The Neuropathy Resident Tutorial 54

The Neuropathy
’54
Med Res Tutorial for Board Exam
Surat Tanprawate, MD, MSc(Lond.), FRCP(T)

Division of Neurology
Chiang Mai University
Tuesday, 31 May 2011

Brachial plexus
Lumbar plexus
History
Physical examination
Acute symmetrical (1) _ _ _ _ _ _ _

predominant motor axonal
polyneuropathy (2) associated finding
Final diagnosis Investigation

Prognosis
-EDX
Severity assessment
Treatment -Biopsy
F/U -Other lab
Step by step: polyneuropathy
1. Localization into the peripheral nervous
system
2. To determine the anatomical pattern of the
neuropathy
3. What is the primary pathology and fiber
involved?
4. Predominated and associated neurological
pattern
5. Temporal course of neuropathy
1. Localized into the LMN and
nerve

2. To determine the anatomical
pattern of the neuropathy
Cranial Non-cranial
Monoradiculopathy
Radiculopathy Polyradiculopathy
-Cranial
mononeuropathy
Motor neuronopathy
Neuronopathy
Ganglinopathy
-Multiple cranial Plexopathy Brachial plexopathy
neuropathy Lumbosacral plexopathy
Peripheral
-Mononeuropathy
-Multifocal mononeuropathy
-Polyneuropathy

Historical, Physical
examination
4 limb <4 limb
Symm >1 limb 1 limb

• Polyneuropathy
• Polyradiculopathy
• Asym.polyneuropathy Suspected
(polyradiculopathy)
entrapment
• Multiple mononeuropathy
Asymm • Multiple nerve entrapment
neuropathy
• Polyneuropathy
• Polyradiculopathy
• Multiple
mononeuropathy
Focal neuropathy
Classes of polyneuropathy according to which part
of the nerve cell is mainly affected
Distal axonopathy, or "dying-back neuropathy
Metabolic or toxic disturbances

-diabetes, renal failure
Deficiency syndromes
-malnutrition and alcoholism
Toxin or drugs
-chemotherapy
Myelinopathy, or "demyelinating
polyneuropathy"
Immune mediated neuropathy
Neuronopathy
Motor neuron disease, neuronopathies (HZV, chemotherapry)

When the distribution is
symmetrical, is it proximal or distal
• Distal
• toxic, metabolic (from dying back process)
• Proximal
• rare, include porphyria, GBS
• Lead neuropathy is an exception
• initially affects motor fibers in radial and

peroneal distribution

Predominated and associated
neurological pattern
• Predominantly motor manifestations

• Neuropathies with facial nerve involvement
• Neuropathies with autonomic nervous
system involvement
• Small-fiber neuropathies
• Sensory ataxic neuropathies
Predominantly motor symptom
• Multifocal motor neuropathy
• Guillain-Barre syndrome
• Acute motor axonal neuropathy
• Porphyric neuropathy
• Chronic inflammatory polyradiculopathy
• Neuropathy with osteosclerotic myeloma
• Diabetic lumbar radiculoplexopathy
• Hereditary motor sensory neuropatthies (Charcot-

Marie-Tooth disease)
• Lead intoxication

Neuropathies with facial
nerve involvement
• Lyme disease
• Sarcoidosis
• HIV-1 infection
• Gelsolin famillial amyloid
neuropathy(Finnish)
• Tangier disease
Neuropathy with autonomic
nervous system involvement
• Chronic
• Acute • Diabetes neuropathy
• Acute dysautonomia • Amyloid neuropathy
• Guillain-Barre • Paraneoplastic sensory

neuropathy(malignant
syndrome inflammatory sensory
polyganglionopathy)
• Toxic: vincristine • HIV related autonomic neuropathy
• Hereditary sensory and autonomic

neuropahty

Small fiber
neuropathies
• Idiopathic small fiber neuropathy
• Diabetes mellitus and impaired glucose

tolerance
• Amyloid neuropathy
• HIV associated distal sensory neuropathy
• Hereditary sensory and autonomic

neuropathies
• Sjogren’s syndrome
Sensory ataxic neuropathy
• Sensory neuropathies (polyganglinopathies)
• Paraneoplastic sensory neuronopathy
• Toxic polyneuropathies
• Cisplatin and analog
• Vitamin B6 excess
• Demyelinating polyradiculoneuropathies
• Immunoglobulin M monoclonal gammopathy of

undetermined significance

3. What is the primary
pathology and fiber involved?
• Primary pathology
• axonal, demyelination, mixed
• Nerve fiver involved
• sensory, motor, autonomic, mixed
• large fiber, small fiber
Clinical features suggesting
axonal vs demyelination
• Axonal • Demyelination
• length-dependent
neuropathy(dying
• relatively sparing of
temperature and PPS
back neuropathy)
• early generalized loss of
• ascending extends
proximally
reflexes
• disproportionately mild
• sensory loss in a
stocking like pattern
muscle atrophy in the
presence of proximal
and distal weakness
• distal muscle
weakness, and
atrophy
• neuropathic tremor
• palpable enlarged nerve

When a nerve biopsy may be
useful
• Inflammatory neuropathies
• Dysproteinaemic neuropathies
• Genetic neuropathies
• Metabolic disorders, with distinctive features and

storage inclusions
• Tumour infiltration
• Toxic neuropathies, with characteristic changes,

e.g. amiodarone, solvent abuse.
Practical Neurology, 2003, 3, 306–313

Enlarged
nerve
-Leprosy
-Hereditary motor and
sensory neuropathy
-Neurofibromatosis
-Refsumʼs disease
-Perineuroma/localized
hypertrophic
neuropathy
-Nerve tumours
-Amyloidosis
Michael Donaghy. Practical Neurology, 2003, 3, 40–45
5. Temporal course of
neuropathy
• The temporal course of a

neuropathy varies, based on
the etiology

DDx cause of neuropathy
Hereditary Acquire
• CMT • Inflammatory demyelinating

polyrediculoneuropathy
• Hereditary neuropathy
• Peripheral neuropathy associated
with liability to pressure
palsy with monoclonal protein
• Other • Neuropathy associated with

systemic disorder
• Diabetes, malignancy, connective

tissue disease, alcohol and
nutritional deficiency
• Toxic/drug neuropathy

Differential diagnosis of neuropathies by clinical course

Chronic progressive
symmetrical sensorimotor
axonal polyneuropathy with
family history

Inflammatory demyelinating neuropathies and related disorders
R. A. C. Hughes. J. Anat. (2002) 200, pp331–339

Common drug Axonal
induced neuropathy Vincristine
Paclitaxel (Taxol)
Colchicine
Isoniazid
Hydralazine
Metronidazole
Pyridoxine
Didanosine
Lithium
Alfa interferon (Intron A)
Dapsone
Phenytoin (Dilantin)
Demyelinating Cimetidine
Amiodarone (Cordarone) Disulfiram
Chloroquine Chloroquine
Suramin Ethambutol
Gold Amitriptyline
Multiple
mononeuropathy
• Multiple mononeuropathy is a asymmetric
asynchronous sensory and motor
peripheral neuropathy involving isolated
damage to at least 2 separate nerve areas
• Disease: DM, vasculitis, amyloidosis, direct
tumor involvement, PAN, RA, SLE,
paraneoplastic syndrome

Neuropathy of
Diabetes

Definition
Diabetic neuropathy is defined as
“The presence of symptoms and signs of

peripheral nerve dysfunction in individuals
with diabetes after the exclusion of other
causes.”
CIDP, vitamin B12 deficiency, alcoholic
neuropathy, endocrine neuropathy

The risk of developing
symptomatic neuropathy in
patients without neuropathic
symptoms or signs at the time of
initial diagnosis of diabetes is
estimated to be
“4% to 10% by 5 years”

“50% by 25 years”

Classification of Diabetes Neuropathies
Symmetrical polyneuropathies
- Distal sensory or sensorimotor polyneuropathy (DSDP)
- Small-fiber neuropathy
- Autonomic diabetic neuropathy(DAN)
- Large-fiber neuropathy
Asymmetrical neuropathies
- Cranial neuropathies (single or multiple)
- Truncal neuropathy (thoracic radiculopathy)
- Limb mononeuropathy (single or multiple)
- Lumbosacral radiculoplexopathy (asymmetrical proximal
motor neuropathy)
- Focal limb neuropathies (including compression and
entrapment neuropathy)
Combinations
- Polyradiculoneuropathy
- Diabetic neuropathic cachexia
- Symmetrical polyneuropathies
- Small-fiber neuropathy 3/4 of all
- Autonomic diabetic neuropathy(DAN)
motor neuropathy)
Combinations
Clinical Pattern of Diabetic
neuropathy

Distal Symmetrical
Polyneuropathy
• Most common
• Clinical features:
• sensory deficit predominate
• autonomic symptoms correlated with
severity
• minor motor symptom affecting the distal
lower extremity muscles

Sub-classification

Sub-classification
Pure small Pure large

fiber fiber

Sub-classification

fiber fiber
-pain of a deep, burning , stinging,
aching character, allodynia to light
touch
-accompanied by autonomic
neuropathy
-impaired pain and temp, but
relatively spared joint position,
vibration and muscle stretch
reflex
Sub-classification

fiber fiber
-pain of a deep, burning , stinging, -painless paresthesia beginning at
aching character, allodynia to light toes and feet
touch -impairment of vibration and joint
-accompanied by autonomic position sense
neuropathy -diminish muscle stretch reflex
-impaired pain and temp, but -often asymmetric
relatively spared joint position, -sensory ataxia (advance case)
vibration and muscle stretch
reflex
Complication of distal
symmetrical sensory
polyneuropathy
• Charcot’s joint
• Painless trauma and burn
• Trophic change and plantar
ulcer

Charcot’s joint
(Neuropathic osteoarthropathies)
• 1868: Jean-Martin Charcot described of the

neuropathic aspect of arthralgia as a
complication of syphilis
• 1936: Jordan linked neuropathic joint to
diabetes which is the most common etiology
now

Joint dislocation, pathologic fractures and debilitating deformities
Picture from Br J Sports Med 2003;37:30–35
Charcot’s joint
Lateral radiograph of a patient
with diabetes with Charcot
foot disease.
Notice the midfoot collapse,
leaving the patient with an
inverted arch
Picture from www.Medscape.com

Foot ulceration
•the lifetime incidence may

be as high as 25%
•50-70% of all non-traumatic
lower extremity amputations
can be attributed to diabetes
Clinical assessment in diabetic
symmetrical polyneuropathy (DSDP)
• Look for any deformity, callus or foot ulcer,

infection or fissure
• Absent ankle reflexed
• Test all sensory modalities: vibration,

neurofilament test
• Weakness of small foot muscles (EHL, EDB)
• Check resting pulse and BP lying and standing
• Check peripheral pulses

Diabetic Neuropathy
Diagnosis
• The diagnosis of peripheral neuropathy can be
made only after a careful clinical examination with
more than 1 test (the American Diabetes
Association recommendation)
• Vibration perception (using a 128-Hz tuning

fork)
• pressure sensation (using a 10-g monofilament at
least at the distal halluces)
• ankle reflexes
• pinprick
Symptomatic
autonomic neuropathy
• although symptomatic autonomic
neuropathy is relatively uncommon, but
specific autonomic function tests show
abnormality in 97% of DSDP patients
• If there is a prominent autonomic
neuropathy in diabetic with no or mild
DSDP, think of another cause of autonomic
disturbance
Llewelyn JG. JNNP 2003;74(Suppl II):ii15–ii1

What atypical features might suggest
an alternative neuropathy?
1. Severe autonomic neuropathy with

mild DSDP
• Amyloid neuropathy
2. Rapidly progressive motor component
• Chronic inflammatory demyelinating
polyneuropathy (CIDP)
Llewelyn JG. JNNP 2003;74(Suppl II):ii15–ii1

- Small-fiber neuropathy
- Autonomic neuropathy
motor neuropathy)
Combinations
Clinical
scenario
“Diabetic
amyotrophy”
“A middle age diabetic patients develop severe

aching or burning and lancinating pain in the hip
and thigh. This is followed by weakness and
wasting of the thigh muscles, which occur
asymmetrically.”
“Diabetic amyotrophy”
Diabetic lumbosacral radiculoplexus

neuropathy (Brun-Garland syndrome)
• Bruns described the syndrome in 1890, and

Garland rediscovered and coined the term
“amyotrophy”
• Common in older patients with type 2 DM
• Clinical feature and evolution are variable
Clinical Pattern of Diabetic
neuropathy

DCCT: Result Summary
Improved control of blood glucose reduces

the risk of clinically meaningful
• Retinopathy 76% (P<0.002)
• Nephropathy 54% (P<0.04)
• Neuropathy 60% (P<0.002)
DCCT: risk of DPN and DAN are reduced with

improved blood glucose control(DM type 1 and 2)
DCCT Research Group. N Eng J Med. 1993;329:977-986.

Entrapment
neuropathy

Entrapment neuropathy
• To remember
• Remember as the a muscle group (set)
• Remember: actions, muscles, roots,
nerves
• Understand terms and pathways of
innervation
• Approaching process of “drop” symptoms
• Clinical skill practice

Muscle groups
Upper Lower
extremities extremities
•Shoulders •Hips
•Abduction •Flex, Extend, Adduct,
Proximal •Elbows Proximal Abduct
•Knee
•Flexion, Extension •Flexion, Extension
•Wrists •Ankles
•Extension, Flexion •Dorsiflexion, Eversion,
Inversion, Plantar
Distal •Fingers Distal flexion
•Extension, Flexion
•Toes
•Abduction
•Great toe dorsiflexion
Nerve innervate only
proximal muscle group
•Muscle: bicep
•Cutaneous: lateral
cutaneous nerve of Axillary nerve
forearm
•Pass under axillar
Musculocutaneous nerve •Muscle: deltoid
Nerve innervate
extensor muscle group
Radial nerve
• Radial nerve:
• run around radial

groove
• Form:
• Posterior
interosseous n.
• Superficial radial n.

Radial nerve
Tricep
• Radial nerve:
Brachioradialis
• run around radial
groove Extensor
carpiradialis
• Form:
• Posterior
interosseous n. Extensor
digitorum
• Superficial radial n. pollicis indices

Nerve innervate distal
muscle group
Median nerve
• Median nerve:
• run medial part of

arm
• Form:
• Median n.
• Anterior interosseous
n.

Median nerve Flexor carpi
radialis
• Median nerve: Flexor digitorum

superficialis
• run medial part of
arm
• Form: Flexor digitorum

profundus 1 & 2
LOAF
muscle
group
• Median n.
• Anterior interosseous Pollicis longus

n.

Nerve innervate distal
muscle group
Ulnar nerve
• Ulnar nerve:
• run ulnar side of the

arm
• Innervate:
• Most intrinsic hand

muscle, except LOAF
muscle group

Ulnar nerve
Flexor carpi
ulnaris
• Ulnar nerve:
• run ulnar side of the arm

Flexor digitorum
• Innervate:
profundus 3 & 4
• Most intrinsic hand

muscle, except LOAF
muscle group Intrinsic hand
muscle except
LOAF

Obturator nerve
-adductor group
Femoral nerve
-quadricep femoris
Superior gluteal
nerve
-gluteus medius
-gluteus minimus

Sciatic nerve
Tibial nerve
-Gastrocnemius
-Tibialis posterior
Common peroneal
nerve
-Deep peroneal nerve
Tibialis anterior
Extensor pollicis longus
-Superficial peroneal
nerve
Peroneus longus
Peroneus brevis

Physical examination
• Action>>Muscle>>Roots>>Nerve
• Remember group by group
• Need to know the basic knowledge of
nerve innervation into the muscle

shoulder abduction deltoid C5,6 axillary n.
elbow flex biceps C5,6 musculocutaneus n.
elbow flex brachioradialis C5,6 radial n.
elbow extension triceps C7,8 radial n.
wrist flex FCR C6,7 median n.
wrist extension ECR longus C5, C6 radial n.
Ext. Digitorum
finger extension C7 PIN
communis
FPL+FDP(index) C8 AIN
finger flex
FDP(ring+little) C8 Ulnar
1 DI T1 Ulnar
finger abduction
APB T1 Median
Hip flex Iliopsoas L1, 2 Femoral n.
Hip adduct Adductor L2, 3 Obturator n.

Gluteus medius,
Hip Abduct L4, 5 Superior gluteal n.
minimus
Hip extension Gluteus maximus L5, S1 Sciatic n.
Knee extension Quadricep L3, 4 Femoral n.
Knee flexion Hamstring L5, S1 Sciatic n.
Ankle dorsiflex TA L4, 5 DPN
Ankle eversion Peronei L5, S1 SPN
Ankle inversion TP L4, 5 Tibial n.
Plantar flexion Gastrocnemius S1, S2 Tibial n.
Big toe extension EHL L5 DPN

Approaching process
Aim: identify site of lesion:
root, plexus, nerve
(awareness the UMN: pyramidal weakness)
1. What’s action and muscle causing “drop”

2. What’s root and nerve innervated that “weak” muscle
3. To test key muscles !
-Same root, but different nerve
-Same nerve, but different root
4. Evaluated sensory loss
5. Give a diagnosis
Entrapment of upper extremi/es
• Clinical approach
–Proximal arm weakness
–Wrist drop
–Hand atrophy

Proximal arm weakness
• Proximal arm muscle: key • PaGern involved
muscle – Deltoid alone
– Deltoid:C5,6‐ axillary n. • axillary n. lesion
– Bicep: C5, 6‐ – Deltoid, bicep,
musculocutaneous n. brachioradialis
involved(spare tricep)
– Tricep: C7,8‐ radial n.
• C5,6 root
– Brachioradialis: C5, 6‐
– Involve alls muscle
radial n.
• Brachial plexus
• Cord

Wrist drop
• Wrist drop
– Extexsor carpi radialis
longus(C5, C6 and radial
nerve)
– Extensor carpi ulnarlis( C7,
C8 and posterior
interosseous branch of
radial nerve)

Key muscle: nerve, root
• Key muscle: radial • Key muscle: C5,6
distribu/on – Deltoid: axiallary n
– Radial n: tricep, – Bicep: musculocutaneous
brachioradialis n.
– P.I.N: extensor digitorum, – Tricep: radial n.(C6,7,8)
extensor carpi ulnaris – FCR: median n.(C6,7)

• Generalized weakness: esp.
weakness of deltoid, tricep,
wrist ext, finger ext.
– UMN: cor/cospinal tract lesion
Interpret
• Selected weakness
– C7,C8 root or plexus
– Radial nerve lesion
– Posterior interosseous
nerve lesion

Out stretch arm test

Tricep Brachioradialis Wrist Finger Finger
extension extension flexion
PIN lesion Normal Normal Normal Weak Normal

(finger drop (radial
with radial divia/on)
devia/on)
C7,8 or Weak Normal Radial Weak Weak
brachial divia/on
plexus lesion
Radial nerve Normal Weak Weak Weak Normal
lesion(radial
groove)
(wrist drop)
C5,6 or Weak Weak Weak Normal Normal
Brachial
plexus

• Muscle of hand • PaGern of weakness
– LOAF(median n.), other – Only APB: median n.
than LOAF(ulnar n.) lesion‐test other flexor m.
– Key muscle: 3 muscle group
• APB(for LOAF), ADM and 1 – Only ADM and 1DI: ulnar n.
DI(for other than LOAF) lesion
• Root innerva/on – Weak all 3 muscle: many
causes
– APB: C8 T1
– 1DI: C8, T1
– ADM: C8, T1

• 3 muscle plus finger extensor, tricep, finger flexor
– C7,8,T1 root
• Fail arm+ all sensa/on
– Brachial plexus
• Fail arm and cape distribu/on sensory loss
– Spinal cord
• Generalized
– MND
– Polyneuropathy

Lower extremi/es
• Lumbosacral plexus
• Proximal
–Anterior: obturator n., femoral n.
–Posterior: gluteal n., scia/c n(hamstring m.)
• Distal
–Anterior: peroneal nerve(deep VS superficial)
–Posterior: /bial nerve

Proximal weakness of legs
• Key muscle • Weak
– Iliopsoas m: L1, 2‐ femoral
• Iliopsoas+quadricep
n. – Femoral n. lesion
– Quadricep m: L2,3‐ femoral • Iliopsoas+quadricep+ hip
n.
adduc/on
– Adductor m: L3,4‐
– L2,3, 4 lesion
obturator n.
– Hamstring m: L5, S1,2‐
scia/c n.
– Gluteus maximus m: L5,
S1,2‐ inferior gluteal n

Foot drop
• Due to weakness of /bialis anterior
• Key muscle
–Tibialis anterior m: L4,5‐DPN
–EHL: L5, S1‐ DPN
–Peroneus m: L5, S1‐ SPN
–Tibialis posterior m: L4,5 ‐ /bial n.
–Gastrocnemius m: S1,2 ‐ /bial n.

PaGern of foot drop
• weakness of • Alls movement of foot
• Dorsiflex+eversion+EHL – Peripheral neuropathy
– Common peroneal n. – Scia/c n. lesion
lesion – Plexus lesion
• Dorsiflex+inversion+ hip – Cauda equina lesion
abduc/on – Anterior horn cell disease
– L4, 5 root or plexus lesion

Differen/al diagnosis of foot drop

Tremor short case
1.Identify tremor type

2.Test associated neurological signs
and general physical signs

Tremor type: 3
position

Tremor at rest
• Test sign of parkinsonism
• Bradykinesia: finger tapping, writing
(micrographia), walking
• Cogwheel rigidity: muscle tone of arm
• Postural instability
• associated signs: Gabellar tapping
Tremor at postural
position-2 ท่า
• Thyroid palpation
• pulse rate
• Look lid lag, lid retraction
• Hair
• Skin
• reflexes
Kinetic tremor
(esp.intention tremor)
• Check other sign of cerebellar dysfunction
• eye movement
• listen to voice(for dysarthria)
• rapid alternating movement
• heel knee chin
• gait, and tandem walk
• Other neuro sign ถ้าเวลาเหลือ

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The Neuropathy

Surat Tanprawate, MD, MSc(Lond.), FRCP(T)

Tuesday, 31 May 2011

Acute symmetrical (1) _ _ _ _ _ _ _

Final diagnosis Investigation

Tuesday, 31 May 2011

Tuesday, 31 May 2011

4 limb <4 limb

Symm >1 limb 1 limb

Metabolic or toxic disturbances

Motor neuron disease, neuronopathies (HZV, chemotherapry)

Tuesday, 31 May 2011

• toxic, metabolic (from dying back process)

• rare, include porphyria, GBS

• Lead neuropathy is an exception

• initially affects motor fibers in radial and

Tuesday, 31 May 2011

• Predominantly motor manifestations

• Acute motor axonal neuropathy

• Chronic inflammatory polyradiculopathy

• Neuropathy with osteosclerotic myeloma

• Diabetic lumbar radiculoplexopathy

• Hereditary motor sensory neuropatthies (Charcot-

Tuesday, 31 May 2011

• Acute • Diabetes neuropathy

• Acute dysautonomia • Amyloid neuropathy

• Guillain-Barre • Paraneoplastic sensory

• Hereditary sensory and autonomic

Tuesday, 31 May 2011

• Diabetes mellitus and impaired glucose

• HIV associated distal sensory neuropathy

• Hereditary sensory and autonomic

• Paraneoplastic sensory neuronopathy

• Cisplatin and analog

• Immunoglobulin M monoclonal gammopathy of

Tuesday, 31 May 2011

Tuesday, 31 May 2011

• Metabolic disorders, with distinctive features and

• Toxic neuropathies, with characteristic changes,

Tuesday, 31 May 2011

• The temporal course of a

Tuesday, 31 May 2011

• CMT • Inflammatory demyelinating

• Other • Neuropathy associated with

• Diabetes, malignancy, connective

Tuesday, 31 May 2011

Tuesday, 31 May 2011

Tuesday, 31 May 2011

R. A. C. Hughes. J. Anat. (2002) 200, pp331–339

Tuesday, 31 May 2011

Tuesday, 31 May 2011

“The presence of symptoms and signs of

Tuesday, 31 May 2011

“4% to 10% by 5 years”

Tuesday, 31 May 2011

Tuesday, 31 May 2011

Tuesday, 31 May 2011

Tuesday, 31 May 2011

Pure small Pure large

Tuesday, 31 May 2011

Pure small Pure large

Pure small Pure large

Tuesday, 31 May 2011