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DECREASED MENTAL STATUS AND CENTRAL DEMYELINATING EMERGENCIES
Bradley Osterman
Pediatric neurology – R4
Summer lecture series 2012
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PLAN
Introduction
Consciousness & impaired consciousness
Coma
Etiologies of coma
DD of coma-like states
Demyelinating emergencies
ADEM
NMO
Demyelinating w/u of a CIE
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INTRODUCTION
Patient brought into the ER unconscious
First reflex
The « ABC »
But don’t forget about the « D » !
Disability
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CONSCIOUSNESS
What does it mean to a neurologist?
Product of two basic brain functions (1) Arousal – wakefulness
ARAS (ascending reticular activating system)
(2) Awareness encompasses multiple functions - attention, memory & executive function
Impaired consciousness can result from derangement in :
(1) arousal,
(2) awareness,
(3) or both, in varying degress
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IMPAIRED CONSCIOUSNESS
There is a wide spectrum
Avoid confusing, imprecise terms such as : Somnolence, stupor, obtundation, lethargy
Use specific statements to describe the patient’s LOC: Ex. « patient responds to painful sternal rub by grimacing & moving R arm »
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GLASCOW COMA SCALE
Has its limitations.
Originally designed for TBI
GCS 12-14 – mild alteration of
consciousness
GCS 9-11 – moderate
GCS 8 and below – severe
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COMA
The term « coma » is actually a specific state of consciousness
characterized by : A complete absence of both (1) arousal & (2) awareness
For ≥ 1 hour
Absence of normal sleep-wake cycle
No spontaneous or stimulus-induced eye-opening
No purposeful mvts
Be familiar with the DD of coma-like conditions
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ETIOLOGIES OF COMA
(1) Direct insult to the CNS
Structural causes
Supratentorial (ie. Brain tumor, hemorrage, encephalitis)
Subtentorial (ie. Midbrain infarction, acute demyelination of brainstem )
Seizures (NC SE)
Hydrocephalus/raised ICP
Traumatic brain injury (TBI)
(2) Metabolic/toxic causes
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DD OF COMA-LIKE CONDITIONS
Condition Arousal Awareness Motor function Breathing Sleep-Wake
Locked-in syndrome N N
No (exc. eye blinking & vertical
eye mvt) N N
Akinetic mutism N partially present decreased mvts N N
Minimally conscious state N
partially present (minimally)
nonpurposeful (or very limited) N N
Vegetative state N absent nonpurposeful N N
Coma absent absent nonpurposeful N absent
Brain death absent absentNo (exc. spinal
reflexes) absent absent
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LOCKED-IN SYNDROME
Occurs with brainstem injuries sparing the midbrain
Patients have normal (1) arousal & mostly intact (2) awareness,
but have a severely limited ability to communicate due to paralysis of voluntary muscles & anarthria
Can be seen with : Pontine glioma
Basilar artery occlusion
Profound neuromuscular dysfunction (GBS, SMA, botulism, organophosphate toxicity)
Trauma
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AKINETIC MUTISM
State of profound apathy with partially intact awareness, and paucity and
slowness of voluntary mvts (1) Normal arousal, and (2) mostly intact awareness (normal attentive pursuit)
On the verge of initiating speech or motor activity (but never happens)
Seen with bilateral injuries to the midbrain, basal diencephalon, or inferior
frontal lobes, occuring in : Tumors or tumor resection (mainly posterior fossa)
TBI
Hydrocephalus
CNS infections
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MINIMALLY CONSCIOUS STATE
Relatively new term
(1) Arousal present & (2) minimally present awareness
Patients capable of reproducible & purposeful (albeit very limited) motor
movements or affective behaviour Ex. following simple commands,
reaching accurately for an object,
crying or smiling in response to emotional stimuli
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VEGETATIVE STATE
Arousal present
Awareness absent (self & environment)
No voluntary or purposeful mvts
Preserved respiratory function & sleep-wake cycles
Including periods of spontaneous eye-opening
Intact brainstem & hypothalamic function
Considered permanent if lasts :
> 12 months after TBI or
> 3 months after non-traumatic brain injury
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BRAIN DEATH
Complete & irreversible loss of (1) brain and (2) brainstem
function, characterized by :
Loss of consciousness
Loss of cranial nerve function
Loss of motor function
Loss of breathing activity
Loss of sleep-wake cycle
Specific criteria – complete brain death exam
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DD OF COMA-LIKE CONDITIONS
Condition Arousal Awareness Motor function Breathing Sleep-Wake
Locked-in syndrome N N
No (exc. eye blinking & vertical
eye mvt) N N
Akinetic mutism N partially present decreased mvts N N
Minimally conscious state N partially present
nonpurposeful (or very limited) N N
Vegetative state N absent nonpurposeful N N
Coma absent absent nonpurposeful N absent
Brain death absent absentNo (exc. spinal
reflexes) absent absent
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DEMYELINATING EMERGENCIES
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ADEM (ACUTE DISSEMINATED ENCEPHALOMYELITIS)
An autoimmune-mediated inflammatory episode involving the CNS Usually monophasic (5-20% cases are multi-phasic)
Usually following an infection (or vaccination; 3-6% of cases) Up to 4 weeks prior to sx (gen. 1-20 days)
Children > adults (peak ages 5-8 y/o) Incidence 8/1,000,000; seasonal increase in spring & winter months
Described equally in all racial, ethnic groups and genders (unlike MS)
Mortality rate – 5% Full recovery – 50-75%
Average time to recovery – 6 months
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ADEM – CLINICAL PRESENTATION
Abrupt onset
With rapid progression – maximum deficits < 1 week (average 4.5 days)
Encephalopathy
Mild to severe alteration in mental status (irritability to coma)
Convulsive seizures (25%)
Focal neuro aN
Weakness, ataxia, visual aN, sensory aN, cranial nerve aN, sphincter dysfunstion
Constitutional sx
H/A, No/Vo, malaise
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ADEM – PHYSICAL EXAM
Alteration in mental status (>90%)
Cranial nerve aN (35-50%) (w/ possible brainstem dysfunction)
Meningeal signs (20%)
Weakness (50-75%)
Hemiparesis, diaparetic, generalized
Long tract signs (80%)
Clonus, increased DTR, upgoing toes
Ataxia (35-60%)
Sensory deficits (15-20%) (w/ possible sensory level)
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ADEM – MRI
ADEM – axial T2-weighted images
Large, T2-hyperintense, patchy, poorly circumscribed
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ADEM – MRI
ADEM – sagittal cervical spine & axial T2-weighted brain images
Large, T2-hyperintense, patchy, poorly circumscribed
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ADEM – MRI
Typically involves the gray-white junction
ADC map – consistent w/ vasogenic edema
Gado-enhancing (30-90%)
Compared to MS
Periventricular lesions (30-45%) and corpus callosum
lesions (20-25%) are less common in ADEM
Additional lesions found in:
optic nerves, basal ganglia (30-40%), the thalamus (30-
40%), the brainstem (45-55%), the cerebellum (30-40%) &
spinal cord
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ADEM – INVESTIGATIONS
Lumbar puncture
leukocytosis (80%); N/elevated OP, protein often > 1.0; N glucose; absent OCB
CSF cultures, serologies typically negative
EEG – diffuse slowing
Work-up for other Demyelinating disorders & Mimics
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ADEM – TREATMENT
1st line tx – high-dose IV corticosteroids
Methylprednisolone (Solumedrol) – 30 mg/kg/day x 5 days
Up to max 1 G Solumedrol daily x 5 days (ie. Like in adults)
Followed by 3-6 weeks of tapering prednisone
Less than 3 week taper has a higher risk of relapse
2nd line – IVIG
and/or plasma exchangev(PLEX)
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ADEM – EVOLUTION/CLASSIFICATION Monophasic (same acute episode)
With fluctuating/subsequent sx as long as the relapse is : < 3 months of onset < 1 month following steroid tx
Recurrent ADEM Relapse with identical sx ≥ 3 months or ≥ 1 month following steroid tx
Multi-Phasic Relapse with new features and change in mental status ≥ 3 months or ≥ 1 month of steroid tx MRI must show new areas of involvement & partial resolution of previous lesions
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ADEM – FLOWCHART
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NMO – NEUROMYELITIS OPTICA
NMO or Devic’s disease
Idiopathic, inflammatory, necrotizing demyelination of the CNS
Simultaneous or successive involvement of :
optic nerves & spinal cord
Mediated by anti-NMO-IgG antibodies
Unlike MS, not T-cell mediated
Targets the protein aquaporin 4 in the cell membrane of astrocytes
Impaired water transport across the membrane leading to inflammatory demyelination
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NMO – CLINICAL PRESENTATION
Visual loss (painful & subacute)
Optic neuritis (ON)
Spinal cord dysfunction
Transverse myelitis (TM)
weakness
sensory deficits (w/ level)
sphincter dysfunction
Unilateral ON (45%); simultaneous & bilateral ON (25-30%)
Isolated TM (10-15%)
Simultaneous ON & TM (10-15%)
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NMO – DIAGNOSTIC CRITERIA
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NMO – TREATMENT
High-dose glucocorticod therapy
IVIG/PLEX
Obtain sample for anti-NMO ab prior
Immunosuppressant therapy
Ex. Imuran
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NMO – PROGNOSIS
High rate of recurrence (> 90%)
Relapse within first year (50%), and within 5 years (90%)
Relapse interval generally < 3 months
Deficits (ON/TM) tend to be severe & recovery incomplete
Permanent leg paralysis and/or blindness in > 50%
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DEMYELINATING (CIS) WORK-UP – AT MCH
Serum
CBC, Glucose, Lactate, TSH, T4, vit B12, IgG index, VLCFA
ESR, CRP, ANA, dsDNA, APLA, ACE (vasculitic w/u)
Viral serologies (HIV, VDRL, HSV)
Lyme (serology +/- PCR)
CSF
Cell count (WBC), Glucose, Protein, Lactate (on ice)
OCB (w/ serum IgG index)
Viral culture (enterovirus, CMV), Bacterial culture
Herpes PCR, Mycoplasma PCR
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QUESTIONS?