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#1013 Approaching Neuropathies. January 18 to 21 Steven M. Nash, MD Assistant Professor of Clinical Neurology Department of Neurology The Ohio University Medical Center Isabelle Periquet, MD Assistant Professor of Neurology Department of Neurology The Ohio University Medical Center.

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1013 approaching neuropathies
#1013 Approaching Neuropathies

January 18 to 21

Steven M. Nash, MD

Assistant Professor of Clinical Neurology

Department of Neurology

The Ohio University Medical Center

Isabelle Periquet, MD

Assistant Professor of Neurology

Department of Neurology

The Ohio University Medical Center


Steven M. Nash, MD

Assistant Professor of Clinical Neurology

Department of Neurology

The Ohio University Medical Center

1


Profile
Profile

Mr. Winkleman

  • 67 year old male

    Symptoms

  • Falling down frequently

  • Unexplained weight loss

  • Low back, hip, and leg pain

  • Tingling in right side of trunk

  • Right foot drop

2


Profile1
Profile

Mr. Winkleman

Evaluation

  • Weakness in multiple muscle groups

    - Asymmetrical from side to side

  • Less severe sensory loss

  • EMG showed active, asymmetrical sensory

    motor polyneuropathy

  • Sural nerve biopsy revealed vasculitic

    neuropathy

    Diagnosis: Vasculitic neuropathy

2A


3


Key points
Key Points

  • Neuropathies may present in many

    different ways

  • Neuropathy is a result of some other

    pathology

  • Treatment requires identification and

    removal of the underlying cause

  • Work-up includes a careful history

    and physical exam, blood work,

    and EMG

4


Neuropathies
Neuropathies

  • Mononeuropathy (including

    radiculopathy, plexopathy)

  • Multiple mononeuropathies

  • Neuronopathy

  • Axonal polyneuropathy

  • Demyelinating polyneuropathy

5


Peripheral motor neurons
Peripheral Motor Neurons

  • Cell bodies

    located in anterior

    horn of spinal

    cord or in

    brainstem nuclei

  • Axons myelinated

  • Axons terminate

    on skeletal

    muscle fibers

6


Peripheral motor neurons1
Peripheral Motor Neurons

  • Cell bodies in dorsal

    root ganglia

    (pseudounipolar)

  • Both myelinated

    (proprioception) and

    unmyelinated

    (pain/temperature)

    axons

  • Terminate in

    sensory receptors

7


Peripheral autonomic neurons
PeripheralAutonomic Neurons

  • Cell bodies (second order

    neurons) in spinal cord nuclei.

    Axons terminate on third order

    neurons in autonomic ganglia

  • Axons are unmyelinated (slow)

  • Axons of third order neurons

    terminate in glands and smooth

    muscle

8


Symptoms of neuropathy
Symptoms of Neuropathy

  • Numbness

  • Imbalance

  • Burning, stinging pain

    dysesthesia)

  • Insomnia

  • Depression

  • Weakness

9


Signs of neuropathy
Signs of Neuropathy

  • Loss of position and vibration

    sensitivity

  • Pain and temperature loss

  • Romberg sign

  • Weakness and loss of reflexes

  • Trophic changes of skin, hair loss,

    decrease / increase of sweating

10


Common causes of neuropathy
Common Causesof Neuropathy

  • Diabetes

  • Alcohol abuse

11


Presentations of diabetic neuropathy
Presentations ofDiabetic Neuropathy

  • Mononeuropathy (including

    cranial nerves, lumbosacral

    plexus)

  • Multiple mononeuropathies

  • Distal sensorimotor

    polyneuropathy

12


Alcoholic neuropathies
Alcoholic Neuropathies

  • Direct toxic effect

  • Secondary nutritional effects

    (vitamin deficiencies)

13


Uncommon causes of neuropathy
Uncommon Causesof Neuropathy

  • Nutritional (vitamin deficiencies)

  • Guillain-Barre syndrome

  • Toxic (drugs, hexacarbons,

    heavy metals)

  • Hereditary

  • Rheumatologic disease

  • Amyloid

14


Other uncommon causes
Other Uncommon Causes

  • Paraneoplastic (Anti-Hu)

  • Infection

  • Systemic disease (uremia,

    hypothyroid, etc)

  • Tumors (Especially in

    neurofibromatosis, type1)

15


Guillain barre syndrome
Guillain-Barre Syndrome

  • “Ascending paralysis”, loss of reflexes

  • Elevated CSF protein, normal cell count

  • Slowing on nerve conduction studies

  • Diagnosis often requires high index of

    suspicion

  • Most recover with prompt care

  • Plasmapheresis / IVIg both speed

    recovery

16


Neuropathy due to vasculitis
Neuropathy Due toVasculitis

  • May be isolated to peripheral nerves

  • Sometimes associated with

    rheumatologic diseases

  • Multiple mononeuropathies

  • Requires nerve biopsy for definite

    diagnosis

  • Treated with corticosteroids;

    cyclophosphamide often required

17


Blood work in work up of neuropathy patients
Blood Work in Work-upof Neuropathy Patients

  • Glucose, BUN, creatinine, liver

    enzymes, TSH, ESR, hemoglobin A1C,

    serum protein electrophoresis

  • ANA, RF, ANCA in selected patients

  • Gene testing in selected patients

  • Antibody testing in selected patients

18


Antibody testing in polyneuropathies
Antibody Testingin Polyneuropathies

  • Anti-Hu useful in sensory

    neuronopathies

  • Anti-GM1 only for motor

    neuropathies in the absence of

    conduction block on NCS

  • Anti-MAG, anti-sulfatide not helpful

    for diagnosis or treatment

19


Electromyography nerve conduction studies
Electromyography / NerveConduction Studies

  • Used to evaluate function of the

    large, myelinated peripheral nerve

    fibers

  • All patients with clinical evidence of

    polyneuropathy should be studied

    to determine distribution, type

    (axonal vs. demyelinating), severity,

    and activity

20


Conclusions
Conclusions

  • Neuropathies may present in many

    different ways

  • Neuropathy is a result of some other

    pathology

  • Treatment requires identification and

    removal of underlying cause

  • Work-up includes a careful history and

    physical exam, blood work, and EMG

21


Summary
Summary

Mr. Winkleman

Treatment

  • IV corticosteroids

  • Tapering dose of oral Prednisone

  • Six months of oral Cytoxan

  • Physical therapy

  • Not back to baseline, but improving

  • Re-gained some weight

    Prognosis: Good

22


Isabelle Periquet, MD

Director, Peripheral Neuropathy Center

Assistant Professor of Neurology

Department of Neurology

The Ohio University Medical Center

23


Profile2
Profile

Mrs. Blanton

  • 57 year old female

    Symptoms

  • 8 year history of burning foot pain

  • Tingling sensations

24


Profile3
Profile

Mrs. Blanton

Evaluation

  • Strength preserved

  • Reflexes preserved

  • Sensory testing:

    - Normal vibration, position and light touch

    sensation

    - Diminished pin sensation

  • Normal EMG

    Diagnosis: ?

24 A



Evaluation
Evaluation

  • Is this a neuropathy or is it

    something else?

  • How can I confirm a diagnosis

    of neuropathy?

  • What laboratory tests are needed

    to evaluate for a cause?

  • How do I treat this patient?

26


Painful sensory neuropathy prospective evaluation using skin biopsy
Painful Sensory NeuropathyProspective Evaluation Using Skin Biopsy

  • 140 consecutively referred patients

  • Inclusion criteria

    - Pain in the extremities as a

    primary complaint

    - No significant weakness

    - No identified cause

27


Evaluation emg ncs
Evaluation: EMG / NCS

114 Patients

EMG / NCS

Abnormal Normal

60/114 (53%) 54/114 (47%)

QST

AUTO

Skin Biopsy

28


Evaluation qst
Evaluation: QST

  • Computerized method of

    determining vibration

    threshold (large fiber

    function) and temperature

    threshold (small fiber

    function)

  • QST was abnormal in 72%

    of patients with normal

    EMGs

29


Evaluation autonomic testing
Evaluation: Autonomic Testing

  • Battery of tests evaluating sweat

    function (QSART), heart rate and blood

    pressure responses to deep breathing,

    valsalva and tilt

  • QSART was

    abnormal in

    59% of patients

    with normal EMG’s

30



Evaluation blood studies
Evaluation: Blood Studies

  • Routine blood studies

    - CBC, lytes, ESR, BUN, Cr, Ca++,

    LFTs, TSH, HgbA1C, B12 (MMA, HC),

    chol, TG

  • Immune / infectious blood studies

    - HIV, FTA, ANA, ENA, RF, IEP with IF

    - Nerve antibodies (GM1, MAG, SGPG,

    Hu, sulfatide)

  • DNA - PMP22 mutations, FAP (met 30)

32


Differential diagnosis
Differential Diagnosis

  • Large Fiber Sensory Neuropathy

    - Hereditary 5

    - Connective tissue disease 3

    (Sjogren’s, MCTD)

    - Monoclonal gammopathy 2

    - Amyloidosis 2

    - Cancer (CML) 1

    - Vasculitis (non-systemic) 1

    - Ganglionitis 1

    - Old GBS 1

    - Drug-induced (Taxol) 1

    - Creutzfeld-Jacob disease 1

    - Idiopathic 42

33


Differential diagnosis1
Differential Diagnosis

  • Small Fiber Sensory Neuropathy

    - Hereditary 1

    - Monoclonal gammopathy 1

    - PROMM 1

    - Idiopathic 41

  • Need also to consider - diabetes,

    AIDS, uremia, porphyria, Tangier

    Fabry

34


Treatment
Treatment

  • Non-Pharmacologic Measures

    - TENS

    - Immersion in cold / warm water

    - Application of creams

    (Lidocaine cream)

    - Massage

    - Dorsal column stimulation

35


Treatment1
Treatment

  • Tricylic antidepressants (amitriptyline,

    nortriptyline, desipramine)

  • Anticonvulsants (carbamazepine,

    phenytoin, gabapentin, clonazepam,

    lamotrigine, topiramate)

  • Antiarrythmics (mexiletine, lidocaine drip)

  • SRIs (fluoxetine, paroxetine, ventrafaxine)

  • Opiates

  • Others (tramadol, baclofen,

    transdermal / intrathecal clonidine)

36


Press pound 71 on your phone keypad to speak with dr periquet and dr nash
Press: # (pound) + 71on your phone keypad tospeak with Dr. Periquet, and Dr. Nash

Questions on this subject?

Visit OMEN OnLine

http://omen.med.ohio-state.edu

37


1014 ulcer disease update

NEXT WEEK

#1014 Ulcer Disease Update

January 25 to 28

Hagop S. Mekhjian, MD

Professor of Internal Medicine

Division of Digestive Diseases

Medical Director, OSU Hospitals

The Ohio University Medical Center

E. Christopher Ellison, MD

Zollinger Professor of Surgery and

Interim Chair, Department of Surgery

The Ohio University Medical Center


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