1:MDS4019 Vik Johal

Motor Neuron Disease

Introduction
 MND is a cluster of neurodegenerative diseases affecting 6/100,000 people with 3:2 male-to-female ratio
 Characterised by selective loss of neurons in the Motor Cortex, Cranial Nerve Nuclei & Anterior Horn Cells
 Upper & Lower motor neurons can be affected but there is no sensory loss or sphincter disturbance
o Thus, distinguishing MND from Multiple Sclerosis & Polyneuropathies
 MND never affects eye movements, distinguishing it from Myasthenia

Types of MND
Amyotrophic Lateral Sclerosis (ALS)
 Archetypal MND (80%) with motor neuron loss in Motor Cortex & Anterior Horn Cells  UMN & LMN Signs
 Worse Prognosis if:
o Bulbar Onset  Dysarthria/Dysphagia
o ↑ Age
o ↓ FVC (Spirometry)

Progressive Bulbar Palsy

 Constituting 10-20% of MND cases only affecting Cranial Nerves IX-XII (Medulla)
o Bulbar Palsy
 LMN lesion of the tongue & muscles of talking and swallowing
 Flaccid & Fasciculating Tongue
 ↔/↓ Jaw Jerk Reflex
 Speech: Quiet, Hoarse or Nasal
o Corticobulbar (‘Pseudobulbar’) Palsy
 UMN lesion of muscles of swallowing and talking
 2o to bilateral lesions above the Mid-Pons (Corticobulbar Tract)
 Slow Tongue Movements
 Slow Deliberate Speech
 ↑ Jaw Jerk
 ↑ Pharyngeal & Palatal Reflexes
 Pseudobulbar Affect (PBA): Emotional incontinence without mood change
 Weeping unprovoked by sorrow or mood-incongruent giggling

Progressive Muscular Atrophy (<10%)

 2o to an Anterior Horn Cell Lesion  LMN Signs only
 Affecting Distal Muscle Groups before Proximal
o Flail Arm/Foot Syndrome
 Better prognosis than ALS

Primary Lateral Sclerosis (Rare)

 2o to loss of Betz Cells in the Motor Cortex  Predominantly UMN Signs
o Marked Spastic Leg Weakness
o Brisk Reflexes
o Extensor Plantar Responses
o Corticobulbar/Pseudobulbar Palsy
o No Cognitive Decline
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Lower Motor Neuron Lesions (LMNLs)

 Flaccid Paralysis that is Ipsilateral to and at the level of the lesion characterised by:
o Absent Voluntary/Reflex Contraction of Denervated Muscles (Paralysis)
o Absent/Suppressed Muscle Stretch Reflexes
 Hyporeflexia, Areflexia or Hypotonicity
o Fasciculations
 Random twitches of denervated motor units visible beneath the skin
o Atrophy of Denervated Muscles
o Fibrillations (1-5 ms potentials)
 Detected via electromyography

Upper Motor Neuron Lesions (UMNLs)

 Spastic Weakness/Paresis that is Ipsilateral or Contralateral to and always below the lesion characterised
by:
o Elevated Muscle Stretch Reflexes (Hyperreflexia/Hypertonicity)
 UMNs have a net overall inhibitory effect on muscle stretch & inverse muscle stretch
reflexes
 ∴ a UMNL results in loss of inhibition of stetch reflexes  Hyperreflexia
o 2o to loss of inhibition of γ-motor neurons by reticulospinal medullary UMNs
o Clonus
 Seen in hyperactive muscle stretch reflexes
 Rapid successive reflex contractions & relaxations of agonists & antagonist in reflex testing
o Clasp Knife Reaction
 Indicative of UMN inhibition loss of the inverse muscle stretch reflex
o Loss of Skilled Finger/Toe Movements
o Babinski Sign
 Extensor Plantar Response to Cutaneous Stimuli
 Hallux Extension & Fanning of Toes
o Absent Cutaneous Reflexes
 Abdominal Reflex (T8-12)
 Cremasteric Reflex (L1)
o Disuse Atrophy of Weakened Muscles
 Spinal Cord UMNLs
o Spastic Paresis 2o to UMN Axonal Lesions within the spinal cord
 Ipsilateral & Below Lesion Level
 UMNLs between Cerebral Cortex & Medulla
o Spastic Paresis 2o to UMNLs occurring above the Pyramidal Decussation (Medulla)  Cerebral Cortex
 Contralateral & Below Lesion Level
 Decorticate Posturing
o 2o to lesions in UMN tracts (e.g. corticospinal) Above Red Nucleus (Midbrain)
 Contraction of Upper Limb Flexors
 Contraction of Lower Limb Extensors
 Decerebrate Posturing
o 2o to Brainstem UMNLs Below Red Nucleus  Postural Extension at joints of both upper & lower
limbs