_____ diseases produce spastic tone, hyperactive tendon reflexes, pathological reflexes (ie Babinski), and emotional lability (inappropriate laughing and crying).
Upper motor neuron (UMN)
Upper motor neuron (UMN) diseases produce what signs/symptoms?
- spastic tone
- hyperactive tendon reflexes
- pathological reflexes (ie Babinski)
- emotional lability (inappropriate laughing and crying)
______ diseases produce muscle atrophy, fasciculations, diminished tone, and reduced or absent reflexes.
Lower motor neuron (LMN)
Lower motor neuron (LMN) diseases produce what signs/symptoms?
- muscle atrophy
- diminished tone
- reduced or absent reflexes
If the pt complains about sensory changes with accompanied weakness, this suggest problems with?
- a nerve root disorder
- plexus disorder
- peripheral nerve disorder
Rapidly developing weakness (hours to days) is characteristic of _______.
neuromuscular junction disorders
Name 3 distribution patterns of weakness.
How is a neuromuscular disorder different than normal atrophy?
- in normal atrophy, the muscle remains strong
- In an NMJ disorder, the muscle is strikingly weak
Name 4 anterior horn cell disorders.
- amyotrophic lateral sclerosis (ALS)
- spinal muscular atrophy
- West Nile virus
Name 2 radiculopathies and plexopathies.
- cervical and lumbosacral nerve root compression
- brachial and lumbosacral plexopathies
Name 3 types of peripheral neuropathies.
- mononeuropathy multiplex
Name 4 neuromuscular junction disorders.
- Myasthenia gravis
- Lambert Eaton syndrome
- Organophosphate poisoning
- progressive weakness and wasting degeneration of brainstem and spinal cord neurons
- asymmetric limb weakness
- foot drop or hand deformity
- pathological reflexes present
- normal sensory exam
Amyotrophic lateral sclerosis (ALS)
What is the average survival after diagnosis of ALS?
How is ALS treated?
- primarily symptomatic
- braces and durable medical equipment
How does someone get Charcot-Marie Tooth disease?
What is the most common form of Charcot-Marie Tooth disease type 1? What causes it?
CMT1A; duplication of the PMP22 gene (peripheral myelin protein gene)
What does a duplication of the PMP22 gene (peripheral myelin protein gene) cause?
Charcot-Marie Tooth disease type 1A (CMT1A)
Most pts with CMT present with 3 phenotypes. What are they, and what is the most common?
- normal onset of walking, distal weakness and sensory loss in first 2 decades** most common
- impaired as infant, delayed walking
- adult onset appearing around age 40
What is the cutoff between demyelinating and axonal forms of CMT?
What is the medication tx for CMT?
none, just pain mgmt
_____% of diabetics have diabetic neuropathy.
What is the most common type of diabetic neuropathy?
distal sensory or sensorimotor polyneuropathy
Name some autonomic dysfunction seen in association with diabetic neuropathy.
- postural HTN
- urinary retention
- increased sweating
_____ is characterized by acute onset of asymmetrical proximal weakness and pain in the legs.
Lumbosacral plexopathy is characterized by?
acute onset of asymmetrical proximal weakness and pain in the legs