Age and Ageing 2020; 49: 487–489
doi: 10.1093/ageing/afaa010
Published electronically 7 March 2020

© The Author(s) 2020. Published by Oxford University Press on behalf of the British Geriatrics Society.
All rights reserved. For permissions, please email: journals.permissions@oup.com

CASE REPORT

A curable pseudo-dementia related to an
atypical presentation of giant cell arteritis

1

Unité mobile de Gériatrie, CHU Clermont-Ferrand, 63000 Clermont-Ferrand, France
Département de Gériatrie, APHP, Hôpital Bichat, 75018 Paris, France
3
Département de Gériatrie, APHP, Hôpital de la Pitié-Salpêtrière, 75013 Paris, France
2

Address correspondence to: Clément Lahaye. Tel: +33 4 73 75 45 94; Fax: +33 4 73 75 45 99.
Email: clahaye@chu-clermontferrand.fr

Abstract
A 70-year-old patient was admitted with rapidly progressive cognitive decline associated with limitations in activities of daily
living, weight loss and cerebellar ataxia. The diagnosis of giant cell arteritis (GCA) with vascular involvement was made, based
on the presence of a metabolically active vasculitis of the brachiocephalic trunk on 18 FDG-PET imaging. Temporal artery
biopsy also revealed pan-arteritis. A progressive regression of cognitive disorders occurred under corticosteroid treatment and
immunosuppressive therapy. Previously published case reports concerning this atypical presentation of GCA are scarce. They
suggest that numerous cognitive symptoms, such as impairment of short-term memory, disorientation, delirium, impaired
attention or visual hallucinations might be related to GCA. Thus, this diagnosis should be considered as a curable cause of
unexplained cognitive impairment associated with weight loss and systemic inflammation.

Keywords: cerebral vasculitis, dementia, giant cell arteritis, older people, reversible cognitive impairment
Key points
• Rapid cognitive decline associated with systemic inflammation should suggest the diagnosis of an atypical giant cell arteritis
(GCA).
• Positron emission tomography affirms the presence of active large vessel vasculitis.
• Glucosteroid therapy may cure giant cell arteritis but also restore cognitive function.

Case report
Giant cell arteritis (GCA) is the most common form of
vasculitis in older adults [1]. The typical clinical presentation associates headache, scalp tenderness and jaw claudication inflammatory arthralgia and constitutional symptoms.
High-dose corticosteroid therapy generally allows a rapid
recovery. However, atypical forms of GCA also exist, and
a delayed diagnosis may lead to potentially serious consequences, especially in older patients.
A 70-year-old patient was admitted for rapidly progressive
cognitive decline. He had a history of alcohol abuse, type

2 diabetes since 2015 and hypertension. Clinical examination revealed psychomotor slowing, apathy, asthenia, weight
loss of 15 kg in 6 months, axial extrapyramidal syndrome
without resting tremor, cerebellar ataxia and sarcopenia.
There were neither disturbed alertness, nor fasciculation,
abnormal movements, orthostatic hypotension, headache,
abnormality of cranial nerves, sensitive or motor deficit. His
Mini Mental State Examination (MMSE) score was 20/30,
Frontal Assessment Battery (FAB) score was 13/18. He also
presented with limitations in activities of daily living, both
for basic and instrumental activities (4/6 on the Katz scale
and 1/4 on the Lawton scale, respectively). Symbolic gestures

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Clément Lahaye1 , Manuel Sanchez2 , Audrey Rouet3 , Ariane Gross2 , Nathalie Faucher2 ,
Agathe Raynaud-Simon2 , Matthieu Lilamand2

C. Lahaye et al.

and visuo-constructive praxis were abnormal. There was no
obvious depressive syndrome.
Blood analysis showed elevated C-reactive protein
(27 mg/L). Thyroid-stimulating hormone, vitamins B1
(134 nmol/L), B6, B9 and B12 and liver enzymes were
normal. Nuclear antibodies, anti-neutrophil cytoplasmic
antibodies, serologies for HIV, TPHA/VDRL, Bartonella,
rickettsiosis, Coxiella and brucellosis were negative. Three
sputum tests for Mycobacterium tuberculosis as well as
interferon-γ release assays excluded the diagnosis of
tuberculosis. Cerebrospinal fluid analysis (CSF) showed
0.44 g/l of proteins, 3.3 mmol/l of glucose, 6 cells/mm3 ,
negative culture. The polymerase chain reaction tests for
John Cunningham virus, Herpes simplex virus 1 and
2 and varicella-zoster virus were negative, as well as
antineuronal antibody, acetylcholine anti-receptor antibody
and Tropheryma Whipplei polymerase chain reaction analysis.

488

The CSF levels of protein Aβ42 (1109 pg/mL) and P-tau
were normal (46 pg/mL), whereas total tau was elevated at
2019 pg/mL (N < 350 pg/mL).
Electroencephalogram and electromyogram of the four
limbs were normal. Medullary MRI excluded spinal cord
compression or myelopathy. Thoracoabdominopelvic computed tomography showed no specific abnormality. Brain
MRI found cerebellar atrophy with multiple microinfarcts;
besides, vascular MRI revealed a left vertebral stenosis
with a V2 segment occlusion as well as an extensive right
vertebral stenosis (Figure 1). The hypothesis of vascular
dementia remained possible regarding the cerebellar lesions,
although the timeline of cognitive decline seemed atypical.
Furthermore, the presence of rapid weight loss argued
against this diagnosis. Wernicke’s encephalopathy was
excluded, given the absence of delirium, ocular abnormality
or vitamin B1 deficiency. However, the diagnosis of

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Figure 1. Brain and vascular imaging of the patient. Top left: cerebelar atrophy (MRI, T1 turbo spin echo, sagittal). Top
right: microinfarcts of right middle cerebellar peduncle and pons (MRI, multiplanar reconstruction, axial). Bottom left: extensive
right vertebral stenosis (magnetic resonance angiography). Bottom right: bilateral hypermetabolism of the arterial walls of the
brachiocephalic arterial trunk, subclavian arteries, vertebral, maxillary and temporal arteries (Fluorine-18-Fluorodeoxyglucose
positron emission tomography).

Atypical presentation of giant cell arteritis
paraneoplasic encephalitis was hypothesised. 18 FDG-PET
examination highlighted a metabolically active vasculitis of
the brachiocephalic trunk, involving subclavian, vertebral
and temporal arteries. Temporal artery biopsy showed an
inflammatory pan-arteritis characterised by a destruction of
the inner elastic lamina, an infiltration of the muscularis
media and giant cells. Therefore, the final diagnosis was
GCA with vertebral artery involvement, associated with
multiple cerebellar strokes. The patient was prescribed three
boluses of methylprednisolone, followed by 0.7 mg/kg/day of oral prednisone, associated with six courses of
cyclophosphamide IV followed by azathioprine 100 mg/j.
Inflammatory syndrome and anorexia disappeared within
1 week; cognitive performance and autonomy gradually
improved (at 4 months MMSE 29/30, FAB 14/18, Katz
scale 6/6 and Lawton scale 3/4).
GCA has exceptionally been described as a potentially
curable dementia. Clinical manifestations from case reports
suggest impairment of short-term memory, disorientation,
delirium, impaired attention or visual hallucinations [2–
4]. These neuropsychiatric symptoms are usually preceded
by the classical manifestations of GCA. Several hypotheses
have been evoked to explain these cognitive disorders:
local inflammation due to vasculitis of the intracranial
or meningeal vessels, systemic inflammation or subacute
ischemia of the temporal and occipital lobes, derived from
extracranial involvement of the vertebral arterial system
[5, 6]. Although often incomplete, a gradual recovery of
neuropsychiatric symptoms is expected under corticosteroid
therapy.

Funding: None.

Declaration of Conflicts of Interest: None.

Received 30 May 2019; editorial decision 19 October 2019

Declaration of Consent: Written informed consent for
publication of their clinical details and clinical images was
obtained from the patient.

References

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