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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.