PENYAKIT SUSUNAN

SARAF TEPI

SYARIF DARWIN ANSORI

 KLASIFIKASI PENYAKIT
Med.Spinalis: Transverse myelitis, absces,
tumor.
Kornu ant.or: Poliomyelitis
Saraf perifer: Sindroma Guillain Barre, poli
neuritis, neuropati.
Myo-neu junct: Myastenia gravis, botulism,
keracunan fosfat.
Otot: Myositis, myopati.
 Acut Flaccid Paralysis (AFP)
AFP = Lumpuh Layuh Mendadak
Acut = klinis 14 hr sesudah lumpuh
Flaccid = R F / (-), R P (-), Klonus (-)
Paralysis = kekuatan < 5, penyebab bukan o.k. trauma.
Penyebab: kelainan / penyakit pd Sistim
Saraf Tepi (SST)
Angka kejadian: 1 per 100.000 anak < 15 th
Penampang Medula spinalis
SEL NEURON
MYELIN
 POLIOMIELITIS
Etiologi: Virus polio (liar / vaksin)
Patogenesis: Virus menembus sel masuk ke
RNA merusak sel ( pd kornu ant. or / nukleus
nervi kranialis )
Masa inkubasi 8-12 hr (5-35 hr), paralise pd 3-8 hr
sesudah gejala pertama muncul.
Klinis: - abortive 90-95%
- non paralitik 4-8 %
- paralitik 1-2 %.
 GEJALA PARALITIK
SPINAL: kaku + nyeri pd leher, tl.belakang,
tungkai tanda tripod, head drop, hiper /
parestesi.
BULBER: parese nn. Kranialis (stridor, dis
fagi), ggn autonom (tek.drh, berkeringat dll)
ENSEFALITIS: kesadaran,( gelisah, tre -
mor, kejang )
LCS :prot.= n /sedikit , pleositosis ringan
(PMN )
 PENATALAKSANAAN
ISTIRAHAT pd fase akut
SIMPTOMATIK: analgetik, antipiretik
Pada fase paralitik, waspadai gagal nafas,
ggn sirkulasi, retensio urine.
FISIOTERAPI ( > 2 minggu )
ROBORANSIA
IMUNISASI AKTIF.
 ACUTE TRANSVERSE MYELITIS
Etiologi: auto-immun, bakteri, virus, spiroke-
ta,Peny Lyme, malformasi pemb.drh
Klinis: kelemahan ext.as inf. secara cepat,
sensoris terganggu, inkonitensia urine
Penatalaksanaan:- methylprednisolone do-
sis tinggi (1g/1.73m/i.v), suportif, simptoma
tik, fungsi kembali normal 1-6 bln (60%).
TRANSVERSE MYELITIS
 GUILLAIN BARRE SYNDROME
Acute Inflammatory demyelinating polyneuropathy
Etiologi: auto immune
Patogenesis: infeksiantigenmielin
Klinis:-Paralisis(1-2mg),simetris,asen
den, terjadi 2mg post inf.
-Ggn fungsi sensoris
-Lcs: disosiasi sito-albumin
 PENATALAKSANAAN
ISTIRAHAT TOTAL 2MG PERTAMA
AWASI TANDA VITAL
PLASMAPHERESIS
IVIG (immuno globulin)
SUPORTIF Stabilisasi, antipiretik, analge-
tig, fisioterapi dll.
>4 mg CIDP kortikosteroid
Myoneural Junction
 MYASTHENIA GRAVIS
A CHRONIC DISEASE

CHARACTERISTIC: Fatigability of Volun

tary muscle
FORMS AGE AT ONSET:
- JUVENILE M. G.
- CONGENITAL M. G.
- TRANSIENT NEONATAL M.G.
 PATHOLOGIE ANATOMY
ELECTRON MICROSCOPY:
- The Nerve Terminal are Small
- Cleft the Nerve-Subneural Widened
- Receptor Asetylcholinesterase <<
THYMUS Lymphoid Hyperplasia
( 70 80 % )
MYOCARDIAL ABNORMALITIES ( 10%)
 PATHOGENESIS
An Antibody-Mediated Autoimmune

Act.Chol.Recptr (N-M Junct) Destruction

Sensitization

Extracts of Thymi
(Acetylcholine Rec.Antibodie )
 CLINICAL MANIFESTATION
TRANSIENT NEONATAL 1-7 days of life
- PARESIS of the Lower Bulbar Muscle
- WEAK CRY
- DIFFICULTY IN Sucking / Swallowing
- GENERALIZED HYPOTONIE
- Born to mother with Myasthenia Gravis
 CLINICAL MANIFESTATION
CONGENITAL M - G

- BEFORE 2 YEARS OF AGE

- MORE THAN ONE SIBLING

- MILD SYMPTOMS SPONTANEUS

REMISSIONS
 CLINICAL MANIFESTATION
JUVENILE M - G
- PR > LK ( 1 : 2/3 )
- Extra ocular Musc / Repeated contraction
- Best in the morning progressively weak
er during the day
- TENDON JERKS ARE NORMAL
- ASSOSIATED: Rheumatoid Arthritis, Juv.
Diabetes Mellitus, Thyroid Dis., SLE.
 DIAGNOSIS
- Prompt Improvement with Anticholinestera
se (TENSILON TEST) :
* 5 mg iv (tensilon) 1 improvement
* 0,5 1,5 mg neostigmin i.m.10-15
- Acetylcholin receptor antibodies (+) in
serum
- PLASMAPHERESIS
- EMG single fiber (+)
 TREATMENT
SYMPTOMATIC: Anti cholinesterase
Pyridostigmine Bromide (mestinon) 15
mg 2 3 x a day
IMMUNOLOGIE: prednison oral 1-2 mg /
kgBB / day tapering when improv.
PLASMAPHERESIS unrespon other Th/
IMMUNOSUPPRESSION cyclosporine
IMUNO GLOBULIN i.v
THYMECTOMY maximal improvement