher [151], a 33-year-old man
with anorexia and insomnia. Carotid angiography showed narrowing of the supraclinoid ICA
as well as narrowing of two convexity vessels in the Sylvian fissure. At postmortem
examination there was marked eccentric left fronto-pareital region arterial narrowing due to
fibroblastic proliferation of the intima with large numbers of inflammatory cells below the
elastica. Headache was not mentioned in two other histopathologically confirmed patients
with tuberculous vasculitis. The fourth case was Patient 2 of Leher [151], a 33-year-old man
presented with fever and stiff neck. Cerebral angiography demonstrated irregularity of the
supraclinoid ICA and reduction in the caliber of the convexity MCA vessels. He died shortly
thereafter and postmortem examination disclosed tuberculous leptomeningitis and arteritis of

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