Transcript Appearance Of Seasonal Allergens

#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
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
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
• 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
• Mononeuropathy (including
radiculopathy, plexopathy)
• Multiple mononeuropathies
• Neuronopathy
• Axonal polyneuropathy
• Demyelinating polyneuropathy
5
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 Neurons
• Cell bodies in dorsal
root ganglia
(pseudounipolar)
• Both myelinated
(proprioception) and
unmyelinated
(pain/temperature)
axons
• Terminate in
sensory receptors
7
Peripheral
Autonomic 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
• Numbness
• Imbalance
• Burning, stinging pain
dysesthesia)
• Insomnia
• Depression
• Weakness
9
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
• Diabetes
• Alcohol abuse
11
Presentations of
Diabetic Neuropathy
• Mononeuropathy (including
cranial nerves, lumbosacral
plexus)
• Multiple mononeuropathies
• Distal sensorimotor
polyneuropathy
12
Alcoholic Neuropathies
• Direct toxic effect
• Secondary nutritional effects
(vitamin deficiencies)
13
Uncommon Causes
of Neuropathy
• Nutritional (vitamin deficiencies)
• Guillain-Barre syndrome
• Toxic (drugs, hexacarbons,
heavy metals)
• Hereditary
• Rheumatologic disease
• Amyloid
14
Other Uncommon Causes
• Paraneoplastic (Anti-Hu)
• Infection
• Systemic disease (uremia,
hypothyroid, etc)
• Tumors (Especially in
neurofibromatosis, type1)
15
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
• 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
• 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
• 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
• 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
• 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
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
Profile
Mrs. Blanton
• 57 year old female
Symptoms
• 8 year history of burning foot pain
• Tingling sensations
24
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
25
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
• 140 consecutively referred patients
• Inclusion criteria
- Pain in the extremities as a
primary complaint
- No significant weakness
- No identified cause
27
Evaluation: EMG / NCS
114 Patients
EMG / NCS
Abnormal
60/114 (53%)
Normal
54/114 (47%)
QST
AUTO
Skin Biopsy
28
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
• 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
31
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
• Large Fiber Sensory Neuropathy
- Hereditary
- Connective tissue disease
(Sjogren’s, MCTD)
- Monoclonal gammopathy
- Amyloidosis
- Cancer (CML)
- Vasculitis (non-systemic)
- Ganglionitis
- Old GBS
- Drug-induced (Taxol)
- Creutzfeld-Jacob disease
- Idiopathic
5
3
2
2
1
1
1
1
1
1
42
33
Differential Diagnosis
• Small Fiber Sensory Neuropathy
- Hereditary
- Monoclonal gammopathy
- PROMM
- Idiopathic
1
1
1
41
• Need also to consider - diabetes,
AIDS, uremia, porphyria, Tangier
Fabry
34
Treatment
• Non-Pharmacologic Measures
- TENS
- Immersion in cold / warm water
- Application of creams
(Lidocaine cream)
- Massage
- Dorsal column stimulation
35
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
Questions on this subject?
#
Press:
(pound) + 71
on your phone keypad to
speak with Dr. Periquet, and Dr. Nash
Visit OMEN OnLine
http://omen.med.ohio-state.edu
37
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