Samenvatting Physiotherpeutic Theory of ALS

Physiotherapeutic theory for neurorehabilitation
of ALS
Clinical presentation of ALS
Amyotrofische Laterale Sclerose

Prevalence
Prevalence: 3-8/100.000
• In Belgium: 1000 patients
• Incidence: 0,4-3/100.000 year
• Male > female
• Age onset: 58-63y
Disease duration
• Survival: 2-5y
o Familiary (hereditary) ALS: <2y
o Sporadic (non-hereditary) ALS: 3-4y

Pathology




UMN involvement
• Corticospinal tract
• Corticobulbar tract




LMN involvement
• Spinomuscular tract




Genetic features
Often sporadic and unknown geneticcause
• 10% familial, 90% sporadic
• 15% genetic cause known, 85% unknown
Genes
• C9orf72: 50% fALS, 7% sALS
• SOD1: 20%fALS, 1% sALS
• FUS & TARDBP: 2-3% fALS

, Disease onset
Spinal onset
à Best survival
• Often asymmetrical weakness
• Distal > proximal
• Arms > legs
• Hypotonia or spasticity
UL onset: arms à thoracic à bulbar à legs
LL onset: legs à thoracic à arms à bulbar

Bulbar onset
• Dysphagia
• Dysarthria
• Emotional lability
• Weakness respiratory muscles
• Dyspnea
• Orthopnea à morning headache

Respiratory onset
• Weakness of respiratory muscles
• Dyspnea
• Orthopnea à morning headache
- Following with upper limb and trunk weakness




ALS variants
PLS: primary lateral sclerosis
Only upper motor neuron
• Corticospinal/corticobulbar tract involvement
• Slower progression, survival of 30y
• 75% evolves to ALS within 4 years

PMA: progressive muscular atrophy
Lower motor neuron abected
• Flail leg syndrome: dropping of knees, hyperextension of knees
• Flail arm syndrome: stooped posture, arms hang loose
• Progression to ALS first 4 years, if not better survival