Flashcards in Neuropathy and myopathy Deck (75)
Diseases in the anterior horn lead to LMN or UMN disease?
What is the classic disease of the anterior horn?
UMN and LMN s/sx = what disease?
Which has hyperactive, and which hypoactive reflexes: UMN and LMN lesions
UMN = hyperactive
LMN = hypoactive
What is the MOA of botulinum toxin?
Cleavage of SNARE proteins prevents the release of vesicles containing ACh
What is bulbar weakness?
The bulb is part of the lower pons, so dysarthria and dysphagia
Which has greater proximal and which has greater distal weakness: myopathy vs neuropathy?
Neuropathy = greater distal weakness
Myopathy = greater proximal weakness
Stocking glove distribution of sensation loss is usually a LMN or UMN lesion?
What defines acute vs chronic weakness of neuromuscular disorders?
Acute = days-weeks
Chronic = months to years
Anterior horn cell damage = what type of deficit?
Motor only--no sensory
How do you differentiate between demyelination and cell body nerve problems?
Demyelination can have relapses, while cell body lesions do not.
UMN lesions what is the diagnostic modality that is especially helpful?
LMN lesions what is the diagnostic modality that is especially helpful?
Nerve conduction test
Fasciculations is a hallmark of UMN or LMN lesions?
What is the amyotrophic part ALS?
symptoms of weakness, atrophy, and fasciculations
What is the lateral sclerosis part of ALS?
UMN in the DC/ML pathway, producing s/sx of hyperactive DTRs, clonus, and babinski signs
Which has a better prognosis: the lateral sclerosis or the amyotrophic form of ALS?
What is the mean age of onset of ALS?
What is the incidence of ALS?
What is the prognosis with ALS?
Variable--Respiratory involvement is much worse than limb involvement
Bulbar involvement with ALS leads to what?
Flaccid dysarthria, tongue atrophy, fasciculations
True or false: for the most part, ALS spares cognition, EOMs, and sphincters
How does ALS usually present initially?
Single limb issue
What are fasciculations, generally?
"Worm-like" movement beneath the skin of a patient that does not move a limb
How do you diagnose ALS?
-Clinical diagnosis is correct more than 95% of the time
What are the antibodies that can be tested for in multi-focal motor neuropathy?
Which usually has eye findings: MG or ALS?
How often are fasciculations in people in general (without ALS)? How do you differentiate this with ALS?
What is the diagnostic criteria for ALS?
UMN and LMN signs with sensattion spared in:
-bulbar region and 2 spinal regions
-three spinal regions
What is the cause of ALS?
Unknown--can be familial, but usually sporadic
What is the symptomatic treatment for ALS?
What is the MOA of Riluzole in treating ALS?
What are the usual causes of mononeuropathies?
Single nerve damaged, leading to deficits in a single nerve distribution
What is mononeuritis multiplex?
When two or more (typically just a few, but sometimes many) separate nerves in disparate areas of the body are affected it is called "mononeuritis multiplex,"
What is allodynia?
Neuropathic pain following nonpainful stimuli
What is dysesthesia?
Unpleasant sensation that is either spontaneous or evoked by pressure, movement, or touching
What is hyperalgesia?
heightened pain sensation provoked by painful stimulus
What are paresthesias?
Irritating spontaneous sensation
What are the motor signs of peripheral neuropathies?
Distal weakness and foot drop
What are the sensory findings with peripheral neuropathies?
Loss of vibratory, position, and/or pin
What are reflexes like with peripheral neuropathies?
Hypoactive to absent
What is the role of EMG in diagnosing peripheral neuropathies?
Helpful in distinguishing axonal vs demyelinating neuropathy
What is the most common type of neuropathy?
What is toxic neuropathies?
Uremia induced neuropathy, or extrinsic toxins of neuropathy
What are the two major extrinsic toxins that can cause neuropathies?
What are the two major immune mediated neuropathies?
AIDP-(acute inflammatory demyelinating polyneuropathy) = GB
CIDP (Chronic Inflammatory Demyelinating Polyneuropathy)
What is Charcot-Marie-Tooth disease (hereditary motor sensory neuropathy)?
AD disease caused by mutations that cause defects in neuronal proteins involved in the production and function of peripheral nerves
What are the classic foot findings of CMT disease?
Pes cavus and hammer toe
What is the expressivity of CMT?
What is the frequency of CMT?
What is the most common inherited neurological disorder?
What is the mode of inheritance of CMT?
What is the usual presentation of CMT?
Slow, progressive distal weakness; difficulty walking
What is a steppage gait?
Bring the knees high to clear the anterior tibialis
What are the two types of CMT? What are the EMG findings of each?
Demyelinating (slow NCV)
Axonal (normal NCV)
How do you definitely diagnose CMT?
GB is a motor or sensory neuropathy?
Motor--sensation is intact
What is the classic CSF finding of GB?
Albuminocytological dissociation (no WBC increase, but marked protein)
What are the two viruses that can cause GB?
What are the two bacteria that predispose to GB?
What are the usual s/sx of GB?
What fraction of GB syndrome patients develop respiratory issues?
What is the Fisher syndrome variant of GB syndrome?
Starts with ophthalmoplegia, ataxia, and loss of DTRs
What is the time course of GB disease?
50% reach nadir in 2 weeks, with slow recovery over months
What percent of patients who develop GB have residual symptoms?
Suspected GB syndrome, but with marked pleocytosis = ?
Presence of fibrillations with GB implies a shorter or longer course?
What is the treatment for GB syndrome?
Plasma exchange or IVIG
Which usually has more pronounced atrophy: LMN lesions or UMN lesions?
The cell body of a LMN is located where?
ventral horn of the spine cord
What is the MOA of tetanus toxin?
Cleaves synaptobrevin, leading to blocked release of GABA and glycince across the synaptic cleft
True or false: the tongue is never affected with ALS
False--can have hypoglossal nerve involvement, leading to atrophy of the tongue
Which nerves are more myelinated:DC/ML nerves, or spinothalamic tract nerves?
Inverted champagne bottle appearance of the leg = which disease?