ND II - ALS

Amyotrophic Lateral Sclerosis (ALS) is a devastating neurodegenerative disease without a cure. According to the El Escorial World Federation of Neurology, a definitive diagnosis of ALS requires:

• LMN + UMN signs in 2 regions

• LMN + UMN signs in 3 regions

• UMN signs and an identified mutated gene

• LMN + UMN signs in 2+ regions & bulbar signs

Primary Lateral Sclerosis refers to

• UMN damage only

• LMN damage only

• ipsilateral LMN + UMN signs

• LMN signs in at least 1 region

Spinal Muscular Atrophy or Progressive Muscular Atrophy refer to

• UMN damage only

• LMN damage only

• UMN damage only in 1+ regions

• irregular fibrillations and fasciculations on the ipsilateral side

T/F. Sensation is spared in patients w/ ALS.

• T

• F

Which of the following risk factors would MOST LIKELY contribute to a diagnosis of ALS.

• environmental exposure

• mutation of the genes C9ORF72, SOD1, TARDBP

• mutation of the genes: BRCA1, BRCA2

• high fitness level

• high BMI

The 2 most common theories about motor neuron degeneration in ALS are ___ & ___.

• inflammation/infection; excessive glutamate

• viral infection; bacterial infection

• nicotine exposure; inflammation

• excessive sodium; limited glutamate

Which bulbar signs are MOST LIKELY to develop d/t ALS?

• difficulty swallowing

• difficulty w/ speech production

• clonus triggered by movement

• head drop

• pseudobulbar affect

Which UMN signs are MOST LIKELY to develop d/t ALS?

• flaccidity

• Hoffman's and Babinski

• neck extensor weakness

• slow tongue movement

• tongue fasciculations

Which LMN signs are MOST LIKELY to develop d/t ALS?

• dysphagia

• focal muscle atrophy

• muscle weakness

• orthopnea

• focal muscle fasciculations

Which of the following are MOST LIKELY to remain intact during ALS?

• sensation

• extraocular movements

• various grip abilities (power grip, pincer grip, etc.)

• volume of voice

• bowel/bladder function