Transcript 90 yof with weakness

Neuropathy
Morning Report
January 5th, 2010
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Recognizing neuropathy
Classifying neuropathy
Myriad neuropathies
CIDP
 Diagnosis
 Spectrum of disease
 Treatment
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History:
 Distal motor neuropathy:
▪ tripping over toes, dropping coffee cups.
 Proximal motor neuropathy:
▪ trouble climbing stairs, getting up from sitting, raising arms
to brush teeth and hair.
 Sensory neuropathy:
▪ burning, lancinating pain, paresthesias, dysesthesias, a tight
band-like sensation around wrists or ankles, hypesthesia,
restless legs, numbness.
 Acute vs. chronic
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Physical Exam:
 Motor Strength:
▪ 5= full strength, 4= falls to moderate resistance, 3= falls to
minimal resistance, 2= falls to gravity, 1= muscle contraction,
0= no movement.
 Sensation:
▪ vibration/proprioception (large myelinated axons) vs.
pain/temperature (small myelinated axons)
▪ Light touch shared by both fiber types.
 Trophic Changes:
▪ pes cavus, kyphoscoliosis, loss of hair, ulceration, thinning of
phalanges, charcot joints (neurogenic arthropathy).
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Neuronopathy
 Cell body damage of the motor neuron or dorsal root
ganglion.
 SMA, ALS, CMT (sometimes).
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Axonopathy
 Traumatic, toxic, metabolic
 Often length dependent- “die back”: symmetric,
distal.
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Myelinopathy
 Hereditary or inflammatory
 Non-length dependent.
▪ Patchy or segmental (inflammatory)
▪ Diffuse (hereditary)
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Axonopathy
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Myelopathy
 Loss of amplitude of AP
 Slowed conduction
 Evidence of
 Prolonged distal latency
denervationfasciculation
 Conduction block
 Temporal dispersion
 Prolonged minimum f-
wave latency.
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Metabolic
Toxic
Inflammatory
Infectious
• Diabetes
• Hypothyroidism
• Alcohol
• Lead
• Platinum chemo, tacrolimus
amiodarone, chloroquine.
• Sarcoidosis
• Vasculitis: RA, SLE
• Non-systemic vasculitis
• Leprosy
• Borreliosis (Lyme)
• HIV
Nutritional
Deficiency
• B12 (cyanocobalamine), B6
(pyridoxine), B1 (thiamine)
• E (alpha-tocopherol)
Paraneoplastic
• Carcinoma-associated
• Lymphoma-associated
• Monoclonal gammopathy
Hereditary
Compressive
• SMA
• CMT
• ALS
• Carpal tunnel, ulnar, …
• Myxedema,
• RA, Acromegaly
Demyelinating
•Guillain-Barré
•CIDP
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C = progresses over >1 month.
I = presumed to have an autoimmune origin
D= demyelinating
P = neuropathy that is poly Motor and sensory
 Symmetrical (or more than one limb)
 Non-length-dependent
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American Academy of Neurology Criteria
 Clinical: 2 more more months, hypo/areflexia,
motor/sensory involvement, >1 limb.
 EMG/NCS: 1. Conduction block, 2. decr
conduction velocity, 3. incr distal latency, 4. incr Fwave latency. (Must have 3 of 4)
 Pathologic: unequivocal evidence of
demyelination, remyelination.
 CSF: WBC < 10, VDRL neg, elevated protein.
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Distal Acquired Demyelinating Symmetric (DADS) Neuropathy
 Men over 50
 Mostly distal sensory loss, mild distal weakness, unsteady gait. 2/3rds
have a IgM paraproteinemia.
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Multifocal Motor Neuropathy (MMN)
 Asymmetric, all weakness, no sensory loss
 Some have antiganglioside antibodies
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Multifocal Acquired Demyelinating Sensory and Motor (MADSAM)
Neuropathy (Lewis-Sumner Syndrome)
 Motor and sensory, elevated CSF protein, asymmetric, some with
antiganglioside antiboties.
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Axonal chronic inflammatory demyelinating polyneuropathy, pure
sensory chronic inflammatory demyelinating polyneuropathy,
multifocal acquired sensory and motor axonopathy (MASAM).
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Goal: block the immune process
Course: continue until maximal improvement
and then maintain.
 IVIg: 0.4g/kg qday x 5 days, then monthly x 3
 Plasma exchange
 Corticosteroids
 Mycophenolate mofetil, azothioprine,
cyclophosphamide, etanercept, rituximab
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Koller, H. et al. (2005) Chronic Inflammatory
Demyelinating Polyneuropathy. NEJM 352(13):
1343-1356.
Poncelet, AM. (1998) An Algorithm for the
Evaluation of Peripheral Neuropathy. AFP, Feb
15th, 1998.
Said, G. (2006) Chronic Inflammatory
Demyelinating Polyneuropathy. Neuromusc
Disorders 16:293-303.
Sander, HW et al. (2003) Research Criteria for
defining patients with CIDP. Neurology 60(Suppl
3):S8-S15.