Cyclic Vomiting Syndrome

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Basics

Description

  • An idiopathic chronic functional GI disorder characterized by discrete, recurrent, stereotypical paroxysmal episodes of high-intensity nausea and vomiting lasting hours to days
  • Subsets
    • Cyclic vomiting syndrome (CVS) plus two or more neuromuscular disorders in association
    • Catamenial CVS: associated with menstrual cycle
    • Note: Cannabis hyperemesis syndrome (CHS) is distinct from CVS.
  • CVS has four distinct phases:
    • Interepisodic: symptom-free period
    • Prodromal: minutes to hours of nausea with or without abdominal pain
    • Vomiting: hours to days
    • Recovery: Nausea remits and patient recovers appetite, strength, and energy (1)[B].

Epidemiology

Incidence

  • 3 per 100,000 annually in children
  • Limited data in adults

Prevalence

  • 0.04–2% in general population
  • 1–2% in children (2)
  • Female > male (55:45)
  • More common in children; mean age of diagnosis is 35 years in adults and ages 3 to 7 in children.
  • Average of 3 years from symptom onset to diagnosis

Etiology and Pathophysiology

  • Strong link between CVS and migraine: similar symptoms, frequent family history of migraines, and effectiveness of antimigraine therapy
  • One proposed mechanism:
    • Heightened neuronal excitability owing to enhanced ion permeability, mitochondrial deficits, or hormonal state → increased susceptibility to physical or psychological trigger → release of corticotropin-releasing factor (CRF) → vomiting
    • Vomiting perpetuated by altered brainstem regulation → sustained vomiting
  • Possible maternal inheritance, based on family history of migraines and link to mitochondrial DNA (mtDNA) mutations
  • Multiple theories:
    • GI motility dysfunction
    • Autonomic dysfunction: sympathetic (1)[B]
    • Food allergy or intolerance

Genetics

  • Likely matrilineal inheritance, especially with childhood onset
  • A3243G or other mtDNA mutations including mitochondrial dysfunction
  • Ion channel mutations
  • Several polymorphisms have been identified. 165119T more common in children with CVS

Risk Factors

  • Family history of migraine headaches
  • Depression and/or anxiety
  • Chronic cannabis use
  • Possibly food allergies
  • Hypothalamic-pituitary-adrenal axis dysfunction

General Prevention

  • No primary prevention measures exist.
  • Secondary prevention of attacks relies on multidisciplinary approach to trigger avoidance, comorbidity treatment, and prophylactic medication use.

Commonly Associated Conditions

  • Irritable bowel syndrome (67%)
  • Headaches (52%)
  • Motion sickness (46%)
  • Migraines (11–40%)
  • Seizure disorder (5.6%)
  • Cannabis use

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Basics

Description

  • An idiopathic chronic functional GI disorder characterized by discrete, recurrent, stereotypical paroxysmal episodes of high-intensity nausea and vomiting lasting hours to days
  • Subsets
    • Cyclic vomiting syndrome (CVS) plus two or more neuromuscular disorders in association
    • Catamenial CVS: associated with menstrual cycle
    • Note: Cannabis hyperemesis syndrome (CHS) is distinct from CVS.
  • CVS has four distinct phases:
    • Interepisodic: symptom-free period
    • Prodromal: minutes to hours of nausea with or without abdominal pain
    • Vomiting: hours to days
    • Recovery: Nausea remits and patient recovers appetite, strength, and energy (1)[B].

Epidemiology

Incidence

  • 3 per 100,000 annually in children
  • Limited data in adults

Prevalence

  • 0.04–2% in general population
  • 1–2% in children (2)
  • Female > male (55:45)
  • More common in children; mean age of diagnosis is 35 years in adults and ages 3 to 7 in children.
  • Average of 3 years from symptom onset to diagnosis

Etiology and Pathophysiology

  • Strong link between CVS and migraine: similar symptoms, frequent family history of migraines, and effectiveness of antimigraine therapy
  • One proposed mechanism:
    • Heightened neuronal excitability owing to enhanced ion permeability, mitochondrial deficits, or hormonal state → increased susceptibility to physical or psychological trigger → release of corticotropin-releasing factor (CRF) → vomiting
    • Vomiting perpetuated by altered brainstem regulation → sustained vomiting
  • Possible maternal inheritance, based on family history of migraines and link to mitochondrial DNA (mtDNA) mutations
  • Multiple theories:
    • GI motility dysfunction
    • Autonomic dysfunction: sympathetic (1)[B]
    • Food allergy or intolerance

Genetics

  • Likely matrilineal inheritance, especially with childhood onset
  • A3243G or other mtDNA mutations including mitochondrial dysfunction
  • Ion channel mutations
  • Several polymorphisms have been identified. 165119T more common in children with CVS

Risk Factors

  • Family history of migraine headaches
  • Depression and/or anxiety
  • Chronic cannabis use
  • Possibly food allergies
  • Hypothalamic-pituitary-adrenal axis dysfunction

General Prevention

  • No primary prevention measures exist.
  • Secondary prevention of attacks relies on multidisciplinary approach to trigger avoidance, comorbidity treatment, and prophylactic medication use.

Commonly Associated Conditions

  • Irritable bowel syndrome (67%)
  • Headaches (52%)
  • Motion sickness (46%)
  • Migraines (11–40%)
  • Seizure disorder (5.6%)
  • Cannabis use

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