Warning: imagejpeg(C:\Inetpub\vhosts\kidney.de\httpdocs\phplern\28381508
.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Neurology
2017 ; 88
(18
): 1736-1743
Nephropedia Template TP
gab.com Text
Twit Text FOAVip
Twit Text #
English Wikipedia
Clinical manifestations of the anti-IgLON5 disease
#MMPMID28381508
Gaig C
; Graus F
; Compta Y
; Högl B
; Bataller L
; Brüggemann N
; Giordana C
; Heidbreder A
; Kotschet K
; Lewerenz J
; Macher S
; Martí MJ
; Montojo T
; Pérez-Pérez J
; Puertas I
; Seitz C
; Simabukuro M
; Téllez N
; Wandinger KP
; Iranzo A
; Ercilla G
; Sabater L
; Santamaría J
; Dalmau J
Neurology
2017[May]; 88
(18
): 1736-1743
PMID28381508
show ga
OBJECTIVE: To report the presentation, main syndromes, human leukocyte antigen
(HLA) association, and immunoglobulin G (IgG) subclass in the anti-IgLON5
disease: a disorder with parasomnias, sleep apnea, and IgLON5 antibodies.
METHODS: This was a retrospective clinical analysis of 22 patients. The IgG
subclass was determined using reported techniques. RESULTS: Patients' median age
was 64 years (range 46-83). Symptoms that led to initial consultation included
sleep problems (8 patients; 36%), gait abnormalities (8; 36%), bulbar dysfunction
(3; 14%), chorea (2; 9%), and cognitive decline (1; 5%). By the time of diagnosis
of the disorder, 4 syndromes were identified: (1) a sleep disorder with
parasomnia and sleep breathing difficulty in 8 (36%) patients; (2) a bulbar
syndrome including dysphagia, sialorrhea, stridor, or acute respiratory
insufficiency in 6 (27%); (3) a syndrome resembling progressive supranuclear
palsy (PSP-like) in 5 (23%); and (4) cognitive decline with or without chorea in
3 (14%). All patients eventually developed parasomnia, sleep apnea, insomnia, or
excessive daytime sleepiness. HLA-DRB1*10:01 and HLA-DQB1*05:01 were positive in
13/15 (87%) patients; the DRB1*10:01 allele was 36 times more prevalent than in
the general population. Among 16 patients with paired serum and CSF samples, 14
had IgLON5 antibodies in both, and 2 only in serum (both had a PSP-like
syndrome). Twenty of 21 patients had IgG1 and IgG4 antibodies; the latter
predominated in 16. CONCLUSIONS: Patients with IgLON5 antibodies develop a
characteristic sleep disorder preceded or accompanied by bulbar symptoms, gait
abnormalities, oculomotor problems, and, less frequently, cognitive decline. IgG4
subclass antibodies predominate over IgG1; we confirm a strong association with
the HLA-DRB1*10:01 allele.
free
free
free
