approach for peripheral neurology 31 may 2011
TRANSCRIPT
8/6/2019 Approach for Peripheral Neurology 31 May 2011
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Siwaporn Chankrachang MD
Chiang Mai University
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How to Approach Peripheral
Neurology(Nerve ,Neuromuscular junction & Muscle)
31 May 2011
Siwaporn Chankrachang
Chiang Mai University
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Over view
� Anatomy & physiology
�
Clinical manifestation
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Anatomy of peripheral nervous system
� Cell body ± Anterior horn cell
± Sensory ganglion cell
± Autonomic ganglion cell
± Cranial nerve ganglion cell
� Nerve fiber ± Axon
± Myelin
� Sensory and autonomic end organ
� Neuromuscular junction
� Muscle
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Cell body
Anterior horn cellSensory ganglion cell Autonomic ganglion cellCranial nerve ganglion cell
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�Nerve fiber ±
Axon ±Myelin
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ensory
&otors
*
Sen
sor
yFib
ers
**
Dia
met
er
(nm
)
Velo
city
(m/s)Functions
- Ia10-
20
50-
120
Motor: alpha motor
neurons
Sensory: muscle spindleafferents
- Ib10-
20
50-
120
Sensory: Golgi tendon
organs, touch, pressure
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Sensor
y andMotoe
rs*
Sen
sor
yFib
ers
**
Dia
met
er
(nm
)
Velo
city
(m/s)Functions
A- III 2-8 10-50
Motor: Small gamma
motorneurons to
intrafusal muscle fibers
A- III 1-5 3-30Sensory: small touch,
pain, temperature fibers
Motor: small
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Anatomy of peripheral nervous system
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Principal components of Peripheral nervous system
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Clinical syndrome
Brain
Nerve
Muscle
Spinal cord
Motor
Sensory
Reflex
ANS
NMJ
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weakness
Brain
Spinal cordNerve
NMJMuscle
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Peripheral nerve ±
motor, sensory, autonomic,
reflex
Root ± proximal
muscle weakness,
winging scapula
Spinal Cord ±
LMN at level ,
UMN below level ,
Suspended sensory
level
Cortex lesion ±
cortical sensation,
other cortical signs
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Clinical syndrome
Neuro
Muscular Junction
Peripheral
Nerve
Muscle
Motor
Sensory
Reflex
ANS
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Nerve NMJ Muscle
Motor
Sensory
Reflexes
ANS
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Nerve NMJ Muscle
Motor Proximal
Distal
Focal
Sensory Pain
Numbness
Reflexes Decreased /
Normal
ANS Small fiber
ANS
Neuropathy
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Nerve NMJ Muscle
Motor Proximal
Sensory Mild S/S
Reflexes Normal /
Decreased
ANS Presynaptic
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Nerve NMJ Muscle
Motor Proximal
Sensory Normal
Reflexes Normal/
Decreased
ANS Normal
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Distribution of weakness
Cranial musculature
Extremities
Axial musculature
Combination
� Symmetrical - Asymmetrical
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Nerve NMJ Muscle
Motor Proximal
Distal
Focal
Proximal Proximal
Sensory Numbness
Pain
Cramp
Mild S/S Normal
Pain
Cramp
Reflexes Decreased /
Normal
Normal /Decreased
Normal/
Decreased
ANS Small fiber
Presynaptic Normal
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Pattern of PNS lesion
� Polyneuropathy
�
Radiculopathy� Polyradiculopathy
� Plexopathy
� Mononeuropathy
� Mononeuropathy multiplex
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Nerve NMJ Muscle
Motor Proximal
Sensory Mild S/S
Reflexes Normal /
Decreased
ANS Presynaptic
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Nerve NMJ Muscle
Motor Proximal
Distal
Focal
Proximal Proximal
Sensory NumbnessPain
Cramp
Mild S/S NormalPain
Cramp
Reflexes Decreased /
Normal
Normal /Decreased
Normal/
Decreased
ANS Small fiber
Presynaptic Normal
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Nerve NMJ Muscle
Motor Proximal
Distal
Focal
Proximal Proximal
Sensory Pain /crampNumbness
Mild S/S NormalPain/cramp
Reflexes Decreased /
Normal
Normal /
Decreased
Normal/
Decreased
ANS Small fiber
ANS
Neuropathy
Presynaptic Normal
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Nerve NMJ Muscle
Motor Proximal
Distal
Focal
Proximal Proximal
Sensory PainNumbness
Mild S/S Normal
Reflexes Decreased /
Normal
Normal /
Decreased
Normal/
Decreased
ANS Small fiber
ANS
Neuropathy
Presynaptic Normal
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The neuromuscular synapseRich in ion channels and associated proteins
vulnerable to antibody-mediated attacks
Voltage-gated
Potassium
channel (VGKC)
Voltage-
gated Calcium
channel (VGCC )
Acetyl choline
receptor (AChR)
acetylcholine
Voltage-gated
Sodiumchannel
Muscle pecifickinases (MUSK
rapsyn
Blood brain barrier
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Characteristic signs in M gravis
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Test for Fatiguability
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Enhancing test
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Give diagnosis
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It¶s fun to hidehahahaaaa
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Describe Abnormal Findings
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Botulism exotoxin actionBotulism exotoxin action
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High rate stimulation
Ach quantaReleased byCa++ which was
previously blockedby toxin becomesmore important
> 10 Hz
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Repetitive nerve stimulation test 2 weeks after
30 stimulation
30 HZ
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Syndromes with Neuropathy &
Myopathy
� Hereditary
±Debrancher deficiency
±Lamin A/C mutations
± Congenital muscular dystrophy: Merosin
deficiency
±
Marinesco-Sjögren ± Mitochondrial: MNGIE Syndrome
±Tyrosinemia
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Periodic paralysis
� Symmetric weakness that reverses within 1 day
± Asymmetrical weakness may occur due to excessive utilization
± No respiratory failure
± Periodic attack is very common
± Attack may related to over exercise or high carbohydrate intake
± Normal CSF and CK
± Associated with hyperthyroidism in young oriental male
±Associated with renal tubular acidosis in north-eastern Thailand
± Positive family history may occur
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Guillain-Barré syndrome
±
Symmetric weakness ±Generalized areflexia
±Distal tingling with little sensory loss
± Extraocular weakness
± Respiratory failure
± Elevated cerebrospinal fluid (CSF) protein
± Anteccdent infection
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"Classic" Guillain-Barré syndrome
C linical features
� Onset ±Weakness: Most often
symptomatic in legs ±Pain: Low back & legs
±
Paresthesias: Distal
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"Classic" Guillain-Barré syndrome
� Cranial Nerves (70%)
±VII
� Symmetric:
Occurs early in parallel withweakness
� Asymmetric
±Occurs later in disease course
±Other weakness may be stable or improving
± Extra-ocular: Overlap with Miller-Fisher
± Tongue: Symmetric; Common (50%)
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"Classic" Guillain-Barré syndrome
� Tendon reflex loss
± Early in most (70%) but not all patients
± Progressive reduction during 1st week
± Distribution: Ankles most frequently lost;
Biceps most frequently spared
± Spared reflexes all during disease course
suggests another diagnosis
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Motor neuron diseases
Amyotrophic lateral sclerosis
± asymmetric weakness proximal or distal
± Weakness with atrophy and fasciculations
± Upper motor neuron signs ± Preservation of reflexes unless severe weakness
Spinal muscular atrophy
± Symmetrical weakness
± Associated features� Kenedy syndrome
± gynecomastia, diabetes
� Madras pattern ± Deafness
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Neuromuscular Junction Disease
� Myasthenia gravis
� Lambert-Eaton myasthenic
syndrome
� Congenital myasthenic syndrome
� Drug-induced myasthenicsyndrome
Classification of Myasthenia gravis and Congenital
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Acquired NMJ disorders Congenial and familial
Myasthenia gravis Congenital Myasthenic Syndrome
Sero Positive Pre synaptic
Anti AChR Positive CMS with episodic apnea
Anti MuSK Positive Paucity of synaptic vescicles
Drug induced (pencillamine) Simulating Lambert Eaton
MGwith Thymoma Synaptic
Neonatal MG AChE deficiency
Sero Negative Post synaptic
AChR Negative Kinetic abnormalities with
Normal, reduced or
Increased AChR MuSK,Rapsyn,Agarin,
Plectin mutations
Classification of Myasthenia gravis and Congenital
myasthenic syndrome
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Distinctive Features Myasthenia Gr avis Congenital Myasthenic
Syndr ome
Familial occurrence Extremely r are(2-5%) Common
Pattern of inheritance Mostly Autosomal recessive
Consanguinity Favours diagnosis
Age of onset Unknown below 2 years,except
tr ansient neonatal myasthenia
gr avis
Neonatal period to adulthood
S pontaneous remission Can occur Does not occur
Association of other autommune disease Favours diagnosis Not seen
Char acteristic facial a ppear ance a part
f r om ptosis
Nil Elongated face,
pr ongnathism,high arched
palate
Response to immuno modulatory
treatment
Positive Nil
Differentiating features of Myasthenia gravis and Congenital Myasthenic syndrome
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Selection of diagnostic tests based on clinical features
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Selection of diagnostic tests based on clinical features
Bedside
Test
Sensitivity,%
Edx Test
(muscle)
Sensitivity
%Immunologi
c
Sensitivity,%
Ocular Distribution
of Weakness
Edrophoni
um
60-95 SFEMG
(facial)
90 AChR-Ab 50
Ice pack
89
Oropharyn
geal.
None RNS
(limb/crania
l)
60 AChR-Ab 85
SFEMG
(limb/facial) 99 MuSK-Ab(if above
negative)
40-50
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Selection of diagnostic tests based on clinical features
Distribution
of Weakness
Bedsid
e Test
Sensitivity
,%
Edx Test
(muscle)
Sensitivity,
%
Immunol
ogic
Sensiti
vity,%
Generalized
(hyporeflexia,
autonomic
signs),suspect
LEMS
None RNS(hand)
SFEMG
(limb)
98
100
VGCC-Ab 90
Suspect
botulism
None RNS(limb) ? None
Suspect CMS None RNS(limb) ? None*
Edx,electrodiagnostic;SFEMG,singlr-fiber electromyography;AChR-Ab,acetlcholine receptor antibody;RNS,repetitive
nerve stimulation;MuSK-Ab,muscle-specific receptor tyrosine kinase antibody;MG, myasthrnia
gravis;LEMS,Lambert-Eaton myasthenic syndrome;VGCC-Ab, voltage-gated calcium channel
antibody;CMS,congenital myasthenic syndrome.
*Muscle biopsy
MGFA li i l l iti ti f th i i
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Jaretzki et al Neurology 2000Class I-Ocular
Class II-Mild general
IIa-Predominantly limb/axial muscles
IIb-Predominantly oropharyngeal/respiratory musclesClass III-Moderate general
IIIa- Predominantly limb/axial muscles
IIIb- Predominantly oropharyngeal/respiratory muscles
ClassIV- Severe generalIVa- Predominantly limb/axial musclesIVb- oropharyngeal/respiratory muscles (feeding tube)
MGFA clinical classitication of myasthenia gravis
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Myasthenia gravis: treatment recommendattions
1st Line Mestinon Mestinon
Prednisone Prednisone
Thymectomy Thymectomy
2 nd Line Azathioprine Azathioprine
Myocphenolate mofetil Cyclosporine
Cyclosporine IVIg
3 rd Line IVIg Myocphenolate mofetil
Plasmapheresis Plasmapheresis
4 th Line Tacrolimus
5 th Line Rituximab cyclophophamide
BeforeBefore 20072007
M th i i t t t d tti
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Myasthenia gravis: treatment recommendattions
1st Line Mestinon Mestinon
Prednisone Prednisone
Thymectomy Thymectomy
2 nd Line Azathioprine Azathioprine
Myocphenolate
mofetil
Cyclosporine
Cyclosporine IVIg
BeforeBefore 20072007
M th i i t t t d tti
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Myasthenia gravis: treatment recommendattions
3 rd Line IVIg Myocphenolate mofetil
Plasmapheresis Plasmapheresis
4 th Line Tacrolimus
5 th Line Rituximab cyclophophamide
BeforeBefore 20072007
MGF A : M gravis FoundationMGF A : M gravis Foundation of Americaof America
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History
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Pain testing (1)
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Sensory testing (2)
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Reflexes
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Q uestion
� Where is the lesion?
1.Nerve2.Neuromuscular lesion
3.Muscle
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Syndrome in peripheral nerve
Weakness
Muscle painDecreased muscle tone
Decreased deep tendon reflex
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S t i l i l t
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Symmetrical involvement
Neuropathic skin changePain & Numb
Decreased reflexes
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Motor
predomi
nate
Less
sensorys/s
Plexopathy
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Mononeuropathy
Multiple mononeuropathy /
mononeuropathy multiplex
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Radiculopathy
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Dorsal scapular to rhomboid
Long thoracic nerve to seratus anterior
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Motor
predomi
nate
Less
sensorys/s
Plexopathy
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Muscles to test
for brachial plexuslesion
1. Supraspinatous
2. Infraspinatous
3. Pectoraris major
4. Pectoraris minor
5. Subscapularis
6. latissimus dorsi
1 and2
3
3 and 4
56
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Mononeuropathy
Multiple mononeuropathy /
mononeuropathy multiplex
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Median N.
M di N
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Median N.
APBFPB
OpponenLumbrical 1&2
Pronator Teres
Flexor Carpi Radialis
Palmaris LongusFlexer Digitor um superficialis
Flexer digitor um
prof undus
Flexer pollicislongus
Anterior
interosseous
nerve
Median N
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Median N.
APBFPB
OpponenLumbrical 1&2
Flexer digitor um prof undusFlexer pollicis longus
Anterior
interosseous
nerve
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Sensory Changes in Median Nerve lesion
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Ulnar Nerve
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N & M l
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Nerve & MuscleMedian Thumb and thenar eminence
Ulnar Little finger and hypothenar
eminence
Radial Wrist-drop
Femoral (weak hip flexion
& knee extension)
Peroneal Foot-drop
Sciatic Weak gastrocnemius
Nerve NMJ Muscle
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Motor
Sensory NumbnessPain
Cramp
MildS
/S
Normal /Pain
Cramp
Reflexes
ANS
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S t
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Sensory symptoms
Pain� Nerve
� Muscle
� NMJ
Pain (1)
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Pain (1)
�I nflammatory process ±Inflammatory myopathies
� Systemic connective tissue disease
� Childhood dermatomyositis ±Muscle infections
�Viral myositis
� Pyomyositis� Toxoplasmosis
� Trichinosis
P i (2)
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Pain (2)
� Denervation muscle painsymmetrical or asymmetricle withweakness
±Guillain Barre syndrome
±Acute anterior horn cell disease�
� Rhabdomyolysis ± Metabolic disorder
Pain (3)
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Pain (3)
InfectionsViral & post-viral syndromes
Brucellosis; Leptospirosis;
Falciparum malariaSystemic connective tissue
disorders: S jögren's
Drugs & ToxinsPolyneuropathy: Small fiber
Cramp
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Cramp� Nerve
Motor system disorders� Amyotrophic Lateral Sclerosis
�
Motor neuropathies� Hereditary motor neuron disorders
Other denervation� S
pinal stenosis� Polyneuropathies
Cramp
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Cramp� Nerve
Spontaneous activity syndromes� Episodic ataxia 1 (EA1)
� Neuromyotonia (Isaac's syndrome)
� Stiffman syndrome
� Cramp-fasciculation syndromes
� Muscle ± Becker muscular dystrophy
± Type II muscle fiber predominance
Fasiculations
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F asiculations� Along the course of the axon� Causes
±Benign
� Common in gastrocnemius
� Normal strength
� Exacerbated by exercise, hyperventilation &
tension� May be associated with tendency to cramp
Fasiculations 2
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F asiculations 2
±Motor neuron disorders & Motorneuropathies
±
Metabolic disorders:Hyperparathyroid; Hyperthyroid;Hypomagnesemia
±
Pharmacologic� Anti-Cholinesterase; Caffeine;
Theophylline; Terbutaline; Lithium
Primary reflexes
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Primary reflexes
1. Ankle jerk S1 Gastrocnemius
2. Knee jerk L2,L3,L4 Quadriceps
3. Biceps C5,C6 Biceps
4. Triceps C7,C8 Triceps
Root & muscle
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Root & muscle
C5 Deltoid
C5 Infraspinatus
C5,C6 Biceps
C6 Extensor carpi radialis &
ulnaris
C7 Extensors digitor um &
triceps
C8,T1 Inetrossei and lumbricals
Root & muscle
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Root & muscle
L2,L3,L4 Quadriceps & iliopsoas
L5 Anterior tibial andextensor hallucis
S1 Gastrocnemius
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C5
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C6
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C6
C7
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C7
T1
Nerve & Muscle
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Nerve & MuscleMedian Thumb and thenar eminence
Ulnar Little finger and hypothenar
eminence
Radial Wrist-drop
Femoral Absent knee jerk (weak hip
flexion & knee extension)
Peroneal Foot-dropSciatic Pain down lateral thigh
and leg , absent ankle jerk
Deep tendon reflexes 1
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Deep tendon reflexes 1
� Reflexes are lost before weakness ±Demyelinating neuropathies
±Loss of large myelinated sensoryaxons: Ankle reflexes lost early
� Reflexes are lost with weakness ±
Reflexes are generally lost inproportion to weakness inpolyneuropathy
Deep tendon reflexes 2
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Deep tendon reflexes 2
� Preserved after weakness in myopathy
� lost proximally in muscular dystrophies orsevere proximal myopathy
� hyper-reflexia in myasthenia gravis� hypo-reflexia in myasthenic syndrome
� Ankle reflexes preserved in porphyria
Nerve NMJ Muscle
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Motor
Sensory
Reflexes Decreased /
Fasciculation/Normal
Normal / may
be increased
and may bedecreased
Normal/
Decreased
ANS
How to get Diagnosis ?
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How to get Diagnosis ?
History taking
Physical examinationLab investigations
History (1)
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History (1)Course ±Acute: Days to Weeks
±Subacute : Weeks to months
±Chronic: Months to Years
±Episodic
History (2)
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History (2)Age at onset Pediatric: Neonatal; Childhood
Adult: 20 to 60 years;Geriatric
Hereditary: family history or examination of relatives
Physical examination
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Physical examination
� Motor� Sensory
� Reflexes� ANS
Clinical
Syndrome
Onset
progression
locationHistory of
Clinical syndrome
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Clinical syndrome
� Acute generalized weakness
� Subacute generalized weakness
� Acute or sunacute or chronic
focal S/S
� Relapsing� Fluctuating
Acute generalized weakness
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� GBS� Neuromuscular junction diseases
� Food-borne botulism
� Acute necrotizing myopathy
� Acute poliomyelitis syndrome
� Periodic paralysis
Subacute or chronic generalized
k
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weakness
� Motor neuron diseases
� Chronic demyelinating
polyneuropathy� Neuromuscular junction diseases
� Myopathies
Acute or subacute or chronic
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weakness or numbness
limited to one limb
Radiculopathy ,
Plexopathy
Compressive neuropathy
Mononeuritis multiplex
RELAPSING deficit
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RELAPSING deficit ± I
mmune mediated disease� CIDP
± Hereditary
� Neuropathy Liability to Pressure Palsy
� Brachial Plexopathy
� Porphyria
Fluctuation
Myasthenic syndrome
I nvestigations
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g
in
P eripheral nerve disease
Types of Investigations
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Where is the
lesion ?
What is the
lesion ?
Objectives
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Objectives� localization
�Function components
�Physiology
�Pathology�Follow up
Investigation of
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Peripheral nerveNerve
& MuscleBiopsy
�NCV
�EMG
MRI
MRI
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MRI
� painless.
� identify axonal damage
� identify a lesion site
Magnetic resonance imaging of
human peripheral nerve disease
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human peripheral nerve disease
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Motor conduction velocity
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CV = distance / time
Median nerve
Sensory conduction velocity
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Ulnar nerve
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Pathology
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Pathology
� Segmental demyelination
� Wallerain degeneration
� Axonal degeneration
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Segmental demyelination
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�
destr uction of the myelinsheath leaving the axon intact,
� Although axonal degeneration may
also be present in demyelinatingneuropathies
� and secondary primary segmental
demyelination may be seen in axonaldegeneration.
Pathological diagnosis
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g g
3 major pathologicalprocesses
� axonal degeneration,� segmental demyelination,
� neuronopathy.
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Wallerian type degenrtation
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Pathology
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gy
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Chronic inflammatory neuropathy. Patchy loss of myelinated fibres ina fascicle of the sural nerve (Semi-thin resin section, toluidine blue).
The lower rectangle is an area with a significant loss of large
myelinated fibres. There are several other thinly myelinated and
entirely demyelinated fibres. The upper rectangle is a more densely
populated area.
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� V asculitis and severe axonal neuropathy in a peripheral nerve. CD68
immunohistochemical staining tovisualize digestion chambers and macrophage in the acutely degenerating nerve.
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