MRI and neuropsychological evaluation in systemic sclerosis

Systemic sclerosis (SSc) is an autoimmune connective tissue disease. Although
initially, involvement of the
central nervous system is not considered as a typical feature of the disease,
recently there has been increasing
evidence that neuropsychiatric manifestations can be clinical manifestations of
SSc. In a systematic review it
was described that SSc has a significant effect on mental health and fatigue
levels, and that SSc affects
cognitive performance, especially visual-spatial and problem solving abilities.
So far there is limited information
on cerebral damage responsible for these symptoms. Previously, researchers from
the LUMC departments of
Rheumatology, Radiology and Neurology have demonstrated that using advanced
neuroimaging techniques the
detection and understanding of pathophysiological processes underlying cerebral
manifestations of rheumatic
diseases (RA, SLE) can be improved. The general aim of this pilot study is to
assess whether brain damage can
be detected using advanced MRI techniques in SSc patients and to correlate
cerebral damage to clinical
characteristics (fatigue, cognitive impairment, disease acitivity and
neuropsychological investigation). All subjects will
undergo psychiatric and neuropsychological testing as well as an advanced MR
imaging protocol. If
brain damage can be detected and correlated with neuropsychological symptoms,
it would pave the way for a
more comprehensive study into the nature of the observed damage, and it would
provide essential ammunition
for a intervention and therapeutical strategy studies.

Aim of the project
1) To detect cerebral structural changes in SSc patients.
2) To investigate the influence of chronic inflammation and chronic
vasculopathy on the cerebral metabolism
and haemodynamic function in SSc patients.
3) To correlate the MRI changes to clinical characteristics, disease activity
parameters and neuropsychological
investigation.

The Psychiatric assessment will be done by a psychiatrist and includes a
detailed psychiatric history and mental state examination assessing behaviour,
cognition, perception and thinking, as well as mood and affect in a
standardized manner. Additionally, the following measures are used for
assessment of quality of life, presence of anxiety- and/or depressive
disorders, presence of dissociation, and presence of neuropsychiatric symptoms:
fatigue index (MVI-20) ,the Hospital Anxiety and Depression Scale (HADS), the
Dissociation Experience Scale (DES), and the Neuropsychiatric Inventory (NPI).

The following functional neuropsychology tests are performed at the department
of Neurology: a) global
functioning (assessed by Mini mental state examination (MMSE), b) memory
(Wechsler Memory scale), c)
executive testing (Trails, Stroop, Wais-R substitution, number strike through
test, d) language: (Word Fluency,
naming pictures, writing and calculating) and e) praxis (drawing a clock, map
and cube).

Advanced neuroimaging methods will be performed at the Department of Radiology
and comprises: a) a set of
conventional MRI sequences (T1-, T2-, FLAIR-, and susceptibility-weighted
sequences), b) a quantitative
structural sequences (Diffusion Weighted Imaging, DWI), c) a technique for
detecting metabolic changes (Single
Voxel Proton Magnetic Resonance Spectroscopy, H1-MRS), d) a technique to detect
and quantify changes at
the level of the cerebral microvasculature (challenged and unchallenged
perfusion MRI), and e) a technique to
assess presymptomatic functional brain changes (resting-state functional MRI).

The patients are doing extra investigations , ( half a day) : MRI of the
brain, neuropsychological tests and completing questionaires.