Autoimmune Encephalitis – KnowNeuropsychology

These learning questions are brought to you by KnowNeuropsychology & the Association for Doctoral Education in Clinical Neuropsychology (ADECN).

*This quiz has 6 multiple-choice questions.

1 / 6

What are the least common cognitive impairments among neural-IgG autoimmune encephalitis groups?

Episodic memory

Processing speed

Attention (sustained, selective, basic)

Language, reaction time, visuoperception

Some executive functions (e.g., problem solving, executive control, go/no-go, Stroop inhibition, planning); complex figure copy

2 / 6

Which statement is not true regarding anti-leucine glioma inactivated 1 (LGI-1) autoimmune encephalitis?

Characterized by memory disturbances in most patients

Characterized by seizures (typically faciobrachial dystonic seizures) which are poorly responsive to anti-seizure drug therapy)

Can show hypoatremnia and/or REM sleep behavior disorder

Typically effects pediatric females

Is the 2nd most common cause of autoimmune encephalitis

MRI usually shows typical limbic encephalitis findings

3 / 6

Which statements are true about anti-N-methlyl-D aspartate receptor (anti-NMDAR) encephalitis?

Usually the most well clinically known, hypothesized if caught early less cognitive burden (though this is unclear)

Primarily in young adults and children, very predicable course

81% of the individuals diagnosed are female (most likely because strongly associated with ovarian teratoma in 45% of patients)

Frequently associated with HSV encephalitis, a more destructive and necrotic process (27% develop anti-NMDA antibodies within 3 months)

All of the above are true

4 / 6

Which are the most common antibodies that we see in adults and children related to autoimmune encephalitis?

Adults (NMDA-R; GAD65; LGH); Children (NMDA-R; MOG; GAD65); LGI1- more associated with older males; NMDA-R- more likely young females

Adults (NMDA-R; CASPR2-igG; GFAP-igG); Children (NMDA-R; PCA-Tr-igG; CRMPS-IgG); CASPR2-igG more likely older males; CRMPS-IgG more likely older females

Adults (NMDA-R; GAD65; LGH); Children (PCA-Tr-igG; MOG; GAD65); CRMPS-IgG more likely younger males and older females

Adults (CASPR2-igG; GAD65; LGH); Children (NMDA-R; MOG; GAD65); no age/gender differences

5 / 6

When considering autoimmune encephalitis with antibodies against intracellular antigens (classical paraneoplastic antibodies), which antigens is considered to have an “unclear diagnostic category,” and overall more rare, when compared to other antigens?

Anti-Hu (ANNA-1)

Anti-Ri (ANNA-2)

CV2 (CRMP5)

Anti-GAD antibodies

Amphiphysin

MA (MA1, MA2)

6 / 6

Which of the following are true about autoimmune encephalitis due to antibodies due to intracellular antigens (vs. due to cell surface or synaptic proteins)

There is a strong cancer association

There is a poor prognosis due to irreversible neuronal destruction, and other factors associated with cancer

Antibodies are not directly pathogenic

All of the above are true

Your score is

Neuropsychology of Autoimmune Encephalitis

Rachel Galioto, PhD

Cleveland Clinic Lerner College of Medicine