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

Decreased mental status and central Demyelinating emergencies

Decreased mental status and central Demyelinating emergenciesBradley OstermanPediatric neurology R4Summer lecture series 2012PlanIntroductionConsciousness & impaired consciousnessComaEtiologies of comaDD of coma-like statesDemyelinating emergenciesADEMNMODemyelinating w/u of a CIE

IntroductionPatient brought into the ER unconscious

First reflexThe ABC

But dont forget about the D !DisabilityConsciousnessWhat does it mean to a neurologist?Product of two basic brain functions(1) Arousal wakefulnessARAS (ascending reticular activating system)(2) Awareness encompasses multiple functions - attention, memory & executive functionImpaired consciousness can result from derangement in :(1) arousal, (2) awareness, (3) or both, in varying degressARAS a network of neural tissues originating in the tegmentum of the pons and midbrain & projecting to the hyothalamus, thalamus and cerebral cortex4Impaired consciousnessThere is a wide spectrum

Avoid confusing, imprecise terms such as :Somnolence, stupor, obtundation, lethargy

Use specific statements to describe the patients LOC:Ex. patient responds to painful sternal rub by grimacing & moving R armGlascow coma scale

Has its limitations. Originally designed for TBI

GCS 12-14 mild alteration of consciousnessGCS 9-11 moderateGCS 8 and below severe

6ComaThe term coma is actually a specific state of consciousness characterized by :A complete absence of both (1) arousal & (2) awarenessFor 1 hour Absence of normal sleep-wake cycleNo spontaneous or stimulus-induced eye-openingNo purposeful mvts

Be familiar with the DD of coma-like conditionsEtiologies of coma(1) Direct insult to the CNSStructural causesSupratentorial (ie. Brain tumor, hemorrage, encephalitis)Subtentorial (ie. Midbrain infarction, acute demyelination of brainstem )Seizures (NC SE)Hydrocephalus/raised ICPTraumatic brain injury (TBI)

(2) Metabolic/toxic causes

DD of coma-like conditionsConditionArousalAwarenessMotor functionBreathingSleep-WakeLocked-in syndromeNNNo (exc. eye blinking & vertical eye mvt)NNAkinetic mutismNpartially presentdecreased mvtsNNMinimally conscious stateNpartially present (minimally)nonpurposeful (or very limited)NNVegetative stateNabsentnonpurposefulNNComaabsentabsentnonpurposefulNabsentBrain deathabsentabsentNo (exc. spinal reflexes)absentabsentLocked-in syndromeOccurs with brainstem injuries sparing the midbrainPatients have normal (1) arousal & mostly intact (2) awareness, but have a severely limited ability to communicatedue to paralysis of voluntary muscles & anarthriaCan be seen with :Pontine gliomaBasilar artery occlusionProfound neuromuscular dysfunction (GBS, SMA, botulism, organophosphate toxicity)Trauma

Akinetic mutismState 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)TBIHydrocephalusCNS infections

Minimally conscious stateRelatively new term

(1) Arousal present & (2) minimally present awareness

Patients capable of reproducible & purposeful (albeit very limited) motor movements or affective behaviourEx. following simple commands, reaching accurately for an object, crying or smiling in response to emotional stimuli

Vegetative stateArousal presentAwareness absent (self & environment)No voluntary or purposeful mvtsPreserved respiratory function & sleep-wake cyclesIncluding periods of spontaneous eye-openingIntact brainstem & hypothalamic functionConsidered permanent if lasts :> 12 months after TBI or > 3 months after non-traumatic brain injuryBrain deathComplete & irreversible loss of (1) brain and (2) brainstem function, characterized by :Loss of consciousnessLoss of cranial nerve functionLoss of motor functionLoss of breathing activityLoss of sleep-wake cycle

Specific criteria complete brain death examDD of coma-like conditionsConditionArousalAwarenessMotor functionBreathingSleep-WakeLocked-in syndromeNNNo (exc. eye blinking & vertical eye mvt)NNAkinetic mutismNpartially presentdecreased mvtsNNMinimally conscious stateNpartially presentnonpurposeful (or very limited)NNVegetative stateNabsentnonpurposefulNNComaabsentabsentnonpurposefulNabsentBrain deathabsentabsentNo (exc. spinal reflexes)absentabsentDemyelinating EmergenciesADEM (acute disseminated encephalomyelitis)An autoimmune-mediated inflammatory episode involving the CNSUsually 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 monthsDescribed equally in all racial, ethnic groups and genders (unlike MS)Mortality rate 5%Full recovery 50-75%Average time to recovery 6 monthsADEM clinical presentationAbrupt onsetWith rapid progression maximum deficits < 1 week (average 4.5 days)EncephalopathyMild to severe alteration in mental status (irritability to coma)Convulsive seizures (25%)Focal neuro aNWeakness, ataxia, visual aN, sensory aN, cranial nerve aN, sphincter dysfunstionConstitutional sxH/A, No/Vo, malaiseADEM physical examAlteration in mental status (>90%)Cranial nerve aN (35-50%) (w/ possible brainstem dysfunction)Meningeal signs (20%)Weakness (50-75%)Hemiparesis, diaparetic, generalizedLong tract signs (80%)Clonus, increased DTR, upgoing toesAtaxia (35-60%)Sensory deficits (15-20%) (w/ possible sensory level)

20ADEM MRIADEM axial T2-weighted imagesLarge, T2-hyperintense, patchy, poorly circumscribed

21ADEM MRIADEM sagittal cervical spine & axial T2-weighted brain imagesLarge, T2-hyperintense, patchy, poorly circumscribed

22ADEM MRITypically involves the gray-white junctionADC map consistent w/ vasogenic edemaGado-enhancing (30-90%)Compared to MSPeriventricular lesions (30-45%) and corpus callosum lesions (20-25%) are less common in ADEMAdditional lesions found in: optic nerves, basal ganglia (30-40%), the thalamus (30-40%), the brainstem (45-55%), the cerebellum (30-40%) & spinal cord

23ADEM InvestigationsLumbar puncture leukocytosis (80%); N/elevated OP, protein often > 1.0; N glucose; absent OCBCSF cultures, serologies typically negativeEEG diffuse slowing

Work-up for other Demyelinating disorders & Mimics24ADEM treatment1st line tx high-dose IV corticosteroidsMethylprednisolone (Solumedrol) 30 mg/kg/day x 5 daysUp to max 1 G Solumedrol daily x 5 days (ie. Like in adults)Followed by 3-6 weeks of tapering prednisoneLess than 3 week taper has a higher risk of relapse

2nd line IVIG and/or plasma exchangev(PLEX)25ADEM evolution/classificationMonophasic (same acute episode)With fluctuating/subsequent sx as long as the relapse is :< 3 months of onset< 1 month following steroid tx

Recurrent ADEMRelapse with identical sx 3 months or 1 month following steroid tx

Multi-PhasicRelapse with new features and change in mental status 3 months or 1 month of steroid txMRI must show new areas of involvement & partial resolution of previous lesions26ADEM flowchart

27NMO neuromyelitis opticaNMO or Devics diseaseIdiopathic, inflammatory, necrotizing demyelination of the CNSSimultaneous or successive involvement of : optic nerves & spinal cordMediated by anti-NMO-IgG antibodiesUnlike MS, not T-cell mediatedTargets the protein aquaporin 4 in the cell membrane of astrocytesImpaired water transport across the membrane leading to inflammatory demyelination

28NMO clinical presentationVisual loss (painful & subacute)Optic neuritis (ON)Spinal cord dysfunctionTransverse myelitis (TM)weaknesssensory deficits (w/ level)sphincter dysfunction Unilateral ON (45%); simultaneous & bilateral ON (25-30%)Isolated TM (10-15%)Simultaneous ON & TM (10-15%)

ONTT w/ N MRI 25% progress to MS over 15 years (risk); 75% w/ aN MRI 29NMO Diagnostic criteria

Brain mri can show NS WM lesions (16%)30NMO treatmentHigh-dose glucocorticod therapy

IVIG/PLEX Obtain sample for anti-NMO ab prior

Immunosuppressant therapyEx. Imuran

31NMO prognosisHigh 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 incompletePermanent leg paralysis and/or blindness in > 50%

32Demyelinating (CIS) work-up at MCHSerumCBC, Glucose, Lactate, TSH, T4, vit B12, IgG index, VLCFAESR, CRP, ANA, dsDNA, APLA, ACE (vasculitic w/u)Viral serologies (HIV, VDRL, HSV)Lyme (serology +/- PCR)CSFCell count (WBC), Glucose, Protein, Lactate (on ice)OCB (w/ serum IgG index)Viral culture (enterovirus, CMV), Bacterial cultureHerpes PCR, Mycoplasma PCR

33Questions?

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