The Neuropathy Resident Tutorial 54

The Neuropathy ’54

Surat Tanprawate, MD, MSc(Lond.), FRCP(T)Division of NeurologyChiang Mai University

Med Res Tutorial for Board Exam

Tuesday, 31 May 2011

Brachial plexus

Lumbar plexusTuesday, 31 May 2011

History

Physical examination

(1) _ _ _ _ _ _ _

(2) associated finding

Investigation-EDX-Biopsy-Other lab

Final diagnosisPrognosisSeverity assessmentTreatmentF/U

Acute symmetrical predominant motor axonal

polyneuropathy


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


Cranial Non-cranial

-Cranialmononeuropathy

-Multiple cranialneuropathy

Radiculopathy

Plexopathy

Neuronopathy

Peripheral -Mononeuropathy-Multifocal mononeuropathy-Polyneuropathy

MonoradiculopathyPolyradiculopathy

Motor neuronopathyGanglinopathy

Brachial plexopathyLumbosacral plexopathy

2. To determine the anatomical pattern of the neuropathy


4 limb <4 limb

Symm• Polyneuropathy• Polyradiculopathy

Asymm• Polyneuropathy• Polyradiculopathy• Multiple

mononeuropathy

1 limb>1 limb

Suspected entrapment neuropathy

• Asym.polyneuropathy

(polyradiculopathy)• Multiple mononeuropathy• Multiple nerve entrapment

Historical, Physical examination

Focal neuropathyTuesday, 31 May 2011

Classes of polyneuropathy according to which part of the nerve cell is mainly affected

Distal axonopathy, or "dying-back neuropathy

Myelinopathy, or "demyelinating polyneuropathy"

Neuronopathy

Metabolic or toxic disturbances-diabetes, renal failureDeficiency syndromes-malnutrition and alcoholismToxin or drugs-chemotherapy

Immune mediated neuropathy

Motor neuron disease, neuronopathies (HZV, chemotherapry)


• 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

When the distribution is symmetrical, is it proximal or distal


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

• Acute

• Acute dysautonomia


• Toxic: vincristine

• Chronic

• Diabetes neuropathy

• Amyloid neuropathy

• Paraneoplastic sensory neuropathy(malignant inflammatory sensory polyganglionopathy)

• HIV related autonomic neuropathy

• Hereditary sensory and autonomic neuropahty


Small fiber neuropathies

• Idiopathic small fiber neuropathy

• Diabetes mellitus and impaired glucose tolerance


• 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

• length-dependent neuropathy(dying back neuropathy)

• ascending extends proximally

• sensory loss in a stocking like pattern

• distal muscle weakness, and atrophy

• Demyelination

• relatively sparing of temperature and PPS

• early generalized loss of reflexes

• disproportionately mild muscle atrophy in the presence of proximal and distal weakness

• 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


Enlargednerve

Michael Donaghy. Practical Neurology, 2003, 3, 40–45

-Leprosy-Hereditary motor and sensory neuropathy-Neurofibromatosis-Refsumʼs disease-Perineuroma/localized hypertrophic neuropathy-Nerve tumours-Amyloidosis


5. Temporal course of neuropathy

•The temporal course of a neuropathy varies, based on the etiology


DDx cause of neuropathy

Hereditary Acquire

• CMT

• Hereditary neuropathy with liability to pressure palsy

• Other

• Inflammatory demyelinating polyrediculoneuropathy

• Peripheral neuropathy associated with monoclonal protein

• 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, pp331–339Tuesday, 31 May 2011

Common drug induced neuropathy

AxonalVincristinePaclitaxel (Taxol)ColchicineIsoniazid HydralazineMetronidazole PyridoxineDidanosine LithiumAlfa interferon (Intron A)DapsonePhenytoin (Dilantin)CimetidineDisulfiramChloroquine Ethambutol Amitriptyline

DemyelinatingAmiodarone (Cordarone)ChloroquineSuramin Gold


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



Symmetrical polyneuropathies - Distal sensory or sensorimotor polyneuropathy (DSDP) - Small-fiber neuropathy - Autonomic diabetic neuropathy(DAN) - Large-fiber neuropathy



3/4 of all


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 fiber

Pure large fiber


Sub-classification

Pure small fiber

Pure large 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

Pure small fiber

Pure large 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

-painless paresthesia beginning at toes and feet-impairment of vibration and joint position sense-diminish muscle stretch reflex-often asymmetric-sensory ataxia (advance case)


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


Charcot’s jointPicture from Br J Sports Med 2003;37:30–35

Joint dislocation, pathologic fractures and debilitating deformities

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


http://www.Medscape.com

http://www.Medscape.com

Foot ulceration

•the lifetime incidence maybe 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


2. Rapidly progressive motor component

• Chronic inflammatory demyelinating polyneuropathy (CIDP)

Llewelyn JG. JNNP 2003;74(Suppl II):ii15–ii1



Symmetrical polyneuropathies - Distal sensory or sensorimotor polyneuropathy (DSDP) - Small-fiber neuropathy - Autonomic neuropathy - Large-fiber neuropathy




Clinical scenario

“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

“Diabetic amyotrophy”


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 Research Group. N Eng J Med. 1993;329:977-986.

DCCT: risk of DPN and DAN are reduced with improved blood glucose control(DM type 1 and 2)


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 extremities

Lower extremities

Proximal

Distal

•Shoulders

•Abduction

•Elbows

•Flexion, Extension

•Wrists

•Extension, Flexion

•Fingers

•Extension, Flexion

•Abduction

Proximal

Distal

•Hips

•Flex, Extend, Adduct, Abduct

•Knee

•Flexion, Extension

•Ankles

•Dorsiflexion, Eversion, Inversion, Plantar flexion

•Toes

•Great toe dorsiflexion


Musculocutaneous nerve

Axillary nerve

•Pass under axillar•Muscle: deltoid

Nerve innervate only proximal muscle group

•Muscle: bicep•Cutaneous: lateral cutaneous nerve of forearm


Radial nerve

• Radial nerve:

• run around radial groove

• Form:

• Posterior interosseous n.

• Superficial radial n.

Nerve innervate extensor muscle group


Radial nerve

• Radial nerve:

• run around radial groove

• Form:

• Posterior interosseous n.

• Superficial radial n.

Tricep

Brachioradialis

Extensor carpiradialis

Extensor digitorum

pollicis indices


Median nerve

• Median nerve:

• run medial part of arm

• Form:

• Median n.

• Anterior interosseous n.

Nerve innervate distal muscle group


Median nerve

• Median nerve:

• run medial part of arm

• Form:

• Median n.

• Anterior interosseous n.

Flexor carpi radialis

Flexor digitorum superficialis

Flexor digitorum profundus 1 & 2

Pollicis longus

LOAF muscle group


Ulnar nerve

• Ulnar nerve:

• run ulnar side of the arm

• Innervate:

• Most intrinsic hand muscle, except LOAF muscle group

Nerve innervate distal muscle group


Ulnar nerve

Flexor carpi ulnaris

Flexor digitorum profundus 3 & 4

Intrinsic hand muscle except

LOAF

• Ulnar nerve:

• run ulnar side of the arm

• Innervate:

• Most intrinsic hand muscle, except LOAF muscle group


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 nerveTibialis anteriorExtensor pollicis longus

-Superficial peroneal nerve

Peroneus longusPeroneus 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.

finger extension Ext. Digitorum communis C7 PIN

finger flex FPL+FDP(index)FDP(ring+little)

C8C8

AINUlnar

finger abduction 1 DIAPB

T1T1

UlnarMedian


Hip flex Iliopsoas L1, 2 Femoral n.

Hip adduct Adductor L2, 3 Obturator n.

Hip Abduct Gluteus medius, minimus

L4, 5 Superior gluteal n.

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

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

Aim: identify site of lesion:root, plexus, nerve

(awareness the UMN: pyramidal weakness)


Entrapment of upper extremi/es

• Clinical approach–Proximal arm weakness

–Wrist drop

–Hand atrophy


Proximal arm weakness

• Proximal arm muscle: key muscle–Deltoid:C5,6‐ axillary n.–Bicep: C5, 6‐ musculocutaneous n.

–Tricep: C7,8‐ radial n.–Brachioradialis: C5, 6‐ radial n.

• PaGern involved–Deltoid alone

• axillary n. lesion

–Deltoid, bicep, brachioradialis involved(spare tricep)• C5,6 root

– Involve alls muscle• 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 distribu/on–Radial n: tricep, brachioradialis

–P.I.N: extensor digitorum, extensor carpi ulnaris

• Key muscle: C5,6–Deltoid: axiallary n–Bicep: musculocutaneous n.

–Tricep: radial n.(C6,7,8)–FCR: median n.(C6,7)


Interpret

• Generalized weakness: esp. weakness of deltoid, tricep, wrist ext, finger ext.– UMN: cor/cospinal tract lesion

• Selected weakness–C7,C8 root or plexus–Radial nerve lesion–Posterior interosseous nerve lesion


Out stretch arm test


Tricep Brachioradialis Wrist extension

Finger extension

Finger flexion

PIN lesion

(finger drop with radial devia/on)

Normal Normal Normal (radial

divia/on)

Weak Normal

C7,8 or brachial

plexus lesion

Weak Normal Radial divia/on

Weak Weak

Radial nerve lesion(radial groove)

(wrist drop)

Normal Weak Weak Weak Normal

C5,6 or

Brachial plexus

Weak Weak Weak Normal Normal


• Muscle of hand–LOAF(median n.), other than LOAF(ulnar n.)

–Key muscle: 3 muscle• APB(for LOAF), ADM and 1 DI(for other than LOAF)

• Root innerva/on–APB: C8 T1–1DI: C8, T1–ADM: C8, T1

• PaGern of weakness–Only APB: median n. lesion‐test other flexor m. group

–Only ADM and 1DI: ulnar n. lesion

–Weak all 3 muscle: many causes


• 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– Iliopsoas m: L1, 2‐ femoral n.

–Quadricep m: L2,3‐ femoral n.

–Adductor m: L3,4‐ obturator n.

–Hamstring m: L5, S1,2‐ scia/c n.

–Gluteus maximus m: L5, S1,2‐ inferior gluteal n

• Weak • Iliopsoas+quadricep

–Femoral n. lesion

• Iliopsoas+quadricep+ hip adduc/on–L2,3, 4 lesion


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

• Dorsiflex+eversion+EHL–Common peroneal n. lesion

• Dorsiflex+inversion+ hip abduc/on–L4, 5 root or plexus lesion

• Alls movement of foot–Peripheral neuropathy–Scia/c n. lesion–Plexus lesion–Cauda equina lesion–Anterior horn cell disease


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


The Neuropathy Resident Tutorial 54

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