NEUROPATHY

Bagian neurologi FK UISU

DEFINISI
NEUROPATI ADALAH GANGGUAN FUNGSIONAL
ATAU ORGANIK DARI SARAF PERIFER
GANGGUAN INI DAPAT MENGENAI :
SARAF SENSORIK
SARAF MOTORIK
SARAF OTONOM
KOMBINASI

CLASSIFICATION
1. BASED ON THE ONSET OF NEUROPATHY:
ACUTE NEUROPATHY
eg. : ACUTE IDIOPATHIC POLYNEUROPATHY
CHRONIC NEUROPTHY

eg.

: BERI BERI
DIABETES MELLITUS
LEPROSY

2. BASED ON SEVERITY

1. MILD NEUROPATHY :
SENSORY ONLY
2. MODERATE NEUROPATHY :
SENSORY, MOTOR, AND DECREASE
OF
TENDON REFLEXES
3. SEVERE NEUROPATHY :
SENSORY, MOTOR, DECREASE OF TENDON
REFLEXES, MUSCLE ATROPHY

3.

BASED ON THE NUMBER OF

NERVES INVOLVED

1. MONONEUROPATHY SIMPLEX :
ONLY ONE PHERIPHERAL NERVE INVOLVED.
2. MONONEUROPATHY MULTIPLEX :
SEVERAL NERVES INVOLVED IN DIFFERENT AREAS
AND USUALLY ASSYMMETRIC.
3. POLYNEUROPATHY :
SEVERAL NERVES INVOLVED, SYMMETRICAL,
SAME ONSET AND DISTAL PREDOMINANT.

4. BASED ON LESION SITE

1 DISTAL AXONOPATHY :
AXONAL LESION
2. MYELINOPATHY :
DISORDER OF MYELIN SHEATH.
3. NEUROPATHY :
DISORDER OF CELL BODY AT ANTERIOR
HORN CELLS, SPINAL CORD
OR DORSAL
ROOT GANGLION.

ETIOLOGY
1. IDIOPATHIC INFLAMMATORY NEUROPATHIES
-

ACUTE IDIOPATHIC POLYNEUROPATHY

(GUILLAIN BARRE SYNDROME)
- CHRONIC INFLAMMATORY DEMYELINATING
POLYNEUROPATHY
2. METABOLIC AND NUTRITIONAL NEUROPATHIES
- DIABETES, HYPOTHYROIDI, ACROMEGALY
- UREMIA
- LIVER DISEASES
- VIT B1, OR VIT B12 DEFICIENCY

ETIOLOGY
3. INFECTIVE AND GRANULOMATOUS
NEUROPATHIES:
AIDS, LEPROSY. DIPHTHERY, SARCOIDOSIS
4. VASCULITIS NEUROPATHIES:
- POLYARTERITIS NODOSA
- RHEUMATOID ARTHRITIS
- SYSTEMIC LUPUS ERYTHEMATOSUS

ETIOLOGY
5. NEOPLASTIC AND PARAPROTEINEMIC
NEUROPATHIES:
- COMPRESSION AND IRITATION BY TUMOR
- PARANEOPLASTIC SYNDROME
- PARAPROTEINEMIAS
- AMYLOIDOSIS

ETIOLOGY
6. DRUGS INDUCED AND TOXIC NEUROPATHIES
- DAPSONE, ISONIAZIDE, PHENYTOIN, PIRIDOXYNE,
VINCRISTIN, HIDRALAZINE.
- ALCOHOL
- TOXINS : ORGANOPHOSPHAT
ARSENIC
LEAD
THALIUM
GOLD

ETIOLOGY (cont.d)
7. HEREDITARY NEUROPATHIES
- IDIOPATHIC
HEREDITARY MOTOR AND SENSORY NEUROPATHIES
HEREDITARY SENSORY NEUROPATHIES
FAMILIAL AMYLOIDOSIS

- METABOLIC
PORPHYRIA
METACHROMATIC LEUCODYSTROPHY
ABETALIPOPROTEINEMIA

ETIOLOGY
8. ENTRAPMENT NEUROPATHIES
- UPPER LIMBS
MEDIAN NERVE (CARPAL TUNNEL SYNDROME)
ULNAR NERVE
RADIAL NERVE

- LOWER LIMBS
PERONEAL NERVE
FEMORAL NERVE
OBTURATOR NERVE

PATHOPHYSIOLOGY
ADA BEBERAPA PROSES PATOLOGI YANG
MENGENAI SERABUT SARAF a.l.:
1. WALLERIAN DEGENERATION
TERJADI DEGENERASI AKSON DAN SELUBUNG
MIELIN KEARAH DISTAL DARI LESI.
DEGENERASI BISA JUGA KE PROKSIMAL SATU ATAU
DUA SEGMEN.

PATHOPHYSIOLOGY
2. SEGMENTAL DEMYELINATION
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. PRIMARY AXONAL DENERATION

DISEBUT JUGA DENGAN AXONOPATHY.
DEGENERASI AKSON INI BIASANYA DI IKUTI OLEH
DEMIELINISASI SEGMENTAL YANG SEKUNDER.
SERING PADA UREMIA, KERACUNAN ALKOHOL,
LEPRA, KARSINOMA.

PATHOPHYSIOLOGY
KERUSAKAN SARAF DIBAGI 3 TINGKAT
PENTING UNTUK MENENTUKAN
PROGNOSE.
1. NEUROPRAKSIA:
- KERUSAKAN PALING RINGAN
- HANYA TERJADI GANGGUAN HANTARAN
- TANPA GANGGUAN KONTINUITAS
- PEMULIHAN TERJADI DALAM BEBERAPA MENIT
SAMPAI BEBERAPA MINGGU

PATHOPHYSIOLOGY
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%

CLINICAL SYMPTOMS
1. SENSORY SYMPTOMS :
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

CLINICAL SYMPTOMS
2. MOTOR SYMPTOMS :
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

CLINICAL SYMPTOMS
3. CHANGE OF TENDON REFLEXES
The tendon reflexes supplied by the affected
nerve are depressed or absent.

Contoh :
-

REFLEKS TENDON BISEPS

REFLEKS TENDON TRISEPS
KPR
APR

CLINICAL SYMPTOMS
4. AUTONOMIC :
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.

CLINICAL SYMPTOMS
GANGGUAN OTONOMIK (lanjutan) :
- GANGGUAN KANDUNG KEMIH :
ATONI KANDUNG KEMIH, RESIDU URINE
- IMPOTENSI
- GANGGUAN KARDIOVASKULER:
HIPOTENSI ORTOSTATIK, SINKOP
- GANGGUAN BERKERINGAT
- CARDIO RESPIRATORY ARREST

DIAGNOSA
1. GEJALA KLINIK
2. LABORATORIUM
3. FOTO THORAKS
4. PUNKSI LUMBAL
5. EKG
6. BIOPSI : paling sering n. suralis atau n. cutaneus
radialis
7. ELEKTROFISIOLOGI: EMG
NCV

ELEKTRO MIOGRAFI
ELEKTRODA DITUSUKKAN KEDALAM SUATU OTOT SKELET
UNTUK MEMPELAJARI PERUBAHAN POTENSIAL LISTRIKNYA.
INDIKASI:
GANGGUAN LOWER MOTOR NEURON, YANG LESINYA DI:
1.
2.
3.
4.
5.
6.

KORNU ANTERIOR
RADIKS
PLEKSUS
SARAF PERIFER
NEUROMUSCULAR JUNCTION
OTOT

DIABETIC NEUROPATHY

Neuropati diabetik :
adanya gejala dan atau tanda disfungsi saraf perifer pd orang
dgn diabetes setelah dieksklusikan penyebab lain.

Prevalensi : 10 - 20 % (simtomatik)

Neuropati diabetik :
50% pasien diabetes
tipe 1 lebih cepat dr tipe 2
sensorimotor kronik bentuk paling sering.
50% polineuropati diabetik kronik asimtomatis
10-20% mengganggu & membutuhkan terapi spesifik.

PATHOGENESIS
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 glucose6-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 intraaxonal 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

CLINICAL SYMPTOMS

DIAGNOSIS

THERAPY
Intensive diabetic therapy
Maintain ideal body weight
Adjuvant analgetics :
TCA antidepressants
carbamazepine
gabapentin
intravenous lidocaine, etc

Adjuvant Analgetics