Amyotrophic Lateral Sclerosis Essay

2937 words - 12 pages

Part I: Amyotrophic Lateral Sclerosis (ALS) is an age-related disorder which results in rapid,

selective deterioration of motor neurons of the central nervous system. ALS was first described by

Jean-Martin Charcot in the 1870s and is the most common motor neuron disease, with an

incidence of 2 per 100,000 people. It predominantly includes primary lateral sclerosis, a disease

restricted to upper motor neurons and progressive muscular atrophy, which is limited to lower

motor neurons. The onset of the disease is typically first observed in limb and bulbar muscles,

eventually spreading into respiratory myotomes, followed by respiratory failure. These clinical

features are noted due to progressive degeneration of LMN's in the medulla and anterior horn of

the spinal cord as well as UMN's in the cerebral cortex. In addition to the motor neurons, damage

to the corticospinal tracts, frontal and temporal lobes leading to genetic and pathological overlap

with a condition called frontotemporal dementia (FTD) is also observed. Neuronal damage in

motor neurons occurs due to aggregation of misfolded proteins and subsequent conformational

instability of the non-neuronal, supporting cells such as microglia, astroglia and oligodendrocytes

(Sreedharan & Brown, 2013). Defective RNA processing, poor axonal transport, genetic

mutations, followed by increased neurotoxicity are some of the key features implicated in the

lethal prognosis of the disease. Relatively better prognosis and longer survival rates are observed

with younger age, stable weight, higher breathing capacity and better motor function. Typical

signs and symptoms associated with loss of LMN's cause fasciculations, cramps, muscle atrophy

and increased muscle weakness which are generally more debilitating to patients than UMN signs

of spasticity, hyperreflexia and modest weakness. Other predictable UMN degeneration symptoms

include positive Babinski and Hoffmann signs. Foot drop, loss of hand dexterity, loss of ability to

walk and frequent falls are some of the symptoms noted in our patient Mr. Stone and will most

likely make him dependent on long-term, palliative caregivers. Dropped head and increased

2

kyphosis due to axial weakness, dysarthria and dysphagia are mostly typical of bulbar type ALS.

Weak coughs, morning headaches, pain due to contractures, immobility and bed sores, shortness

of breath are mixed sensory and motor findings that lead to depression and anxiety in some cases

(Sreedharan & Brown, 2013).Ten percent of all ALS cases are inherited, whereas most of the

cases are sporadic. Some environmental risk factors include tobacco smoking, trauma, viral

infections and exposure to toxins. As in Mr. Stone's case, advancing age is one of the major risk

factors associated with ALS.
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