29 Questions
ALS can occur at any age, with the average age at onset being the mid-to-late ______s
50s
After age 65, the prevalence of ALS is equal between males and females, with a ratio of ______
1.7:1
One of the risk factors for ALS is gene mutation of ______
SOD1
Individuals with certain occupations may be at higher risk due to neurotoxicant ______
exposure
Vigorous physical activity, heavy manual labor, and trauma are all potential risk factors for ALS due to overuse and ______
strain
The degeneration of the corticospinal tracts, neurons in the motor cortex, and brainstem are all associated with the pathophysiology of ALS, involving glutamate excitotoxicity and ______
oxidative stress
The 50% survival rate for patients with primary bulbar symptoms is only 1 year post ______
diagnosis
UMN manifestations include spasticity, hyperreflexia, clonus, Babinski sign, Hoffmann sign, and muscle ______
weakness
LMN manifestations include flaccidity, areflexia/hyporeflexia, hypotonicity, muscle atrophy, and muscle ______
cramping
Cervical extensor weakness is a common sign in patients with ______
ALS
Muscle weakness can lead to secondary complications such as joint subluxation, ambulation problems, high risk for falls, contractures, deformities, and ADL ______
problems
Healthy, intact surrounding axons can sprout and reinnervate the partially denervated muscle, in essence assuming the role of the degenerated motor neuron and preserving strength and function early in the disease; however, the surviving motor units undergo ______.
enlargement
Reinnervation can compensate for the progressive degeneration until motor unit loss is about 50%, and electromyography (EMG) studies have found evidence of motor unit reinnervation in individuals with ______.
ALS
As the disease progresses, reinnervation cannot compensate for the rate of degeneration and a variety of ______ develop.
impairments
Average duration of the disease is 27 to 43 months. Age at time of onset has the strongest relationship to ______.
prognosis
Patients less than 35 to 40 years of age at onset had better 5-year survival rates than older individuals. Poor prognostic factors include: older age at time of onset, bulbar and/or pulmonary dysfunction early in the clinical course of the disease, short time period from symptom onset to diagnosis, and predominance of LMN findings at the time of ______.
diagnosis
Young males with ALS may have a longer life expectancy but overall the median 50% ______.
survival
FTD associated with ALS is mostly characterized by cognitive decline, functioning impairments, hard time in planning, organizing, and concept abstract, as well as behavior changes. Patients with FTD are also reported to have cognitive impairments, such as verbal fluency, language comprehension, memory abstract reasoning, and intellectual function.
Frontotemporal Dementia
Even without FTD, patients may still experience ______ impairments. This may also be caused by respiratory impairments because there is not enough oxygen in the brain of the patient.
cognitive
Pseudobulbar affect is characterized by pathological weeping, ______, and yawning that is inappropriate or excessive to the emotional state of the patient. It is considered to be a result of loss of frontal lobe inhibition of brainstem generated emotional responses.
laughing
Differential Diagnosis includes variants of ______ neuron diseases. The relationship between ALS and other variants is not entirely clear, but there is strong evidence that these disorders are at a minimum tightly related.
Motor
The start of muscle weakness in ALS is distal to proximal, meaning it begins in the _______ parts of the body.
distant
There can be underlying causes such as Sialorrhea or ______ impairment due to muscle weakness. Cognitive impairments may include difficulties in planning, organizing, and concept abstract, as well as behavior changes. Patients with FTD are also reported to have cognitive impairments, such as verbal fluency, language comprehension, memory abstract reasoning, and intellectual function.
respiratory
The revised El Escorial criteria with the Awaji modification forms the diagnostic criteria generally used currently. Principles is the requirement para ma-diagnose si pt with ___
ALS
Most commonly used imaging technique is ___
MRI
Ang pag-spread ng ALS is continuous; parang spider-web, region per region; not diffused. Board-sensitive ang diagnostic categories ng ___
ALS
Upper motor neuron (UMN) and lower motor neuron (LMN) findings in the same limb are very suspicious for ___
ALS
Electrodiagnostic testing is the cornerstone of identification of LMN loss in all MNDs, but diagnosis of ALS requires clinical correlation with examination features of UMN involvement. No laboratory or imaging test can make the diagnosis of ___ in isolation
ALS
In a patient with clinical features of ALS, no diagnostic test can rule out ___. Most patients suspected of having ALS should undergo testing to exclude mimicking conditions
ALS
Test your knowledge on the chapter about ALS in the De Lisa book authored by Dr. Lisa S. Krivickas. Learn about the epidemiology, prevalence rates, and challenges associated with the diagnosis of ALS. Also explore the impact of movements like the Ice-bucket challenge on raising funds for ALS research and treatment.
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