Long-term persistence of NMDAR antibodies after encephalitis with de novo occurrence of demyelinating disorder
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Long-term persistence of NMDAR antibodies after encephalitis with de novo occurrence of demyelinating disorder Maria Tappatà 1
&
Patrizia Riguzzi 1 & Lilia Volpi 1 & Luca Albini Riccioli 2 & Roberta Pantieri 1 & Roberto Michelucci 1
Received: 1 June 2020 / Accepted: 15 September 2020 # Fondazione Società Italiana di Neurologia 2020
Abstract The issue of persistence of NMDAR antibodies after encephalitis is not fully elucidated and their relationship with demyelinating disorders has been suggested. A female patient showed at the age of 18 an acute neurological disorder (with psychiatric symptoms, focal seizures, orofacial dyskinesias and hypoventilation requiring ventilatory support) clinically mimicking antiNMDAR encephalitis. At that time specific laboratory tests were not available, CSF revealed oligoclonal bands and MRI was negative. The patient had full recovery after first line immunotherapy (i.v. steroids and immunoglobulins). Fifteen years later, at the age of 33, she was hospitalized with subacute right hemiparesis and MRI disclosed multiple T2 hyperintensities in the white matter, one of them in the left midbrain showing contrast enhancement. Serum and CSF NMDAR antibodies were positive while MOG and AQP4 antibodies were negative. Intravenous methylprednisolone led to complete recovery. This case report provides evidence of a long-term persistence of NMDAR antibodies even 15 years after the encephalitis and raises the suspicion of a possible causal relationship between NMDAR antibodies and demyelinating disorders in the form of multiple sclerosis. Keywords NMDAR antibodies . Encephalitis . Clinical course
Introduction Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is the most common autoimmune encephalitis with a typical and homogeneous clinical picture, frequently recognizable on clinical grounds [1]. This condition is prevalent in young women, and its manifestations include a wide range of psychiatric symptoms early in the course of the disease, followed by neurological symptoms, such as memory problems, seizures, and movement disorders [1]. The clinical course is overall severe, being commonly accompanied by a decreased level of consciousness, autonomic dysfunction, and hypoventilation often requiring ventilatory support [1]. Nonetheless, the outcome is usually favorable with around 80% of patients presenting a substantial recovery at 24 months [1]. This condition is associated in up to 58% of women with * Maria Tappatà [email protected] 1
IRCCS Istituto delle Scienze Neurologiche di Bologna, Unit of Neurology, Bellaria Hospital, Bologna, Italy
2
IRCCS Istituto delle Scienze Neurologiche di Bologna, Unit of Neuroradiology, Bellaria Hospital, Bologna, Italy
an ovarian teratoma, while in a minority of cases, it may be triggered by a herpetic encephalitis [1]. In recent years, a link between the anti-NMDAR encephalitis and demyelinating diseases (DemD) has been described, usually in the form of neuromyelitis optica spectrum disorders (NMOSD) [2] or, more rarely
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