Autonomic Dysregulation, Cognitive Impairment, and Symptoms of Psychosis as an Unusual Presentation in an Anti-Aquaporin 4-Positive Patient

Abstract

We present the unusual case of a patient with an aquaporin 4 antibody-seropositive neuromyelitis optica spectrum disorder who presented with autonomic dysregulation, cognitive impairment, and symptoms of psychosis. Only a few previous cases have been described with similar psychiatric symptoms. Brain MRI showed an abnormal hyperintense T2 signal of the hypothalamus and, to a lesser extent, a minor hyperintense signal of the right optic nerve. Her symptoms and MR abnormalities improved after high-dose methylprednisolone.

Overlap syndrome of anti-aquaporin-4 positive neuromyelitis optica spectrum disorder and systemic lupus erythematosus: A systematic review of individual patient data

“…The myriad of neuropsychiatric presentations of NMOSD can mimic all those encountered clinically in lupus, and we suggest after ruling infections in lupus, it's worthwhile testing for AQP4 positivity, as it portends a relapsing course, culminating in damage accrual, and a progressive neurological disability. Unusual presentations for NMOSD including psychosis, altered sensorium, cognitive disturbances, apraxia, catatonia, autonomic disturbances, syndrome of inappropriate diuretic secretion (SIADH), and symptomatic narcolepsy [25][26][27][28] can also overlap with neuropsychiatric manifestations of lupus. 4,29 Testing for AQP4 becomes imperative in any neuropsychiatric manifestations of lupus, especially the refractory or relapsing subset, to rule out concomitant NMOSD.…”

Brain anomalies in early psychosis: From secondary to primary psychosis

“…The strong relations between biological stress, its anatomical substrates in the HPA axis, and psychosis, may contribute to the occurrences of secondary psychoses implicating these structures. In one case, incoherent and delusional thinking with self-neglect was observed in a patient who presented with hypothalamic lesions in the context of aquaporin 4 antibody seropositive neuromyelitis optica spectrum disorder (Ruiter et al 2017). In another, a hypothalamic hamartoma caused ictal psychosis, which manifested by episodes of sudden outbursts of aggressive and agitated behavior, with paranoid delusions.…”

Hypothalamic Dysfunction, Bilateral Vertical Gaze Palsy, and Psychiatric Symptoms in Neuromyelitis Optica Spectrum Disorders: A Case Report

“…A number of patients experienced hypothalamic dysregulation (1). However, only a few cases reported patients with hypothalamic lesions and psychiatric symptoms (1,2). Our study found a patient with prominent hypothalamic dysfunction presented typical diencephalic lesions in the initial episode, which was not observed until 2 months after she was transferred to a specialized hospital.…”