Neurology revision Neurology revision

11 March 201411 March 2014

Antony ThomasAntony Thomas

Consultant NeurologistConsultant Neurologist

HistoryHistory

General ApproachGeneral Approach– Is this neurological?Is this neurological?– If so where in the neuroaxis: central or If so where in the neuroaxis: central or

peripheral?peripheral?– Above or below foramen magnum?Above or below foramen magnum?– Above or below the tentorium?Above or below the tentorium?– What might be the nature of the problem?What might be the nature of the problem?– Differential diagnosisDifferential diagnosis– Handedness Handedness

History TakingVital importanceGood listenerFocused Lateral thinkingAnatomical and Pathological Diagnosis Age / Occupation / HandednessTemporal features of a symptom : 1.Onset 2.Progression 3.Duration 4.Recovery 5.FrequencyWeakness of one side of the bodyNumbness of hands and legs

Direct QuestionsPain

Headache

Facial, neck, back and limb pain

Disturbance of consciousness

Blackouts, faints, fits

Altered sleep pattern

Cognitive & affective dysfunction

Memory, language

Depression, irritability

Direct QuestionsCranial Nerve symptoms

Loss of vision, blurring, diplopia

Hearing, sense of taste and smell

Facial muscle weakness

Vertigo, dizziness, giddiness

Bulbar muscles ( swallowing , articulation of speech)

QuestionsLimb symptoms

Difficulty lifting , gripping, fine finger movements, clumsiness

Gait disorder, leg weakness, stiffness, balance problems

Loss of sensation, altered sensation, numbness

Involuntary movements, incordination

Bladder, bowel, sexual dysfunction

Initial ImpressionGait

Facial Expression

Handshake

Speech

Arm swinging

Positive symptom and negative symptom

HistoryHistory

Speed of onsetSpeed of onset– InstantaneousInstantaneous– MinutesMinutes– HoursHours– DaysDays– Weeks/MonthsWeeks/Months– Months/YearsMonths/Years

Anatomically lesions localised to Where is the lesion?

Meninges (Venus Sinus) Spinal fluidCortexSubcortex (Basal Ganglia, Thalamus, Hypothalamus)Brain Stem (Midbrain, Pons & Medulla oblongata)CerebellumForamen magnum (Craniocervical Junction)Cranial NervesSpinal Cord (ends at Lower border L1)Anterior Horn Cell DisorderNerve root (Dorsal & Ventral)PlexusPeripheral Nerve Neuromuscular JunctionMuscle

HistoryHistory

InstantaneousInstantaneous– ““Electrical events”Electrical events”

EpilepsyEpilepsy

Myoclonic jerksMyoclonic jerks

Neuralgic painNeuralgic pain

– Vascular eventsVascular eventsSubarachnoid H’age (SAH)Subarachnoid H’age (SAH)

Intracerebral H’ageIntracerebral H’age

HistoryHistory

Maximal over minutesMaximal over minutesVascular eventsVascular events

Migranous eventsMigranous events

Maximal over HoursMaximal over HoursInfective eventsInfective events

Inflammatory: GBS, MyelitisInflammatory: GBS, Myelitis

Vascular: strokeVascular: stroke

Vasculitic:GCA, Mononeuritis multiplexVasculitic:GCA, Mononeuritis multiplex

HistoryHistory

Maximal over DaysMaximal over Days

Intoxication: IatrogenicIntoxication: Iatrogenic

Infection: HSV encephalitis, Infection: HSV encephalitis, MeningitisMeningitis

Inflammation: MS, GBSInflammation: MS, GBS

HistoryHistory

Maximal over weeks/monthsMaximal over weeks/months

Brain tumoursBrain tumours

Expanding unruptured aneurysmsExpanding unruptured aneurysms

Degenerative: CJDDegenerative: CJD

Some polyneuropathiesSome polyneuropathies

Some myopathies: Steriod inducedSome myopathies: Steriod induced

HistoryHistory

Maximal over months/years Maximal over months/years – NeurodegenerativeNeurodegenerative

Parkinson’s (PD)Parkinson’s (PD)

Alzheimer’sAlzheimer’s

Cerebellar ataxiasCerebellar ataxias

Motor Neurone Disease (MND)Motor Neurone Disease (MND)

Most NeuropathiesMost Neuropathies

Most myopathiesMost myopathies

Single or multipleSingle or multiple

MigraineMigraine

EpilepsyEpilepsy

TIATIA

SyncopeSyncope

Trigeminal NeuralgiaTrigeminal Neuralgia

Multiple Sclerosis (Relapsing Remitting) Multiple Sclerosis (Relapsing Remitting)

Documenting HxDocumenting Hx

No different in NeurologyNo different in Neurology Presenting complaint (PC)Presenting complaint (PC) Hx of the PCHx of the PC Past Medical: Injuries, Psychiatric, Op, Past Medical: Injuries, Psychiatric, Op,

ArteriopathArteriopath Medication: Recreational useMedication: Recreational use Social/Employment: Driver, Smoker / AlcoholSocial/Employment: Driver, Smoker / Alcohol Family Hx: Stroke, MND, PD, Dementias, Family Hx: Stroke, MND, PD, Dementias,

Tremors, DM, MSTremors, DM, MS

Common presenting complaints in Common presenting complaints in NeurologyNeurology

Funny turnsFunny turnsSeizures and LOCSeizures and LOCHeadachesHeadachesDizziness & VertigoDizziness & VertigoConfusionConfusionWeakness of arms / legsWeakness of arms / legsAbnormal movementsAbnormal movementsLoss of balanceLoss of balanceWalking difficultiesWalking difficultiesNumbness and tingling, pins and needlesNumbness and tingling, pins and needlesVisual failure, diplopiaVisual failure, diplopia

HPCHPC

As much detail as possibleAs much detail as possible

When and whereWhen and where

Previous episodesPrevious episodes

Witness accountsWitness accounts

Exacerbating and relieving factorsExacerbating and relieving factors

Treatments and changes to RxTreatments and changes to Rx

Associated symptomsAssociated symptoms

Recurrent attacks of LOCRecurrent attacks of LOC

Postures and manoeuvresPostures and manoeuvres

Drugs/AlcoholDrugs/Alcohol

PalpitationsPalpitations

Prodromal featuresProdromal features

Post-ictal confusional statesPost-ictal confusional states

Eye witness accountEye witness account

TreatmentsTreatments

ExaminationExamination

Higher Mental FunctionsHigher Mental FunctionsCranial NervesCranial NervesMotorMotorSensorySensoryCerebellarCerebellarGaitGaitSphinctersSphinctersSkull and SpineSkull and SpineNeck stiffnessNeck stiffnessNeurocutaneous markersNeurocutaneous markersGeneral examinationGeneral examinationOther systemsOther systems

Higher Mental FunctionsHigher Mental Functions

Appearance and behaviourAppearance and behaviourMood and AffectMood and AffectThought form and contentThought form and contentSensorium (GCS) and CognitionSensorium (GCS) and Cognition– AwarenessAwareness– SleepSleep– DrowsinessDrowsiness– StuporStupor– ComaComa

Perceptual disturbances: HallucinationsPerceptual disturbances: HallucinationsMMSEMMSESpeech and LanguageSpeech and Language

Cranial nervesCranial nerves1. Olfactory1. Olfactory

2. Optic2. Optic

3. Occulomotor3. Occulomotor

4. Trochlear4. Trochlear

5. Trigeminal5. Trigeminal

6. Abducens6. Abducens

SmellSmell

VisionVision

Elevate, depress and Elevate, depress and adduct, pup: constrictadduct, pup: constrict

Depression, adduction, Depression, adduction, intorsionintorsion

Face sensation, muscles Face sensation, muscles of masticationof mastication

AbductionAbduction

Cranial NervesCranial Nerves

7. Facial7. Facial

8. Vestibulocochlear8. Vestibulocochlear9. Glossopharyngeal9. Glossopharyngeal

10. Vagus10. Vagus

11. Spinal Accessory 11. Spinal Accessory

12. Hypoglossal12. Hypoglossal

Muscles of facial expression, Muscles of facial expression, Anterior 2/3 tongue tasteAnterior 2/3 tongue tasteHearing and balanceHearing and balanceTaste posterior 1/3 tongue, gag Taste posterior 1/3 tongue, gag reflexreflexGag reflex, motor to soft palate, Gag reflex, motor to soft palate, pharynx, larynx. Autonomic fibres pharynx, larynx. Autonomic fibres to oesophagus, stomach, small to oesophagus, stomach, small intestine, heart, trachea, visceraintestine, heart, trachea, viscera

Sternocleidomastoid, TrapeziusSternocleidomastoid, Trapezius

Motor control tongueMotor control tongue

Motor SystemMotor System

Bulk and nutritionBulk and nutrition

WastingWasting

ToneTone

PowerPower

ReflexesReflexes

Babinski Babinski

DTRDTR

00 AbsentAbsent

+/- Present with reinforcement+/- Present with reinforcement

+ Reduced+ Reduced

2+ Normal2+ Normal

3+ Increased Brisk Exaggerated3+ Increased Brisk Exaggerated

4+ Pathologically brisk with clonus4+ Pathologically brisk with clonus

Sensory SystemSensory System

Side to sideSide to sideProximal to distalProximal to distalPin prickPin prickTouchTouchVibrationVibrationJoint position senseJoint position senseRomberg’sRomberg’sCortical sensationCortical sensation

Cerebellar signsCerebellar signs

Intention TremorsIntention TremorsTitubationTitubationAtaxiaAtaxiaTruncal ataxiaTruncal ataxiaDysdiachokinesis Dysdiachokinesis Slurred speech and dysarthria Slurred speech and dysarthria Hypotonia Hypotonia Past pointing DysmetriaPast pointing DysmetriaNystagmus Nystagmus Tandem walking heel-toe walkingTandem walking heel-toe walkingRebound phenomenonRebound phenomenonPendular knee jerkPendular knee jerkHyporeflexiaHyporeflexiaFinger nose / Heel shin co-ordination (watch out for weakness)Finger nose / Heel shin co-ordination (watch out for weakness)

GaitGait

NormalNormalHemiplegic / CircumductionHemiplegic / CircumductionParkinsonianParkinsonianCerebellarCerebellarHigh stepping/ steppage or stampingHigh stepping/ steppage or stampingWaddling / TrendelenburgWaddling / TrendelenburgSpasticSpasticScissor gaitScissor gaitAntalgicAntalgicFunctionalFunctional

Diagnostic testsDiagnostic tests

CSF analysis (LP)CSF analysis (LP)EEGEEGEvoked PotentialsEvoked PotentialsEMGEMGNCSNCSCTCTMRMRDATDATSPECTSPECTBloodsBloods

Typical Cerebrospinal Fluid Findings in Various Types of Meningitis Typical Cerebrospinal Fluid Findings in Various Types of Meningitis

Test Test Bacterial Bacterial Viral Viral Fungal Fungal TubercularTubercular

Opening pressureOpening pressure Elevated Elevated Usually normal Usually normal Variable Variable Variable Variable

WBCWBC ≥≥1,000 per mm3 1,000 per mm3 <100 per mm3 <100 per mm3 Variable Variable Variable Variable

Cell differentialCell differential Predominance of Predominance of Predominance of Predominance of Predominance Predominance Predominance Predominance

PMNs* PMNs* lymphocyteslymphocytes†† of lymphocytes of lymphocytes of lymphocytes of lymphocytes

Protein Protein Mild to marked Mild to marked Normal to elevated Normal to elevated Elevated Elevated Elevated Elevated elevation elevation

CSF-to-serum glucoseCSF-to-serum glucose Normal to marked Normal to marked Usually normal Usually normal Low Low Low Low

ratio decreaseratio decrease

CSF = cerebrospinal fluid; PMNs = polymorphonucleocytes.CSF = cerebrospinal fluid; PMNs = polymorphonucleocytes. *—Lymphocytosis present 10 percent of the time.*—Lymphocytosis present 10 percent of the time. †—†—PMNs may predominate early in the course. PMNs may predominate early in the course.

EEGEEG

EncephalitisEncephalitis

Seizure DisorderSeizure Disorder

EncephalopathyEncephalopathy

Anoxic brain injuryAnoxic brain injury

Degenerative conditions (CJD)Degenerative conditions (CJD)

Trimodality EPsTrimodality EPs

Visual Evoked ResponsesVisual Evoked Responses

Brain Stem Auditory Evoked ResponseBrain Stem Auditory Evoked Response

Somatosensory EPSomatosensory EP

EMG/NCSEMG/NCS

Muscle vs Motor NeuronMuscle vs Motor Neuron

Demyelinative vs AxonalDemyelinative vs Axonal

Nerve root vs PlexopathyNerve root vs Plexopathy

Localisation of mononeuropathyLocalisation of mononeuropathy

NMJ disorders: MG, LEMSNMJ disorders: MG, LEMS

Entrapment NeuropathyEntrapment Neuropathy

NeuropathyNeuropathy

DemyelinatingDemyelinating– Slowed conductionSlowed conduction– Preserved amplitudePreserved amplitude

AxonalAxonal– Reduced amplitudeReduced amplitude– Normal NCVNormal NCV

NeuroradiologyNeuroradiology

CT Head +/- contrastCT Head +/- contrast

MRI (MRA, MRV)MRI (MRA, MRV)

DWI (acute stroke)DWI (acute stroke)

PWIPWI

FLAIRFLAIR

MR AngiogramMR Angiogram

PET/SPECTPET/SPECT

CortexCortex

Cortical AreasCortical Areas

Brain blood supplyBrain blood supply

CirculationCirculation

Brainstem supplyBrainstem supply

Spinal cordSpinal cord

Cord blood supplyCord blood supply

Neurological EmergenciesNeurological Emergencies

Status EpilepticusStatus EpilepticusComaComaTraumatic Brain Injury (TBI)Traumatic Brain Injury (TBI)Acute StrokeAcute StrokeInfections (Meningitis)Infections (Meningitis)Subarachnoid HaemorrhageSubarachnoid HaemorrhageRaised intracranial pressure Raised intracranial pressure Herniation HerniationAcute Spinal cord compressionAcute Spinal cord compressionAcute Neuromuscular respiratory paralysisAcute Neuromuscular respiratory paralysisAcute Visual lossAcute Visual lossDeliriumDelirium

Clinical scenarioClinical scenario

35 years old lady35 years old lady

2/7 ago started with pins and needles in 2/7 ago started with pins and needles in feet followed by difficulty walking then in feet followed by difficulty walking then in the last 24 hours unable to hold a cup in the last 24 hours unable to hold a cup in her hands and could not get out of the bedher hands and could not get out of the bed

Past: Had diarrhoeal illness2 weeks ago.Past: Had diarrhoeal illness2 weeks ago.

O/E:-O/E:-

O/EO/E

HypotoniaHypotonia

Faccid weaknessFaccid weakness

AreflexiaAreflexia

Bilateral Bell’s palsyBilateral Bell’s palsy

No UMN signsNo UMN signs

Glove and stocking sensory disturbanceGlove and stocking sensory disturbance

Diagnosis ??Diagnosis ??

GBSGBS

HistoryHistoryExaminationExamination– Flaccid weaknessFlaccid weakness– HypotoniaHypotonia– HyporeflexiaHyporeflexia– Cranial nerves involvementCranial nerves involvement– Respiratory muscle involvementRespiratory muscle involvement– Autonomic involvementAutonomic involvement– Sensory disturbanceSensory disturbance– No UMN signsNo UMN signs

GBSGBS

Mortality rate 3 to 5 %Mortality rate 3 to 5 %Symmetric rapidly progressive, ascending, flaccid Symmetric rapidly progressive, ascending, flaccid paralysis from a demyelinating poly radiculoneuropathyparalysis from a demyelinating poly radiculoneuropathyPost infective, post inflammatoryPost infective, post inflammatory10% starts in ULs10% starts in ULsProgresses over the initial days up to 4 weeksProgresses over the initial days up to 4 weeksPlateaux and then improves afterwardsPlateaux and then improves afterwardsProximal weaknessProximal weaknessBells palsy in 50%Bells palsy in 50%Prior infection GIT/RespPrior infection GIT/Resp

Diagnosis of GBSDiagnosis of GBS

Classical history & findingsClassical history & findingsNeurophysiology: Slowing of nerve conductionNeurophysiology: Slowing of nerve conductionSerology: Campylobactor, CMV, EBV, HSV, Serology: Campylobactor, CMV, EBV, HSV, MycoplasmaMycoplasmaAntibodies: Anti GM1, Anti GQ1bAntibodies: Anti GM1, Anti GQ1bCSF analysis: High protein with normal cells CSF analysis: High protein with normal cells (Albumino-cytological dissociation)(Albumino-cytological dissociation)(? Neuro-imaging)(? Neuro-imaging)Papilledema in GBSPapilledema in GBS

TreatmentTreatment

Admit and observe Admit and observe HDU/ITU HDU/ITU

Monitor FVCMonitor FVC

Artificial ventilation in 23% patientsArtificial ventilation in 23% patients

Mortality from cardiac causes and Mortality from cardiac causes and respiratory infectionsrespiratory infections

IVIGIVIG

Plasma exchangePlasma exchange

GBS - IVIGGBS - IVIG

Easy administrationEasy administration

Safety profileSafety profile

0.4 g/kg/day for 5 days0.4 g/kg/day for 5 days

IVIG vs PlasmapheresisIVIG vs Plasmapheresis

Studies showed equal efficacyStudies showed equal efficacy

IVIG alone, Plasmapheresis alone and IVIG alone, Plasmapheresis alone and Plasmapheresis followed by IVIG : - have Plasmapheresis followed by IVIG : - have equal outcome.equal outcome.

Clinical ScenarioClinical Scenario

70 years old male70 years old maleH/o difficulty chewing foodH/o difficulty chewing foodChoking and coughing on foodChoking and coughing on foodCT normal, MR scan normalCT normal, MR scan normalSent home ? StrokeSent home ? StrokeSymptoms continued with good days and Symptoms continued with good days and

bad daysbad daysDiagnosis ??Diagnosis ??