4 (Motor Neuron Disease) Flashcards
(20 cards)
UMN VS LMN
UMN: More tone, reflex, disuse atrophy (late), babinsky, clonus
LMN: Less tone, reflex, wasting atrophy (early), fasciculation
Exception?
*at first of spine injury, spinal shock can cause LMN symptoms, and later on turn into UMN.
Patient with motor neuron disease with recurrent aspiration pneumonia, dyspnea when lying flat
gastrostomy Radiologically inserted (can’t percutaneous due to dyspnea while lying flat)
headache and not refreshing after sleep
Hypercapnia, NIV
Both UMN, LMN, bulbar
Amyotrophic lateral sclerosis (ALS)
UMN Bulbar ALS
Spastic tongue, brisk jaw jerk, emotional lability
LMN Bulbar ALS
wasting fasciculation tongue, nasal voice, dysarthria, dysphagia
Diabetic, little reflex leg, hyper reflex hand
ALS
Pure UMN (2)
Progressive lateral sclerosis PLS (proximal+face), Hereditary spastic paraparesis (distal
Pure LMN
PMA (Progressive muscular atrophy)
SMA (Spinal Muscular atrophy)
Progressive bulbar palsy
Spinal bublar palsy
Types of SMA?
1: death few weeks after birth
3: Juvenile chronic form, 1.5y, normal life expectancy, proximal weakness, bulbar palsy at late causing pneumonia
Which disease may later be diagnosed as ALS?
Progressive bulbar palsy
Other name for Kennedy?
Spinal Bulbar palsy
Kennedy Disease Pattern?
X Recessive (males), CAG
Kennedy Features?
LMN, peri oral fasiculation, androgen insensivity, testicular atrophy infertility gyncomastia
Foster kennedy?
Frontal Meningtioma, Ipsilateral Optic atrophy, Contralateral papiledema, Central scotoma, Anosmia,
Multifocal Motor Neuropathy
MND (distal unilateral)+ Demyelination (conduction block), Anti GM1, IVIG
Huntington Pattern?
Autosomal dominant, genetic anticipation, CAG chromosome 4
Huntintgton features?
Chorea/fidgety movements, parkinsonism, short term memory, depression and suicide, dystonia saccadic eye movements
Huntington survival?
death 20y after symptoms
