NEUROPATI

Definisi
Gangguan fungsional atau organik dari saraf perifer
Gangguan ini dapat mengenai :
Saraf sensorik
Saraf motorik
Saraf otonom
Kombinasi

Klasifikasi
Banyak klasifikasi dari neuropati
I. Menurut Onset Serangan
1. Neuropati Akut
Mis. : Acute idiopathic polyneuropathy
2. Neuropati Kronik
Mis. : Beri-beri, diabetes, leprosy
II. Menurut Derajat
1. Ringan : sensorik saja
2. Sedang : sensorik, motorik, penurunan refleks
3. Berat : sensorik, motorik, penurunan refleks, atrofi otot
III. Menurut Jumlah Saraf Yang Terlibat
1. Mononeuropati Simpleks
Gangguan pada satu saraf perifer saja
C/: Carpal Tunnel Syndrome n. Medianus
2. Mononeuropati Multipleks
Mengenai beberapa saraf tepi, biasanya tidak berdekatan
dan tidak simetris
3. Polineuropati
Beberapa saraf tepi, simetris dan serentak
Biasanya predominan daerah distal
IV. Menurut Letak Lesi
1. Distal Axonopathies gangguan pada akson
2. Myelinopathies gangguan pada myelin sheath
3. Neuropathies gangguan pada nerve cell bodies di cornu anterior, medula
spinalis atau pada dorsal root ganglion

Etiologi
I. Idiopathic Inflammatory Neuropathies
Acute idiopathic polyneuropathy (Guillain Barre Syndrome)
Chronic Inflammatory Demyelinating Polyneuropathy

II. Metabolic and Nutritional Neuropathies

Diabetes, Hypotyroidism, Acromegali
Uremia
Liver Disease
Vit. B1, Vit. B12 deficiency

III. Infective and Granulomatous Neuropathies

AIDS, Leprosy, Diphteria, Sarcoidosis

IV. Vasculitis Neuropathies

Polyarteritis Nodosa
Rheumatoid Arthritis
Systemic Lupus Erithematosus

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V. Neoplastic and Paraproteinemic Neuropathies
Compression and Iritation by tumor
Paraneoplastic syndrome
Paraproteinemias
Amyloidosis

VI. Drugs Induced and Toxic Neuropathies

Dapsone, Isoniazide, Phenitoin, Pyridoxine, Vincristine,
Hidralazine
Alcohol
Toxin : organophosphat, arsenic, lead, thalium, gold

VII. Hereditary Neuropathies

Idiopathic
Hereditary Motor and Sensory neuropathies
Hereditary Sensory neuropathies
Familial Amyloidosis
Metabolic
Porphiria
Metachromatic leucodistrophy
Abetalipoproteinemia

VIII. Entrapment Neuropathies

Upper Limbs
Median nerve (carpal tunnel syndrome)
Ulnar nerve
Radial nerve
Lower Limbs
Peroneal nerve
Femoral nerve
Obturator nerve

Most Common Diseases Affecting The Peripheral Nerves

DANG THE RAPIST
Diabetes Trauma Rheumatic(Collagen Vascular)
Alcohol Hereditary Amyloid
Nutritional Environmental Paraneoplastic
Guillan Barre toxin and drugs Infections
Systemic diseases
Tumors

Patofisiologi
Ada beberapa proses patologi yang mengenai serabut saraf a.l.:
1. Degenerasi Wallerian
Terjadi degenerasi akson dan selubung mielin kearah distal dari lesi.
Degenerasi bisa juga ke proksimal satu atau dua segmen
2. Demielinisasi Segmental
Timbul bila terjadi lesi pada sel schwann
Proses dimulai di daerah nodus ranvier dan meluas tak teratur mengenai
segmen-segmen internodus lain.
Akson dapat mengalami degenerasi atau tidak terganggu sama sekali.
3. Degenerasi Akson Primer
Disebut juga dengan aksonopati.
Degenerasi akson ini biasanya di ikuti oleh demielinisasi segmental yang
sekunder.
Sering pada uremia, keracunan alkohol, lepra, karsinoma

Kerusakan saraf dibagi 3 tingkat penting untuk menentukan prognose.

1. Neuropraksia :
Kerusakan paling ringan
Hanya terjadi gangguan hantaran
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 Tanpa gangguan kontinuitas
Pemulihan terjadi dalam beberapa menit sampai beberapa minggu

2. Aksonotmesis :
Kerusakan pada akson disertai degenerasi
Tanpa kerusakan endoneural
Regenerasi kemungkinan dapat terjadi dengan hasil yang baik

3. Neurotmesis :
Saraf terputus total atau sebagian
Pengobatan dgn penyambungan
Kemungkinan perbaikan 50%

Gejala Klinik
1. Gangguan Sensorik
Involvement of sensory axons produces impairment of sensation with
dysesthesias or paresthesias.
Rasa kaku, dingin, pedas
Gatal dan kebas-kebas
Nyeri seperti ditusuk jarum
Rasa terbakar
Rasa berjalan di atas kapas
Rasa tersandung waktu berjalan
Rasa tidak stabil

2. Gangguan Motorik
Involvement of motor axons produces muscle wasting and weakness followed by
atrophy and fasciculations
Kelemahan bersifat lmn
Sulit memutar kunci pintu
Sulit membuka kancing baju
Sulit memutar tutup botol
Foot drop
Wrist drop
Gangguan gerakan tangkas

3. Ganguan Refleks Tendon

The tendon reflexes supplied by the affected nerve are depressed or absent.
Contoh :
Refleks tendon biseps
Refleks tendon triseps
KPR
APR

4. Ganguan Otonomik
Involvement of axons supplying autonomic function produces loss of sweating,
alteration in bladder fuction, constipation, and impotence in male
Contoh :
Gangguan gastrointestinal : diare, konstipasi, dilatasi lambung, mual
dan muntah.
Gangguan kandung kemih : atoni kandung kemih, residu urine
Impotensi
Gangguan kardiovaskuler : hipotensi ortostatik, sinkop
Gangguan berkeringat
Cardio respiratory arrest

Predominantly Motor Neuropathies

Guillain-Barre Syndrome
Diphtheric neuropathy
Dapsone-induced neuropathy
Porphyria and multifocal motor neuropathy
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Predominantly Sensory Neuropathies
Drug toxicity : pyridoxine, doxorubicine
Autoimmune : paraneoplastic, Sjogren syndrome, etc.
Infectious : diphtheria, HIV
Deficiency : vit. E
Inherited : abetalipoproteninemia

Diagnosa
1. Gejala klinik
2. Laboratorium
3. Foto thoraks
4. Punksi lumbal
5. EKG
6. Biopsi : paling sering n. suralis atau n. Cutaneus, n. radialis
7. Elektrofisiologi : EMG, NCV

ELEKTROMIOGRAFI
Elektroda ditusukkan kedalam suatu otot skelet untuk
mempelajari perubahan potensial listriknya

Indikasi : gangguan lower motor neuron, yang lesinya di :

1. Kornu anterior
2. Radiks
3. Pleksus
4. Saraf perifer
5. Neuromuscular junction
6. Otot

Manfaat EMG :
1. Membantu diagnosa secara dini
2. Menentukan letak lesi
3. Membedakan lesi miogen atau neurogen
4. Menentukan lesi parsial atau total
5. Membedakan sensorik atau motorik
6. Evaluasi pengobatan
7. Membantu menentukan prognose

NERVE CONDUCTION VELOCITY (NCV)/KECEPATAN HANTAR SARAF

Nilai normal :
N. Ulnaris = 47-72 m/s
N. Medianus = 46-72 m/s
N. Peroneus = 42-63 m/s
N. Tibialis = 40-67 m/s

Distal Latency (DL)

Nilai normal n. Medianus : 2,7 + 0,3 m/s

Manfaat pengukuran KHS :

Mengikuti perjalanan penyakit
Mengevaluasi efek pengobatan
Menentukan prognose, apakah masih mungkin diperoleh perbaikan lagi.

EMG dan KHS pada neuropati :

Dijumpai penurunan KHS
Pemanjangan distal latency
Penurunan amplitudo gelombang M
Durasi yang memanjang
Potensial polifasik
Fibrilasi

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NEUROPATI DIABETIK
Prevalensi : 10 - 20 % (simtomatik)
KHS 80 % abnormal
Klinis dapat mengenai :
Sensorik
Motorik
Otonomik
Kombinasi

Patogenese Neuropati Diabetik

The etiology is uncertain.
4 hypothesis (not necessarily exclusive) :
1. Hyperglycemia-polyol-myoinositol hypothesis.
2. Microvascular hypothesis
3. Structural changes at the node of Ranvier.
4. Vasculitic neuropathy.

1. HYPERGLYCEMIA-POLYOL-MYOINOSITOL HYPOTHESIS
Normal : glucose hexokinase glucose-6-phosphate Krebs
cycle.
Hyperglycemia saturates hexokinase activity glucose
shunted to polyol pathway production of sorbitol assoc w/ a decrease in
intracelluler myoinositol defective Na/K ATPase activity defect axon
transport slowing NCV

2. MICROVASCULAR HYPOTHESIS
DM : thickening of capillary basement membrane
increase in the size and number of capillary endothelial cells
Microangiopathy increase number of closed capillaries in
peripheral nerves progressive hypoxia secondary changes in axons and
Schwann cells

3. STRUCTURAL CHANGES AT THE NODE OF RANVIER

Na/K ATPase defiency increase intra-axonal Na and nodal
axonal swelling detachment of myelin myelin retraction from the nodal
area slowing of axonal conduction.
Exposure of paranodal K channels leakage of K impairment
of axonal conduction.
Impairment of axonal transport gradual dying back of axons
starting at the distal axons and progressing proximally.

4. VASCULITIC NEUROPATHY
Some cases of NIDDM and proximal diabetic have a inflammatory
vasculopathy with perivascular collections of lymphocytes and axonal
neuropathy

PAINFUL DIABETIC NEUROPATHY

Cranial nerve neuropathy
Acute thoracoabdominal neuropathy
Acute distal sensory neuropathy
Acute lumbar radiculoplexopathy
Chronic distal small-fiber neuropathy

Terapi
Intensive diabetic therapy
Maintain ideal body weight
Adjuvant analgetics :
TCA antidepressants
carbamazepine

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 gabapentin
intravenous lidocaine, etc

CARPAL TUNNEL SYNDROME

Characterized by : fluctuating numbness, paresthesia and pain in the hand due to
compression of the median nerve at the wrist.
80% in women, a common temporary phenomenon during pregnancy
Pressure to the nerve when passing beneath the flexor retinaculum obstruction
of venous circulation and edema ischemia increasing pressure on the nerve
ischemic atrophy of nerve fibers

Etiologi
1. Hereditary : HMSN type III
2. Traumatic : Dislocation, fracture, hematoma, wrist sprain
3. Infection : Tenosynovitis, TBC, sarcoidosis
4. Metabolic : Amyloidosis, gout
5. Endocrine : Acromegaly, DM, hypothyroidism, pregnancy
6. Neoplastic : Ganglion cysts, lipoma , myeloma
7. Collagen vascular diseases : RA, polymyalgia rheumatica, SLE
8. Degenerative disease : OA
9. Iatrogenic : Radial artery puncture, shunt for dialysis, anticoagulant therapy

Gejala Klinis
The earliest symptoms : numbness and paresthesias in the sensory
distribution of the median nerve in the hand (thumb, index, middle and lateral
half of the ring finger)
Later on : pain, worst at night
Late : inability to screw bottle caps or grip properly

Terapi
Identified causes should be treated
Corticosteroid injection around the median nerve in the carpal tunnel.
Surgical division of the transverse ligament (flexor retinaculum)
Endoscopic carpal tunnel release

GUILLAIN - BARRE SYNDROME (GBS)

Acute Inflammatory Post Infectious
Polyneuropathy
Insiden : 2 PER 100.000 POPULASI PERTAHUN
1-3 minggu setelah infeksi : virus, bakteri, imunisasi
Inflamasi terhadap serabut saraf merupakan respon autoimmun baik melalui
reaksi antibodi maupun cell mediated response
Terjadi demielinasi segmental disertai dengan kerusakan akson bila prosesnya
berat
Dijumpai infiltrasi limfosit perivaskuler pada saraf perifer dan nerve roots
Limfosit dan makrofag menghasilkan sitotoksin yang merusak mielin
Kelumpuhan keempat anggota gerak
Umumnya dimulai dari tungkai, meluas keatas, lengan, otot leher dan wajah
kadang-kadang otot menelan
Sebagian besar kasus mengeluh parastesi pada ekstremitas inferior
Gangguan otonomik dijumpai pada 25% kasus
Pada LP dijumpai disosiasi sitoalbumin

Kriteria Diagnostik
Dijumpainya 5 dari 6 kriteria ini :
1. Diffuse flaccid paralysis
2. Gangguan sensorik < gangguan motorik
3. Remisi sempurna dalam 6 bulan
4. Peningkatan protein pada CSF dalam 2 minggu
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5. Demam (-) atau suhu sedikit meninggi
6. Leukosit normal hanya kemungkinan LED sedikit meninggi

Predictors Of Severe Disease and Poorer Outcome

Old age
Rapid onset of severe tetraparesis
Need for early artificial ventilation
Severely decreased compound muscle action potential (<20% normal)
Acute motor-sensory axonal form of the disease

Terapi
1. Plasmapharesis
2. Immunoglobulin IV 0,4 gr/kg BB selama 5 hari
3. Perawatan umum
4. Fisioterapi
5. Perawatan di ICU bila terjadi gagal nafas

KSY-183 Mira Yulianti (01-107)