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EVALUATION AND MANAGEMENT OF PERIPHERAL NEUROPATHY

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Title: EVALUATION AND MANAGEMENT OF PERIPHERAL NEUROPATHY


1
EVALUATION AND MANAGEMENT OF PERIPHERAL NEUROPATHY
  • NAYEEM KARIM, DO
  • Diplomate, American Board of Psychiatry and
    Neurology
  • October 27, 2012

2
OBJECTIVES
  • PERIPHERAL NEUROANOTOMY NEUROPHYSIOLOGY
  • CLINICAL FEATURES
  • PHYSICAL EXAM FEATURES
  • DIAGNOSTIC APPROACH
  • ELECTRODIAGNOSTIC EVALUATION
  • TREATMENT OPTIONS

3
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4
GBS IN KIDS
  • Most common cause of acute flaccid paralysis in
    childhood, affecting 1 in 100,000 children each
    year.
  • Several forms besides acute inflammatory
    demyelinating polyradiculoneuropathy (AIDP),
    including Miller Fisher syndrome, acute motor and
    sensory axonal neuropathy (AMSAN), and acute
    sensory axonal neuropathy (ASAN).

5
  • Ascending weakness is the hallmark of GBS, but
    pain may be the initial manifestation in almost
    half of affected children.
  • Ataxia and cranial nerve findings are more
    frequent than in adults with this syndrome.

6
  • The most common serious complications are
    weakness of the respiratory muscles and autonomic
    instability.
  • Pneumonia, respiratory distress syndrome,
    septicemia, pressure sores, and pulmonary embolus
    are also important complications.

7
  • In general, the outcome of GBS is more favorable
    in children than adults.
  • The recovery period is longer than the duration
    of the acute illness, often weeks to months, with
    a median estimated recovery time of 7 months.

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9
CASE STUDIES
  • Seven children with GBS treated with IVIg
    compared to eight children treated with
    plasmapheresis alone had similar clinical
    results. However, the need for admission to PICU
    and duration of ICU stay were lower in the IVIg
    group. IVIg recommended as initial therapy for
    pediatric GBS, because it was equally as
    effective as plasmapheresis and associated with
    fewer complications.
  • (J Child Neurol. 1997 Sep)

10
AMERICAN ACADEMY OF NEUROLOGY
  • Both IVIg and plasma exchange are class B
    treatment option recommendations for children
    with severe GBS.

11
PERIPHERAL NEUROANATOMY
  • Cranial Nerves
  • Spinal nerve roots
  • Dorsal root ganglia
  • Peripheral nerve trunks branches
  • Peripheral autonomic nervous system

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15
Brachial Plexus
16
LUMBAR PLEXUS
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19
Structure of Peripheral Nerves
20
A Typical Neuron Overview
  • Dentrites
  • Cell Body
  • Axon
  • Terminal

21
Action Potential Stages Overview
22
Saltatory Conduction
23
PATHOPHYSIOLOGY
24
WALLERIAN DEGENERATION
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26
CLINICAL HISTORY
  • PMHx infection, cancer, connective tissue,
    vascular, endocrine
  • Medications
  • Trauma
  • Drugs, ETOH, exposure to heavy metals/ solvents/
    toxins
  • HIV risk factors
  • Foreign Travel
  • Diet (nutrition)
  • Vitamin Use
  • Family History

27
CLINICAL FEATURES OF SMALL FIBER NEUROPATHIES
  • Small fiber burning/ lancinating pain, aching,
    dysesthesias, allodynia, paresthesias,
    hyperesthesia
  • In chronic Axonal Neuropathies, the longer axons
    are affected first, resulting in symptoms that
    begin in the LE. Slow insidious progression,
    sensory symptoms precede motor symptoms, pain is
    often a predominant component. Primarily
    affecting the small unmyelinated fibers
    (Spinothalamic Tracts)
  • As the syndrome progresses, mild weakness of the
    distal extremities and numbness may extend
    proximally
  • Classic stocking and glove distribution of
    sensory loss
  • Autonomic involvement

28
Small-fiber Neuropathies
  • Leprosy
  • Diabetes
  • Alcoholic Neuropathy
  • Amyloidosis
  • AIDS
  • Hereditary

29
Neuropathies with Autonomic Involvement
  • Diabetic Polyneuropathy
  • Amyloidosis
  • Porphyria
  • Paraneoplastic Syndromes
  • GBS (Acute panautonomic neuropathy)
  • HIV/ AIDS

30
Neuropathies with Cranial Nerve Involvement
  • Diabetes
  • GBS (Miller-Fisher syndrome)
  • HIV
  • Lyme
  • Sarcoidosis

31
Clinical Features of Large Fiber Neuropathies
  • Large fiber myelinated fibers (Posterior
    Columns) decreased VS proprioception, motor
    related complaints secondary to reduced
    proprioception. Imbalance, tripping, gait
    instability, weakness, loss of grip strength,
    difficulty climbing stairs, getting out of chair
  • Demyelinating Neuropathies affects
    predominantly motor nerve fibers. So weakness
    rather than sensory loss is typically one of the
    earlier findings. Eventually complain of
    dysesthesias distally in the legs or arms.
    Clinical course is variable. On exam distal
    muscles are predominantly affected.

32
3 TYPES OF PERIPHERAL NEUROPATHY
  • Mononeuropathy symptoms and signs related to
    the particular nerve
  • Polyneuropathies trouble turning keys in locks,
    having to use both hands to start ignition in
    car, weakness of feet/ ankles resulting in foot
    drop or ankle sprains
  • Mononeuropathy Multiplex

33
Neuropathic Pain Descriptors
  • Burning
  • Electricity
  • Jabbing
  • Broken Glass
  • Spasms
  • Icy Cold
  • Unpleasant Paresthesia
  • Squeezing
  • Deep Aching
  • Sharp
  • Cramping
  • Sunburn
  • Frostbite
  • Dysesthesias
  • Intense Itching

34
NEUROLOGIC EXAM
  • General Physical Exam orthostatic hypotension,
    respiratory rate, vital capacity, organomegaly,
    skin lesions
  • Cranial Nerves
  • Fundoscopic
  • Pattern of Weakness
  • Pattern of Sensory Loss stocking/ glove, focal,
    dermatomal/ nerve root distribution
  • Type of Sensory Loss pain and temp vs light
    touch and proprioception

35
Neurologic Exam
  • DTRs reduced/ absent (axonal vs demyelinating)
  • Musculoskeletal Exam pes cavus, claw toes,
    hammer toes, high arches, foot drop,
    kyphoscoliosis, peroneal muscular atrophy,
    wasting of distal extremities
  • Coordination gait ataxia, romberg,
    incoordination secondary to proprioceptive loss

36
Common Mononeuropathies
  • Median at the Wrist (CTS)
  • Ulnar at the Elbow (Cubital Tunnel Syndrome)
  • Facial (Bells Palsy)
  • Radial at the Spinal Groove (Wrist Drop)
  • Sciatic in the Thigh
  • Peroneal at Fibular Head (Foot Drop)
  • Lateral Femoral Cutaneous
  • Femoral
  • Spinal Accessory

37
Mononeuritis Multiplex Differential Diagnosis
  • Churg-Strauss
  • Diabetes
  • HIV
  • Ischemia
  • Sarcoidosis
  • Rheumatoid Arthritis
  • Neoplastic Infiltration
  • CMV
  • Giant Cell Arteritis
  • Leprosy
  • SLE
  • Vasculitis
  • Trauma
  • Polyarteritis Nodosa

38
Peripheral Neuropathies Etiologies
  • Alcohol abuse
  • Endocrine DM, Hypo T4
  • Nutritional Thiamine, Pyridoxine, B12, Vitamin E
  • Metabolic Uremia, Liver Disease
  • Inflammatory GBS, CIDP, MMM, AMAN, AMSAN,
    Sarcoidosis, Critical illness Polyneuropathy

39
  • Connective Tissue Lupus, RA, Sjogrens Syndrome,
    PAN
  • Infectious Diseases HIV, Lyme, Hepatitis,
    Leprosy
  • Hematalogic Paraproteinemia (MGUS),
    Cryoglobulinemia, Porphyria, Amyloidosis,
    Multiple Myeloma, Leukemia, Waldenstroms
    macroglobulinemia
  • Paraneoplastic

40
Inherited Neurogenetic Diseases
  • Giant axonal neuropathy
  • Familial dysautonomia syndromes
  • Spinocerebellar ataxias
  • Dentatorubral-pallidoluysian atrophy
  • Ataxia with vitamin E deficiency
  • Abetalipoproteinemia
  • Hereditary Neuropathy with Predilection to
    Pressure Palsies

41
INBORN ERRORS OF METABOLISM
  • Mitochondrial disorders
  • MELAS, MERRF, Leigh disease, Kearns-Sayre
    syndrome
  • Peroxisomal disorders
  • Refsum disease, Adrenoleukodystrophy
  • Lysosomal storage diseases
  • Krabbes disease, Metachromatic leukodystophy,
    Niemann-Pick type C
  • Leukodystrophies
  • Alexander, Canavan, Pelizaeus-Merzbacher

42
DRUG INDUCED NEUROPATHIES
  • AXONAL
  • Vincristine
  • Taxol
  • Colchicine
  • Isoniazid
  • Pyridoxine
  • Dapsone
  • Dilantin
  • DEMYELINATING
  • Amiodarone
  • Chloroquine
  • Gold

43
Drug Induced Neuropathies
  • Statins
  • Nitrofurantoin
  • Cisplatin
  • Thalidomide
  • Ara-C

44
DIFFERENTIAL BY CLINICAL COURSE
Acute onset (within days) Subacute (wks to mnths) Chronic Course
Ischemic Toxins/ meds Endocrine
GBS Nutritional Hereditary
Acute Int. Porphyria Metabolic Metabolic Diseases
Critical illness polyneuropathy Paraneoplastic
Connective tissue Infection
Traumatic
45
Subtypes of CMT Neuropathies
Disease Name Pathology Inheritance Prop. of CMT
CMT1 Abnormal myelin AD 50
CMT2 Axonopathy AD 20-40
Intermediate form Combination AD Rare
CMT4 Myelinopathy or Axonopathy AR Rare
CMTX Axonopathy with myelin changes XLD 10-20
46
CLINICAL FEATURES OF CHARCOT-MARIE TOOTH DISEASE
47
CLINICAL FEATURES OF CHARCOT-MARIE TOOTH DISEASE
48
Charcot-Marie Tooth
49
Peripheral Neuropathy Questionnaire
  • Family Member
  • (Circle yes or no)


  • High arches YES
    NO
  • Curled toes
    YES NO
  • Soak feet
    YES NO
  • Twist ankles easily YES
    NO
  • Use of cane/crutches YES
    NO
  • Foot surgery YES
    NO
  • Casting of feet as a child YES
    NO
  • Corrective shoes
    YES NO
  • Cramps
    YES NO
  • Skinny legs
    YES NO
  • Numbness of feet/hands YES
    NO
  • Difficulty buying shoes YES
    NO
  • Burning of feet YES
    NO
  • Painful feet
    YES NO

50
NEUROPATHOLGY OF CMT
51
  • NORMAL ONION BULB
  • FORMATION

52
Hereditary Motor and Sensory Neuropathy
Type Inheritance Onset Neuro-pathy
HMSN1 (CMT1) AD 17 Childhood D
HMSN2 (CMT2) AD 1p Second decade Axonal
HMSN3 (Dejerine-Sottas) AR 1q or 17p Infancy D
HMSN4 (Rousy-Levy) AD Infancy D
HMSN5 (Refsum) AR Childhood D
53
Hereditary Neuropathy with Autonomic Features
Type Inheri- tance Onset Pathology
HSAN1 AD 2nd decade DRG neurons
HSAN2 AR Infancy Absence of myelinated fibers
HSAN3 AR Birth Absence of myelinated fibers
HSAN4 AR Birth Absence of DRG neurons
Friedreich ataxia AR Before age 20 Loss of large myelinated fibers
Ataxia telangiectasia AR Before age 5 Loss of large unmyelinated fibers
54
LABORATORY EVALUATION
  • CBC, CMP
  • HgA1c, TSH, ESR, SPEP, ANA
  • Rheumatologic Screen
  • HIV testing
  • ACE level
  • Paraneoplastic Panel
  • 24hr urine for heavy metals
  • CSF analysis

55
ADDITIONAL TESTING
  • EMG/ NCS
  • Sural Nerve Biopsy
  • Skin Punch Biopsy (Immunoflourescence of epidural
    nerve density)

56
NERVE CONDUCTION STUDIES
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58
NERVE CONDUCTION STUDIES
  • AMPLITUDE size of the response after the muscle
    / nerve is stimulated. Often corresponds to
    axonal injury.
  • LATENCY time interval from nerve stimulation to
    recorded response. Corresponds to demyelinating
    injury.

59
  • NERVE CONDUCTION VELOCITY (m/s) Distance (m) /
    Time (s)
  • (Proximal latency Distal latency) /
  • (distance between proximal distal site)
  • 8.5 ms 3.5 ms/ 23cm 46m/s

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61
Polyneuropathy Treatment
  • Proper diet, strict blood glucose control
  • Correction of hormone deficiency / over secretion
  • Parenteral B12
  • Removal of toxins
  • Corticosteroids
  • Genetic counseling for familial neuropathy
  • Vitamin supplementation
  • Immunosuppression
  • Plasma exchange
  • Intravenous IgG
  • Treatment of HIV, infection, lyme, leprosy
  • Physical therapy / orthotics

62
Treatment of Neuropathic Pain
  • NSAIDs
  • Antidepressants (cymbalta, elavil, pamelor)
  • Anticonvulsives (neurontin, lyrica, lamictal)
  • Topical neuropathic creams
  • Epidural spinal cord stimulation
  • Anodyne therapy
  • SSRIs

63
Neroupathic Pain
  • Pregabalin
  • Dosage range 25 mg to 900 mg per day
  • Effective for pain, sleep, and mood
  • Side effects dizziness, somnolence, and weight
    gain
  • Gabapentin
  • Dosage range 100 mg to 4800 mg per day
  • Effective for shooting and burning pain,
    allodynia, hypesthesia, and sleep
  • Side effects drowsiness, fatigue, imbalance,
    cognitive issues

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65
  • TOPICAL PAIN CREAMS
  • ANTI-INFLAMMATORY CREAMS
  • (Ketorolac, Diclofenac, Cyclobenzaprine,
    Baclofen)
  • NEUROPATHIC PAIN CREAMS
  • (Gabapentin, Imipramine, Amitriptyline)
  • COMBINATION PAIN CREAMS
  • (Baclofen, Diclofenac, Gabapentin, Lidocaine,
    Nifedipine)

66
THANKS
  • Abd-Allah SA. IVIg as therapy for pediatric GBS.
    J Child Neurol. 1997.
  • Bracker MD. The Numb Arm and Hand. Am Fam
    Physician 1995 103-16.
  • Donofrio P. Peripheral Neuropathy Diagnosis and
    Treatment, ACNS Annual Course in Clinical EEG
    and Electrophysiology 2008.
  • Hughes RAC. Practice parameter immunotherapy for
    GBS. Neurology 2003.
  • Maria B. GBS. Current Management in Child
    Neurology 2005 630-633.
  • McLeod JG. Investigation of Peripheral
    Neuropathy. J Neurol Neurosurg Psychiatry 1995
    274-83.
  • Poncelet A. An Algorithm for the Evaluation of
    Peripheral Neuropathy, American Family Physician
    1998.
  • Thomas PK, Ochoa J. Symptomology and
    Differential Diagnosis of Peripheral Neuropathy
    1993 749-74.
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