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AND OTHER
MOTOR SYSTEM DISEASE
MND
• UNKNOWN CAUSE
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
• PSEUDOBULBAR/BULBAR ;
PROGRESSIVE BULBAR PALSY
BRAIN STEM INVOLVEMENT, PREDOMINANTLY SPASTIC: (PSEUDOBULBAR)
PREDOMINANTLY FLACCID (BULBAR)
Amyotrophic Lateral Sclerosis
SURYADI
ALS merupakan :
• 1. Peny. degeneratif neuron motorik progresif usia tua
• 2. Motor neuron disease dgn gejala LMN & UMN
bersamaan.
• ALS ditandai dgn perubahan pada kornu anterior MS, inti
motorik BO, traktus kortikospinalis.
• ALS = progresif, fatal, ditandai pengecilan otot &
kelemahan gagal nafas kematian.
• Perlu Dx tepat & edukasi untuk pasien dan keluarga.
AMYOTROPIC LATERAL SCLEROSIS
the most common form of motor neuron disease
• No sensory signs
CERVICAL SPONDYLOSIS
SYRINGOMYELIA
ACUTE STAGE OF OLIOMYELITIS
NEURALGIC AMYOTROPHY
THYROTOXIC MYOPATHY
BULBAR SYMPTOMS:
SELECTIVE SWALLOWING DIFFICULTIES
WEAKNESS AND NASAL SPEECH
FASCICLATION AND ATROPHY THE TOGUE
DIAGNOSIS
CLINICAL FEATURES
May leave a little doubt
CONFIRMED BY ELECTROMYOGRAPHY
If indicated muscle biopsy
MYELOGRAM ?
(High cervical lesion)
CFS EXAMINATION
(Neurosyphilis)
Epidemiologi
• ALS jarang dijumpai
• Insidensi : 1 - 5 / 100.000
penduduk.
• Laki : Perempuan = 2 : 1.
• Usia onset 40 - 70 thn.
Etiologi
• Umumnya etiologinya : 85 % tidak diketahui.
• Faktor-faktor yg mendasari.
• 1. Faktor genetik : 5 - 15 %. Autosomal dominan mutasi gen
untuk enzim SOD 1
• radikal bebas rusak sel motor neuron
• 2. Lingkungan.
• 3. Kelebihan Glutamat.
• 4. Infeksi virus.
• 5. Faktor penuaan.
• 6. Sistim Imunitas.
• 7. Logam berat.
Patofisiologi
Medula spinalis
Otot
Konus Medularis
Terima Kasih