in children - EPNS€¦ · behavioral responses to visual, auditory, tactile, or noxious stimuli...
Transcript of in children - EPNS€¦ · behavioral responses to visual, auditory, tactile, or noxious stimuli...
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comaproblems of consciousness
in children
Kees BraunUMC Utrecht
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definitions
consciousness
state of full awareness of the self and one’s relationship to
the environment
consiousness ≠ responsiveness
level of wakefullness / alertness
content or quality: awareness of self/environment,
including various and overlapping functions,
such as attention, perception, memory
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definitions
coma
- a state of deep, unarousable, sustained pathologic
unconsciousness with the eyes closed
- persisting for at least 1 hour
- lack of both wakefulness and awareness
- patient cannot be aroused to respond appropriately to
painful stimuli
E1M5V2
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coma – in general
requires dysfunction of:
Ascending (Reticular)
Arousal (or Activating)
System ([A]RAS) upper brain stem
diencephalon
or
both hemispheres
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coma – in general
requires dysfunction of:
Ascending (Reticular)
Arousal (or Activating)
System ([A]RAS) upper brain stem
diencephalon
or
both hemispheres
caused by:
structural lesions
compressive
destructive
diffuse/metabolic causes
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paramedian midbrain
dorsolateral pons
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no coma
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asessing coma – level of consciousness GCS
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pitfalls of the GCS
10 year old girl
eyes open
normal pupillary and corneal reflexes
conjugate eye deviation – left
does not obey commands
extends R arm, localizes with L arm
makes sounds to pain
E4M5V2
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pitfalls of the GCS
10 year old girl
eyes open
normal pupillary and corneal reflexes
conjugate eye deviation – left
does not obey commands
extends R arm, localizes with L arm
makes sounds to pain
E4M5V2
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pitfalls of the GCS
L MCA infarct
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pitfalls of the GCS
5 year old boy
after breakfast: “sleepy”
within 2 hours: progressive paresis R, later L, unresponsive
E4 / M5(L) M2(R) / V2
no words, no movements of face/arms/legs at request
eye deviation to R, no roving movements
PR +/+, corneal reflexes -/-
oculocephalic reactions: INO
bilateral Babinski’s sign
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pitfalls of the GCS
locked-in syndrome, pontine infarction, basilar artery thrombosis
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pitfalls of the GCS
insufficiently examined too little pain
not bilaterally
discrepancy E-M-V scores
aphasia
anarthria
tetraplegia
coma – mimics
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LOC / coma – mimics
Ashwal Brain Dev 2003
awake
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vegetative state
Ashwal NeuroRehabil 2004
Criteria
all of the following:
– no evidence of awareness of themselves or their environment; they are
incapable of interacting with others
– no evidence of sustained, reproducible, purposeful, or voluntary
behavioral responses to visual, auditory, tactile, or noxious stimuli
– no evidence of language comprehension or expression
– intermittent wakefulness manifested by the presence of sleep-wake
cycles.
– sufficiently preserved hypothalamic and brain stem autonomic functions
to survive if given medical and nursing care
– bowel and bladder incontinence
– variably preserved cranial nerve (pupillary, oculocephalic, corneal,
vestibulo-ocular, gag) and spinal reflexes
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minimally conscious state
Ashwal Brain Dev 2003
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psychogenic unresponsiveness
lie with eyes closed
normal reflexes and ventilatory patterns
oculocephalic reflexes absent (due to visual fixation)
caloric testing: nystagmus away, no (little) tonic reaction
passive eye opening: upward deviation, active resistance
no slow roving eye movements
normal tone, no active resistance to passive movements
no motor reaction to pain, arm drop: avoids hitting the face
normal EEG
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physical examination
vital signs
(airway / breathing / circulation / temp / seizures)
evidence of trauma
(monocle sign / battle sign / hematoma)
evidence of acute or chronic systemic illness?
(jaundice, anemia, cyanosis, rash, petechiae)
evidence of drug ingestion(needle marks, alcohol on breath)
nuchal rigidity(once cervical trauma excluded)
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neurological examination
GCS – consciousness + laterality
(spontaneous – verbal commands – pain)
start with A, C, or D: M2, M3, M5 (check asymmetry)
if no response: B, bilaterally! M1-5 (check asymmetry)
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neurological examination
motor responses – posturing
(spontaneous – pain) (+ tone + tendon + plantar
reflexes)
“decortication”
“decerebration”
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breathing patternmidbrain
hepatic coma
sepsis
metabolic acidosis
or Ondine’s curse
forebrain
diencephalon
metabolic encephalopathy
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brain stem reflexes
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brain stem reflexes
pupillary reflexes
metabolic coma:
long retained
after seizures:
transiently
absent
hypoxia/ischemia:
large + fixed
opiates:
pinpoint (~pons)
naloxone reverses
thalamus lesions:
complex oculomotor
disturbances
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eyelids – corneal responses
in coma: closed
after passive opening: slow and gradual closing
during seizures: often opened
alternated opening: vegetative state
unilateral ptosis: Horner syndrome, III nerve palsy
blink reflexes to light/threat: may be present in vegetative state
corneal reflex:
intact afferent (n. V) and efferent paths (n. VII and n. III - Bell’s phenomenon)
contact lenses!
neurological examination – brainstem reflexes
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neurological examination – brainstem reflexes
frontal eye field
pons
midbrain
VIVI
IIIIII
PPRFPPRF
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neurological examination – brainstem reflexes
frontal eye field
pons
midbrain
VIVI
IIIIII
PPRFPPRF
X
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neurological examination – brainstem reflexes
frontal eye field
pons
midbrain
VIVI
IIIIII
PPRFPPRF
X
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neurological examination – brainstem reflexes
frontal eye field
pons
midbrain
VIVI
IIIIII
PPRFPPRF
X
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neurological examination – brainstem reflexes
frontal eye field
pons
midbrain
VIVI
IIIIII
PPRFPPRF
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neurological examination – brainstem reflexes
frontal eye field
pons
midbrain
VIVI
IIIIII
PPRFPPRF X
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neurological examination – brainstem reflexes
frontal eye field
pons
midbrain
VIVI
IIIIII
PPRFPPRF X
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neurological examination – brainstem reflexes
frontal eye field
pons
midbrain
VIVI
IIIIII
PPRFPPRF
XMLF
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neurological examination – brainstem reflexes
frontal eye field
pons
midbrain
VIVI
IIIIII
PPRFPPRF
XMLF
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spontaneous eye movements
- often slight exophoria
- metabolic coma: often spontaneous roving movements
- conjugate lateral deviation
* seizure (ictally: away from, postictally: towards lesion)
* gaze paralysis (hemispheric: towards lesion
pons: away from lesion)
- disconjugate: brain stem or III / VI nerve lesions
- skew deviation: brain stem
- bobbing, dipping, ping/pong: different localizations
neurological examination – brainstem reflexes
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oculocephalic reflex
neurological examination – brainstem reflexes
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brainstem intact, metabolic encephalopathy
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R pontine lateral lesion
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bilateral INO/MLF lesion
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caloric responsesreserved for the examination of brain death
neurological examination – brainstem reflexes
5 min 10ml/min
when awake and trying to fixate: + nystagmus to midline
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psychogenic unresponsiveness
disoriented to time, space and self, retain new information
lie with eyes closed
normal reflexes and ventilatory patterns
oculocephalic reflexes absent (due to visual fixation)
caloric testing: nystagmus away, no (little) tonic reaction
passive eye opening: upward deviation, active resistance
no slow roving eye movements
normal tone, no active resistance to passive movements
no motor reaction to pain, arm drop: avoids hitting the face
normal EEG
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causes of coma in children
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causes of coma in children
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causes of coma – in general
requires dysfunction of:
Ascending (Reticular)
Arousal (or Activating)
System ([A]RAS) upper brain stem
diencephalon
or
both hemispheres
structural lesions
compressive
destructive
diffuse/metabolic causes
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structural causes of coma
both hemispheresanox./isch., trauma, hydrocephalus, meningitis, SAH, ICP, central herniation
one hemisphere – secundary brain stem/ARAS involvementmass lesion, subfalcine or uncal herniation
posterior fossa – brainstem compressionmass lesion, tonsillar herniation
secundary hydrocephalus
intrinsic brainstem lesionspace-occupying
infarct, demyelination
focal signs !
brain stem signs !
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herniation syndromes – pressure gradient
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herniation syndromes – pressure gradient
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herniation syndromes
symmetry asymmetry
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herniation syndromes
Edlow et al. Lancet 2014
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herniation syndromes
central tentorial
herniation
diencephalic stage
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central tentorial
herniation
midbrain/pons stage
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3 year old boy
E1M3V2, small reactive pupils
CR +/+ OCR +/+, posture: flexion arms/hands, extended
legs, bilateral Babinski sign
diencephalic stage, central transtentorial herniation
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herniation syndromes
uncal
herniation
early III nerve stage
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uncal
herniation
late III nerve stage
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4 months old boy
vomited, became drowsy, opisthotonus
E1M4V1
dilated non-reactive R pupil
R: withdraws, L: extends
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tonsillar herniation
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tonsillar herniation
after 2 days:
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diffuse/metabolic causes of coma
Stevens et al. Crit Care Med 2006
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diffuse/metabolic causes of coma
Stevens et al. Crit Care Med 2006
false localizing signs may occur in metabolic coma
non-reactive pupils
focal deficits in hypoglycaemic coma
bilateral Babinski’s sign
symmetrical posturing
+
nonconvulsive generalized status epilepticus
immune-mediated syndromes (NMDA, Hashimoto)
inborn errors of metabolism
genetic causes (FHM)
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ancillary investigations in coma
blood glucose, electrolytes, urea, ammonia, lactate
arterial blood gases
blood cultures
metabolic screen
tox screen
CT
lumbar puncture
EEG
MRI, CTV, MRV, MRA
specific investigations: endocrine, auto-immune antibodies
DNA, cultures, PCR
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glucose, electrolytes, urea, ammonia, lactate, blood gas
urgent CT
lumbar puncture, blood cultures (meningitis suspected: treat!)
toxicology screen
EEG (NCSE? focal / metabolic causes?)
metabolic screen
MRI/MRA (brain stem signs: posterior circulation AIS? pont. myel.?
ADEM?)
MRV/CTV (signs of high ICP: sinovenous thrombosis?)
auto-immune antibodies, genes......
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diagnostic approach – summary
is the patient in a coma?
GCS (exclude aphasia/anarthria/tetraplegia)
coma mimic (vegetative or minimally conscious state)
psychogenic unresponsiveness
structural cause?
bilateral or focal
sequence of events – herniation?
focal signs / brain stem signs
diffuse or metabolic cause?
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Edlow et al. Lancet 2014
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literature
Plum and Posner’s diagnosis of stupor and coma4th edition, Oxfor University Press 2007
Sejersen T, Wang CH (eds). Acute pediatric NeurologySpringer 2014, ISBN 978-0-85729-490-6
Edlow JA et al. Diagnosis of reversible causes of comaLancet 2014;384:2064-2076
Kirkham FJ. Non-traumatic coma in childrenArch Dis Child 2001;85:303-312
Ashwal S. Pediatric vegetitive stateNeurorehabilitation 2004;19:349-360
Stevens RD et al. Approach to the comatose patientCrit Care Med 2006;34:31-41
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