1. THE QUEER STRIKING THE YOUNG- ANTI NMDA RECEPTOR ENCEPHALITIS GARGI SASMAL, PRABHAT KUMAR, RATNAKAR SAHOO, HARISH GUPTA, SAKSHI MITTAL PGIMER, DR. RAM MANOHAR LOHIA HOSPITAL, NEW DELHI
INTRODUCTION, AIMS & OBJECTIVES:
Anti N-methyl-D-aspartate (NMDA) receptor encephalitis (NMDARE) is a severe form of encephalitis associated with antibodies against NR1 and NR2 subunits of the NMDA receptor.
it was first discovered in 2007 in 4 young females with neuropsychiatric symptoms and underlying ovarian teratomas.
3 proposed possible mechanisms of NMDA autoimmunity-
Expression of NMDAR in ovarian teratomas leads to a downstream autoimmune response. Therefore, considered to be one of the Paraneoplastic encephalitic disorders.
However, 40% NMDARE patients have no tumor! Only 2% have tumor other than ovarian teratoma
Autoantibodies to infectious agents by epitope similarity.
Loss of self-tolerance to the receptor due to some tissue damage outside the CNS.
Detection of NMDAR antibodies in serum/CSF is GOLD STANDARD diagnostic test –
100% have anti NMDAR in CSF while 85% in serum.
MANAGEMENT:
1. Dealing with acute effects of disease,
2. Removal of underlying tumour
3. Induction of immunosuppressive therapy
4. Management of residual effects.
METHODS & RESULTS:
17 year old female, known case of PCOD, on OCPs for 01 year, came with complaints of:
Altered sensorium X 02 weeks (muttering religious chants, behaving violently, auditory sensations.
H/o of episode of runny nose and low grade fever lasting for 2-3 days a week before the onset of these complaints.
Past history of frequent quarrels in the family.
Admitted under the Dept. of Psychiatry with the provisional diagnosis of dissociative disorder …
But, steady deterioration of symptoms
Two episodes of GTCS followed 02 days later by high grade fever.
Patient received in altered state with Oro- Facial Dyskinteic movements.
On Evaluation: CSF study revealed mild pleocytosis (2-4 cells/mm3, all mononuclear). HIV, HSV serology, ANA, RF, urine for PBG were negative, thyroid profile was normal & anti TPO was negative.
CEMRI Brain was suggestive of chronic calcified granuloma in right Cerebellar hemisphere.
EEG showed abnormal awake record with generalized β activity.
Anti- NMDA Antibodies in serum & CSF were positive.
CECT abdomen with pelvis was done to rule out malignancy. It was normal.
MANAGEMENT:
DISCHARGED ON: oral Steroids &
/Mycophenolate Mofetil.
CONCLUSIONS:
NMDARE is a new, under recognized and a potentially treatable neurological disorder.
Treatment essentially involves immunotherapy (1st line steroids and IVIG) & supportive management of symptoms.
Second line Treatment: initated after 10 days of awaiting response to primary therapy.
Rituximab, Cyclophosphamide, Azathiprine, MMF.
Immunosuppression can be continued for at least one year with steroid sparing agents after initial immunosuppressive therapy.
PROGNOSIS:
1. Non-neoplastic patients have poorer prognosis
2. Recovery occurs in reverse order of symptom presentation
3. Need several months of physical and behavioural rehabilitation
4. 47% patients have complete recovery, 18% left with severe deficits
5. Relapses upto 0.52 relapses per patient per year, after months
6. 4% mortality due to sepsis, sudden cardiac arrest, acute respiratory distress, refractory status epilepticus, tumour progression.
Prodrome (70%)
Psychiatric symptoms (68-77%)
Neurological symptoms
Wakeful unresponsiveness
Gradual return of responsiveness
Pulse Methyl-
prednisolone
IVIG & oral
Steroids
Clinical
improvement