Neuropathy

dr. Sumarnita Tarigan, SpS

Departemen Neurologi

1
 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. :
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.
 PATHOPHYSIOLOGY

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
 PATHOPHYSIOLOGY

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 autonomic (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. KORNU ANTERIOR
2. RADIKS
3. PLEKSUS
4. SARAF PERIFER
5. NEUROMUSCULAR JUNCTION
6. 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  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
CLINICAL SYMPTOMS
DIAGNOSIS
 THERAPY

• Intensive diabetic therapy

• Maintain ideal body weight
• Adjuvant analgetics :
TCA antidepressants
carbamazepine
gabapentin
intravenous lidocaine, etc
Adjuvant Analgetics
 Carpal tunnel syndrome

Definition

Compression neuropathy of the median nerve in

wrist area ( tardy median nerve palsy)

Described in 1854 by Sir James Paget

 anatomy

The median nerve

travels from the
forearm into your
hand through a
“tunnel” in your wrist.
 Carpal tunnel syndrome

Pressure on the median nerve can result

in; sensations of numbness, tingling,
pain and clumsiness of the hand.( typical
median N. distribution in the radial three and one – half
digits).

The combination of these symptoms is

called carpal tunnel syndrome
 Carpal tunnel syndrome
Most often 30 -60 years old
Five times more common in women
Older, overweight, and physically
inactive people
Carpal tunnel syndrome
 Etiology
1-primery or Idiopathic
2- secondary
A: Local etiology I: Anatomical malformation
II: Tumors
III: Infections
IV: Bone prominence
B: Systemic etiology
obesity, diabetes mellitus, thyroid
dysfunction, R.A
Clinical finding
 History often is more important than the physical
examination in making the diagnosis of CTS
 Numbness and tingling
hands fall asleep or things slip from the fingers
without the person's noticing (loss of grip, dropping
things), as well as numbness and tingling
 Symptoms are usually intermittent and are
associated with certain activities (i.e., driving,
reading the newspaper, crocheting, painting)
 Nocturnal symptoms that wake the individual are
more specific of CTS, especially if the patient
relieves symptoms by shaking the hand/wrist
Clinical finding

If pressure
continues- thenar
muscles can weaken
and atrophy
 diagnosis
History
Clinical examination I: Tinel's nerve percussion test
II: Phalen's wrist flexion test
III: Tourniquet test
IV: Carpal compression test
V: Tethered median nerve
stress test
Para clinical examination
diagnosis
Reverse Phalen Test
Electrodes are placed on the forearm and a mild electrical
current is passed through the arm.
EMG
 diagnosis

Electromyography

90% sensitive and 60% specific

Measurement of how fast & how well the median

nerve responds indicates if there is damage to
the nerve.
treatment

1- Non surgical treatment

2- Surgical treatment
Endoscopic release
1-age over 50
2-duration longer than 10 months
3-constant paresthesia
4- stenosing flexor tenosynovitis
5-positive phalen test less than 30 seconds
 Treatment
• splinting or bracing
• steroid injection
• activity modification
• physiotherapy
• regular massage therapy treatments
chiropractic
• medications
• surgical release of the transverse carpal
ligament
Non surgical treatment
Non surgical treatment
Surgical treatment

Surgical Decompression
Open or endoscopic (similar
success)
Only means of definitive cure (American
Academy of Neurologists)
Up to 86% improvement in pain
Complication 1-2% (higher in endoscopic)
Wilson JK, Sevier TL. A review of treatment for carpal tunnel syndrome. Clinical
Rehabilitation. 2003; 25:3:113-119.
Surgical complication

1- Infection
2- Nerve injury
3- Reflex Sympathetic Dystrophy
4- Painful scar
5- Bowstringing
6- Muscle weakness
7- Skin necrosis
Guillain-Barre Syndrome
 GBS
Is an acute autoimmune disease
Peripheral nerve myelin is target of an
immune attack
unknown cause / idiophatic
 Eventually get widespread patchy
demyel= increased paralysis
 Classification

1 2 4
miller

3
  AIDP :
as the traditional form as described previously,
dominant with motor symptom
 AMAN:
selective involv of motor nerves, more common in
Japan/China, almost all preceded by Campylobacter
infxn
 AMSAN:
more severe form of AMAN +sensory.
Poor prognosis.
 Miller Fisher Syndrome:
opthalmoplegia, ataxia, and areflexia.
 APN (Acute Pandysautonomic Neuropathy):
Encephalophati (rare). Symphatetic and
parasympathetic nerve failure.
 Pathophysiology
Usually postinfectious
Immune-mediated: infectious agents
thought to induce Ab production against
specific gangliosides/glycolipids
Lymphocytic infiltration of spinal
roots/peripheral nerves & then
macrophage invade myelin sheath caused
the axon unveiled
Result: defects in the propagation of
electrical nerve impulses, with eventual
conduction block and flaccid paralysis
 Clinical Features:
• Progressive, fairly symmetric muscle
weakness
• typically starts in distal legs
• In severe cases, resp muscle weakness
could occur.
• May include, sensory,motor, and
autonomic symptoms.
 How to diagnose GBS

• Check diagnostic criteria

• Exclude other causes
• Consider:
• Routine laboratory tests
• CSF examination
• Electrophysiological studies
 Diagnosis:

History and neurological.

Albuminocytologic dissociation: elevated
CSF protein w/ normal WBC.
Electromyography (EMG) helps confirm
diagnosis = prolonged or absent F waves
 When to suspect

• Rapidly progressive bilateral limb

weakness and/or sensory deficits
• Hypo/areflexia
• Facial or bulbar palsy
• Ophthalmoplegia and ataxia
DDX OF POLYNEUROPATHY:

Arsenic poisoning
N-Hexane (glue sniffing)
Vasculitis
Lyme Disease
Tick paralysis
Sarcoidosis
Leptomeningeal Dz
Paraneoplastic Dz
Critical Illness
 DISEASE MODIFYING TREATMENT

 IVIG : typically given for 5 d at 0.4 gram/kg/d

(may need to extend course depending on
response)
 PLASMAPHERESIS: usually 4-6 treatments over
8-10 days

The choice b/w PLASMA EXCHANGE AND IVIG is

dep on availability, pt contraindications, etc.
Because of ease of administration, IVIG is
frequently preferred. The cost and efficacy of the
2 treatments are comparable.

Glucocorticoids have NO ROLE!!

Outcomes:
 65% can walk independently @ 6 mos
 Overall, 80% usually recover completely
 5-10% have prolonged course w/ incomplete
recovery, ~3% wheelchair bound
 Approx 5% die despite ICU care
 2% will develop chronic relapsing Chronic
Inflammatory Demyelinating
Polyradiculoneuropathy (CIDP)
ASSALAMMUALAIKUM