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Cerebral amyloid angiopathy revealed by rapidly progressing leptomeningeal

lesions

Article in Journal of Neurology · May 2014

DOI: 10.1007/s00415-014-7378-8 · Source: PubMed

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Fahed Zairi Emilie Le Rhun

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J Neurol (2014) 261:1432–1435
DOI 10.1007/s00415-014-7378-8

LETTER TO THE EDITORS

Cerebral amyloid angiopathy revealed by rapidly progressing

leptomeningeal lesions
Armelle Chouraki • Adeline Rollin-Sillaire • Vincent Deramecourt •

Fahed Zairi • Emilie Le Rhun • Charlotte Cordonnier •

Christine Delmaire • Claude-Alain Maurage • Florence Pasquier

Received: 10 February 2014 / Revised: 13 May 2014 / Accepted: 14 May 2014 / Published online: 25 May 2014
Ó Springer-Verlag Berlin Heidelberg 2014

Dear Sirs, The patient was referred to our hospital because of

Cerebral amyloid angiopathy (CAA) causes intracere- repeated and stereotyped episodes of numbness in the
bral haemorrhages and is associated with cognitive tongue with dysarthria followed by progressive paresthesia
impairment and Alzheimer’s disease. In autopsy series, the and hypoesthesia of the left arm and lower facial area in
estimated prevalence of CAA is high (20–40 % in non- the last 3 days. Examination revealed epicritic hypoaes-
demented subjects; 50–60 % in dementia) [1]. Brain thesia and slight hypopallesthesia of the left hemibody.
magnetic resonance imaging (MRI) usually reveals cere- Partial ictal manifestations were confirmed by a right
bral microbleeds (CMB), white matter (WM) changes, temporal epileptic focus on electroencephalography. Initial
lobar haemorrhages and silent acute cortical ischemic brain MRI showed an aspect of leptomeningitis in the right
lesions. Convexity subarachnoid haemorrhage (cSAH) and frontal (Fig. 1a, b) and cSS in the right central sulcus
cortical superficial siderosis (cSS) have been recently (Fig. 1c). Angio-MRI was normal. Three cerebrospinal
described [2]. Rarer, inflammatory forms (CAAi) are fluid (CSF) tests showed only a slight hyperproteinorachia
characterized by the presence of extensive WM and men- (0.7 g/L). All tests in search of an inflammatory, infectious,
ingeal lesions [3]. neoplastic or a paraneoplastic cause were negative except
Here, we report the case of a 64-year-old woman suf- for a positron emission tomography showing uptake of two
fering from a non-inflammatory CAA presenting with an small axillary lymph nodes (benign). Twelve days after
MRI suggestive of leptomeningitis in the clinical context of admission, MRI showed a bilateral extension of the pial
partial seizure. lesions (Fig. 1d, e, f) still progressing one month after

A. Chouraki
A. Rollin-Sillaire (&)
V. Deramecourt
E. Le Rhun

F. Zairi
E. Le Rhun
C. Cordonnier
C. Delmaire
Neurooncology, Lille University Hospital, 59037 Lille, France
C.-A. Maurage
F. Pasquier
Univ Lille Nord de France, UDSL, 59000, Lille, France E. Le Rhun
e-mail: [email protected] Department of Medical Oncology, Oscar Lambret Center,
59020 Lille, France
A. Chouraki
A. Rollin-Sillaire
V. Deramecourt
F. Pasquier
Laboratory of Excellence DISTALZ, Memory Clinic, EA 1046, C. Cordonnier
Lille University Hospital, 59000 Lille, France Stroke Unit, Neurology Department, Lille University Hospital,
59000 Lille, France
V. Deramecourt
C.-A. Maurage
Department of Pathology, Lille University Hospital, 59000 Lille, C. Delmaire
France Department of Neuroradiology, Lille University Hospital,
59000 Lille, France
F. Zairi
Department of Neurosurgery, Lille University Hospital,
59000 Lille, France

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J Neurol (2014) 261:1432–1435 1433

Fig. 1 Successive MRI done by the patient. First row: MRI on areas and appearance of hyperintensities in the left parietal and
admission, a Right frontal leptomeningeal hyperintensities on FLAIR occipital regions. f Progression of the Gadolinium-enhanced menin-
sequence. No corresponding hypointensities in T2* or hyperintensi- geal lesions. We considered this aspect and its progression as linked
ties on T1 were observed making the possibility of a large cSAH to a possible inflammatory process caused by the microglial activation
unlikely. b Gadolinium contrast enhancement of the right frontal detected at the biopsy possibly provoked by the amyloid deposits in
meninges; c Cortical superficial siderosis of the right central sulcus the vessels walls. Third Row MRI one month after admission. g, h, i
evoking cSS. Second row Second MRI. d, e Progression of the FLAIR Progression of the leptomeningeal lesions
hyperintensities to the right frontal, temporal, parietal and occipital

admission (Fig. 1g, h, e). The left occipital region was This case illustrates an unusual presentation of CAA with
biopsied. Histological examination revealed Ab-positive partial seizures, corresponding to Transient Focal Neuro-
CAA with intense leptomeningeal and cortical perivascular logical Symptoms and Signs (TFNSSs), and rapidly pro-
microglial activation without lymphocytic or granuloma- gressive leptomeningeal MRI abnormalities. When CAA is
tous angiitis (Fig. 2). revealed by leptomeningeal abnormalities, it is usually

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1434 J Neurol (2014) 261:1432–1435

Fig. 2 Brain and meningeal

biopsy of the left occipital
region. a Brain and meningeal
biopsy stained with
hematoxylin-eosin reagent.
Note the thickened,
hypercellular aspect of the pial
mater (asterisk). The
leptomeningeal arteriolar walls
were eosinophilic and laminated
(arrows). b Immunostaining of
Ab pathological staining of the
leptomeningeal and cortical
arteriolar walls.
c Immunostaining of CD68,
revealing infiltration of the pial
matter by macrophages.
d Immunostaining of CD3,
revealing the presence of only a
few scattered T lymphocytes.
Scale bar 50 l

associated with angiitis, clinically characterized by a con- The clinical and neuroradiological presentations of CAA
fusional state or an impaired level of consciousness, a rapidly are heterogeneous and emphasize the need to update the
progressive cognitive impairment, sometimes seizures, definition of this condition. It would be interesting to test
headaches and/or hallucinations [3–5]. Brain MRI shows the diagnostic value of combining clinical features with
WM hyperintensities, sometimes with multiple CMB, usu- MRI markers and CSF biomarkers (such as Ab40) and
ally without leptomeningeal enhancement [3, 4, 6, 7]. A compare these improved criteria with the neuropathologi-
cerebro-meningeal biopsy shows angiodestructive, some- cal examination.
times granulomatous inflammation, infiltrated by lympho-
cytes and multinucleated giant cells [3], absent here. Conflicts of interest On behalf of all authors, the corresponding
author states that there is no conflict of interest.
However, we noticed intense microglial activation in the
leptomeninges and perivascular spaces which has been
Ethical standard The manuscript submitted for publication has
reported in patients with sporadic severe CAA and lobar
been performed in accordance with the ethical standards laid down in
cerebral haemorrhage, multiple cortical infarction/CMB and the 1964 Declaration of Helsinki and its later amendments.
interpreted as a reaction towards the amyloid deposits in the
vessels walls [8]. Nonetheless, we cannot totally exclude
CAAi since only one biopsy was performed and has maybe References
missed the characteristic inflammation.
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