Anti-Dipeptidyl-Peptidase-Like Protein 6 (DPPX) Encephalitis Progressive Encephalomyelitis with Rigidity and Myoclonus

Alternative name(s) : anti-DPPX autoimmunity, DPPX autoimmune encephalitis

Gene_locus : human-DPP6

Mutation : No mutation

OMIM : 126141 , 616311

Comment
Dipeptidyl-peptidase-like protein-6 (DPPX) encephalitis is a rare autoimmune disorder characterized by the presence of antibodies directed against DPPX, a subunit of the Kv4.2 potassium channel. Most patients demonstrate a classic triad of chronic diarrhea\/weight loss, cognitive dysfunction, and central nervous system (CNS) hyperexcitability

References (30)

Title : The effect of double filtration plasmapheresis and corticosteroids on patients with anti-dipeptidyl-peptidase-like protein 6 encephalitis - Wan_2024_Ther.Apher.Dial_28_141
Author(s) : Wan W , Pan Y , Chen Y , Bai S , Yao X , Lin Y , Wu J , Ni L , Mei Y , Qiu H , Zhou Y , Hao Y , Guan Y
Ref : Ther Apher Dial , 28 :141 , 2024
Abstract :
PubMedSearch : Wan_2024_Ther.Apher.Dial_28_141
PubMedID: 37461148
Gene_locus related to this paper: human-DPP6

Title : A rare case of anti-DPPX encephalitis combined with neuroleptospirosis - Jin_2024_BMC.Neurol_24_34
Author(s) : Jin Y , Lan W , Chen X , Liu W , Luo W , Chen S
Ref : BMC Neurol , 24 :34 , 2024
Abstract :
PubMedSearch : Jin_2024_BMC.Neurol_24_34
PubMedID: 38243162
Gene_locus related to this paper: human-DPP6

Title : Colocalization of Increased Midbrain Signals in Neuroinflammation and Tau PET Imaging Suggests the Diagnosis of Progressive Supranuclear Palsy - Lu_2024_Clin.Nucl.Med__
Author(s) : Lu J , Ge J , Yu H , Zhao G , Chen X
Ref : Clin Nucl Med , : , 2024
Abstract :
PubMedSearch : Lu_2024_Clin.Nucl.Med__
PubMedID: 38271226
Gene_locus related to this paper: human-DPP6

Title : Expanding the clinical spectrum of anti-DPPX encephalitis: a multicenter retrospective study - Gao_2024_Front.Neurosci_18_1379933
Author(s) : Gao Y , Zhang Y , Chunyu H , Xu Y , Wang Y , Liu S , Chang J , Tang B , Xu C , Lu Y , Zhou J , Kong X , Zhu X , Chen S , Zhou Q , Meng H
Ref : Front Neurosci , 18 :1379933 , 2024
Abstract :
PubMedSearch : Gao_2024_Front.Neurosci_18_1379933
PubMedID: 38756408
Gene_locus related to this paper: human-DPP6

Title : Neuropsychiatric Presentation of Anti-DPPX Progressive Encephalomyelitis with Rigidity and Myoclonus -
Author(s) : Neo RJ , Mehta AR , Weston M , Magrinelli F , Quattrone A , Gandhi S , Joyce EM , Bhatia KP
Ref : Movement Disordersord Clin Pract , 11 :97 , 2024
PubMedID: 38291842
Gene_locus related to this paper: human-DPP6

Title : A Devastating Neurological Disorder: Anti-Dipeptidyl-Peptidase-Like Protein 6 (DPPX) Encephalitis Causing Rapidly Progressive Dementia - Esechie_2023_Cureus_15_e51123
Author(s) : Esechie A , Thottempudi N , Patel C , Shanina E , Li X
Ref : Cureus , 15 :e51123 , 2023
Abstract :
PubMedSearch : Esechie_2023_Cureus_15_e51123
PubMedID: 38274926
Gene_locus related to this paper: human-DPP6

Title : Long term outcomes in patients with anti-DPPX autoimmunity - Dominguez_2023_J.Neuroimmunol_384_578214
Author(s) : Dominguez L , McKeon A , Tobin WO , Lopez-Chiriboga S
Ref : Journal of Neuroimmunology , 384 :578214 , 2023
Abstract :
PubMedSearch : Dominguez_2023_J.Neuroimmunol_384_578214
PubMedID: 37806046
Gene_locus related to this paper: human-DPP6

Title : Clinical and imaging analysis to evaluate the response of patients with anti-DPPX encephalitis to immunotherapy - Xiao_2022_BMC.Neurol_22_129
Author(s) : Xiao J , Fu PC , Li ZJ
Ref : BMC Neurol , 22 :129 , 2022
Abstract :
PubMedSearch : Xiao_2022_BMC.Neurol_22_129
PubMedID: 35382765
Gene_locus related to this paper: human-DPP6

Title : [Weakness and weight loss in a 62 year-old patient] -
Author(s) : Legendre P , Couture P , Ponsoye M , Marroun I , Ackermann F , Comarmond C
Ref : Rev Med Interne , 43 :444 , 2022
PubMedID: 35606201

Title : Case report: Pain in anti-DPPX encephalitis - Bjerknes_2022_Front.Neurol_13_1091688
Author(s) : Bjerknes TL , Steihaug OM , Haugen M , Hjelland IE , Vedeler CA
Ref : Front Neurol , 13 :1091688 , 2022
Abstract :
PubMedSearch : Bjerknes_2022_Front.Neurol_13_1091688
PubMedID: 36588910
Gene_locus related to this paper: human-DPP6

Title : Anti-dipeptidyl-peptidase-like protein 6 encephalitis with pure cerebellar ataxia: a case report - Lin_2022_BMC.Neurol_22_242
Author(s) : Lin J , Zhu M , Mao X , Jin Z , Zhou M , Hong D
Ref : BMC Neurol , 22 :242 , 2022
Abstract :
PubMedSearch : Lin_2022_BMC.Neurol_22_242
PubMedID: 35778696
Gene_locus related to this paper: human-DPP6

Title : DPPX autoimmune encephalitis: A short report on two patients presenting with classic features of this rare but treatable disease -
Author(s) : Mbonde AA , Gritsch D , Arca K , Blech B , McKeon A , Carter J
Ref : Mult Scler Relat Disord , 52 :102934 , 2021
PubMedID: 33901970
Gene_locus related to this paper: human-DPP6

Title : Treatable Rapid Progressive Dementia: A First Case Report of Anti-dipeptidyl-peptidase-like Protein 6 Encephalitis in Taiwan - Tsai_2021_Acta.Neurol.Taiwan_30(3)_118
Author(s) : Tsai MC , Shih HK , Lin HH , Tsai MY , Sung YF
Ref : Acta Neurol Taiwan , 30(3) :118 , 2021
Abstract :
PubMedSearch : Tsai_2021_Acta.Neurol.Taiwan_30(3)_118
PubMedID: 34841508
Gene_locus related to this paper: human-DPP6

Title : Clinical Features and Prognosis in Chinese Patients With Dipeptidyl-Peptidase-Like Protein 6 Antibody-Associated Encephalitis - Miao_2021_Front.Neurol_12_817896
Author(s) : Miao A , Shi Y , Wang X , Ge J , Yu C
Ref : Front Neurol , 12 :817896 , 2021
Abstract :
PubMedSearch : Miao_2021_Front.Neurol_12_817896
PubMedID: 35095748
Gene_locus related to this paper: human-DPP6

Title : Utilization of video oculography to quantify oculomotor dysfunction in anti-DPPX encephalitis: A case study - Blackburn_2020_Mult.Scler.Relat.Disord_46_102460
Author(s) : Blackburn K , Vernino S , Sguigna P
Ref : Mult Scler Relat Disord , 46 :102460 , 2020
Abstract :
PubMedSearch : Blackburn_2020_Mult.Scler.Relat.Disord_46_102460
PubMedID: 32932166
Gene_locus related to this paper: human-DPP6

Title : Oro-Bucco-Lingual Dyskinesia, Weight Loss, and Cognitive Decline in Anti-DPPX Antibody-Mediated Encephalitis -
Author(s) : Deuel LM , Yu CH , Vaughan CL , Piquet AL
Ref : Movement Disordersord Clin Pract , 7 :S80 , 2020
PubMedID: 33015231
Gene_locus related to this paper: human-DPP6

Title : Co-occurrence of antibodies against dipeptidyl-peptidase-like protein-6 and aquaporin-4 during a case of paraneoplastic encephalitis -
Author(s) : Bien CG , Schanzer A , Dargvainiene J , Dogan-Onugoren M , Woermann F , Strickler A
Ref : Clin Neurol Neurosurg , 197 :106093 , 2020
PubMedID: 32702576
Gene_locus related to this paper: human-DPP6

Title : Dipeptidyl-peptidase-like protein 6 encephalitis treated with immunotherapy - Ye_2020_Proc.(Bayl.Univ.Med.Cent)_34_114
Author(s) : Ye L , Schnegelsberg M , Obermann M
Ref : Proc (Bayl Univ Med Cent) , 34 :114 , 2020
Abstract :
PubMedSearch : Ye_2020_Proc.(Bayl.Univ.Med.Cent)_34_114
PubMedID: 33456166
Gene_locus related to this paper: human-DPP6

Title : Anti-dipeptidyl-peptidase-like protein 6 encephalitis, a rare cause of reversible rapid progressive dementia and insomnia - Zhou_2020_J.Neuroimmunol_339_577114
Author(s) : Zhou Q , Zhu X , Meng H , Zhang M , Chen S
Ref : Journal of Neuroimmunology , 339 :577114 , 2020
Abstract :
PubMedSearch : Zhou_2020_J.Neuroimmunol_339_577114
PubMedID: 31775073
Gene_locus related to this paper: human-DPP6

Title : Teaching Video NeuroImages: Opsoclonus in anti-DPPX encephalitis -
Author(s) : Micieli JA , Newman NJ , Kase CS , Biousse V
Ref : Neurology , 92 :e2298 , 2019
PubMedID: 31061220
Gene_locus related to this paper: human-DPP6

Title : Pruritus in anti-DPPX encephalitis -
Author(s) : Wijntjes J , Bechakra M , Schreurs MWJ , Jongen JLM , Koppenaal A , Titulaer MJ
Ref : Neurol Neuroimmunol Neuroinflamm , 5 :e455 , 2018
PubMedID: 29616232
Gene_locus related to this paper: human-DPP6

Title : Anti-DPPX encephalitis: prominent nystagmus reflected by extraocular muscle FDG-PET avidity -
Author(s) : Doherty L , Gold D , Solnes L , Probasco J , Venkatesan A
Ref : Neurol Neuroimmunol Neuroinflamm , 4 :e361 , 2017
PubMedID: 28626782
Gene_locus related to this paper: human-DPP6

Title : [Progressive encephalitis due to anti-DPPX antibodies: a case with good response to treatment with rituximab] -
Author(s) : Valero-Lopez G , Martin-Fernandez JJ , Hernandez-Clares R , Baidez-Guerrero AE , Martinez-Hernandez E
Ref : Rev Neurol , 65 :525 , 2017
PubMedID: 29178111
Gene_locus related to this paper: human-DPP6

Title : DPPX antibody-associated encephalitis: Main syndrome and antibody effects - Hara_2017_Neurology_88_1340
Author(s) : Hara M , Arino H , Petit-Pedrol M , Sabater L , Titulaer MJ , Martinez-Hernandez E , Schreurs MW , Rosenfeld MR , Graus F , Dalmau J
Ref : Neurology , 88 :1340 , 2017
Abstract :
PubMedSearch : Hara_2017_Neurology_88_1340
PubMedID: 28258082
Gene_locus related to this paper: human-DPP6

Title : Anti-DPPX encephalitis: Pathogenic effects of antibodies on gut and brain neurons - Piepgras_2015_Neurology_85_890
Author(s) : Piepgras J , Holtje M , Michel K , Li Q , Otto C , Drenckhahn C , Probst C , Schemann M , Jarius S , Stocker W , Balint B , Meinck HM , Buchert R , Dalmau J , Ahnert-Hilger G , Ruprecht K
Ref : Neurology , 85 :890 , 2015
Abstract :
PubMedSearch : Piepgras_2015_Neurology_85_890
PubMedID: 26291285
Gene_locus related to this paper: human-DPP6

Title : Prednisolone and azathioprine are effective in DPPX antibody-positive autoimmune encephalitis -
Author(s) : Stoeck K , Carstens PO , Jarius S , Raddatz D , Stocker W , Wildemann B , Schmidt J
Ref : Neurol Neuroimmunol Neuroinflamm , 2 :e86 , 2015
PubMedID: 25798450
Gene_locus related to this paper: human-DPP6

Title : Neuropathologic features of anti-dipeptidyl-peptidase-like protein-6 antibody encephalitis -
Author(s) : Stokin GB , Popovic M , Gelpi E , Kogoj A , Dalmau J , Graus F
Ref : Neurology , 84 :430 , 2015
PubMedID: 25520315
Gene_locus related to this paper: human-DPP6

Title : Progressive encephalomyelitis with rigidity and myoclonus: a new variant with DPPX antibodies - Balint_2014_Neurology_82_1521
Author(s) : Balint B , Jarius S , Nagel S , Haberkorn U , Probst C , Blocker IM , Bahtz R , Komorowski L , Stocker W , Kastrup A , Kuthe M , Meinck HM
Ref : Neurology , 82 :1521 , 2014
Abstract :
PubMedSearch : Balint_2014_Neurology_82_1521
PubMedID: 24696508
Gene_locus related to this paper: human-DPP6

Title : DPPX potassium channel antibody: frequency, clinical accompaniments, and outcomes in 20 patients - Tobin_2014_Neurology_83_1797
Author(s) : Tobin WO , Lennon VA , Komorowski L , Probst C , Clardy SL , Aksamit AJ , Appendino JP , Lucchinetti CF , Matsumoto JY , Pittock SJ , Sandroni P , Tippmann-Peikert M , Wirrell EC , McKeon A
Ref : Neurology , 83 :1797 , 2014
Abstract :
PubMedSearch : Tobin_2014_Neurology_83_1797
PubMedID: 25320100
Gene_locus related to this paper: human-DPP6

Title : Encephalitis and antibodies to dipeptidyl-peptidase-like protein-6, a subunit of Kv4.2 potassium channels - Boronat_2013_Ann.Neurol_73_120
Author(s) : Boronat A , Gelfand JM , Gresa-Arribas N , Jeong HY , Walsh M , Roberts K , Martinez-Hernandez E , Rosenfeld MR , Balice-Gordon R , Graus F , Rudy B , Dalmau J
Ref : Annals of Neurology , 73 :120 , 2013
Abstract :
PubMedSearch : Boronat_2013_Ann.Neurol_73_120
PubMedID: 23225603
Gene_locus related to this paper: human-DPP6