a case presentation for a patient with autoimmune encephalitis presented with symptoms similar to hypomania with acute onset

1. Case presentation
By:
Shokry Alemam, MD.

2. • 17 year old female patient, single, student.
• She lives with her parents, and one brother and one sister,
she is the eldest.
• One month before her presentation to my clinic, 2 days
before her final exams, she started to be introverted, less
talkative than before, not concentrating well, and she
used to sleep 4-5 hours at this time because of her study
and exams, but she went to her exams.

3. • After finishing her exams and on the same day, she woke
up screaming and shouting and said that Allah came to
her dream to pray 40 times per day and it is too difficult to
her.
• Later on, she visited her aunt to have some fun with her
cousins, and she developed disorientation to persons and
called her father to come and take her, and after
sometime on the same day she recognized them and
didn’t remember what happened.
• After few days, she started to complain from headache
and low back pain, then she became aggressive and also
didn’t recognize any of her family.

4. • All this time she had less sleep time, and irritability,
talkativeness with repetitive headache, so they went to
one hospital as she developed fever, and sore throat as
well and they admitted her, MRI and LP were done.
• LP was normal and MRI showed nonspecific changes in
frontal lobe white matter; however, they started her on
antibiotic and antiviral??? to cover any possibility to
have meningitis or encephalitis??!!

5. • The patient developed another attack of disorientation
and aggressive behavior for few hours, and after that she
didn’t remember any of it.
• She was discharged as neurology and psychiatry doctors
said she was free.
• After discharge by one day she developed 2 attacks of
GTCs without losing consciousness for few minutes, then
they admitted her to a hospital after calling me suspecting
it is a psychiatric problem.

6. • She was admitted and after admission she got 5 attacks of
GTCs, was witnessed by doctors on-call and was
transferred to ICU with loading phenytoin and midazolam.
EEG was done and it was free while she got GTCs while
doing it; however, she was controlled and then transferred
back to the ward , then MRI brain was done for the second
time.
• The family mentioned that every time before she develops
the bizarre behavior or seizures she starts to stare strongly
and not responsive to people talk to her.

7. • O/E: She was conscious, alert, cooperative, oriented to
time, place and person.
• She was irritable, easily distracted, interrupting others
speech, repeated hand shaking with me “against her
nature according to the family” trying to make comments
“flirting”, decreased concentration, repeating questions,
has ideas of grandiosity that she has some gifts.
• No flight of ideas.
• Denied hallucinations and delusion, while her mother told
me she tells them that they love her siblings more than
her and treat her in a bad way.
• Neurological examination was free.

8. • Family history: irrelevant
• Medical history: irrelevant
• Premorbid personality: extroverted with good relationships
with her family and friends at school.
• The patient was on “IV phenytoin + depakine and acyclovir
+ ceftriazone” at the time of doing EEG.

11. • the patient was diagnosed as
Autoimmune encephalitis “VGKC”
• IVIG and IV Solumedrol were commenced and the
patient showed dramatic improvement in her behavior.
• She was discharged on phenytoin 100 mg BID, Depakin
chrono 500 mg BID, and prednisolon 60 mg OD

12. her images after one week of IVIG

13. Autoimmune Encephalitis
• a new and rare disease, characterized by brain inflammation and
circulating autoantibodies.
Types:
• N-methyl-D-aspartate receptors (NMDAR)
• alpha-amino-3-hydroxy-5-methyl-4- isoxazolepropion acid receptor
(AMPAR)
• leucine-rich glioma inactivated 1 (Lgi1)
• Contactin-associated protein-like 2 (Caspr2)
• glutamate decarboxylase (GAD)
• gamma-aminobutyric acid type B receptor (GABA R)
ChefdevilleA,HonnoratJ,HampeCS,DesestretV.NeuronalCNSsyndromeslikelymediatedbyautoantibodies. EurJNeurosci.2016;43:1532–1552.

14. Psychiatric presentation
• Grandiose and paranoid delusions.
• Hallucinations (visual and auditory).
• Bizarre behavior, agitation, fear, insomnia, confusion, and short-
term memory loss.
• These manifestations are generally considered as acute psychosis,
mania (with psychotic features), or onset of schizophrenia*
• limbic dysfunction and prominent psychiatric symptoms such as
confusion, disorientation, confabulation, agitation, combativeness,
and perseveration
*KayserMS,DalmauJ.Anti-NMDAreceptorencephalitis,autoim-munity,andpsychosis.SchizophrRes.2016;176(1):36–40

15. Neurological presentation
• 70% of patients, clinical course begins with viral-like prodromes
(fever, nausea, diarrhea) occurring 1 or 2 weeks before psychiatric
and/or neurologic symptoms.
• seizures, dyskinesias, dysautonomic symptoms until a comatose
phase.
• Fatal outcomes due to respiratory complications were frequent in
the past.
TitulaerMJ,MccrackenL,GabilondoI,etal.Treatmentandprognosticfactorsforlong-termoutcomeinpatientswithanti-NMDAreceptorencephalitis:anobservationalcohortstudy. LancetNeurol.2013;12(2):157–
165.

16. How to diagnose autoimmune
encephalitis in psychiatric departments?
• Neurological examination “Memory deficits, seizures, dyskinesias
and movement disorders, and headaches should be particularly
questioned”*
• EEG: In a context of brain MRI “even if a normal EEG does not
exclude diagnosis of autoimmune encephalitis”
• Autoantibodies characterization “IgG autoantibodies in the
patients’ serum or CSF”
CarvalhoF,MassanoJ,CoelhoR.Neuropsychiatricsymptomsinautoimmuneencephalopathies:aclinician’sguide. IntJClinNeurosciMentHeal.2014;1:1–11.

17. Management of psychiatric manifestations
• Atypical and more sedative antipsychotics rather than typical
antipsychotics as dopamine antagonists that aggravate agitation.
• mood symptoms, valproic acid was advised for sedation, sleep, and
seizure.
• Uses of lithium and benzodiazepines are also reported in the
literature but do not cause significant changes.
KuppuswamyPS,TakalaCR,SolaCL.Managementofpsychiatricsymptomsinanti-NMDARencephalitis:acaseseries,literaturereviewandfuturedirections. GenHospPsychiatry.2014;36(4):388–391.

18. Thank you