好色先生

好色先生

Explore the latest content from across our publications
Join The AAN

Log In

Forgot Password?
Create New Account

Loading... please wait

Abstract Details

Aggressive Expansile Medullary Lesion as a Near-fatal Presentation of Neuromyelitis Optica
Autoimmune Neurology
P1 - Poster Session 1 (8:00 AM-9:00 AM)
2-009
To report a rare case of neuromyelitis optica (NMO) presenting as an expansile medullary lesion, characterized by a severe multisystem clinical course and notable diagnostic challenges. 
The most frequent NMO presentations are longitudinally extensive transverse myelitis and optic neuritis, with brainstem involvement accounting for fewer than 10% of cases. NMO lesions in the brainstem rarely exhibit expansile, tumor-like features with rapidly progressive symptoms necessitating critical care. Such aggressive manifestations increase diagnostic uncertainty and delay targeted immunotherapy.
Case report.
A 35-year-old woman developed progressive bilateral lower extremity and left upper extremity weakness alongside right eye pain, diplopia, and dysphagia. Initial evaluation for stroke included a non-contrast MRI, which revealed a left medullary lesion. The exam showed severe vertical nystagmus, dysarthria, dysphagia, left vocal cord paralysis, allodynia, marked bilateral hip flexion and left wrist weakness, and hyperreflexia. She subsequently experienced urinary retention and respiratory distress. Repeat contrasted MRI of the neuroaxis in five days demonstrated a larger patchily enhancing expansile lesion from the rostral medulla to C1, predominately involving the dorsal left medulla and occupying two-thirds of the axial space. CSF analysis confirmed CSF-specific oligoclonal bands; infectious workup was negative. High-dose IV methylprednisolone followed by a prolonged taper and IVIG elicited no clinical improvement. Biopsy was considered but deferred due to involvement of eloquent brainstem tissue. The patient’s course was complicated by prolonged respiratory failure requiring intubation, severe dysautonomia, and ileus. Eventually, Aquaporin-4 Immunoglobulin-G antibodies (AQP4-IgG) testing was positive and sustained immunosuppressive therapy produced gradual neurologic recovery.
This case illustrates an uncommon, monophasic, aquaporin-4 antibody-positive presentation of NMO as an aggressive, expansile evolving medullary lesion with respiratory compromise. Three other cases in literature exist describing medulla oblongata involvement with an expansile lesion. Early recognition of atypical tumor-like medullary NMO is crucial for timely immunotherapy and optimizing patient outcomes.
Authors/Disclosures
Abdur Rehman, MD (Geisinger Medical Center)
PRESENTER 		Dr. Rehman has nothing to disclose.
Reilly M. Graney Ms. Graney has nothing to disclose.
Chichun Sun, DO Dr. Sun has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alector. Dr. Sun has received personal compensation in the range of $0-$499 for serving as a Speaker with 好色先生. Dr. Sun has received personal compensation in the range of $0-$499 for serving as a Speaker with Pennsylvania Neurological Society.