Variations of movement disorders in anti-N-methyl-D-aspartate receptor encephalitis

Abstract

Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is one of the most common autoimmune encephalitis that presents with a wide variety of movement disorders. The purpose of our study is to review the manifestations and duration of movement disorders in different ages with NMDAR encephalitis.A retrospective cohort of 28 patients (20 females and 8 males) with positive cerebrospinal fluid (CSF) anti-NMDAR antibody in a 5-year period from major hospitals in Taiwan was enrolled. They were categorized into 3 age groups: 7 patients were ≤10 years, 14 patients were 10 to 18 years, and 7 patients were >18 years.Total 28 patients (20 females and 8 males) with age ranging from 8 months to 38 years were enrolled. Nearly all patients (n = 27/28, 96%) presented with at least 2 types of disorders, including orofacial–lingual dyskinesia (OFLD; n = 20), catatonia (n = 19), tremor (n = 11), bradykinesia (n = 11), dystonia (n = 11), choreoathethosis (n = 9), and ballism (n = 3). Only 1 patient below 10 years presented with isolated periodic choreoathethosis without other movement disorders. OFLD was common in all age groups. Choreoathetosis was most common in patients aged ≤10 years, while catatonia was most common in patients aged >10 years (P = 0.001 and 0.020, respectively). Bradykinesia was also more common in patients aged >10 years (P = 0.020). The clinical presentations of movement disorders were not significantly different in the age of 10 to 18 years and those >18 years. Neither patient ≤10 years old nor male patients had associated tumors. All patients’ movement disorders were improved after treatment, while female patients with tumors had worse short-term outcome (P = 0.014). Compared with other disorders, choreoathetosis persisted significantly longer in patients ≤10 years (P = 0.038), while OFLD and catatonia last longer in patients >10 years (P = 0.047 and 0.002, respectively).Our study shows that hyperkinetic movements such as choreoathetosis are more common and last longer in younger age groups, whereas hypokinetic movements such as catatonia and bradykinesia are more common and last longer in older age groups. Female patients with ovarian tumors had worse short-term outcome.

Herpes simplex virus‐induced anti‐N‐methyl‐d‐aspartate receptor encephalitis: a systematic literature review with analysis of 43 cases

“…With regard to the overall clinical manifestations during HSV‐induced anti‐NMDAR encephalitis, our literature review shows that the clinical picture is similar to that of anti‐NMDAR encephalitis not preceded by HSE, including the known age‐specific features of the disease. Indeed, psychiatric symptoms were more frequent in adults in our literature cohort, whereas neurological manifestations, such as movement disorder, were more represented in children (Table SI), similarly to what was previously reported in patients with anti‐NMDAR encephalitis not preceded by HSE . Although it should be taken into account that under‐reporting of psychiatry and cognitive features in young children is possible, we also observed a slightly more severe disease in children than in adults, and a shorter latency between HSE and autoimmune encephalitis in children.…”

“…Indeed, psychiatric symptoms were more frequent in adults in our literature cohort, whereas neurological manifestations, such as movement disorder, were more represented in children (Table SI), similarly to what was previously reported in patients with anti-NMDAR encephalitis not preceded by HSE. 11,45,46 Although it should be taken into account that under-reporting of psychiatry and cognitive features in young children is possible, we also observed a slightly more severe disease in children than in adults, and a shorter latency between HSE and autoimmune encephalitis in children.…”

Catatonia secondary to anti-N-methyl-D-aspartate receptor (NMDAr) encephalitis: a review

“…Studies have shown that children tend to have neurological symptoms more frequently 82 as opposed to psychiatric ones, and appear to have a higher incidence of movement disorders, with numbers of children noted to have a movement disorder ranging from 60% 83 to 90%, 84,85 which included unilateral dystonia, speech disturbance, status epilepticus, 78 and gait disturbances. 86 Granata et al 87 compared the movement disorders in children and teenagers (>12 years of age) with anti-NMDAr encephalitis and found that catatonic symptoms were much more prevalent in adolescents than in children, as other studies 88 had shown before. In children, especially those under 12, behavioral changes may present themselves with increased temper tantrums, hyperactivity, or irritability as opposed to frank psychosis.…”

Movement disorders associated with neuronal antibodies

“…The most common MD reported in literature is orofacial lingual dyskinesia (OFLD), which is characterized by perioral grimacing, chewing, tongue thrusting, lip smacking, and frowning movements. In a series of 28 patients of NMDAR antibody‐positive encephalitis reported by Duan et al, OFLD (20/28) was the most common MDs followed by catatonia (n = 19), tremor (n = 11), bradykinesia (n = 11), dystonia (n = 11), choreoathetosis (n = 9), and ballism (n = 3) . The pathogenesis of this form of movement could be due to the hypersensitivity of the dopaminergic system, the instability between dopamine and cholinergic systems, and impairments of striato‐nigral GABAergic neurons.…”