Peripheral Neuropathy

Dr. Sunil J. Panjwani M.D.

Associate Professor in Medicine,

Medical College, Bhavnagar.

Types of nerve fibers

Diameter microns Conduction Velocity m/s Function

A alpha 1-20 1beta 5-10 5gama 3-6 3delta 2-5 2B <3 C <1.3
Non myelinated

70-110 7030-60 3020-30 2020-30 205-15 0.5-2 0.5-

Motor, Proprioception Touch Fusimotor, spindles Sharp pain Autonomic, pregangl.

Slow pain

Peripheral Nerve

 Myelin  Axon

: Current cannot flow : Not nerves left

The Peripheral Nervous System

 Motor: weakness, atrophy  Sensory loss
 Large Fibers (position)  Small fiber (pain)

 Reflex loss  Autonomic symptoms

 (redness, dizziness, ED)

Mechanisms of damage
 Demyelination
GBS

Myelin sheath disrupted

HSMN

Post Diphtheric

 Axonal degeneration
Toxic neuropathies

Axon damage

 Wallerian degeneration Nerve section Both  Compression Focal demyelination

Entrapment-Carpel tunnel syndrome

 Infarction  Infiltration
Leprosy

Arteritis
Churg-Strauss synd. DM

Polyarteritis nodosa Sarcoidosis

Infiltration

Definitions
 Neuropathy
Pathological process affecting a peripheral nerve/s

 Mononeuropathy
A single nerve affected

 Mononeuritis multiplex
Multiple mononeuropathy or Multifocal neuropathy

 Polyneuropathy
Diffuse symmetrical disease usually beginning peripherally Acute/Chronic Static/Prog. Relapsing/Recovering Motor Sensory Sensrimotor(Mixed) Autonomic Demyelinating Axonal

 Radiculopathy

Nerve root disease

Classification FINDINGS EXAMINATION

 Purely Motor or Sensory or Sensorimotor?  Proximal or distal? Symmetric or asymmetric?  Multifocal, generalized, regional?  Upper limbs, lower limbs, neck, trunk? TIMING  Acute or chronic? ASSOCIATED FINDINGS  Painful or painless?  Hereditary or sporadic? ELECTRODIAGNOSIS  Axonal or demyelinating? LABORATORY  Paraprotein present? Type?  Antibody against nerve?  CSF protein level? HISTOLOGY  Inflammatory Cells

Objectives
 To learn a logical/sequential approach  To know the types and etiologies  To understand the treatment

Background
 Common problem  Logical/sequential approach necessary
 Variable presentation and disparate causes  Evaluation and management

Background
 Categorized by subtype and etiology
 Clinical findings  Electro diagnostic tests  Laboratory investigations

 Classification allows rational assessment

 Prognosis  Treatment options

Epidemiology
 Prevalence
 ~ 2.4%  ~ 8% in people older than 55 years

 DM is most common cause

Epidemiology
 Other common systemic causes
       Metabolic disorders Infectious agents Vasculitis Toxins Drugs Autoimmunity Inherited

Diagnosis
 Most important details to determine
 Distribution  Duration  Course

Diagnosis
 Clinical manifestations vary widely
    Altered sensation Pain Muscle weakness or atrophy Autonomic symptoms

Laboratory Screening for Treatable Neuropathy?

B12 Diabetes ANA,

Not truly length-dependent lengthThis type of neuropathy generally a late finding Screen for connective tissue diseases (late finding) If positive, have you proven anything? If onset is recent Risk Factors medications Big question

chronic disease

screen
TSH ESR HIV Review

Diagnosis
 Electro diagnostic studies
 Sensitive, specific, validated  Extension of neurologic exam  Nerve conduction studies (NCS)  Needle electromyography (EMG)

Diagnosis
 Establish distribution
 Mononeuropathy  Mononeuropathy multiplex  Polyneuropathy

 Determine primary pathology

 Demyelinating  Axonal

Mononeuropathy
 Focal lesion involving a single nerve  Electro diagnostic studies indispensible
 Localize site of injury  Determine severity of lesion

Mononeuropathy
 Causes
 Entrapment  Carpal tunnel syndrome is most common  Foot drop  Focal compression  Trauma

Mononeuropathy Multiplex
 Separate/noncontiguous involvement
 Simultaneously  Serially

 Pattern
 Random  Multifocal

 Frequently evolves quickly

Mononeuropathy Multiplex
 Urgent assessment for vasculitis
 Polyarteritis nodosa  Churg-Strauss disease Churg Connective tissue diseases  Rheumatoid arthritis  Sjogrens syndrome

Polyneuropathy
 Most commonly distal symmetrical
 Fiber effect is length-dependent length Toes and soles affected first  Associations  Systemic diseases  Metabolic disorders  Exogenous toxins

Polyneuropathy
 Diabetes is prototype
 Chronic, sensory and motor  Commonest in developed world  Alcoholism is the second most common

Polyneuropathy
 Early symptoms
 Sensory abnormalities
   

Numbness Burning Paresthesias Dysesthesias

 Distally predominant  Symmetrical

Polyneuropathy
 Evolution is centripetal
 Symptoms spread up legs
 Sensory loss  Dysesthesias

 Ankles jerks are depressed  Patients have trouble walking on their heels  Foot plantar flexion remains strong

Polyneuropathy
 Symptoms noticed in fingertips
 Numbness  Dysesthesias

 Advanced picture is easily recognizable

 Stocking-glove sensory loss Stocking Distal muscle wasting and weakness  Absent tendon reflexes

Polyneuropathy
 Sub classification
 Historical features are indispensible
    

Other medical conditions Symptoms of systemic disease Recent viral or other infectious diseases Recent vaccinations Institution of new medications

Polyneuropathy
 Exposure to toxins
 Alcohol  Heavy metals  Organic solvents

 Family history  Duration and clinical course are helpful

 Acute = days to weeks  Chronic = months to years

Laboratory Investigations
 CBC  SUGAR  TFTs  ESR  CRP  UREA  B12
 Methylmalonic acid  Homocysteine

 Folate

Treatment
 General  Subtype specific
     Diabetes mellitus Renal insufficiency Hypothyroidism Vitamin B12 deficiency Systemic vasculitis

Treatment
 General
 Pain
 Antiepileptic drugs  Antidepressants  Tramadol

Treatment
 Preventative and palliative
 Weight reduction  Assiduous foot care  Good shoes  Ankle-foot orthoses as needed Ankle-

 Several organizations provide support

Objectives Revisited
 Approach
 History
 Distribution  Duration  Course

 Laboratory evaluation
    

 Physical exam
 Sensation  Strength

CBC SUGAR TFTs UREA B12

 Electrodx studies
 NCS  EMG

Objectives Revisited
 Subtypes
 Mononeuropathy
 Entrapment  Focal compression  Trauma

 Polyneuropathy
 DM  Toxin

 Mononeuropathy multiplex
 DM  Vasculitis

Objectives Revisited
 Treatment
 General
 Pain control  Preventative/ Palliative

 Specific
 DM  Renal insufficiency  Vasculitis  B12 deficiency  Hypothyroidism

Chronic Length Dependent Neuropathy

 Begins in toes or feet  Stocking distribution
 Progresses rostrally  Tops and bottoms of feet

 Weakness begins in ankles when sensation reaches calves

Sometimes diagnosable, Never treatable?

Phenotype CIDP

Small Differential

PhenotypePhenotype-MADSAM Neuropathy
 Key DDx:
 Brachial plexopathies  Vasculitis mononeuropathy multiplex  Compression neuropathies  HNPP (genetic testing)

Multifocal Motor Neuropathy (MMN)

 Almost always in hands and wrists  Pattern of weakness is in the distribution of individual peripheral nerves
 i.e. severe involvement in ulnar distribution sparing median

 Lack of atrophy in weak muscles  No pathological reflexes

Uncertainty
 Many cases are not easily definable because of multiplicity of patterns  Cases that are not clearly untreatable are possibly treatable