Aggressive Acquired Demyelinating Neuropathy Caused by NF-155: Initially Treatment-Resistant.
J Clin Neuromuscul Dis
; 25(2): 59-62, 2023 Dec 01.
Article
en En
| MEDLINE
| ID: mdl-37962191
OBJECTIVES: Anti-neurofascin-155 IgG4 (NF-155) antibody disease has previously been associated with a subset of patients with chronic inflammatory demyelinating polyradiculoneuropathy. We report a case of NF-155 positive polyneuropathy that initially presented as an acute inflammatory demyelinating polyradiculoneuropathy. The patient responded appropriately to treatment but subsequently progressed over a 3-month period, resulting in quadriplegia, areflexia, and oculobulbar paralysis. METHODS: Case report and literature review. RESULTS: A 40-year-old male presented with acute bilateral arm and thigh weakness, areflexia, and distal sensory loss. Treatment with intravenous immunoglobulin (IVIg) for acute acquired demyelinating neuropathy resulted in initial improvement but subsequent decline. Lack of response to additional IVIg and plasmapheresis (PLEX) prompted testing for NF-155. Treatment with rituximab and steroids resulted in virtually complete recovery. CONCLUSIONS: Early testing for nodal and paranodal proteins is indicated in patients who present with acute acquired demyelinating neuropathy but fail to respond to conventional treatments, such as IVIg or PLEX. Identification of nodal and paranodal antibodies should prompt treatment with rituximab and steroids to increase likelihood of recovery.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Inmunoglobulina G
/
Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante
Límite:
Adult
/
Humans
/
Male
Idioma:
En
Revista:
J Clin Neuromuscul Dis
Asunto de la revista:
FISIOLOGIA
/
NEUROLOGIA
Año:
2023
Tipo del documento:
Article
Pais de publicación:
Estados Unidos