Apresenta

1
ISOLATED BRAINSTEM SYNDROME AS THE SOLE
MANIFESTATION OF NEUROMYELITIS OPTICA SPECTRUM
DISORDER Gisele 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. References Lana-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
2011171107-1112. 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
2013802210-6.
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Brain MRI FLAIR-sequence. Hyperintense lesion
around fourth ventricle and aqueduct.