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G342(P) Weak at the knees with laughter
  1. S Rao,
  2. J Patel
  1. Deprtment of Paediatric Neurology, Bristol Royal Hospital for Children, Bristol, UK

Abstract

Narcolepsy is a chronic neurological disorder characterised by excessive daytime sleepiness, cataplexy, hallucinations in sleep-wake phase and sleep paralysis.

Case reports (methods) We present a case series of children with narcolepsy from the South-West.

Case 1: A 6 year old boy presented with extreme tiredness and excessive sleep. He was afraid of laughing as it made him weak at knees and he tends to drop to the floor, with little head drops with excitement. Sleep was disturbed with frequent movements and had significant weight gain since onset of symptoms.

He has cataplectic facies with droopy eyelids, open mouth with tongue thrusting. Examination was normal as were investigations including EEG and MRI head. CSF levels of hypocretin were reduced, confirming a diagnosis of Narcolepsy.

Case 2: A 10 yrs athletic boy developed episodes of extreme lethargy, easy fatigability and sleep walking. He had started falling asleep mid-activity. His neurological examination and initial investigations including EEG and Nerve conduction studies were normal. He tested (HLA) DQB1*0602 positive with absent hypocretin in CSF, confirming narcolepsy.

Case 3: 9 yr girl old with tics; ataxia developed excessive sleepiness with significant disruption of night time sleep and behavioural problems at school. She is awaiting results of investigations.

Case 4: A 12 yr old with epilepsy, ADHD presented with episodes of narcolepsy but no cataplexy and investigations are suggestive of a diagnosis of narcolepsy.

Results Narcolepsy is a rare diagnosis in children. Strong association with (HLA) DQB1*0602 has been noted. Diagnosis is by clinical features; polysomnograph followed by multiple sleep latency tests and reduced in CSF hypocretin levels. It is a lifelong condition and management is supportive.

Finnish and Swedish studies have shown higher incidence of narcolepsy with swine flu vaccination (ASO3 adjuvant H1N1 vaccine). UK study showed a 10-fold increased risk of narcolepsy within six months and estimates risk as one in 52,000 in those vaccinated (1).

Conclusion We aim to raise awareness on the recent high incidence of narcolepsy in children attributed to increased risk with swine flu vaccine. We present videos, advances in diagnosis and review of literature.

Reference:

  1. E Miller et al., Risk of narcolepsy in children and young people receiving AS03 adjuvanted pandemic A/H1N1 2009 influenza vaccine: retrospective analysis, BMJ 2013;346:f794

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