Antibodies Flashcards
What are associated neurological disorders with the NMDAR antigen?
Post-herpes simplex encephalitis relapse with chorea; paediatric dyskinetic encephalitis lethargica; idiopathic epilepsy; immunotherapy- responsive dementia.
What are associated neurological disorders with the LGI1 antigen?
Morvan’s syndrome, NMT, epilepsy, REM sleep behaviour disorder.
What are associated neurological disorders with the CASPR2 antigen?
LE, NMT, epilepsy.
What are the main psychiatric features seen in NMDAR antigen encephalitis?
Anxiety, agitation, bizarre behaviour, catatonia, delusional or paranoid thoughts, and visual or auditory hallucinations. Also movement disorder, seizures, autonomic instability.
What are the main psychiatric features seen in LGI1 encephalitis?
Confusion, hallucinations, depression.
What is the antigen description of the NMDAR antigen?
Ligand gated ion channel
What are the main psychiatric features seen in CASPR2 mediated encephalitis?
Confusion, hallucinations, agitation, delusions.
Which main encephalopathy syndrome is CASPR2 associated with?
Morvan’s syndrome:
rare constellation of neurological symptoms, consisting of peripheral nerve hyperexcitability, autonomic instability
What is encephalitis
inflammation of the brain
What is encephalitis divided into?
Infectious encephalitis, immune-mediated encephalitis and unknown
what are the causes of encephalitis?
Infective, non-infective and unknown
What can infective encephalitis be sub-divided into?
Viral, Bacterial, Protozoal, Rickettsial and Fungal
What are some examples of viral encephalitis?
HSV-1,HSV-2, HIV, measles, influenza, VZV, CMV, EBV, dengue, rabies, Zika, JC virus
Give examples of bacterial causes
syphilis, borrelia (Lyme disease), Mycobacterium tuberculosis
What are examples of protozoal causes
malaria, toxoplasma, human African trypanosomiasis (sleeping sickness)
What are examples of non-infective causes
Autoimmune
▪ Paraneoplastic: Hu, Ma, ampiphysin, CV2
▪ Non-paraneoplastic: NMDAR, LGI1, CASPR2, DPPX, IgLON5 ▪ Systemic lupus erythematosus
▪ ADEM
What are some unknown types
Encephalitis lethargica
Rasmussen’s encephalitis
What are symptoms of HSV-1
headache, fever, seizures and confusion
what are the acute psychiatric symptoms of HSV-1?
Agitation
Confusion
Psychosis esp hallucinations Mania
Delirium
Confabulation
Catatonia
what are the chronic psychiatric symptoms of HSV-1?
Cognitive impairment esp anterograde amnesia (25-75%) with variable retrograde component
Executive/frontal dysfunction in 40%
Disinhibition
Aggression
Kluver-Bucy syndrome
What are investigation results for NMDAR encephalitis?
Serum inflammatory markers usually normal
MRI: 33% abnormal (non-specific)
CSF: 80% abnormal
Lymphocytic pleocytosis 75% Oligoclonal bands 53%
EEG: 80-90% abnormal (inc ‘extreme delta brush’) Malignancy found in 39% (figure decreasing)
Briefly what does the anti NMDAR antibody do?
target the receptor, internalizing it, causes NMDA receptor hypofunction
What are signs of VGKC (voltage-gated potassium channel) encephalitis?
Memory deficits, disorientation and medial temporal lobe seizures predominate
What is the clinical diagnostic criteria of AE?
Diagnosis can be made when all three of the following criteria are met: subacute onset of working memory deficits, altered mental status or psychiatric symptoms.
At least one of the following: new focal CNS findings, seizures not explained by a previously known seizure disorder, CSF pleocytosis and MRI features suggestive of encephalitis.
