Amyotrophic Lateral Sclerosis (ALS)

Typical presentation: – Amyotrophic lateral sclerosis (ALS)  is a progressive neurodegenerative disease characterized by gradual loss of upper and lower motor neurons and their pathways usually without affecting the extraocular and sphincter muscles ending in programmed cell death in selective neuronal subpopulations. The typical form of ALS with upper and lower motor neuron involvement and progressive bulbar paralysis with bulbar muscle involvement with difficulty in speaking, swallowing, articulation. Progressive muscle atrophy, where the only lower motor neuron is affected and primary lateral sclerosis with only upper motor neuron damage are rare, familiar forms of ALS (FALS) is usually associated with dementia (frontotemporal lobar dementia), behavioral disorders, cognitive dysfunction, and impairment of executive functions (Stetkarova, & Ehler, 2021). Other non-motor symptoms of GI (sialorrhea, constipation, diarrhea), autonomic (pain, dyspnea, urinary incontinence), psychiatric (depression, anxiety, sleep problems, fatigue, suicidal tendency, cognitive-behavioral impairment), vascular (dyslipidemia), itching, and pressure sores, psychosis and schizophrenia or mood disorders.

Appropriate diagnostic testing and screening: – Clinical presentation of central and peripheral motor neuron involvement in one or more segmental anatomical areas and functional impairment, nerve conduction/needle electromyography studies (EMG) Motor evoked potentials after transcranial magnetic stimulation is to identify impairment of upper motor neurons (Stetkarova, & Ehler, 2021). MRI of the brain reveals early degeneration of upper motor neurons. A biochemical-specific protein called TDP-43. Blood work such as CBC, CMP, CK, Muscle MRI, and muscle biopsy, ESR, CSF studies to exclude other neuro disorders, Chest x-ray, and urine analysis. Genetic tests and screening to rule out other neurological disorders. SMN detection (genetic testing)

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Admission orders: – Admission status based on patient’s airway status. If the patient unable to maintain his oxygenation need supplementary oxygenation and as the respiratory muscles get involved, the respiratory status deteriorated and may need ventilatory support and need ICU management.

-Maintain fluid and electrolytes with IVF or in long-term tube feeding requiring gastrostomy feeding.

-Skin and pressure ulcer prevention measures, care, and management of skin.

-Managing incontinence and complications secondary to incontinence and constipation by using natural fibers, stool softener, or laxative in severe constipation.

-Management of diagnostic tests and imaging and education support and counseling with the diagnosis of ALS regarding the disease, various diagnostic tools, prognosis, end of life care and symptomatic management and finding the resource and support groups on ALS. Monitor for the elevation of WBC, fever, and other signs of infection.

Referrals and Consultations: – Referral to pomologist, neurologist, ENT surgeon to place tracheostomy and gastrostomy placement either by interventionist or surgeon. Rehabilitation services such as PT/OT/ST in the initial part of diagnosis and social service, nutritionist and case management for planning the appropriate therapy as the patient condition deteriorates, and end-of-life care when the disease gets advanced.

Potential Risks and Complications: – People with ALS are prone to breast cancer or lymphoma, noticeable weight loss in 5-10 years, motor weakness leading to split hand syndrome, gait disorders, and bulbar signs associated with vocalization (Stetkarova, & Ehler, 2021).

Interventions and preventive care and geriatric considerations: – No definitive treatment for ALS and many drugs and therapies are still being tried. Drugs such as riluzole and edaravone, are used to slow disease progression (Cruz, 2018), and also stem cell and Sodium phenylbutyrate-taurursodiol were also been tried. Care and management of ALS are directed towards the quality of life and functional independence and physical integrity. Supportive therapy such as airway management by ventilator assistance, nutrition by feeding tube, meeting hygienic, elimination and ADL needs and prevention of complications such as ventilator-associated pneumonia, malnutrition, maintaining fluid and electrolyte balance, and prevention of pressure sores and other complications. Education about nature and counseling the patient and family poor prognosis of the disease due to no successful therapy has a big role in expectation and quality of life and prepare them for transitioning as the patient’s conditions progress. Preventive care is by immunization against common pathogens such as flu vaccine, pneumococcal vaccine, meningitis vaccine, Now Covid vaccine and prevention of therapy-related complication such as pneumonia, pressure injury, infections, contractures, malnutrition and mental counseling to cope up with the disease and major life changes with poor prognosis and nature of the disease. ALS is underrecognized in elderly patients related to frequent presentation with symptoms of dysphagia, frailty, or general weakness for other reasons and ALS is to be considered as a differential diagnosis since it is common than formerly assumed.

Reference.

Stetkarova, I., & Ehler, E. (2021). Diagnostics of Amyotrophic lateral sclerosis: Up to date. Diagnostics, 11(2), 231. Retrieved from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7913557/

Broussalis, E., Grinzinger, S., Kunz, A. B., Killer‐Oberpfalzer, M., Haschke‐Becher, E., Hartung, H. P., & Kraus, J. (2018). Late age onset of amyotrophic lateral sclerosis is often not considered in elderly people. Acta Neurologica Scandinavica137(3), 329-334. Retrieved from https://onlinelibrary.wiley.com/doi/abs/10.1111/ane.12869

Cruz, M. P. (2018). Edaravone (Radicava): a novel neuroprotective agent for the treatment of amyotrophic lateral sclerosis. Pharmacy and Therapeutics43(1), 25. Retrieved from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5737249/

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