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Intern Med. 2019 Jun 7. doi: 10.2169/internalmedicine.2805-19. [Epub ahead of print]

A Unique Shape of Brainstem Lesion that Caused Orthostatic Hypotension in Anti-NMDAR Encephalitis.

Author information

1
Department of Neurology, Fukushima Medical University, Japan.
2
National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, Japan.
3
Department of Neuro-regeneration, Fukushima Medical University, Japan.

Abstract

After experiencing upper respiratory-tract symptoms, a 41-year-old woman developed encephalitis with consciousness disturbance and respiratory failure. She had external ophthalmoplegia and facial diplegia. Magnetic resonance imaging revealed a brainstem lesion with spared longitudinal pontine bundles. Abnormal findings of the brainstem auditory-evoked potentials and blink reflex supported brainstem damage. The patient was positive for anti-N-methyl-D-aspartate receptor (NMDAR) antibodies. Repeated immunological treatments improved her symptoms, but severe orthostatic hypotension emerged. A head-up tilt test revealed no arginine vasopressin response to hypotension. The atypical symptoms of this case highlighted that the brainstem is one of the pivotal regions in anti-NMDAR encephalitis.

KEYWORDS:

anti-NMDAR encephalitis; arginine vasopressin; blink reflex; brainstem auditory-evoked potentials; magnetic resonance imaging; orthostatic hypotension

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