MOTOR NEURON DISEASES

AND OTHER
MOTOR SYSTEM DISEASE
 MND
• Penyebab tidak diketahui

• Diperkirakan karena TRAUMA, EXERCISE yang berlebihan,

KERACUNAN TIMBAL DAN MERCURY, KEGANASAN YANG
TERSEMBUNYI & VIRUS

• Bisa mengenai semua ras

• Dapat mengenai usia muda, tapi sebagian besar pasien berusia
lebih dari 60 tahun
• Pria : Wanita = 1.5 : 1

• Rerata harapan hidup 3-4 tahun

 GEJALA KLINIS
• Kesulitan menjalankan aktivitas :
Memutar kunci, , kelemahan kaki, genggaman lemah atau atrofi beberapa otot

• Riwayat kram, , quivering of muscles, or heaviness,

aching and stiffness of legs

DIAGNOSIS
IS SUGGESTED BY THE PRESENCE OF
FASCICULATION OR WASTING
WITH ENHANCED REFLEXES
AND WITHOUT SENSORY SIGNS
 CHARACTERIZED BY

Progressive degeneration of
• anterior horn cells
• corticospinal fibers, and
• motor nuclei in the medulla

Various levels of the nervous system:

bulbar, cervical, and lumbar may be involved
 All level of the motor system are involved
The most common presentation:
AMYOTROPIC LATERAL SCLEROSIS
 wasting in the upper limbs and spasticity in the lower limbs

• PROGRESSIVE MUSCULAR ATROPHY

 SHOW PREDOMINANTLY LOWER MOTOR NEURONE CHANGES

• PROGRESSIVE LATERAL SCLEROSIS

 PYRAMIDAL TRACT DEGENERATION BEFORE MUSCULAR WASTING

• PSEUDOBULBAR/BULBAR ;
 PROGRESSIVE BULBAR PALSY
BRAIN STEM INVOLVEMENT, PREDOMINANTLY SPASTIC: (PSEUDOBULBAR)
PREDOMINANTLY FLACCID (BULBAR)
 AMYOTROPIC LATERAL SCLEROSIS
the most common form of motor neuron disease

• Atrophy, weakness and fasciculation in their limb

muscle (indicating a lower motor neuron lesion)

• Hyperactive reflexes

• Extensor plantar responses

• No sensory signs

It is this combination of upper and lower motor neuron

signs in all limbs that hallmark of ALS
FASCICULATION OVER AN AREA MAY BE SEEN WITH

 CERVICAL SPONDYLOSIS
 SYRINGOMYELIA
 ACUTE STAGE OF POLIOMYELITIS
 NEURALGIC AMYOTROPHY
 THYROTOXIC MYOPATHY

BULBAR SYMPTOMS:
 SELECTIVE SWALLOWING DIFFICULTIES
 WEAKNESS AND NASAL SPEECH
 FASCICULATION AND ATROPHY THE TONGUE
 DIAGNOSIS

CLINICAL FEATURES
May leave a little doubt

CONFIRMED BY ELECTROMYOGRAPHY
 If indicated  muscle biopsy

MYELOGRAM ?
(High cervical lesion)

CFS EXAMINATION
(Neurosyphilis)
 PROGNOSIS

 IF THE PATIENS ASKS IF THE CONDITION IS

POTENTIALLY LETHAL, THE ANSWER MUST IN
ALMOST EVERY CASE BE ‘YES’.

 Most patients remain mentally alert and are able to

make rational decisions to cope with their increasing
disability
 PARALYTIC POLIO
 Persons infected with polio: > 95% asymptomatic viremia and
spontaneous clearing

 Flulike prodrome  severe generalized myalgias with focal,

often asymmetric fasciculation;
 followed by weakness that often is severe
 the legs often are most affected
although any muscle or region can be
involved including diaphragm and bulbar
muscles

 Recovery typically is incomplete, atrophy and asymmetric weakness is

often permanent
 POST-POLIO SYNDROME

• Occasionally a syndrome develops in former paralytic polio victims

several years following the initial attack

• Patients typically complain a diffuse myalgias and recurrence of

weakness of muscles that were affected in the initial attack

• The lag between the initial attack and development of so-called post-
polio syndrome often is measured in decades
 PARALYTIC POLIO
POST-POLIO SYNDROME

MANAGEMENT ?
 MYASTHENIA
• An acquired autoimmune disorder causing skeletal
muscle fatigue and weakness

• Autoantibodies against the acetylcholine receptor

produce weakness that can affect the entire body or
only eye movement

• Can begin at any time, from early childhood to

extreme old age

• The cause of the autoantibodies is not known. The

thymus is implicated in the inception and generation
of the autoantibodies
 Auto antibodies bind to the acetylcholine receptor and cause
increased receptor degradation.
The combination of the binding and the turnover effects results
loss of receptor so that an action potential in the motor neuron
does not always result in an action potential in the muscle fiber

Thymoma as present in some patients with myasthenia

Onset in non-thymoma cases:

Peak incidence at 10-30 yeas of age, again at 60-70 yeas of age

Myasthenia associated with thymoma:

Peak incidence at 40-50 years of age
Under 40  predominantly affects women
MYASTHENIA WHICH HAS A DIFFERENT MECHANISM

• Neonatal myasthenia
Transient illness, lasting less than 1 month, 1 in 8 babies of myasthenic mothers

• Juvenile myasthenia
Myasthenia in the younger age group, generally similar to those of myasthenia in young adults

• Penicillamine-induced myasthenia
Usually resolves over several month after drug withdrawal

• Lambert-Eaton myasthenic syndrome

A presynaptic disorder characterized by impaired release of Ach from the nerve terminal.
60% cases is associated with small cell lung carcinoma

• Congenital myasthenia
• Familial myasthenia
 SYMPTOMS AND SIGNS
weakness of skeletal muscle is
characteristically increased by exercise,
but is not associated with muscle pain
(in contrast to ‘physiological” fatigue)

emotional stress, pregnancy and infection can also cause an

exacerbation of symptoms

• Ocular muscles
• Limb weakness
• Bulbar muscle weakness
• Respiratory muscle involvement
 CLINICAL CLASSIFICATION

Main groups of acquired myasthenia gravis

Group I: ocular myasthenia gravis (symptoms may remain

persistently confined to the ocular muscles, particularly
when 2 years have elapsed since the onset

Group IIA, B: mild or moderately severe generalized

myasthenia gravis

Group III: acute severe (fulminating) myasthenia gravis

with respiratory muscle involvement

Group IV: late (chronic) severe disease

 INVESTIGATION

• Anti-ACHR antibody
• Antistriated muacle antibody

• Edrophonium chloride test

• (modification?)

• Electromyographic techniques

• Thymoma
 MODES OF THERAPY

• Anticholinesterase therapy:
– Pyridostigmine, 30-120 mg orally
– Neostigmine bromide, 15-30 mg orally every 3 hours except at
night

Higher dose than those given above are seldom indicated and
greatly increase the risk of cholinergic crisis.
Side-effects are caused by para-sympatithetic stimulation and
include: - pupillary constriction
- colic
- diarrhoea
- Increased salivation
- Increased sweating
- Increased lacrimation
- Increased bronchial secretions
MODES OF THERAPY
• Corticosteroids:
Prednisolone - suitable
- once daily on alternate days to avoid
side-effects
- initial dose 10 mg, increased slowly
 out patients: 5-10mg / week
 in patients: 5-10 mg / dose
 to avoid the exacerbation of
symptoms that can occur when the drug is started at
a high dose
- Maximal dose: 1-1.5 mg / kg body weight
- (or symptoms are controlled)
 Thymectomy
 Intravenous immunoglobuln
 Plasma exchange

• Improvement is expected although most patients are

maintained on a low-dose corticosteroid after their
initial tapering

• Crisis may develop requiring hospitalization,

administration or IVIG or PE, and/or transient
increases in corticosteroid
 MYASTHENIC CRISIS
occurs with inadequate treatment and can be
precipitated by infection.

Treatment consist of:

• Control of the airway and assisted ventilation

• Anticholinesterase medication
• Immunosuppressive drug therapy and/or plasma
exchange
 CHOLINERGIC CRISIS
caused by excess anticholinesterase medication

Treatment consist of:

• Control of the airway and assisted ventilation

• Temporary withdrawal of anticholinesterase drugs,
with later reintroduction at a reduced dose regiment
• Immunosuppressive drug therapy and/or plasma
exchange
• Atropine, if not already being given
SINDROMA GUILLAIN BARRE

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PENDAHULUAN
 Sindrom Guillain-Barre (SGB) :
 Penyakit sistem saraf perifer dengan paralisis bersifat akut
dan difus
 Hilangnya segmental mielin pada radiks spinalis , saraf
perifer dan saraf kranialis
 Hilangnya sensorik yang bersifat subyektif
 Penyakit autoimun didahului oleh suatu infeksi
 Sinonim : Landry Guillain Barre Strohl Syndrome, Post
Infectious Polyneuritis, Acute Inflammatory Demyelinating
Polyradiculoneuropathy (AIDP), Acute Immune-Mediated
Polyneuritis (AIMP), Acute Idiopathic Polyneuritis
 Diagnosis SGB ditegakkan  berdasarkan gambaran
klinis, pemeriksaan laboratorium, pemeriksaan cairan
serebro spinal (CSS) dan pemeriksaa elektromielografi
(EMG)
 Belum ada obat yang spesifik
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DEFINISI
 Sindrom Guillain-Barre (SGB)  penyakit autoimun
pada sistem saraf perifer yang ditandai paralisis akut
dan difus, berupa hilangnya segmental mielin pada
radiks spinalis, saraf perifer dan saraf kranialis,
hilangnya sensorik yang bersifat subyektif yang
biasanya didahului oleh suatu infeksi

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INSIDENSI
 4-5 setiap 100.000 penduduk
 Rasio laki-laki : perempuan 1,5:1 (terutama pada ras
caucasians)
 Puncak insiden pada usia remaja atau dewasa muda
(15-35 tahun)
 Puncak kedua pada usia 50-70 tahun mungkin
berkaitan dengan penurunan daya tahan tubuh

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ETIOLOGI
 Belum diketahui → akibat reaksi imunologi yang
diperantarai sel yang diarahkan pada saraf perifer
 > 50% didahului oleh infeksi saluran napas bagian
atas atau gastrointestinal 1-3 minggu sebelumnya
 Keadaan yang berhubungan dengan SGB antara lain :
 Infeksi dengan kampilobakteri jejuni, sitomegalovirus,
Epstein-Barr virus, mikoplasma, virus campak, virus cacar
air
 Infeksi bakteri  demam tifoid, paratifoid, pertusis dan
setelah pemberian vaksinasi influensa, difteri, polio dan
tetanus (DPT) dan HIV

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PATOLOGI
 Proses inflamasi non infeksi multi fokal pada
saraf perifer  demielinisasi sampai terjadi
degenerasi aksonal pada kasus yang berat
 Proses patologi diawali infiltrasi limfosit
perivaskuler  diikuti infiltrasi sel radang
terutama limfosit dan makrofag  makrofag
menembus membrana basalis sel schwan 
memisahkan mielin dari akson  proses
demielinisasi segmental

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PATOGENESIS
 Kecenderungan peranan dasar patogenesis yang
bersifat immunologik
 Bukti-bukti bahwa Immunopathogenesa merupakan
mekanisme yang menimbulkan lesi :
 Didapatnya antibodi atau adanya respon kekebalan seluler
(cell mediated immunity) terhadap agen infeksious pada
sistem saraf tepi
 Adanya autoantibodi atau respon kekebalan seluler
terhadap sistem saraf tepi
 Didapatnya penimbunan kompleks antigen antibodi dari
peredaran pada pembuluh darah saraf tepi

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GAMBARAN KLINIS
 Gejala klinis SGB 60 – 70% didahului infeksi
saluran pernafasan bagian atas atau gastrointestinal
1 – 3 minggu sebelumnya, diikuti oleh :
 Rasa tebal / parestesi oleh sebagian besar penderita, dan
sebagian kecil dengan kelemahan dan parestesi
 Kelemahan anggota gerak yang bersifat bilaeral (simetris).
Kelemahan biasanya dimulai dari anggota gerak bawah
tetapi dapat juga bersama dengan anggota gerak atas,
jarang dimulai dari anggota gerak atas, umumnya bagian
distal lebih parah daripada proksimal.
 Dapat mengenai saraf kranial (VII,IX,X – III,IV,VI – jarang
V,XII)
 Paralisis diafragma dan otot intercostal terjadi pada 7 –
22% kasus, yang menyebabkan terjadinya insufisiensi
pernafasan  kedaruratan  perlu ventilator
 Terjadinya disfungsi otonom 37
38
ETIOLOGI
Diagnosis penyakit SGB ini dapat diketahui dengan :
 Gejala klinik
 Pemeriksaan Laboratorium
 Peningkatan kadar protein dalam LCS > 0,5 g / l tanpa diikuti
peningkatan jumlah sel, keadaan ini disebut : Disosiasi cyto-
albumin
 Terjadi setelah minggu ke-2 dan mencapai puncaknya setelah
3 – 6 minggu
 Pemeriksaan Elektrofisiologi (EMG)
 Kecepatan hantaran saraf motorik dan sensorik melambat
 Distal motor latensi memanjang
 Kecepatan hantaran gelombang F melambat yang
menunjukkan perlambatan pada segmen proksimal dan
radiks saraf
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PENATALAKSANAAN
 Terapi Suportif
 Terapi pada gangguan otonom

 Terapi preventif untuk infeksi nosokomial,

tromboemboli, komplikasi akibat imobilisasi yang
lama
 Rehabilitasi Medik : ROM passive, splinting

 Nutrisi yang adekuat

 Hidrasi yang adekuat

 Stool softeners (obat pencahar)

 Terapi untuk penghilang rasa nyeri

 Terapi Medikamentosa
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PROGNOSIS
 ± 75% sembuh sempurna, 10% dengan gejala sisa
minor, 10% dengan problem yang lebih nyata (mis
drop foot, tremor dll), angka kematian akibat SGB
<5%(2), angka relaps 3-5%.
 Hal-hal yang mempengaruhi prognosis :
 Prediktor Klinis : usia lanjut, perjalanan penyakit dari
onset yang sangat cepat, defisit motorik yang berat,
penggunaan ventilator.
 Prediktor EMG : derajat degenerasi aksonal, nilai CMAP
yang rendah dan menetap, hasil stimulasi saraf minimal
dan terdapat fibrilasi.

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