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ISOLATED BRAINSTEM SYNDROME AS THE SOLE MANIFESTATION OF NEUROMYELITIS OPTICA SPECTRUM DISORDERGisele O. Lima, Natália C. Talim1, Lívia E. C. Talim1, Rodrigo Kleinpaul1, Juliana M. S. S. Amaral1, Márcia Prates1, Carolina R. Araujo1, Cristiane F. Rocha1, Kazuo Fujihara2, Douglas Sato2, Marco A. Lana-Peixoto1.
1CIEM MS Research Center, Federal University of Minas Gerais Medical School, Belo Horizonte, Brazil. 2Tohoku University, Sendai, Japan.
Background
Neuromyelitis optica spectrum disorders (NMOSD)
comprise a group of inflammatory immuno-mediated
disorders of the central nervous system whose hallmark is
involvement of the optic nerves and spinal cord. Cerebral
and brainstem symptoms may occur at disease onset or
during the course of the disease, usually in association with
one of the index events. Occurrence of brainstem symptoms
as the sole clinical manifestation of NMOSD has been rarely
reported. Herein we report an aquaporin 4-IgG seropositive
patient with brainstem symptoms and no clinical evidence of
optic nerve or spinal cord abnormality.
Case report
A 31 YOBF was examined at the CIEM MS Research
Center because of dysgeusia, loss of sensation in the right
teeth and over most part of the right face. Although she had a
partial recovery of the sensation deficit in about 15 days, she
developed during this period diplopia, ptosis of the right
upper lid, bilateral loss of hearing, dysarthria and dysphagia.
Examination revealed no visual deficit and no motor or
sensation abnormality. Brain MRI revealed large
periependymal lesions around the 4th ventricle and
aqueduct. Spinal MRI was normal. CSF analysis was
unrevealing. Serum AQP4-IgG was negative by indirect
immunofluorescence but a repeated search using cell-based
assay yielded a positive result. The patient was given pulses
of IV methylprednisolone and put on prophylactic treatment
with oral prednisone and azathioprine and is doing well since
then.
Conclusion
This patient had brainstem symptoms as the sole clinical
manifestation of NMOSD. The diagnosis of the disease was
confirmed by AQP4-IgG seropositivity as the antibody is
highly specific for NMOSD. Clinicians should include
NMOSD in the differential diagnosis of isolated brainstem
syndrome and use more sensitive assays for detection of
AQP4-IgG than indirect immunofluorescence.
ReferencesLana-Peixoto, Marco A; Callegaro, Dagoberto. The expanded spectrum of neuromyelitis optica: evidences for a new definition. Arq. Neuro-Psiquiatr., São Paulo , v. 70, n. 10, Oct. 2012 .
Kim W, Kim SH, Lee SH, Li XF, Kim HJ. Brain abnormalities as an initial manifestation of neuromyelitis optica spectrum disorder. Mult Scler J 2011;17:1107-1112.
Kremer L, Mealy M, JACOB A, et al. Brainstem manifestations in neuromyelitis optica: a multicenter study of 258 patients. Mult Scler, 2013.
Sato DK, Nakashima I, Takahashi T, Misu T, Waters P, Kuroda H, et al. Aquaporin-4 antibody-positive cases beyond current diagnostic criteria for NMO spectrum disorders. Neurology 2013;80:2210-6.
Brain MRI – FLAIR-sequence. Hyperintense lesion around fourth ventricle and aqueduct.