Presentaties ISMC - Hypersomnia
50 important questions on Presentaties ISMC - Hypersomnia
What are some warnings and strategies related to attacks in Hypocretin-1 deficient individuals?
- Warnings:
- - Can feel an attack coming: 57.0%
- - ‘Strange feeling in the head’ before an attack: 48.5%
- Strategies:
- - Has tricks to prevent an attack: 58.3%
- - Can resist an attack when it starts: 46.2%
- - Resisting changes which body parts get affected: 24%
How often do Hypocretin-1 deficient individuals suffer injuries due to cataplexy?
- Never: 56.6%
- Seldom: 29.2%
- Sometimes: 10.4%
- Often: 3.8%
What does the validation concept for typical cataplexy show regarding hypocretin-1 concentrations?
- No cataplexy
- Typical cataplexy
- Atypical cataplexy
- Median and interquartile ranges are indicated.
- Dashed lines show detection limit (75 pg/mL) and intermediate range (111-200 pg/mL).
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What are the characteristics of typical cataplexy?
- Meets all ICSD3 criteria.
- Bilaterally symmetrical, brief episodes (< 2 min).
- Triggered by strong, positive emotions.
- One or more episodes of muscle tone loss.
- Abrupt return of muscle activity.
- Retained consciousness.
What are the characteristics of falling in Hypocretin-1 deficient individuals?
- Falls straight down: 33.3%
- Falls to the floor within 5 seconds: 58.1%
What is the pattern of muscle weakness in Hypocretin-1 deficient individuals?
- Only complete attacks: 23.8%
- Only partial attacks: 29.5%
- Both partial & complete: 45.7%
What defines atypical cataplexy according to Lammers et al.?
- Meets one or more in addition to or contradicting typical criteria.
- Purely unilateral episodes.
- Prolonged duration (e.g., > 3 min).
- No identifiable or negative emotion triggers.
- Hyperacute generalized weakness.
- Prolonged recovery.
- Exclusively generalized attacks.
What are the top triggers for cataplexy according to the study?
- Laughing excitedly: Often - 32.7%, Always - 29%
- Making a sharp-minded remark: Often - 36.5%, Always - 17.3%
- Telling a joke: Often - 32.7%, Always - 15.4%
- Punch line of a joke: Often - 33.3%, Always - 13.9%
- Being tickled: Often - 26.3%, Always - 15.8%
What is the duration and immediate aftermath of complete attacks?
- Immediate muscle usability after attack: 89.6%
- Complete attacks build up in at least seconds
What percentage of patients experience cataplexy when being startled?
- Never: 45.3%
- Sometimes: 25.5%
- Often: 22%
- Always: 7.3%
How prevalent is self-perceived unilateral paralysis among Hypocretin-1 deficient individuals?
What are some additional aspects of typical attacks noted in the presentation?
- Majority of attacks are partial.
- Common trigger is laughter, but it’s less specific.
- At least one other specific trigger, such as unexpected meetings.
- 'Building up of weakness' observed.
- Ends abruptly and completely.
- Involves jerks.
- Exceptional during consultations.
How frequently do patients experience cataplexy spontaneously?
- Never: 41.7%
- Sometimes: 51.5%
- Often: 5.8%
- Always: 1.0%
What are the characteristics of typical cataplexy?
- Sudden onset of muscle weakness
- Bilateral occurrence
- Preserved consciousness
- Triggered by emotions
- Duration ranges from seconds to minutes
Which muscle groups are most involved during a cataplexy attack?
- Knee, leg: 67.7%
- Neck, head: 57.6%
- Jaw, face: 69.7%
- Shoulder: 17.2%
- Arm: 35.4%
- Hand: 46.5%
- Muscles related to speech: 43.4%
- Other: 8.1%
What triggers include hearing an unexpected sound?
- Never: 63.3%
- Sometimes: 22.1%
- Often: 11.4%
- Always: 3.2%
What are the choices provided for identifying a typical cataplexy scenario?
- A) Cataplexy triggered by reading a funny book.
- B) Cataplexy with sudden and short muscle tone loss.
- C) Cataplexy where muscle tone loss is followed by sleep.
- D) Not cataplexy; lady falls asleep due to a boring book.
What are the consciousness and muscle twitching characteristics during a cataplexy attack?
- Conscious at beginning of attack: 100%
- Muscle twitches during cataplexy: 54.3%
What are the rates of cataplexy triggered by pain?
- Never: 69.8%
- Sometimes: 15.2%
- Often: 11.4%
- Always: 3.6%
What is the focus of the 2011 study in Sleep Medicine by Overeem and colleagues?
- Explores clinical features of cataplexy.
- Uses a questionnaire study.
- Targets narcolepsy patients.
- Compares with and without hypocretin-1 deficiency.
- Published in Sleep Medicine, Volume 12 (2011).
What topics are covered in the interactive video session on cataplexy?
- Determining if symptoms indicate cataplexy.
- Examples of typical and atypical cataplexy.
- Differences in muscle weakness during cataplexy vs. sleep.
- Cataplexy in children and its phenotype changes.
- Differential diagnosis considerations.
What is the ICSD-2 expert definition of the condition depicted?
- Transient episode of bilateral loss of muscle tone.
- Consciousness is preserved.
- Triggered by strong emotions, often laughing or joking.
- Duration is less than 2 minutes.
What are the conclusions regarding the ISCD-2 definition and laughter as a trigger?
- ISCD-2 definition can be more specific based on new data.
- There are more relevant characteristics than those listed.
- Laughter is frequent but not a universal trigger, showing less specificity.
What is the duration of complete cataplexy attacks in hypocretin-1 deficient patients?
- 15min: 1.4%
How does the duration of partial cataplexy attacks vary according to the study?
- 15min: 1%
What are some triggers for cataplexy according to the study?
- Laughing excitedly: 8.4%, 29.9%, 32.7%, 29.0%
- Sharp-minded remark: 11.5%, 34.6%, 36.5%, 17.3%
- Telling a joke: 12.5%, 39.4%, 32.7%, 15.4%
- Before a joke punchline: 15.7%, 37.0%, 33.3%, 13.9%
- Being tickled: 38.9%, 18.9%, 26.3%, 15.8%
- Hearing a joke: 17.0%, 43.4%, 31.1%, 8.5%
- Angry: 30.5%, 33.3%, 29.5%, 6.7%
- Unexpectedly meeting someone known: 35.9%, 29.1%, 25.2%, 9.7%
What are the main themes covered in the recent questionnaire study?
- Triggers: Identified factors causing reactions
- Patterns of muscle weakness: Examined variations in muscle response
- Associated features: Explored warning signs and injury
- Treatment (effect): Evaluated impact of treatments
- Limitations in daily life due to cataplexy: Analyzed effects on routine activities
What are the goals of the questionnaire study?
- Verify the ICSD-2 definition of cataplexy.
- Describe additional unaddressed features.
- Use a well-defined ‘gold standard’:
- - Diagnosis by narcolepsy-expert
- - CSF Hypocretin-1 levels
Why is evoking cataplexy difficult?
- Occurs rarely in a doctor's office.
- Requires a certain amount of relaxation.
- History taking is crucial to identify cataplexy.
What details are presented about the cataplexy event at ISMC 2022?
- Event: ISMC 2022
- Date: June 9th
- Location: St-Michielsgestel
- Speakers: Gert Jan Lammers & Rolf Fronczek
- Affiliations:
- - Leiden University Medical Center
- - Slaap Wake Centre SEIN, Netherlands
What are the key symptoms and features of narcolepsy?
- Excessive daytime sleepiness
- Cataplexy (loss of muscle tone)
- Hypnagogic hallucinations
- Sleep paralysis (inability to move after waking)
- Obesity
- Fragmented nighttime sleep
- Short lasting episodes
- Disturbed vigilance
What are the ICSD-3 criteria for Narcolepsy With Cataplexy (type 1)?
- Excessive Daytime Sleepiness for at least 3 months.
- Typical Cataplexy and a positive MSLT (Multiple Sleep Latency Test).
- Hypocretin-1 Deficiency as an alternative criteria.
What might be the purpose of the wires and electrodes attached to the child's head?
- Likely used for monitoring brain activity.
- Common in medical testing like EEG.
- Can diagnose neurological conditions.
- Electrodes track electrical signals in the brain.
What are some conditions classified under CNS hypersomnolence in ICSD-3?
- Narcolepsy type 1 & 2 (NT1 & NT2)
- Idiopathic Hypersomnia (IH)
- Kleine-Levin Syndrome (KLS)
- Hypersomnia due to:
- - Medical Disorder
- - Medication or Substance
- - Psychiatric Disorder
- - Insufficient Sleep
What role do hypocretin-producing neurons play in narcolepsy, according to the diagram?
- Hypocretin-producing neurons are crucial for maintaining wakefulness.
- Their absence leads to a disturbance in the balance between wake and sleep.
- This results in a tendency to fall asleep easily.
What brain structures and neurotransmitters are associated with Narcolepsy type 1?
- Hypothalamus: Location for the loss of hypocretin-producing neurons.
- Suprachiasmatic nucleus: Regulates circadian rhythms.
- Ventralateral preoptic nucleus: Involved in sleep induction.
- Hypocretin: Deficiency in narcolepsy.
- Neurotransmitters:
- - Histamine
- - Serotonin
- - Norepinephrine
What are potential symptoms of hypersomnolence according to the International Classification of Sleep Disorders?
- Sleepiness: Persistent feeling of sleepiness
- Excessive Daytime Sleepiness (EDS): More sleepiness during the day
- Fatigue: Persistent tiredness
- 'Residual EDS': Ongoing sleepiness even after sleep
- Lowered vigilance/alertness/attention: Difficulty maintaining focus
What are the ICSD-3 criteria for Narcolepsy without Cataplexy (type 2)?
- Narcolepsy without Cataplexy (type 2) requires:
- - Excessive Daytime Sleepiness lasting at least 3 months.
- - A positive result on the MSLT (Multiple Sleep Latency Test).
What are the brain nuclei important for sleep?
- Suprachiasmatic nucleus: Regulates circadian rhythms.
- Ventrolateral preoptic nucleus: Promotes sleep.
- Hypocretin producing neurons: Involved in wakefulness.
- Nucleus tuberomamillaris: Releases histamine.
- Nucleus raphes posterior: Releases serotonin.
- Locus caeruleus: Releases norepinephrine.
What is the difference in the number of hypocretin neurons in normal, cataplexy, and no cataplexy conditions according to Thannickal et al., Sleep 2009?
- Normal: High number of hypocretin neurons.
- Cataplexy: Significant loss of neurons.
- No Cataplexy: Partial loss, indicating potential narcolepsy type 2.
- Research suggests a clear relationship between hypocretin neuron count and cataplexy presence.
What does the graph indicate about hypocretin-1 levels in controls versus patients with narcolepsy?
- Controls: Hypocretin-1 levels range around 300-500 pg/ml.
- Patients: Many have levels below detection limit.
- Narcoleptics show reduced hypocretin-1, indicating a deficiency.
What does the concept "Sleep and Wake out of Balance" refer to?
- Imbalance in sleep and wake cycles.
- Visualized by unevenly stacked stones.
- Represents disrupted sleep routines or disorders.
- Linked to health issues and decreased productivity.
How does hypocretin-1 neuron distribution differ in normal versus narcoleptic individuals?
- Normal: Dense clusters of hypocretin-1 neurons shown.
- Narcoleptic: Sparse or absent hypocretin-1 neurons.
- Suggests neuron loss is associated with narcolepsy.
What is the main topic and who is the presenter of the lecture?
- Title: Central Disorders of Hypersomnolence
- Presenter: Rolf Fronczek, PhD, MD
- Profession: Neurologist/Somnologist
- Affiliation: Leids Universitair Medisch Centrum
- Associated Center: SEIN
What are the cited studies in the analysis of hypocretin-1 levels in narcolepsy?
- Nishino et al., Lancet 2000.
- Thannickal et al., 2000.
- Studies focus on hypocretin-1 deficiency and neuron loss in narcolepsy.
What is the pathophysiology of narcolepsy?
- Narcolepsy involves the loss of hypocretin-producing neurons.
- This occurs in the lateral hypothalamus.
- Hypocretin is crucial for regulating wakefulness and sleep.
What is a positive MSLT for narcolepsy types 1 and 2?
- Mean Sleep Latency Test (MSLT):
- - Narcolepsy Types 1 & 2
- - Positive if mean sleep latency is less than 8 minutes
- - Multiple sleep onset REM periods
What differences in sleep stages are observed between control and narcolepsy subjects?
- Control Group:
- - Consistent wakefulness during the day.
- - Regular sleep stages at night.
- Narcolepsy Group:
- - Frequent day sleep episodes.
- - Disrupted night sleep stages.
- - Quick shifts to REM sleep.
What are the ICSD-3 criteria for Narcolepsy With Cataplexy (type 1)?
- Excessive Daytime Sleepiness (lasting 3 months)
- Typical Cataplexy and Positive MSLT
- Hypocretin-1 Deficiency
What is CNS Hypersomnolence Disorder and its symptoms?
- CNS Hypersomnolence Disorder involves sleeping for an excessively long time.
- Known as Excessive Daytime Sleepiness (EDS).
- Symptoms include:
- - Inability to stay awake during usual wake times.
- - Periods of irresistible sleep and sleep attacks.
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