Cerebral hematoma as an initial presentation of focal cerebral vasculitis Fábio Carvalho (1) ; Mariana Santos (2) ; Cristina Gonçalves (1) ; Teresa Palma.

Cerebral hematoma as an initial

presentation of focal cerebral vasculitis

Fábio Carvalho (1); Mariana Santos (2); Cristina Gonçalves (1); Teresa Palma (1)

1- Unidade Clínica Autónoma Neurorradiologia; 2- Serviço de Neurologia;Hospital Professor Doutor Fernando Fonseca

Introduction:

• Vasculitis describes generalised inflammation of vessels.

Vasculidities can involve almost any organ system.

• Cerebral vasculitis is an unusual disorder with numerous

causes. 

• When restricted to the brain and spinal cord, it can be called

primary angiitis of the central nervous system (PACNS), a rare form of

vasculitis.

• When affecting he central nervous system (CNS) it can lead to

disruption of the normal structural and physiologic characteristics of the

affected vessels, which in turn results in vascular occlusion and/or

formation of aneurysms with consequent ischemia and hemorrhage.

• The etiology and the pathogenesis of PACNS are still unknown,

but it is believed that the fundamental mechanism is immunologic.

• Due to the peculiarities of this disease, and the difficulties

associated with its study, the epidemiological characteristics related to it

are not yet known.

Clinical Case:

38 years old woman with insidious onset of asthenia, myalgia, chills,

weight loss and mild holocranial pulsatile headache, more intense in

frontal region.

The patient was admited with focal motor seizures involving the right

limbs. After their pharmacological control, she presented with right

hypotonic hemiparesis.

Multiple laboratory, imaging and anatomopathologic exams allowed the

definite diagnosis.

First CT:

Hemorrhagic lesion, roughly oval, measuring at its longest axis (transverse) about 16mm in the high fronto-parietal region with a discreet perilesional edema.

MRI Study:

Axial T2WI Axial T2 FLAIR Axial T2*

Axial DWI Cor. T1 post-Gad.

Axial T1 post-Gad.

MRI depicted Intra-axial lesion, with acute hemorrhagic

component, at the high right fronto-parietal region, roughly oval, measuring

at its longest axis (transverse) about 16mm. It was surrounded by marked

vasogenic oedema and presented heterogeneous contrast enhancement

post-gadolinium. No other intra-axial areas of abnormal signal were

detected.

The patient underwent other MRI, which were not instructive as to

the nature of the lesion. The case was discussed with Neurosurgery. It

was decided stereotactic biopsy.

Stereotactic biopsy of the mass lesion revealed mononuclear

inflammatory process (T lymphocytes) vascular transmural (non-

granulomatous or necrotic) associated with hemorrhage.

DSA:

Not observed in the intracranial circulation segmental stenosis suggesting vasculitis. Small area, on the right, of "emptiness" vascular in relation to the region of the biopsy.

Conclusions:

o Central nervous system (CNS) vasculitis is a rare and

diagnostically challenging disorder because patients present with

nonspecific symptoms of variable severity and progression, such as

headache and encephalopathy.

o Not rarely, brain biopsy is necessary to establish the diagnosis,

because imaging, angiography and even cerebrospinal fluid analysis fail.

References:Osborn AG. Osborn’s Brain: Imaging, Pathology, and Anatomy. Wolters Kluwer | Lippincott Williams & Wilkins; 2012.

Alehan FK, Boyyat F, Baskin E, et al, Focal cerebral vasculitis and stroke after chickenpox, European Journal Paediatric Neurology 2002;6(6):331-3.

R.I. Aviv, S.M. Benseler, E.D. Silverman, et al, MR Imaging and Angiography of Primary CNS Vasculitis of Childhood, AJNR Am J Neuroradiol January, 2006 27: 192-199