Dr.Suherman, Sp.S

DEFINISIGANGGUAN FUNGSIONAL ATAU ORGANIKDARI SARAF PERIFER

GANGGUAN INI DAPAT MENGENAI : SARAF SENSORIK SARAF MOTORIK SARAF OTONOM KOMBINASI

KLASIFIKASIBANYAK KLASIFIKASI DARI NEUROPATI.1.MENURUT ONSET SERANGAN: NEUROPATI AKUT MIS : POLINEUROPATI IDIOPATIK AKUT NEUROPATI KRONIK MIS : BERI BERI DIABETES MELLITUS LEPRA

2.MENURUT DERAJATNYA1.NEUROPATI RINGAN:SENSORIK SAJA2.NEUROPATI SEDANG:SENSORIK, MOTORIK, REFLEKS 3.NEUROPATI BERAT :SENSORIK, MOTORIK,REFLEKS , ATROFI OTOT

3. MENURUT JUMLAH SARAF YANG TERLIBAT1. 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 LESI1 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.

ETIOLOGI1. 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:ORGANOPHOSPHATARSENICLEADTHALIUMGOLD

ETIOLOGI (lanjutan)7. HEREDITARY NEUROPATHIES- IDIOPATHICHEREDITARY MOTOR AND SENSORY NEUROPATHIESHEREDITARY SENSORY NEUROPATHIESFAMILIAL AMYLOIDOSIS- METABOLICPORPHYRIAMETACHROMATIC LEUCODYSTROPHYABETALIPOPROTEINEMIA

ETIOLOGI8. ENTRAPMENT NEUROPATHIES- UPPER LIMBSMEDIAN NERVE (CARPAL TUNNEL SYNDROME)ULNAR NERVERADIAL NERVE- LOWER LIMBSPERONEAL NERVEFEMORAL NERVEOBTURATOR NERVE

MOST COMMON DISEASES AFFECTING THE PERIPHERAL NERVE DANG THE RAPIST

Diabetes TraumaRheumatic (collagen vascular)Alcohol HereditaryAmyloidNutritional EnvironmentalParaneoplasticGuillain Barre toxin and drugsInfectionsSystemic diseasesTumors

PATOFISIOLOGIADA BEBERAPA PROSES PATOLOGI YANG MENGENAI SERABUT SARAF a.l.:

1. DEGENERASI WALLERIANTERJADI DEGENERASI AKSON DAN SELUBUNG MIELIN KEARAH DISTAL DARI LESI. DEGENERASI BISA JUGA KE PROKSIMAL SATU ATAU DUA SEGMEN.

PATOFISIOLOGI2. DEMIELINISASI SEGMENTALTIMBUL BILA TERJADI LESI PADA SEL SCHWANNPROSES DIMULAI DI DAERAH NODUS RANVIER DAN MELUAS TAK TERATUR MENGENAI SEGMEN-SEGMEN INTERNODUS LAIN. AKSON DAPAT MENGALAMI DEGENERASI ATAU TIDAK TERGANGGU SAMA SEKALI.

PATOFISIOLOGI3. DEGENERASI AKSON PRIMERDISEBUT JUGA DENGAN AKSONOPATI. DEGENERASI AKSON INI BIASANYA DI IKUTI OLEH DEMIELINISASI SEGMENTAL YANG SEKUNDER. SERING PADA UREMIA, KERACUNAN ALKOHOL, LEPRA, KARSINOMA.

PATOFISIOLOGIKERUSAKAN 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

PATOFISIOLOGI2. AKSONOTMESIS:- KERUSAKAN PADA AKSON DISERTAI DEGENERASI- TANPA KERUSAKAN ENDONEURAL- REGENERASI KEMUNGKINAN DAPAT TERJADI DENGAN HASIL YANG BAIK

PATOFISIOLOGI3. NEUROTMESIS:

- SARAF TERPUTUS TOTAL ATAU SEBAGIAN- PENGOBATAN DGN PENYAMBUNGAN- KEMUNGKINAN PERBAIKAN 50%

GEJALA KLINIK1. 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 KLINIK2. 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 KLINIK3. 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 KLINIK4. 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 KLINIKGANGGUAN OTONOMIK (lanjutan) : - GANGGUAN KANDUNG KEMIH : ATONI KANDUNG KEMIH, RESIDU URINE - IMPOTENSI- GANGGUAN KARDIOVASKULER: HIPOTENSI ORTOSTATIK, SINKOP- GANGGUAN BERKERINGAT- CARDIO RESPIRATORY ARREST

PREDOMINANTLY MOTOR NEUROPATHIESGuillain-Barre SyndromeDiphtheric neuropathyDapsone-induced neuropathyPorphyria and multifocal motor neuropathy

PREDOMINANTLY SENSORY NEUROPATHIESDrug toxicity : pyridoxine, doxorubicineAutoimmune : paraneoplastic, Sjogren syndrome, etc.Infectious : diphtheria, HIVDeficiency : vit. EInherited : abetalipoproteninemia.

DIAGNOSA1. GEJALA KLINIK2. LABORATORIUM3. FOTO THORAKS4. PUNKSI LUMBAL5. EKG6. BIOPSI : paling sering n. suralis atau n. cutaneus radialis7. ELEKTROFISIOLOGI: EMG NCV

ELEKTRO MIOGRAFIELEKTRODA DITUSUKKAN KEDALAM SUATU OTOT SKELET UNTUK MEMPELAJARI PERUBAHAN POTENSIAL LISTRIKNYA.INDIKASI: GANGGUAN LOWER MOTOR NEURON, YANG LESINYA DI:1. KORNU ANTERIOR2. RADIKS3. PLEKSUS4. SARAF PERIFER5. NEUROMUSCULAR JUNCTION6. OTOT

MANFAAT EMGMEMBANTU DIAGNOSA SECARA DINIMENENTUKAN LETAK LESIMEMBEDAKAN LESI MIOGEN ATAU NEUROGENMENENTUKAN LESI PARSIAL ATAU TOTALMEMBEDAKAN SENSORIK ATAU MOTORIKEVALUASI PENGOBATANMEMBANTU 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 / sDISTAL LATENCY ( DL ) NILAI NORMAL N. MEDIANUS 2,7 + 0,3 m/s

MANFAAT PENGUKURAN KHSMENGIKUTI PERJALANAN PENYAKITMENGEVALUASI EFEK PENGOBATANMENENTUKAN PROGNOSE, APAKAH MASIH MUNGKIN DIPEROLEH PERBAIKAN LAGI.

EMG DAN KHS PADA NEUROPATIDIJUMPAI PENURUNAN KHS.PEMANJANGAN DISTAL LATENCYPENURUNAN AMPLITUDO GELOMBANG MDURASI YANG MEMANJANGPOTENSIAL POLIFASIKFIBRILASI

NEUROPATI DIABETIKPREVALENSI : 10 - 20 % (SIMTOMATIK)KHS 80 % ABNORMALKLINIS DAPAT MENGENAI: SENSORIK MOTORIK OTONOMIK KOMBINASI

PATOGENESE NEUROPATI DIABETIKThe etiology is uncertain.

4 hypothesis (not necessarily exclusive) :1. Hyperglycemia-polyol-myoinositol hypothesis.2. Microvascular hypothesis3. 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 RanvierNa/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 neuropathySome cases of NIDDM and proximal diabetic have a inflammatory vasculopathy with perivascular collections of lymphocytes and axonal neuropathy

PAINFUL DIABETIC NEUROPATHY cranial nerve neuropathyAcute thoracoabdominal neuropathyAcute distal sensory neuropathyAcute lumbar radiculoplexopathyChronic distal small-fiber neuropathy

TerapiIntensive diabetic therapyMaintain ideal body weightAdjuvant analgetics :TCA antidepressantscarbamazepinegabapentinintravenous lidocaine, etc

CARPAL TUNNEL SYNDROMECHARACTERIZED 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 PREGNANCYPRESSURE 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

Etiologi1. Hereditary: HMSN type III2. Traumatic: dislocation, fracture, hematoma, wrist sprain3. Infection: tenosynovitis, tbc, sarcoidosis4. Metabolic: amyloidosis, gout5. Endocrine: acromegaly, DM, hypothyroidism, pregnancy6. Neoplastic: ganglion cysts, lipoma , myeloma7. Collagen vascular diseases : RA, polymyalgia rheumatica, SLE8. Degenerative disease : OA9. Iatrogenic: radial artery puncture, shunt for dialysis, anticoagulant therapy

Gejala KlinisThe 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 nightLate : inability to screw bottle caps or grip properly

TerapiIdentified causes should be treatedCorticosteroid 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

G B S (lanjutan)- 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

G B S (lanjutan)- 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

G B S (lanjutan)KRITERIA DIAGNOSTIKDIJUMPAINYA 5 DARI 6 KRITERIA INI :1. DIFFUSE FLACCID PARALYSIS2. GANGGUAN SENSORIK < GANGGUAN MOTORIK3. REMISI SEMPURNA DALAM 6 BULAN4. PENINGKATAN PROTEIN PADA CSF DLM 2 MGG5. DEMAM (-) ATAU SUHU SEDIKIT MENINGGI6. LEUKOSIT NORMAL HANYA KEMUNGKINAN LED SEDIKIT MENINGGI

PREDICTORS OF SEVERE DISEASE AND POORER OUTCOMEOld ageRapid onset of severe tetraparesisNeed for early artificial ventilationSeverely decreased compound muscle action potential (

TERAPI1. PLASMAPHARESIS2. IMMUNOGLOBULIN IV 0,4 gr/kg BB SELAMA 5 HARI3. PERAWATAN UMUM4. FISIOTERAPI5. PERAWATAN DI ICU BILA TERJADI GAGAL NAFAS