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Peripheral

Neuropathy

Darwin Amir
Bgn Neurologi
Fakultas Kedokteran
Universitas Andalas

http://www.apsu.edu/thompsonj/Anatomy%20&%20Physiology/2010/2010%20Exam%20
Reviews/Exam%204%20Review/CH%2013%20Peripheral%20Nerve%20Histology.htm
• Neuropati perifer
Objectives
• The students will recognize major
peripheral sensory and motor neuropathies
including diabetic neuropathy and Guillain-
Barre syndrome and others
• Student will recognize difference between
mono and polyneuropathies.
• Approach to evaluating patients will be
recalled given a case presentation

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• Tujuan
Para siswa akan mengenali neuropati
sensorik dan motorik utama termasuk
neuropati diabetes dan sindrom Guillain-
Barre dan lainnya.
Siswa akan mengenali perbedaan antara
mono dan polyneuropathies.
Pendekatan untuk mengevaluasi pasien
akan diingat mengingat presentasi kasus
Definitions
• Generalized term including disorders of
any cause
• May involve sensory nerves, motor
nerves, or both
• May affect one nerve (mononeuropathy),
several nerves together (polyneuropathy)
or several nerves not contiguous
(Mononeuropathy multiplex)
• May have demyelination or axonal
degeneration
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• Definisi
Istilah umum termasuk gangguan
penyebab apapun
Mungkin melibatkan saraf sensorik, saraf
motorik, atau keduanya
Dapat mempengaruhi satu saraf
(mononeuropati), beberapa saraf bersama
(polyneuropathy) atau beberapa saraf
tidak bersebelahan (Mononeuropathy
multiplex)
The Motor Unit

From Dumitru, D. Electrodiagnostic Medicine, Hanley & Belfus. Philadelphia. 1995


The 3 questions of clinical neurology…

1. Where is the lesion?


. What is the etiology?
. What is the treatment?

www.ama-assn.org/ ama/pub/category/7172.html
# 1. Dimana lesi itu?
# 2. Apa etiologi itu?
# 3. Apa pengobatannya?
The patterns of peripheral
neuropathy…

• Mononeuropathy?

• Polyneuropathy?
multiple nerves
contiguous
typically length dependent
(“stocking-glove”)

Polyneuropathy is common!
2.4% (8% over 55 yr)

www.ama-assn.org/ ama/pub/category/7172.html
• Pola neuropati perifer ...?
Mononeuropati

Polineuropati?
beberapa saraf
berdekatan
Biasanya tergantung panjang ("stocking-
glove")

Polineuropati adalah umum! 2,4% (8% diatas 55


tahun)
Overview of the Lecture –Mastering polyneuropathy

#1. Where is the injury?


The syndrome depends on:
• what modalities are injured,
• what fibers are injured,
• whether axon or myelin (or both) injured.

#2. What is the etiology?


Tricky – hence an approach necessary at the bedside.

#3. What is the treatment?


Depends on reversing the underlying cause.

Three common examples


• Sekilas tentang Kuliah - Memimpin polineuropati
# 1. Dimana luka itu?
Sindrom ini tergantung pada:
modalitas apa yang terluka,
Serat apa yang terluka,
apakah axon atau myelin (atau keduanya) terluka.

# 2. Apa etiologi itu?


Tricky - maka pendekatan yang diperlukan di samping tempat tidur.

# 3. Apa pengobatannya?
Bergantung pada membalikkan penyebab yang mendasarinya.

Tiga contoh umum


Physical exam
• Look for thickening of nerves
(Neurofibromas)
• Decreased pinprick, sensation, or
temperature
• Decreased reflexes
• Motor weakness
• Tinnel’s testing

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• Ujian fisik
Carilah penebalan saraf (Neurofibroma)
Turunkan pinprick, sensasi, atau suhu
Berkurangnya refleks
Kelemahan motor
Pengujian Tinnel
http://fulton.edzone.net/cites/winkler-science/team1/chap8.html http://www.neuro.wustl.edu/neuromuscular/pathol/nervenl.htm
The clinical effect of a polyneuropathy
depends on
1) what modalities involved
2) what fibers are effected
3) whether the injury is axonal or demyelinating.

Adapted from http://www.neuroanatomy.wisc.edu/SClinic/Weakness/Weakness.htm


• Efek klinis dari polineuropati tergantung
pada? 1) modalitas apa yang ada? 2)
serat apa yang terkena? 3) apakah cedera
itu bersifat aksonal atau pelonggaran.
The clinical response to motor nerve injury

Loss of function Disturbed function


“- symptoms” “+ symptoms”

Motor nerves Wasting Fasiculations


Hypotonia Cramps
Weakness
Hyporeflexia
Orthopedic deformity
www.neuro.wustl.edu/neuromuscular/pics/people/patients/Hands/handatrophymnd3.jpg
The clinical response to sensory nerve injury

Loss of function Disordered function


“- symptoms” “+ symptoms”

Sensory ↓ Vibration Paresthesias


“Large Fiber” ↓ Proprioception
Hyporeflexia
Sensory ataxia

Sensory ↓ Pain Dysesthesias


“Small Fiber” ↓ Temperature Allodynia
The clinical response to autonomic nerve injury

Loss of function Disturbed function


“- symptoms” “+ symptoms”

Autonomic nerves ↓ Sweating ↑ Sweating Hypertension


Hypotension
Urinary retention
Impotence
Vascular color changes
http://www.neuro.wustl.edu/neuromuscular/nother/skel.html#nosteo
Physical exam
• Look for thickening of nerves (Neurofibromas)
• Decreased pinprick, sensation, or temperature
• Decreased reflexes
• Motor weakness

Tinnel’s testing
• Tapping over nerve creates tingling
• Examples
– Wrist (Carpal tunnel syndrome)
– Elbow (Funny bone)
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• Ujian fisik
Carilah penebalan saraf (Neurofibroma)
Turunkan pinprick, sensasi, atau suhu
Berkurangnya refleks
Kelemahan motor

Pengujian Tinnel
Mengetuk saraf membuat kesemutan
Contoh
Pergelangan tangan (Carpal tunnel syndrome)
Siku (tulang yang lucu)
Lab evaluation
• Might included CBC,
• Erythrocyte sedimentation rate,
• Urinalysis,
• Glucose serum,
• Serum protein electrophoresis,
• Thyroid function testing

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• Evaluasi lab
Mungkin termasuk CBC,
Tingkat sedimentasi eritrosit,
Urinalisis,
Glukosa serum,
Elektroforesis protein serum,
Pengujian fungsi tiroid
The two types of peripheral neuropathies:
axonopathies and myelinopathies
Electrical diagnosis
• Demyelination
– Slows nerve conduction velocity
– Conduction block possible

• Axonal degeneration
– Decreases amplitude of action potentials

• Helps differentiate between:


– Muscle vs. nerve problem vs. neuromuscular junction
– Root vs. distal nerve location
– Single vs. multiple nerves
– Upper vs. lower motor neuron dz
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• Diagnosis listrik
Demyelination
Memperlambat kecepatan konduksi saraf
Kemungkinan blok konduksi

Degenerasi aksonal
Mengurangi amplitudo potensial aksi

Membantu membedakan antara:


Masalah otot vs saraf vs persimpangan neuromuskular
Lokasi syaraf akar vs distal
Single vs multiple syaraf
Motor neuron atas vs bawah dz
Using nerve conduction studies in polyneuropathy

= Low!

= Slow!

= Slow!

Copyright ©2002 BMJ Publishing Group Ltd.


Hughes, R. A C BMJ 2002;324:466-469 http://www.neuroanatomy.wisc.edu/SClinic/Weakness/Weakness.htm
Myelinopathies

• Unusual by comparison with axonopathies


• Clues: hypertrophic nerves on exam
global arreflexia
weakness without wasting
motor << sensory deficits
NCS can discriminate inherited from acquired

• NCS: Distal motor latency prolonged (<125% ULN)


Conduction velocities slowed (<80% LLN)
May have conduction block
EMG: Reduced recruitment w/o much denervation
• Myelinopathies
Tidak biasa dibandingkan dengan axonopathies
Petunjuk: saraf hipertrofik pada pemeriksaan
arreflexia global
kelemahan tanpa menyia-nyiakan
motor << defisit sensorik
NCS dapat membedakan warisan dari akuisisi

NCS: latency motor distres berkepanjangan (<125% ULN)


Kecepatan konduksi melambat (<80% LLN)
Mungkin ada blok konduksi
EMG: Mengurangi rekrutmen dengan banyak denervasi
Axonopathies

• By far the majority of the toxic, metabolic and


endocrine causes
• NCVs: CMAPs ↓ 80% lower limit of normal w/o
or min velocity or distal motor latency change.
• Legs<< arms.
• EMG: Signs of denervation (acute, chronic) and
reinnervation
• Axonopathies
Sejauh ini sebagian besar penyebab
toksik, metabolik dan endokrin
NCVs: CMAPs ↓ 80% batas bawah
kecepatan normal w / o atau min atau
distorsi motor distal.
Kaki << lengan
EMG: Tanda denervasi (akut, kronis) dan
reinnervasi
Nerve biopsy
• May be required to rule out:
– Vasculitis
– Amyloid
– Leprosy
– Sarcoidosis

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• Biopsi saraf

Mungkin diperlukan untuk


mengesampingkan:
Vaskulitis
Amyloid
Kusta
Sarkoidosis
Normal Nerve Axonal degeneration

http://missinglink.ucsf.edu/lm/ids_104_musclenerve_path/student_musclenerve/nervepath.html
From Kumar: Robbins and Cotran: Pathologic Basis of Disease, 7th ed.
Wallerian Degeneration

http://missinglink.ucsf.edu/lm/ids_104_musclenerve_path/student_musclenerve/nervepath.html
Segmental Demyelination

Normal

Demyelination

Normal

Demyelination

http://www.neuro.wustl.edu/neuromuscular/pathol
Question #2. What is the etiology?

Only a limited number of ways a peripheral


nerve can react to injury, thus a multitude
of different etiologies can cause similar
effects…
• Pertanyaan # 2. Apa etiologi itu?
Hanya sejumlah kecil cara saraf perifer
dapat bereaksi terhadap cedera, sehingga
banyak etiologi yang berbeda dapat
menyebabkan efek yang sama ...
Problem: The multitude causes of peripheral neuropathy!!!

Inherited: e.g. Charcot-Marie-Tooth disease (HMSN)


Infectious: e.g. Leprosy
Inflammatory: e.g. Guillain Barre syndrome (AIDP)
Neoplastic: e.g. Monoclonal gammopathy
Metabolic: e.g. Diabetes
Drug: e.g. Vincristine
Toxic: e.g. Ethanol
• Masalah: Banyaknya penyebab neuropati perifer !!!
Warisan: mis. Penyakit Charcot-Marie-Tooth (HMSN)
Infeksi: mis. Kusta
Peradangan: mis. Sindrom Guillain Barre (AIDP)
Neoplastik: mis. Gammopati monoklonal
Metabolik: mis. Diabetes
Obat: mis. Vincristine
Beracun: mis. Etanol
How then are we to sort through the causes to
make an etiologic diagnosis?...

Use the 6 D’s….


• What is the distribution of the deficits?
• What is the duration?
• What are the deficits (which fibers are involved)?
• What is the disease pathology (axonal or
demyelinating or mixed)
• Is there an inherited (developmental) neuropathy?
• Is there drug/toxin exposure?
• Lalu bagaimana kita bisa memilah-milah penyebabnya untuk
membuat diagnosis etiologi? ... ?? Gunakan 6 D's ....
Berapa distribusi defisitnya?
Berapa lama?
Apa defisit (serat mana yang terlibat)?
Apa patologi penyakit (axonal atau demyelinating atau mixed)
Apakah ada neuropati bawaan (perkembangan)?
Adakah paparan obat / toksin?
1. What is the distribution of the deficits?

• Asymmetry
1. Mononeuropathy
2. Mononeuritis multiplex – e.g. vasculitis

• Symmetric (glove/stocking) = polyneuropathy


• 1. Bagaimana distribusi defisit?
Asimetri
1. Mononeuropati
2. Mononeuritis multipleks - mis.
vaskulitis

Simetris (sarung tangan / kaus kaki) =


polyneuropathy
Mononeuropathies
• Ulnar neuropathy
• Carpal tunnel syndrome
• Tarsal tunnel syndrome
• Bell’s palsy

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• Mononeuropati
Ulnar neuropati
Sindrom terowongan karpal
Sindrom terowongan tarsal
Suara yang rendah
Ulnar neuropathy
• Just distal to the elbow cubital tunnel
entrapped
• Results in a claw hand if severe

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• Ulnar neuropati
Hanya distal ke terowongan cubital siku
yang terperangkap
Hasil di tangan cakar jika parah
Carpal tunnel syndrome
• Perhaps the most common
mononeuropathy
• Entrapment of median nerve in the wrist
• Results in paresthesias of thumb, index,
and middle finger; Weakness of the
abductor pollicus brevis
• Tingling fingers, weak thumb, loss of
“meat” of the APB muscle (atrophy)
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• Sindrom terowongan karpal
Mungkin mononeuropati yang paling
umum
Meraba saraf median di pergelangan
tangan
Hasil di parestesia pasak, indeks, dan jari
tengah; Kelemahan penculik pollicus
brevis
Kesemutan jari, jempol lemah, kehilangan
"daging" otot APB (atrofi)
Carpal tunnel syndrome-Causes
• Usually due to overuse
– Typing probably okay (argued)
• Other causes
– Arthritis
• Osteoarthritis
• Rheumatoid arthritis
– Infiltrative diseases
– Hypothyroidism
– Diabetes
– Pregnancy

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• Sindrom terowongan karpal-penyebabnya
Biasanya karena terlalu sering digunakan
Mengetik mungkin oke (diperdebatkan)
Penyebab lainnya
Radang sendi
Osteoarthritis
Radang sendi
Penyakit infiltrasi
Hipotiroidisme
Diabetes
Kehamilan
Carpal tunnel syndrome-Treatment
• Treatment is usually surgical resection of carpal
ligament
• Other treatments may help, too
– Stretches
– Splints
• Cock-up wrist splints
• Night time use only ?
– Anti-inflammatory medications
• Oral (non-steroidal)
• Injections

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• Sindrom terowongan karpal - perawatan
Pengobatannya biasanya reseksi bedah ligamen karpal
Perawatan lainnya juga bisa membantu
Peregangan
Belat
Cock-up pergelangan tangan splints
Penggunaan waktu malam saja?
Obat anti-inflamasi
Oral (non-steroid)
Suntikan
2. What is the duration?

Ask: Acute or Chronic?

• Most polyneuropathies are chronic – ++months-yrs

• Acute polyneuropathies
e.g. Guillain Barre syndrome
Vasculitis

• Relapses and remissions


e.g. Intermittent toxin exposure
• 2. Berapa durasi?
Tanyakan: Akut atau Kronis?

Kebanyakan polineuropati kronis - ++ bulan-yrs

Polineuropati akut
misalnya Sindrom Guillain Barre
Vaskulitis

Relaps dan remisi


misalnya Paparan toksin intermiten
3. What are the deficits (which fibers affected)?

• If predominant motor fibers think of:


Guillain Barre syndrome
Lead toxicity
Charcot-Marie-Tooth disease

• If pure sensory/ severe proprioceptive deficit, think of sensory


neuronopathy:
Carcinoma (paraneoplastic)
Vitamin B6 toxicity

• If autonomic nerves involved (small fiber) think of:


Diabetes
Amyloid
Drugs like vincristine, ddI, ddC
• 3. Apa defisit (serat yang terpengaruh)? ???

Jika serat motor utama berfikir:


Sindrom Guillain Barre
Toksisitas timah
Penyakit Charcot-Marie-Tooth

Jika defisit proporsional / defisit proprioseptif murni, pikirkan


neuronopati sensorik:
Karsinoma (paraneoplastik)
Toksisitas vitamin B6

Jika saraf otonom terlibat (serat kecil) pikirkan:


Diabetes
Amyloid
Obat-obatan seperti vincristine, ddI, ddC
4. What is the disease pathology?

• The vast majority are axonal.

• Demyelination a key finding because its causes are


relatively few.

• If demyelination uniform the cause is hereditary.


e.g. Charcot-Marie Tooth type I (HMSN)

• If otherwise unremarkable chronic sensorimotor


axonal polyneuropathy… exclude
alchohol, diabetes, hypothyroidism, uremia,
B12 deficiency & monoclonal gammopathy
• 4. Apa patologi penyakitnya?
Sebagian besar adalah aksonal.

Demyelination merupakan temuan kunci karena penyebabnya relatif sedikit.

Jika demyelination uniform penyebabnya turun temurun.


misalnya Charcot-Marie Tooth tipe I (HMSN)

Jika polineuropati sensorimotorik polosiklinik yang tidak biasa ... tidak


diikutsertakan
5. Is there an inherited (developmental) neuropathy?

• Among the most common!


• Clues – orthopedic deformities (feet, spine)
– long duration
– indolent progression
– few “positive” symptoms
– examine/question the family members!
• 5. Apakah ada neuropati bawaan
(perkembangan)?
Di antara yang paling umum!
Petunjuk - deformitas ortopedi (kaki,
tulang belakang)
- durasi panjang
- Perkembangan lamban
- Beberapa gejala "positif"
- memeriksa / mempertanyakan anggota
keluarga!
6. Drug or toxin exposure?

Demyelinating Axonal

e.g. e.g.
Glue sniffing Cancer drugs like vincristine
Arsenic and paclitaxel
Antibiotics like chloroquine,
ethambutol, isoniazid and
metronidazole
Cardiac medications like
amiodarone
• 6. Paparan obat atau toksin?
Demyelinating

misalnya
Lem mengendus
Arsenik

Axonal
misalnya
Obat kanker seperti vincristine dan paclitaxel
Antibiotik seperti klorokuin, etambutol, isoniazid dan metronidazol
Obat jantung seperti amiodarone
History of drug use
• Amiodarone
• Chemotherapeutics
– Cisplatin
– Taxol
• Antibiotics
– Metronidazole
– INH
– Anti-retrovirals
• Heavy metals

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• Sejarah penggunaan narkoba
Amiodarone
Kemoterapi
Cisplatin
Taxol
Antibiotik
Metronidazol
INH
Anti-retroviral
Logam berat
Polyneuropathy Example #1

• 58 year old movie industry executive


• 2 yrs toe numbness, paresthesias and pain
• Stocking numbness of toes with absent ankle jerks
• No medical history or family history or medications
• Multiple consultations & lab testing without etiologic
diagnosis
• Contoh Polyneuropathy # 1
Eksekutif industri film berusia 58 tahun
2 yrs toe mati rasa, parestesia dan rasa
sakit
Menorehkan mati rasa jari kaki dengan
sentakan pergelangan kaki
Tidak ada sejarah medis atau riwayat
keluarga atau pengobatan
Beberapa konsultasi & pengujian
laboratorium tanpa diagnosis etiologi
(A common axonal polyneuropathy)
Ethanol Neuropathy

• Among the most common neuropathies worldwide


• Chronic
• Numbness, paresthesias, pain in stocking distribution
• Sensory <<< Motor
• Loss of ankle reflexes
• History!
• Ethanol toxicity and nutritional deficiency
• Vitamin B1 (thiamine)
• (Polineuropati axonal yang umum)? Etanol Neuropati?
Di antara neuropati yang paling umum di seluruh dunia
Kronis
Mati rasa, parestesia, nyeri dalam distribusi tebar
Sensory <<< Motor
Kehilangan refleks pergelangan kaki
Sejarah!
Toksisitas etanol dan defisiensi nutrisi
Vitamin B1 (tiamin)
Polyneuropathy Example #2

• 23 yr old professional baseball player with no past


medical or family history & no medications.
• Severe pain in back and flank followed by
weakness over hours to inability to walk.
• Severe weakness legs, milder weakness arms
• Arreflexia
• Numbness of feet
• Diarrheal illness 2 weeks ago
• Contoh Polyneuropathy # 2
Pemain baseball profesional berusia 23 thn yang tidak
memiliki riwayat medis atau keluarga sebelumnya dan
tidak memiliki obat.
Nyeri parah di punggung dan sayap diikuti oleh
kelemahan selama berjam-jam agar tidak bisa berjalan.
Kaki lemah parah, lengan lemah lebih ringan
Arreflexia
Mati rasa kaki
Penyakit diare 2 minggu yang lalu
(A common demyelinating polyneuropathy)
Guillain-Barre Syndrome

• Rapid, severe, typically ascending paralysis


• Post infectious in 60%
• Paresthesias, pain, numbness
• Autonomic nerves
• Reflexes lost
• Cytoalbuminologic dissociation in the CSF
• (Polineuropati demyelinating yang
umum)? Guillain-Barre Syndrome?
Kelumpuhan cepat, parah, biasanya
menaik
Post menular di 60%
Parestesia, nyeri, mati rasa
Saraf otonom
Refleks hilang
Disosiasi cytoalbuminologic di CSF
Polyneuropathy Example #3

• 55 year old obese woman


• Family history positive for diabetes
• 4-5 years of nocturia and 1-2 years of polyuria
• Dry skin over the feet
• Stocking numbness in all modalities to the ankles
• Absent ankle reflexes
• Contoh Polyneuropathy # 3?
Wanita gemuk berusia 55 tahun
Riwayat keluarga positif untuk diabetes
4-5 tahun nokturia dan polyuria 1-2 tahun
Kulit kering di atas kaki
Menebar mati semua modalitas ke
pergelangan kaki
Absen refleks pergelangan kaki
(A common mixed axonal & demyelinating polyneuropathy)
Diabetic Polyneuropathy

• Multiple forms of neuropathy in diabetes


• Sensory <<< motor polyneuropathy
• Autonomic involvement common
• CSF protein frequently elevated
• Glucose control!
• Foot care
• (Polineuropati axonal & demyelinating
campuran yang umum) Polineuropati
Diabetik?
Beberapa bentuk neuropati pada diabetes
Sensori <<< polineuropati motor
Keterlibatan otonomi umum terjadi
Protein CSF sering meningkat
Kontrol glukosa
Perawatan kaki
Peripheral Neuropathy in summary…

1. Patterns: mononeuropathy, mononeuropathy multiplex or


polyneuropathy – focal, multifocal or diffuse

2. “Signature” manifestations of a polyneuropathy depend


on what modalities affected (motor, sensory, autonomic)
and whether it is axonal or demyelinating.

3. Examination, NCS/EMG & biopsy can discriminate


axonopathy from myelinopathy

4. The multiple potential etiologies of polyneuropathy are


manageable recognizing patterns of disease by the 6 Ds
• Neuropati perifer secara ringkas ... ??
1. Pola: mononeuropati, mononeuropati multipleks atau polineuropati - fokal,
multifokal atau menyebar

2. Manifestasi "tanda tangan" dari polineuropati tergantung pada modalitas yang


terpengaruh (motorik, sensorik, otonomik) dan apakah itu aksonal atau
demyelinating.

3. Pemeriksaan, NCS / EMG & biopsi dapat membedakan aksonopati dari mielinopati

4. Etiologi polinuropati berpotensi ganda dapat dikelola dengan mengenali pola


penyakit oleh 6 Ds
Plan of the Nervous System
UMN
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vv
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vv

m.
v drg T1-L2
c ^
DorC <
^ o
r < Sy
d ^
Spth

r. III,VII,IX,X
LMN g. S2-4
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Motor Sensory Autonomic <^

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