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Dr. AmruthaVenkateswaran
 Autoimmune encephalitis- group of syndromes that
occur as a result of antibodies against-
- Neuronal cell protein
- Syna...
 Exact incidence not exactly known
 Studies show- comprises 5 to 10% of all encephalitic
syndromes
 May surpass viral e...
 Anti- N-Methyl D- Aspartic acid receptor
encephalitis (Anti NMDAR)
 Anti LG-1 Encephalitis
 CSAPR 2
 GABA –B
 AMPA e...
 The antibodies target the NR1 subunit of the NMDA
receptor
 Second most common cause of autoimmune
encephalitis-
 Most...
 First stage- Prodromal stage, mimics viral flu like
syndrome
 Second stage- Neuropsychiatric features,
behavioural chan...
NMDA Receptors- inhibitory
GABAnergic neurons and glutamergic
synapses
Increased extracellular glutamate
Fronto striatal s...
 Toddlers and infants- present with complex
movement disorders and status epilepticus
 Often labelled as infantile tremo...
 Association with tumors- testicular and ovarian
teratomas
 Seen in more than 40% of females less than 12 years
 <15% i...
 EEG abnormal in most of the cases- 30% of adult
patients show a unique EEG pattern, called
“extreme delta brush” because...
EEG- Shows serrated appearance of delta
waves- due to overriding and fast beta activity
with slowing of theta waves
MRI BRAIN-T2 segment FLAIR bilateral temporal lobe
hyperintense signal
B- follow up shows decrease in intensity
 CSF- Abnormal in approximately 80% of cases,
showing moderate lymphocytic pleocytosis,
increased protein synthesis and o...
 Mortality rate- 7%
 80% full recovery
 Slow recovery- can take 2 years, last to recover-
social interactions, language...
Viral Encephalitis Acute onset, lesser psychosis
and dyskinesias, more
pleocytosis in csf
Relapsing post herpes simplex
en...
PEM- Paraneoplastic
encephalomyelitis
Personality changes,
disorientation, memory loss
Autoimmune post
streptococcal neuro...
 Other D/D
- Limbic encephalitis
- Encephalitis lethargica
- Childhood disintegrative disorder/ late onset autism
- IEM
-...
 Inflammatory process of the limbic system
including, the medial temporal lobes, amygdala,
and cingulate gyri.
 Antibodi...
 Severe short-term memory loss
 Hyponatremia
 Seizures-
myoclonic-like movements (faciobrachial dystonic
seizures)
*Oph...
A) T2 segment FLAIR in medial
temporal lobe
B) Improvement in density on
follow up
C) & D) generalized atrophy on
follow u...
T2 segment FLAIR seen in limbic encephalitis- closest D/D is
Herpes simplex Encephalitis which shows similar picture
 Other types can occur with antibodies against
intracellular antigens (eg, Hu, CRMP5, Ma2) or
against cell surface or syn...
 Defined by the detection of thyroid peroxidase
(TPO) antibodies in patients with acute or subacute
encephalitis that res...
 Stroke-like symptoms
 Tremor, myoclonus
 Transient aphasia
 Sleep and behaviour abnormalities
 Hallucinations, seizu...
 Relevant antibody-associated disorders, such as
GABA(B), LGI1, or NMDA receptor antibodies
 In some instances, plasma e...
 Inflammatory encephalopathy characterized by
progressive refractory partial seizures, cognitive
deterioration, and focal...
 Mechanism still unclear
 Antibodies against the GluR3 subunit of the AMPA
receptor andT-cell mediated mechanisms trigge...
 “Acute Encephalitis with Refractory Repetitive
Partial Seizures (AERRPS)”
 “Fever-Induced Refractory Epileptic
Encephal...
 Presence of autoantibodies, but lack to response to
conventional treatment of autoimmune
encephalitis.
 Residual sympto...
ROHHAD
 Rapid onset obesity with hypothalamic dysfunction,
hypoventilation, and autonomic dysregulation seen
in children ...
 Basal ganglia encephalitis- patients with
predominant or isolated involvement of the basal
ganglia.
 Abnormal movements...
 Pseudomigraine syndrome with CSF pleocytosis
(PMP) or headache with neurologic deficits and
CSF lymphocytosis (HaNDL)- p...
Ophthalmoplegic migraine (recurrent cranial
neuralgia)
 Recurrent bouts of head pain in addition to cranial
nerves III, I...
 Occurs in children and adults although it probably
represents different diseases and pathogenic
mechanisms
 Syndrome us...
 Typical features of opsoclonus characterized by
rapid, chaotic, multidirectional eye movements
without saccadic interval...
 Subacute progressive ophthalmoplegia and ataxia
in addition to drowsiness or hyperreflexia.
 More frequent in adults
 ...
 Idiopathic/ Post infectious
- CSF- no evidence of neuronal antibodies
- Show similar syndrome like anti NMDAR
encephalit...
 Anti NMDAR
 Anti LG-1 Encephalitis
 CSAPR 2
 GABA –B
 AMPA encephalitis
Done in both serum and CSF
 No consensus guidelines
 Pulsed dose IV methyl prednisolone followed by
high dose oral steroids
( 30mg/kg/day upto 1 g/...
 Absence of CSF pleocytosis does not rule
autoimmune encephalitis
 d/d:
- Viral encephalitis
- Mitochondrial disorders
-...
 Should be considered as a differential for acute
encephalitis syndrome
 Presents with neuropsychiatric symptoms in olde...
Autoimmune encephalitis
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Autoimmune encephalitis

Autoimmune encephalitis

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Autoimmune encephalitis

  1. 1. Dr. AmruthaVenkateswaran
  2. 2.  Autoimmune encephalitis- group of syndromes that occur as a result of antibodies against- - Neuronal cell protein - Synaptic receptors * Help in transmission, plasticity and excitability of neurons
  3. 3.  Exact incidence not exactly known  Studies show- comprises 5 to 10% of all encephalitic syndromes  May surpass viral encephalitis if close studies are done
  4. 4.  Anti- N-Methyl D- Aspartic acid receptor encephalitis (Anti NMDAR)  Anti LG-1 Encephalitis  CSAPR 2  GABA –B  AMPA encephalitis
  5. 5.  The antibodies target the NR1 subunit of the NMDA receptor  Second most common cause of autoimmune encephalitis-  Most common in females (80%), around 40% occurs in less than 18 years of age.  In older children and adoloscents, the disease follows a predictable course
  6. 6.  First stage- Prodromal stage, mimics viral flu like syndrome  Second stage- Neuropsychiatric features, behavioural changes- agitation, change in mood, speech disturbances  Third stage- decreased level of consciousness, seizures, limb or oral dyskinesias, choreoathetoid movements, and autonomic instability
  7. 7. NMDA Receptors- inhibitory GABAnergic neurons and glutamergic synapses Increased extracellular glutamate Fronto striatal syndrome- psychatric manifestations Complex movement disorders Central Hypoventilation
  8. 8.  Toddlers and infants- present with complex movement disorders and status epilepticus  Often labelled as infantile tremor syndrome * Often psychiatric disturbance missed due to their young age. • Cerebellar ataxia and hemiparesis • On- Off phenomenon with response to the level of consciousness
  9. 9.  Association with tumors- testicular and ovarian teratomas  Seen in more than 40% of females less than 12 years  <15% in young boys
  10. 10.  EEG abnormal in most of the cases- 30% of adult patients show a unique EEG pattern, called “extreme delta brush” because of its resemblance to the delta brush (beta-delta complexes) pattern in premature infants.  Brain MRI is abnormal in approximately 35% of cases, nonspecific cortical and subcorticalT2 (FLAIR) signal abnormalities, transient cortical or meningeal enhancement; nonspecific white matter abnormalities. Lesions may also involve the spinal cord producing symptoms of myelitis
  11. 11. EEG- Shows serrated appearance of delta waves- due to overriding and fast beta activity with slowing of theta waves
  12. 12. MRI BRAIN-T2 segment FLAIR bilateral temporal lobe hyperintense signal B- follow up shows decrease in intensity
  13. 13.  CSF- Abnormal in approximately 80% of cases, showing moderate lymphocytic pleocytosis, increased protein synthesis and oligoclonal bands.  NMDAR antibodies in CSF or serum
  14. 14.  Mortality rate- 7%  80% full recovery  Slow recovery- can take 2 years, last to recover- social interactions, language and executive functions  15% relapse- partial syndrome
  15. 15. Viral Encephalitis Acute onset, lesser psychosis and dyskinesias, more pleocytosis in csf Relapsing post herpes simplex encephalitis 4-6 weeks post treatment of herpes simplex encephalitis- true viral relapse (PCR +) or Autoimmune (PCR-) New onset psychosis Primary psychiatric disorder Drugs/Toxins Ketamine, phencyclidine, carbon monoxide. Neuroleptic Malignant Syndrome episodes of rigidity, hyperthermia, and autonomic Instability, frequent use of neuroleptics to control the abnormal behavior
  16. 16. PEM- Paraneoplastic encephalomyelitis Personality changes, disorientation, memory loss Autoimmune post streptococcal neurological syndrome PANDAS, PANS,CANS, sydenham chorea,Tourette syndrome ADEM Confusion, ataxia, weakness with visual blurring Hashimoto’s encephalopathy Epilepsy, disorientation, memory problems Rasmussen’s encephalitis Mental deterioration, seizures, hemiparesis
  17. 17.  Other D/D - Limbic encephalitis - Encephalitis lethargica - Childhood disintegrative disorder/ late onset autism - IEM - Monoamine neurotransmitter disorders - Demyelinating disorders - CNS vasculitits
  18. 18.  Inflammatory process of the limbic system including, the medial temporal lobes, amygdala, and cingulate gyri.  Antibodies against neuronal protein called leucine- rich glioma inactivated 1 (LGI1), and a protein called Caspr2 expressed in brain and myelinated nerves. *This disorder was initially identified in 1968 as a paraneoplastic syndrome associated with small-cell lung cancer.
  19. 19.  Severe short-term memory loss  Hyponatremia  Seizures- myoclonic-like movements (faciobrachial dystonic seizures) *Ophelia syndrome- association with Hodgkin’s lymphoma  Develop antibodies against mGluR5, a receptor involved in learning and memory
  20. 20. A) T2 segment FLAIR in medial temporal lobe B) Improvement in density on follow up C) & D) generalized atrophy on follow up after 9 months
  21. 21. T2 segment FLAIR seen in limbic encephalitis- closest D/D is Herpes simplex Encephalitis which shows similar picture
  22. 22.  Other types can occur with antibodies against intracellular antigens (eg, Hu, CRMP5, Ma2) or against cell surface or synaptic proteins.  InvolvesT cell immune mediated reactions  Responds well to immunotherapy unlike the conventional form
  23. 23.  Defined by the detection of thyroid peroxidase (TPO) antibodies in patients with acute or subacute encephalitis that responds to steroids.  “Encephalopathy associated with autoimmune thyroid disease” is considered more accurate- due to normal thyroid function.  Very vague symptoms- unclear course
  24. 24.  Stroke-like symptoms  Tremor, myoclonus  Transient aphasia  Sleep and behaviour abnormalities  Hallucinations, seizures and ataxia.  EEG is usually abnormal  MRI brain- normal  CSF shows pleocytosis with elevated protein  TPO antibodies should be viewed as a marker of autoimmunity rather than a neurologic disease-specific or pathogenic antibody.
  25. 25.  Relevant antibody-associated disorders, such as GABA(B), LGI1, or NMDA receptor antibodies  In some instances, plasma exchange or intravenous immunoglobulin has been equally effective as steroids.
  26. 26.  Inflammatory encephalopathy characterized by progressive refractory partial seizures, cognitive deterioration, and focal deficits that occur with gradual atrophy of one hemisphere  The disorder frequently presents in 6to 8year old children, although adolescents and adults can be affected.
  27. 27.  Mechanism still unclear  Antibodies against the GluR3 subunit of the AMPA receptor andT-cell mediated mechanisms triggered by a viral infection.  High dose methyl prednisolone  Rituximab and intraventricular α-interferon  ‘Functional hemispherectomy’- surgical disconnection of the affected hemisphere.
  28. 28.  “Acute Encephalitis with Refractory Repetitive Partial Seizures (AERRPS)”  “Fever-Induced Refractory Epileptic Encephalopathy Syndrome (FIRES),”  “Devastating Epilepsy of School-aged Children (DESC).”
  29. 29.  Presence of autoantibodies, but lack to response to conventional treatment of autoimmune encephalitis.  Residual symptoms included cognitive impairment, temporal lobe epilepsy, and mesial temporal sclerosis.
  30. 30. ROHHAD  Rapid onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation seen in children upto 2-4 years who are normal.  Followed by episodes of hyperphagia, weight gain, abnormal behaviour, autonomic dysfunction and central hypoventilation  Frequent association to neural crest tumors
  31. 31.  Basal ganglia encephalitis- patients with predominant or isolated involvement of the basal ganglia.  Abnormal movements and neuropsychiatric disease.  Multiple etiologies, including metabolic, toxic, genetic, and infectious processes, an immune- mediated etiology has been postulated.  Antibodies against the dopamine-2 receptor have been identified
  32. 32.  Pseudomigraine syndrome with CSF pleocytosis (PMP) or headache with neurologic deficits and CSF lymphocytosis (HaNDL)- predominantly affects young male adults with a family history of migraine.  Repeated episodes of severe headache with transient neurologic deficits  CSF- lymphocytosis with elevated protein  MRI- normal  EEG- background slowing activity
  33. 33. Ophthalmoplegic migraine (recurrent cranial neuralgia)  Recurrent bouts of head pain in addition to cranial nerves III, IV, and/orVI involvement.  MRI shows focal nerve thickening and contrast enhancement  D/D includes structural, neoplastic, traumatic, metabolic, and infectious disorders.
  34. 34.  Occurs in children and adults although it probably represents different diseases and pathogenic mechanisms  Syndrome usually develops in the 1st 2 yr of life  50% have underlying neuroblastoma  Child often presents with Irritability, ataxia, myoclonus, tremor, and drooling of saliva
  35. 35.  Typical features of opsoclonus characterized by rapid, chaotic, multidirectional eye movements without saccadic intervals  CSF- antibodies against neuronal proteins  Immunosuppressive treatment, including corticosteroids, IVIG, rituximab, and cyclophosphamide  Relapses occur in 50% of the patients
  36. 36.  Subacute progressive ophthalmoplegia and ataxia in addition to drowsiness or hyperreflexia.  More frequent in adults  Mimics GBS in younger children  Serum GQ1b immunoglobulin G antibodies are found in 66% of patients, with MRIT2 FLAIR abnormality.  Treated with steroids, IVIG, and/or plasma exchange, and often have good outcome
  37. 37.  Idiopathic/ Post infectious - CSF- no evidence of neuronal antibodies - Show similar syndrome like anti NMDAR encephalitis  Patients usually have full recovery after treatment with immunotherapy unlike opsoclonus- myoclonus  d/d: listeria, enterovirus, primary CNS lymphoma
  38. 38.  Anti NMDAR  Anti LG-1 Encephalitis  CSAPR 2  GABA –B  AMPA encephalitis Done in both serum and CSF
  39. 39.  No consensus guidelines  Pulsed dose IV methyl prednisolone followed by high dose oral steroids ( 30mg/kg/day upto 1 g/day) for 3 to 5 days followed by 1-2mg/kg/day of oral prednisolone 2g/kg of IV Ig for 2-5 days with plasma exchange  Second line therapy- rituximab and cyclophosphamide
  40. 40.  Absence of CSF pleocytosis does not rule autoimmune encephalitis  d/d: - Viral encephalitis - Mitochondrial disorders - Leukodystrophy  Should be investigated for new antibodies in serum if CSFantibodies are negative.
  41. 41.  Should be considered as a differential for acute encephalitis syndrome  Presents with neuropsychiatric symptoms in older children and with status epilepticus in younger children and infants  Treatable cause of acute encephalitis

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