Conditions in Occupational TherapyJonathan Alonso & Michael Muñoz
ALS also known as Lou Gehrig’s Disease or Motor Neuron Disease (MND). ALS was first described in 1869 by Jean-Martin Charcot, a French neurologist. And was brought to national attention once baseball legend, Lou Gehrig was diagnosed with the disease in 1939.
It is a life threatening disease that negatively affects millions of people each year. It is a progressive neuromuscular condition characterized by muscle weakness (hypotonicity), muscle wasting (atrophy), muscle twitching (fasciculation), and increase reflexes (hyperreflexia) (Aebischer & Kato, 2007). The progression of the disease is different from one person to the next. Important! This a motor neuron disease; sensory, executive functions, and intellect remain intact.
The cause of ALS remains unknown. Theories to the cause of such disease are thought to be genetic, viral, autoimmune disorder. Enzyme deficiency, environmental factors, and neurotoxic hypothesis (Benot-Abreu, Damme, Van Den Bosch & Robberecht). Research postulates concern over neurotoxicity relating to abnormalities of essential neurotransmission anions, calcium and glutamate, entering the neuron, damaging the cell metabolism, and resulting in pathological changes (Wijesekera & Leigh, 2010).
A report on incidence shows that ALS on average affects 1.89 per 100,000 a year and prevalence average of 5.2 per 100,000 (King, Duke, & O’Conner, 2009). The mean age of onset for ALS is about 60 yrs, with a slight male prevalence male/female ratio 1.5:1 Approximately 2/3 of patients with typical ALS have a spinal form of the disease where the symptoms may start either distally or proximally in the UE and LE (King, Duke, & O’Conner, 2009). Most common motor neuron disorder in adults (Atchison & Dirette, 2007).
Depend on the location of the disease ALS divides into three areas: lower motor neuron, corticospinal tract, and corticobulbar tract dysfunctionRegardless the part of the body first affected by the disease, muscle hypotonicity and atrophy spread to other somatic effectors as the disease progresses (Atchison & Dirette, 2007).Patients have increasing problems with moving, swallowing (dysphagia), and speaking or forming words (dysarthria) (Wijesekera & Leigh, 2010).
Patients must have signs and symptoms of both upper and lower motor neuron damage, that’s not attributed to other causes. The process consists of a history and physical exam, repeated at regular intervals, to document hyperreflexia, fasciculation, and upper and lower movement (Bento-Abreu, et. al, 2010). MRI tests document denervation and distinguish benign fasciculation from those of ALS (Atchison & Dirette, 2007).
Currently there is no known medical cure to alter the fatal progression of ALS. Gradual death 1-5 years from diagnosis due to respiratory problems, though course is progressive and rapid. Riluzole remains to be the only compound licensed for use since it reduces damage to motor neurons by decreasing the release of glutamate and modifies the rate of evolution (Corcia & Meininger, 2008). Anti-inflammatory medications Antispasmodic Non-invasive ventilator support Multidisciplinary teams (including OT) may increase quality and length of life.
Occupational Therapy provides a longer duration and better quality of life (QOL). Providinghope for patients retaining purpose in occupational activity (Corcia & Meininger, 2008) Prescribingappropriate equipment, which helps with functional independence within areas of occupation (AOTA, 2008) .
Assistive technology and equipment commonly recommended to minimize energy output and improve performance with self care tasks and other ADLs: Reachers Dressing sticks Long handle shoehorns Long handle sponges Buttonhooks Shower seats Three-in-one commodes
The cause of ALS remains unknown and most common motor neuron disorder in adults Important! This a motor neuron disease; sensory, executive functions, and intellect remain intact. Symptoms include: muscle spasticity, hypotonicity, atrophy, cramps, fasciculation and hyperreflexia. Also, dysphagia, dysarthria, and an overactive gag reflex. Treatments include: Anti-inflammatory and Antispasmodic medications. Also, non-invasive ventilator support Multidisciplinary teams (including OT) may increase quality and length of life. Death from respiratory failure, 1-5 years after Diagnosis due to rapid and progressive nature of the disease.
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