S.

IRWANSYAH

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

KLASIFIKASI
BANYAK KLASIFIKASI DARI NEUROPATI. 1.MENURUT ONSET SERANGAN: NEUROPATI AKUT
MIS : POLINEUROPATI IDIOPATIK AKUT

NEUROPATI KRONIK
MIS : BERI BERI DIABETES MELLITUS LEPRA

2.MENURUT DERAJATNYA
1. NEUROPATI RINGAN : SENSORIK SAJA 2. NEUROPATI SEDANG : SENSORIK, MOTORIK, REFLEKS 3. NEUROPATI BERAT : SENSORIK, MOTORIK, REFLEKS , ATROFI OTOT

3. MENURUT JUMLAH SARAF YANG TERLIBAT

1. MONONEUROPATI SIMPLEKS:
GANGGUAN PADA SATU SARAF PERIFER SAJA.
2. MONONEUROPATI MULTIPLEKS: MENGENAI BEBERAPA SARAF TEPI, BIASANYA TIDAK BERDEKATAN DAN TIDAK SIMETRIS. 3. POLINEUROPATI: BBRP SARAF TEPI, SIMETRIS DAN SERENTAK, BIASANYA PREDOMINAN DI DAERAH DISTAL.

4. MENURUT LETAK LESI

1 AKSONOPATI DISTAL: GANGGUAN PADA AKSON. 2. MIELINOPATI : GANGGUAN PADA SELUBUNG MIELIN. 3. NEURONOPATI : GANGGUAN PADA BADAN SEL SARAF DI CORNU ANTERIOR, MEDULLA SPINALIS ATAU PADA DORSAL ROOT GANGLION.

ETIOLOGI
1. IDIOPATHIC INFLAMMATORY NEUROPATHIES
- POLINEUROPATI IDIOPATIK AKUT (GUILLAIN BARRE SYNDROME) - CHRONIC INFLAMMATORY DEMYELINATING POLYNEUROPATHY
2. METABOLIC AND NUTRITIONAL NEUROPATHIES - DIABETES, HIPOTIROIDI, ACROMEGALI - UREMIA - LIVER DISEASES - VIT B1, OR VIT B12 DEFICIENCY

ETIOLOGI (lanjutan)

3. INFECTIVE AND GRANULOMATOUS NEUROPATHIES: AIDS, LEPROSY. DIFTERI, SARCOIDOSIS

4. VASCULITIS NEUROPATHIES: - POLYARTERITIS NODOSA - RHEUMATOID ARTHRITIS - SYSTEMIC LUPUS ERYTHEMATOSUS

ETIOLOGI (lanjutan)
5. NEOPLASTIC AND PARAPROTEINEMIC NEUROPATHIES: - COMPRESSION AND IRITATION BY TUMOR - PARANEOPLASTIC SYNDROME - PARAPROTEINEMIAS - AMYLOIDOSIS

ETIOLOGI (lanjutan)
6. DRUGS INDUCED AND TOXIC NEUROPATHIES - DAPSON, ISONIAZIDE, PHENYTOIN, PIRIDOKSIN VINCRISTIN, HIDRALAZINE. - ALKOHOL - TOKSIN: ORGANOPHOSPHAT ARSENIC LEAD THALIUM GOLD

ETIOLOGI (lanjutan)
7. HEREDITARY NEUROPATHIES - IDIOPATHIC
HEREDITARY MOTOR AND SENSORY NEUROPATHIES HEREDITARY SENSORY NEUROPATHIES FAMILIAL AMYLOIDOSIS

- METABOLIC
PORPHYRIA METACHROMATIC LEUCODYSTROPHY ABETALIPOPROTEINEMIA

ETIOLOGI
8. 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 NERVE

DANG THE RAPIST

Diabetes Rheumatic (collagen vascular) Alcohol Hereditary Amyloid Nutritional Environmental Paraneoplastic Guillain Barre toxin and drugs Infections Systemic diseases Tumors Trauma

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.

PATOFISIOLOGI
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.

PATHOGENESIS

-Trauma
-Entrapment -Tumor

-GBS
-Metabolic -Nutritional

-Dying Back --Toxin --Nutritional --Collagen

PATOFISIOLOGI
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.

PATOFISIOLOGI
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

PATOFISIOLOGI
2. AKSONOTMESIS: - KERUSAKAN PADA AKSON DISERTAI DEGENERASI - TANPA KERUSAKAN ENDONEURAL - REGENERASI KEMUNGKINAN DAPAT TERJADI DENGAN HASIL YANG BAIK

PATOFISIOLOGI
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

GEJALA KLINIK
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

GEJALA KLINIK
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

GEJALA KLINIK
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.

GEJALA KLINIK
GANGGUAN OTONOMIK (lanjutan) :
- 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

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 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

MANFAAT EMG
MEMBANTU DIAGNOSA SECARA DINI MENENTUKAN LETAK LESI MEMBEDAKAN LESI MIOGEN ATAU NEUROGEN MENENTUKAN LESI PARSIAL ATAU TOTAL MEMBEDAKAN SENSORIK ATAU MOTORIK EVALUASI PENGOBATAN MEMBANTU MENENTUKAN PROGNOSE

NERVE CONDUCTION VELOCITY( NCV) NCV ATAU KHS 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

NEUROPATI DIABETIK
PREVALENSI : 10 - 20 % (SIMTOMATIK) KHS 80 % ABNORMAL KLINIS DAPAT MENGENAI: SENSORIK MOTORIK OTONOMIK KOMBINASI

NEUROPATHY : An Overview Focus on Diabetic Neuropathy

EPIDEMIOLOGY & ETIOLOGY

Mononeuropathy : Median nerve entrapment the most common mononeuropathy Causes : - repetitive motion injury during manual tasks such as keyborad operation (entrapment) - multifocl demyelination - ischemic injury - trauma
CURRENT DIAGNOSIS & TREATMENT NEUROLOGY, 2007

Polyneuropathy : hundreds of potential etilogies DM is the most common cause in the US, affecting at least 1-2% of the population Leprosy remains the most common cause of neuropathy worldwide.

CURRENT DIAGNOSIS & TREATMENT NEUROLOGY, 2007

DIABETIC NEUROPATHY

Prevalence of Diabetes Mellitus

Worldwide: 194 million people USA: 18,2 million (18% of people > 65) About 40 % of U.S. adults ages 40- 74 (41 million people) have abnormal blood glucose levels without having DM. Many will develop type 2 DM in the next 10 years. Total annual cost in 2002: $132 billion (one out of every 10 health care dollars spent

Diabetic Neuropathy
occurs equally in type-1 and type-2 DM

The major morbidity is foot ulceration, the precursor of gangrene and limb loss Neuropathy increases the risk of amputation: 1.7 - fold 12 - fold with deformity (also a consequence of neuropathy) 36 - fold with history of previous ulceration. 85,000 amputations in the US each year, 1 every 2 minutes, and neuropathy is considered to be the major contributor in 87% of cases.

PATHOPHYSIOLOGY OF DIABETIC NEUROPATHY (Bird 2002 )

* Direct glucose neurotoxicity Metabolic derangements Increased polyol pathway activity with accumulation of fructose and sorbitol and reduced nerve inositol Reduced Na+K+ ATPase Slowed axonal transport Intracellular oxidative stress with premature apoptosis Microvascular abnormalities Depressed prostaglandins Decreased nerve blood flow Endoneurial ischemia Trophic agent (nerve growth factor) deficiency Abnormal glycation and glycosylation of proteins Altered nerve proteins Altered endothelial protein functions Autoimmune-mediated neurotoxicity

Types of DN

Focal (Mononeuritis) Entrapment Diffuse Proximal Distal Small- fiber (including autonomic) Large-fiber

DIABETIC NEUROPATHIC SYNDROMES

ISCHEMIC MONONEUROPATHY. - cranial (eg. CNs III, VI,VII) diplopia, pupil-sparing third nerve palsy, hemifacial weakness - Radicular (thoracic, lumbosacral) pain, followed by numbness or weakness in a radicular distribution - Peripheral (eg. Femoral) pain, followed by numbness, weakness or both in territory of a single nerve

CURRENT DIAGNOSIS & TREATMENT NEUROLOGY, 2007

DIABETIC NEUROPATHIC SYNDROMES (cont.d)

SMALL FIBER NEUROPATHY :
- pure small fiber neuropathy numbness, paresthesias, painful dysesthesias, hyperesthesias. - Diabetic neuropathy cachexia subacute, severe neuropathic pain, rapid weight loss - Autonomic neuropathy erectile dysfunction, orthostasis, cardiac dysrhythmia, diarrhea, constipation
CURRENT DIAGNOSIS & TREATMENT NEUROLOGY, 2007

DIABETIC NEUROPATHIC SYNDROMES (cont.d)

DISTAL SYMMETRIC NEUROPATHY : - large fiber sensory neuropathy numbness, paresthesias, dysesthesias, hyperesthesias, ataxia. - sensorimotor neuropathy any of the above plus distal weakness

CURRENT DIAGNOSIS & TREATMENT NEUROLOGY, 2007

DIABETIC NEUROPATHIC SYNDROMES (cont.d) REGIONAL NEUROPATHIC SYNDROMES. - Diabetic amyotrophy subacute weakness and atrophy of proximal leg muscles - Diabetic thoracoabdominal neuropathy. subacute weakness, numbness, and atrophy in thorax and abdomen

CURRENT DIAGNOSIS & TREATMENT NEUROLOGY, 2007

DIAGNOSIS

Status terkendalinya Diabetus Mellitus

Baik

Sedang

Kurang terkendali

Glukosa darah
Puasa 2 jam pp mmol/l Hb A1 Glukosa Urine Kholesterol total % % mg/dl mmol/l mg/dl mmol/l mg/dl mmol/l kg/m2 Pria Wanita mm Hg mg/dl mmol/l mg/dl 4.4 - 8.9 < 8.5 0 < 200 < 5.2 > 40 > 1.1 < 150 < 1.7 80 - 120 4.4 - 6.7 80 - 160 < 10 8.5 - 9.5 < 0.5 < 250 < 6.5 > 35 > 0.9 < 200 < 2.2 < 140 < 7.8 < 180 > 10.0 > 9.5 > 0.5 > 250 > 6.5 < 35 < 0.9 > 200 > 2.2 > 140 > 7.8 > 180

Kholesterol HDL

Trigliserid

BMI

< 25 < 24 < 140/90

< 27 < 26 < 160/95

> 27 > 26 > 160/95

Tekanan darah

Treatment of Diabetic Neuropathy

Best Rx is prevention !!! Diabetics should do three things to help prevent the complications of diabetes: 1) Achieve ideal body weight 2) Maintain regular exercise 3) Maintain good control of glucose level

Treatment of Diabetic Neuropathy (cont.d)

Protect tissues from injury (check feet every day, good shoes, beware of excess heat or cold)

Medications can provide symptomatic relief from pain

Treatment of Diabetic Neuropathy (cont.d)

Pada DN demielinasi segmental Vit B12 menstimulasi sel-sel Schwann memfasilitasi mielogenesis, meningkatkan sintesis protein dan regenerasi syaraf Vitamine B12 - Cyanocobalamine - Mecobalamine - Hydroxycobalamine

Pathogenesis of DN
Heterogenous with causative factors: Microvascular insufficiency oxidative stress nitrosative stress defective neurotrophism autoimmune mediated nerve destruction

Treatment of Diabetic Neuropathy Medications for Neuropathic Pain: Topical capsaicin Antidepressants (amitriptyline, nortriptyline, imipramine) Anticonvulsants (gabapentin, carbamazepine, clonazepam, phenytoin) Narcotics (avoid unless nothing else works)

PAINFUL DIABETIC NEUROPATHY

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

TRAUMA SARAF PERIFER

Proksimal Regenerasi Sprouting * 1 - 5 mm / hari * Distal Degenerasi

DERAJAT TRAUMA SARAF PERIFER

Sunderland
I
Hantar saraf (-) Axon utuh Myelin utuh Perineurium utuh Hantar saraf (-) Axon Putus Wallerian Degen Myelin utuh Perineurium utuh Hantar saraf (-) Axon Putus Wallerian Degen Myelin menipis Perineurium Utuh Hantar saraf (-) Axon Putus Myelin Putus Perineurium Putus Seluruh jar.saraf putus + Jar.sekitarnya putus

Seddon
NEUROPRAXIA

Prognose
Trasient
Regen. 1-5 mm/hari

II

AXONOTMESIS

III

NEUROTMESIS

JELEK

IV V

JELEK

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