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
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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
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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.
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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
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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
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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
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Catatonia |
- Manifestation
- Up to 50% of cases may develop catatonia. Common features include:
- 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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