Please enable JavaScript.
Coggle requires JavaScript to display documents.
Motor Neurone Disease (Pathophysiology (Upper & lower motor neurones…
Motor Neurone Disease
Key Facts
Cluster of major degenerative diseases characterised by selective loss of neurones in motor cortex, cranial nerve nuclei and anterior horn cells
-
-
Aetiology/Risk Factors
-
5-10% cases are familial - linked to a mutation in the free radical scavenging enzyme superoxide dismutase
-
Pathophysiology
Upper & lower motor neurones are affected but there is NO SENSORY LOSS or SPHINCTER DISTURBANCE - this is what distinguishes MND for MS and poly neuropathies
-
-
Caused reactive oxygen species which damage DNA, lipids & proteins
There is relentless and unexplained destruction of upper motor neurones and anterior horn cells in the brain and spinal cord
-
Degenerative condition affecting motor neurones, namely anterior horn cells
Clinical Presentation
Progressive msucular atrophy (PMA). LMN only. Present with weakness, muscle wasting and fasciculations, usually in one limb. Affects distal before proximal
Progressive bulbar pasty (PBP). LMN only. lower cranial nerves and nuclei initially only affected. Dysarthria, dysphagia, nasal regurgitation of fluids.
Amyotrophic lateral sclerosis (ALP). Affects UMN + LMN. Loss of motor neurones in motor cortex & anterior horn of the cord . Weakness and UMN signs.
-
-
-
Diagnosis
-
-
-
Nerve conduction studies (NCS) + electromyography - denervation of muscles due to degeneration of LMN's is confirmed by EMG
Treatment
-
-
Dysphagia - blend food, nasogastric tube, percutaneous catheter gastrostomy
-
Drooling - due to bulbar palsy (wasting of muscle of mastication) - oral propantheline or amitriptyline
Specialist MDT support - neurologist, SLT, OT, specialist nurse, physiotherapist
Antiglutamatergic drugs - oral riluzole (Na channel blocker, inhibits glutamate release), prolongs life by 3 months, raises LFT's