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.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Medicine+(Baltimore)
2016 ; 95
(19
): e3632
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English Wikipedia
Sjögren Sensory Neuronopathy (Sjögren Ganglionopathy): Long-Term Outcome and
Treatment Response in a Series of 13 Cases
#MMPMID27175675
Pereira PR
; Viala K
; Maisonobe T
; Haroche J
; Mathian A
; Hié M
; Amoura Z
; Cohen Aubart F
Medicine (Baltimore)
2016[May]; 95
(19
): e3632
PMID27175675
show ga
Primary Sjögren syndrome (SS) is an autoimmune disease mainly affecting the
exocrine glands causing a sicca syndrome. Neurological manifestations are rarely
seen in SS although they are debilitating. Peripheral neuropathies namely sensory
axonal neuropathy and painful small fiber neuropathy are the most frequent
neurological manifestations. Sensory neuronopathy (SN) is less frequently seen
although leading to more severe handicap.The aim of the study was to analyze the
clinical presentation and treatment efficacy in a series of SS-related SN.We
retrospectively studied patients with SS fulfilling the American-European
Classification Criteria and SN according to recent criteria. Studied variables
were neurological findings, associated autoimmune diseases, biological profiles,
nerve conduction and sensory/motor amplitudes study, treatments received, and
outcomes. Handicap scores were studied at beginning and end of each treatment
using the modified Rankin Scale (mRS).Thirteen patients were included (12 women,
1 man; median age 55 years at SN diagnosis) presenting with SN with a median
follow-up of 3 years (range 2-17). In 11 patients, SN preceded or coincided with
SS diagnosis. Most common neurological findings were ataxia and areflexia
followed by paresthesia and pain. Lower limbs were more affected than upper
limbs, neurological deficits were often symmetric and cranial nerves were
affected in 3 patients. Seven patients were treated with corticosteroids, 7 with
mycophenolate mofetil, 6 with hydroxychloroquine, 5 with intravenous
immunoglobulins, 4 with cyclophosphamide, and 2 patients received other
immunosuppressive drugs. At the beginning and at the end of follow-up, average
mRS was 2.15 (median 2) and 2.38 (median 2), respectively.SS-related SN
progression is heterogeneous but tends to be chronic, insidious, and debilitating
despite treatment. From these data concerning a small number of patients,
treatment strategies with corticosteroids in association with immunosuppressive
drugs, namely mycophenolate mofetil, had positive results. In contrast,
intravenous immunoglobulins had disappointing results.
|*Sensory Receptor Cells
[MESH]
|Adrenal Cortex Hormones/therapeutic use
[MESH]
|Adult
[MESH]
|Aged
[MESH]
|Cyclophosphamide/therapeutic use
[MESH]
|Disability Evaluation
[MESH]
|Enzyme Inhibitors/*therapeutic use
[MESH]
|Female
[MESH]
|Follow-Up Studies
[MESH]
|Humans
[MESH]
|Hydroxychloroquine/therapeutic use
[MESH]
|Immunoglobulins, Intravenous/therapeutic use
[MESH]
|Immunologic Factors/*therapeutic use
[MESH]
|Male
[MESH]
|Middle Aged
[MESH]
|Mycophenolic Acid/therapeutic use
[MESH]
|Peripheral Nervous System Diseases/*drug therapy/etiology
[MESH]