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Determining Prognosis: Patients with End-Stage Neurologic Disease (Reading & Sharing)

The three major subgroups: Alzheimer’s disease and other dementia, cerebrovascular disease, and amyotrophic lateral sclerosis (ALS) and related motor neuron disorders.

Alzheimer’s disease and other dementia

    • Inability to ambulate without assistance (FAST 7-C)
    • inability to speak or communicate meaningfully with speech limited to approximately a half-dozen or fewer intelligible or different words (FAST 7-B)
    • Loss of ADL functions including bathing and dressing (FAST 6)
    • Incontinence of bowel and bladder (FAST 6)
    • one or more of the following comorbid conditions in last 3-6 months
      • Aspiration pneumonia
      • Pyelonephritis or upper urinary tract infection
      • Septicemia
      • Decubitus ulcers, usually multiple and stages II or IV
      • Fever, recurrent after antibiotics
      • An altered nutritional status as manifested by:
        • difficulty swallowing or refusal to eat such that sufficient fluid or caloric intake cannot be maintained and the patient refuses artificial nutritional support
        • OR
        • If the patient is receiving artificial nutritional support (NG or G-tube or parenteral hyperalimentation), there must be evidence of an impaired nutritional status as defined in the General Guidelines (greater or equal 10% loss of body weight)

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Amyotrophic Lateral Sclerosis (ALS) – Reading & Sharing

Definition, Etiology, and Incidence


Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig disease after the famous New York Yankees baseball player, is a devastating neurologic disorder that selectively affects motor function with an unknown origin, results in muscle weakness, disability, respiratory insufficiency, and eventually death. ALS has an annual incidence of 0.5-2 per 100,000 population, is primarily a disorder of middle to late adulthood, affecting people most frequently in their fifties, with men developing the disease nearly twice as often as women. The median survival duration for patient is approximately 3 years, yet approximately 20% of patients survive greater than 5 years, and 10% will survive for greater than 10 years.

As the main area of involvement is the motor neurons of the brain and spinal cord, ASL affects motor neurons in three locations as follows: the anterior horn cells (lower motor neurons – LMNs) of the spinal cord; the motor nuclei of the brain stem, particularly the hypoglossal nuclei; and the upper motor neurons (UMNs) of the cerebral cortex. The death of the LMNs leads to denervation, with subsequent shrinkage of musculature and muscle fiber atrophy. It is this fiber atrophy, called amyotrophy, which appears in the name of the disease. The loss of nerve fibers in lateral columns of the white matter of the spinal cord, along with fibrillary gliosis, imparts a firmness or sclerosis to this CNS tissue. The term lateral sclerosis designates these changes.

A remarkable feature of the disease is that the entire sensory system, the regulatory mechanisms of control and coordination of movement, and the intellect remain intact.

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Hospice & Palliative Care

End Stage Neurological Disorders – Amyotrophic Lateral Sclerosis 肌萎缩侧索硬化 (ALS) (Learning about Hospice)

ALS is a rapidly progressing degenerative neuromuscular disease with an unknown origin, results in muscle weakness, disability, respiratory insufficiency, and eventually death. The median survival duration for patients is approximately 3 years, yet 10% will survive for > 10 years.

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