The Case of the Mysterious Masquerader.
Let us delve into this enigma.
1st ever experience for us.
a 🧵 (1/13)
#neurotwitter #NeuroX #MedTwitter #MedX #Neurology #FOAMmed #Parkinsonsdisease #criticalcare #OSA #tauopathy
Let us delve into this enigma.
1st ever experience for us.
a 🧵 (1/13)
#neurotwitter #NeuroX #MedTwitter #MedX #Neurology #FOAMmed #Parkinsonsdisease #criticalcare #OSA #tauopathy
Sep 2022.
66♂. T2DM. HTN. Hypothyroid.
She was 1st seen by a neurologist for 2y h/o RUE tremors. Dx as Parkinson’s. L-dopa started.
MRI brain normal.
(02/13)
66♂. T2DM. HTN. Hypothyroid.
She was 1st seen by a neurologist for 2y h/o RUE tremors. Dx as Parkinson’s. L-dopa started.
MRI brain normal.
(02/13)
Nov 2022.
Reached ER w/ ↓sensorium, gasping respiration, developed over few hours.
Ventilated. Rapid improvement.
BUT, over 23d, 3 failed extubation attempts.
B/l lower lobe consolidation, later improved.
Needed tracheostomy, Day 21.
Discharged, Day 43.
Was it a happenstance?
Reached ER w/ ↓sensorium, gasping respiration, developed over few hours.
Ventilated. Rapid improvement.
BUT, over 23d, 3 failed extubation attempts.
B/l lower lobe consolidation, later improved.
Needed tracheostomy, Day 21.
Discharged, Day 43.
Was it a happenstance?
Jan 2023.
Recuperating well.
A Month post-discharge again reached ER w/ sudden onset respiratory failure, ↓sensorium over hours w/ significant ‘sweating’.
Ventilated.
Over 4 hours regained sensorium.
Extubated Day 3.
Discharged Day 11.
Dx: ? pneumonia, OSA.
Was it a coincidence?
Recuperating well.
A Month post-discharge again reached ER w/ sudden onset respiratory failure, ↓sensorium over hours w/ significant ‘sweating’.
Ventilated.
Over 4 hours regained sensorium.
Extubated Day 3.
Discharged Day 11.
Dx: ? pneumonia, OSA.
Was it a coincidence?
Mar 2024.
A Year passes, again respiratory failure over hours. Reaches ER of Our hospital gasping.
Ventilated again.
ABG: Type 2 resp failure.
Extubated Day 3.
Neurology ref: No definite conclusion drawn.
Discharged Day 8, on CPAP.
‘Eyes cannot see, what the mind does not know.’
A Year passes, again respiratory failure over hours. Reaches ER of Our hospital gasping.
Ventilated again.
ABG: Type 2 resp failure.
Extubated Day 3.
Neurology ref: No definite conclusion drawn.
Discharged Day 8, on CPAP.
‘Eyes cannot see, what the mind does not know.’
Aug 2024.
5 months pass.
Again, came gasping to Our ER w/ rapid onset respiratory failure.
Ventilated. Again quick improvement.
Extubated.
PSG: Severe OSA (AHI > 30).
We were missing the enemy.
'The Mysterious Masquerader.'
(06/13)
5 months pass.
Again, came gasping to Our ER w/ rapid onset respiratory failure.
Ventilated. Again quick improvement.
Extubated.
PSG: Severe OSA (AHI > 30).
We were missing the enemy.
'The Mysterious Masquerader.'
(06/13)
Neuro ref: Detailed history obtained.
Reviewed all past records.
Had done it last time too though.
Certain peculiar points glared at us this time: 👇
(07/13)
Reviewed all past records.
Had done it last time too though.
Certain peculiar points glared at us this time: 👇
(07/13)
We were looking at some sleep related disorder with additional features.
We had never experienced this disease before.
Studied. #Google
We send for The Test.
We could nail the 'Enemy' at last.
(08/13)
We had never experienced this disease before.
Studied. #Google
We send for The Test.
We could nail the 'Enemy' at last.
(08/13)
Anti-IgLON5 as an entity was 1st elucidated in this seminal paper from @idibaps Barcelona.
thelancet.com
(09/13)
thelancet.com
(09/13)
Anti-IgLON5 disease composite score (ICS) has also been described in 5 distinct domains.
1. Bulbar
2. Sleep disturbances
3. Movement disorders
4. Cognition
5. Others
(11/13)
1. Bulbar
2. Sleep disturbances
3. Movement disorders
4. Cognition
5. Others
(11/13)
Pathophysiology.
Poorly understood.
Is it autoimmune or neurodegenerative?
Is IgLON5 an innocent bystander or perpetrator?
IVIg & RTX started. 🤞🏻
(13/13)
Poorly understood.
Is it autoimmune or neurodegenerative?
Is IgLON5 an innocent bystander or perpetrator?
IVIg & RTX started. 🤞🏻
(13/13)
She also had mild RBD, like flinging/thrashing of limbs in sleep. No V-PSG done though.
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