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2015 ; 94
(31
): e0971
Nephropedia Template TP
gab.com Text
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Movement and Other Neurodegenerative Syndromes in Patients with Systemic
Rheumatic Diseases: A Case Series of 8 Patients and Review of the Literature
#MMPMID26252269
Menezes R
; Pantelyat A
; Izbudak I
; Birnbaum J
Medicine (Baltimore)
2015[Aug]; 94
(31
): e0971
PMID26252269
show ga
Patients with rheumatic diseases can present with movement and other
neurodegenerative disorders. It may be underappreciated that movement and other
neurodegenerative disorders can encompass a wide variety of disease entities.
Such disorders are strikingly heterogeneous and lead to a wider spectrum of
clinical injury than seen in Parkinson's disease. Therefore, we sought to
stringently phenotype movement and other neurodegenerative disorders presenting
in a case series of rheumatic disease patients. We integrated our findings with a
review of the literature to understand mechanisms which may account for such a
ubiquitous pattern of clinical injury.Seven rheumatic disease patients (5
Sjögren's syndrome patients, 2 undifferentiated connective tissue disease
patients) were referred and could be misdiagnosed as having Parkinson's disease.
However, all of these patients were ultimately diagnosed as having other movement
or neurodegenerative disorders. Findings inconsistent with and more expansive
than Parkinson's disease included cerebellar degeneration, dystonia with an
alien-limb phenomenon, and nonfluent aphasias.A notable finding was that
individual patients could be affected by cooccurring movement and other
neurodegenerative disorders, each of which could be exceptionally rare (ie,
prevalence of ?1:1000), and therefore with the collective probability that such
disorders were merely coincidental and causally unrelated being as low as
?1-per-billion. Whereas our review of the literature revealed that ubiquitous
patterns of clinical injury were frequently associated with magnetic resonance
imaging (MRI) findings suggestive of a widespread vasculopathy, our patients did
not have such neuroimaging findings. Instead, our patients could have syndromes
which phenotypically resembled paraneoplastic and other inflammatory disorders
which are known to be associated with antineuronal antibodies. We similarly
identified immune-mediated and inflammatory markers of injury in a psoriatic
arthritis patient who developed an amyotrophic lateral sclerosis (ALS)-plus
syndrome after tumor necrosis factor (TNF)-inhibitor therapy.We have described a
diverse spectrum of movement and other neurodegenerative disorders in our
rheumatic disease patients. The widespread pattern of clinical injury, the
propensity of our patients to present with co-occurring movement disorders, and
the lack of MRI neuroimaging findings suggestive of a vasculopathy collectively
suggest unique patterns of immune-mediated injury.