Motor Neuron Disease and Cerebral Palsy

Question 1

What are the 4 subtypes of MND

Answer 1

Spinal ALS
Bulbar ALS
Progressive Muscular Atrophy
Primary Lateral Sclerosis

Question 2

CLinical features of ALS

Answer 2

typically LMN signs in arms and UMN signs in legs
asymmetric limb weakness is the most common presentation of ALS
wasting of the small hand muscles/tibialis anterior is common (split hand syndrome)
the absence of sensory signs/symptoms
vague sensory symptoms may occur early in the disease (e.g. limb pain) but ‘never’ sensory signs

Question 3

Primary lateral sclerosis has…

Answer 3

UMN signs ONLY

Question 4

Progressive muscular atrophy clinical features

Answer 4

LMN signs only
affects distal muscles before proximal
carries best prognosis

Question 5

Clinical features of Progressive bulbar palsy

Answer 5

palsy of the tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei
carries worst prognosis

Question 6

Investigation of MND

Answer 6

diagnosis of motor neuron disease is clinical
BUT nerve conduction studies will show normal motor conduction and can help exclude a neuropathy

Question 7

What is Cerebral Palsy?

Answer 7

disorder of movement and posture due to a non-progressive lesion of the motor pathways in the developing brain

Question 8

Possible causes of Cerebral palsy

Answer 8

Premature births
Intrauterine infections (TORCH - toxoplasmosis Gondi, other (syphillis, VSV), Rubella, CMV, HSV)
Hypoxia Ischemic insults
meningitis, encephalitis

Question 9

Subtypes of Cerebral palsy

Answer 9

spastic (70%)
subtypes include hemiplegia, diplegia or quadriplegia
increased tone resulting from damage to upper motor neurons
Hyperreflexia, weakness, clonus, increased tone

dyskinetic
caused by damage to the basal ganglia and the substantia nigra
athetoid (slow, smooth, ‘snake-like’) movements and oro-motor problems

ataxic
caused by damage to the cerebellum with typical cerebellar signs

mixed