Motor Neurone Disease

Motor neuron diseases are a heterogeneous group of disorders characterized pathologically by death of motor neuron cells ( Thomas T.Warner , Practical Guide to Neurogenetics , 2009 )

The red flag tool can be assessed via https://static.mndassociation.org/app/uploads/2013/10/19140141/red-flags-final-2.pdf

Subtypes -MND classification is based on those affecting primarily the UMNs , those affecting the LMNs and those affecting both

Amyotrophic lateral sclerosis or Lou Gehrig's disease- Involvement of upper , lower and brainstem motor neurons 70 % of cases of adult onset Fatal disorder - progressive skeletal muscle weakness and wasting or atrophy ( hence amyotrophy ) , spasticity and fasciculations due to degeneration of UMNs, LMNs --> eventual respiratory paralysis Most cases sporadic ( 5-10 % familial ) Median survival 3 yrs

Primary lateral sclerosis-Rare -idiopathic neurodegenerative disorder Pure UMN -predilection for lower limb dis onset Slower dis progression and better prognosis

Progressive bulbar palsy-Disorder of motor nuclei in medulla Atrophy and fasciculations of the lingual muscles , dysarthria and dysphagia May eventually lead to widespread symptoms typically seen in ALS

Hereditary spastic paraparesis-Familial spastic paraplegia or Strumpell-Lorrain disease slowly progressive spastic paraparesis

Spinal muscle atrophy X-linked spinobulbar muscular atrophy ( SBMA ; Kennedy disease ) Postpolio syndrome

Epidemiology- All motor...