Anti-glutamic acid decarboxylase (GAD) positive cerebellar Ataxia with transitioning to progressive encephalomyelitis with rigidity and myoclonus (PERM), responsive to immunotherapy: A case report and review of literature.
Aged
Autoantibodies
/ immunology
Autoantigens
/ immunology
Autoimmune Diseases of the Nervous System
/ etiology
Cerebellar Ataxia
/ complications
Encephalomyelitis
/ etiology
Glutamate Decarboxylase
/ immunology
Humans
Immunocompromised Host
Immunoglobulins, Intravenous
/ therapeutic use
Immunotherapy
Kidney Transplantation
Male
Muscle Rigidity
/ etiology
Plasmapheresis
Postoperative Complications
/ etiology
Remission Induction
Stiff-Person Syndrome
/ etiology
Journal
Journal of neuroimmunology
ISSN: 1872-8421
Titre abrégé: J Neuroimmunol
Pays: Netherlands
ID NLM: 8109498
Informations de publication
Date de publication:
15 07 2019
15 07 2019
Historique:
received:
16
01
2019
revised:
05
03
2019
accepted:
03
04
2019
pubmed:
25
4
2019
medline:
31
3
2020
entrez:
25
4
2019
Statut:
ppublish
Résumé
We present a case of a 65-year-old African American male, immunosuppressed on Tacrolimus, who initially presented with cerebellar ataxia and rapidly developed Progressive Encephalomyelitis with Rigidity and Myoclonus (PERM) with positive anti-glutamic acid decarboxylase (GAD65) antibodies, no underlying malignancy, and normal neuroimaging. PERM is a rare spectrum of Stiff Person Syndrome (SPS), which is strongly associated with anti-GAD antibodies and characterized by flare-ups and remissions of encephalopathy, myelopathy and rigidity with myoclonus. PERM is diagnosed clinically and has been successfully treated with both Intravenous Immunoglobulin (IVIg) and plasmapheresis. Our patient was successfully treated with IVIg. On day 14 after starting IVIg treatment, his neurological symptoms started to improve and ultimately returned to baseline.
Identifiants
pubmed: 31015081
pii: S0165-5728(18)30577-0
doi: 10.1016/j.jneuroim.2019.04.003
pii:
doi:
Substances chimiques
Autoantibodies
0
Autoantigens
0
Immunoglobulins, Intravenous
0
GAD55, human
EC 4.1.1.15
Glutamate Decarboxylase
EC 4.1.1.15
Types de publication
Case Reports
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
135-137Informations de copyright
Copyright © 2019 Elsevier B.V. All rights reserved.