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Anti-NMDA Receptor Encephalitis — Management of Symptoms — Clinical Pathway: Inpatient

Anti-NMDA Receptor Encephalitis Clinical Pathway — Inpatient

Management of Symptoms Associated with Anti-NMDARE

Common management strategies for various symptoms associated with anti-NMDARE are included below.

Several medications such as clonidine and benzodiazepines target multiple symptoms.

Movement Disorders
  • Manifestation
    • Orofacial dyskinesias, Choreoathetosis Dystonia
    • Rigidity, Parkinsonism
  • Treatment
    • Consider medication on a case-by-case basis in consultation with relevant specialists
  • Comments
    • Improves with immunotherapy treatment
    • Poly-pharmacy may lead to over sedation
    • Caution with anticholinergic and dopaminergic medications in children with psychosis
Seizures
  • Manifestation
    • Sub-clinical or clinical
    • Focal or generalized
    • Low threshold for video EEG monitoring
  • Treatment
  • Comments
    • Avoid levetiracetam due to impact on mood and behavior
Behavior Changes or Agitation
  • Manifestation
    • Neuropsychiatric symptoms may initially be intermittent at first, becoming more frequent with disease progression.
      • Irritability
      • Tantrums
      • Anxiety
      • Insomnia
      • Hallucinations
      • Delusions
      • Disorganized thinking
      • Catatonia
      • Aggression
      • Sleep dysfunction
      • Hyperactivity
      • Manic symptoms
    • Overt psychosis is observed more frequently among adolescents.
    • Younger children may exhibit subtle symptoms including
      • Temper tantrums
      • Aggression
      • Progressive speech deterioration
  • Treatment
  • Comments
    • Given antipsychotic medications can worsen the course of anti-NMDARE. Consult Psychiatry if psychosis is present.
Sleep Disturbances
  • Manifestation
    • Insomnia most common in acute phase
    • Hypersomnolence or inverted sleep patterns in recovery stage
    • However, any sleep problem may be associated with Anti-NMDARE
  • Non-Pharmacologic Treatment
    • Consider sleep chart/sleep log
    • Sleep Hygiene
      • Environmental changes (e.g., turn off TV, phone)
      • Minimize interruptions at night
      • Timed light exposure to support circadian rhythms
        • Open blinds during the day, closed at night, turn off artificial lights at night
      • Out of bed during the day
    • Environmental Safety
      • Assess falls risk
      • Bed alarms
      • Safety observer if needed, Mattress low to the ground
  • Treatment
    • Immediate release melatonin, clonidine, trazodone
  • Comments
    • Many medications used for anti-NMDARE play a role in sleep management such as clonidine, benzodiazepines, AEDs 2nd generation antipsychotics
  • Schedule medications to support sleep initiation and avoid daytime somnolence
Paroxysmal Sympathetic Hyperactivity
  • Manifestation
    • Core clinical features of PSH may include:
      • Tachycardia
      • Hypertension
      • Tachypnea
      • Central fever
      • Diaphoresis
      • Increased muscle tone with possible dystonic posturing
  • Treatment
  • Non-Pharmacologic Treatment
    • Avoid noxious stimuli when possible
      • E.g., acute pain, suctioning, loud noises, positioning, urinary retention, increased tone, etc.
    • Identify appropriate treatment and/or prevention of urinary retention and constipation
  • Comments
    • Many pharmacotherapies require a wean prior to discontinuation
    • Consult with clinical pharmacy and/or the managing service to determine an appropriate plan for therapy de-escalation
Feeding/Swallowing Dysfunction
  • Manifestation
    • Coughing, choking, wet vocal quality, throat clearing, watery eyes while eating or drinking
    • Overstuffing or impulsivity with eating
    • Oral pocketing
    • Inability to maintain a constant state of alertness while eating
    • Decrease in O2 saturation with any eating or drinking
    • New onset respiratory symptoms
    • CXR concerning for aspiration pneumonia
  • Treatment
    • Glycopyrrolate or scopolamine for secretion management
      • Glycopyrrolate may worsen tachycardia if child also has paroxysmal sympathetic hyperactivity
  • Non-Pharmacologic Treatment
    • Consider alternative means of nutrition if child is unable to meet all nutrition and hydration needs orally
  • Comments
    • Cognitive deficits, including level of alertness/awareness, impulsivity and poor safety awareness can impact safety with oral feeding in the setting of inadequate or disordered oral motor and swallowing abilities
    • Reevaluate and watch for signs of aspiration that may require NPO or alternative feeding
Catatonia
  • Manifestation
    • Up to 50% of cases may develop catatonia. Common features include:
      • Mutism
      • Staring
      • Echolalia
    • A minority of children will develop motor stereotypies.
  • Treatment
    • Benzodiazepines
      • Higher doses of benzodiazepines may be required. Consider consult with Psychiatry, Neurology, and PM&R.

 

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