Decreased mental status and central D emyelinating emergencies

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DECREASED MENTAL STATUS AND CENTRAL DEMYELINATING EMERGENCIES Bradley Osterman Pediatric neurology – R4 Summer lecture series 2012

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Decreased mental status and central D emyelinating emergencies. Bradley Osterman Pediatric neurology – R4 Summer lecture series 2012. Plan. Introduction C onsciousness & impaired consciousness Coma Etiologies of coma DD of coma-like states Demyelinating emergencies ADEM - PowerPoint PPT Presentation

Transcript of Decreased mental status and central D emyelinating emergencies

Page 1: Decreased  mental  status  and central  D emyelinating  emergencies

DECREASED MENTAL STATUS AND CENTRAL DEMYELINATING EMERGENCIES

Bradley OstermanPediatric neurology – R4Summer 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?