Lecture 6 - Neuromuscular Diseases Flashcards by Alexander Lee

The type of neuropathy a patient has depends on the ___________

type of fibers affected

are they myelinated or unmyelinated fibers?

Are they A Delta, A beta, …..?

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You’re more likely to have a ______ injury than an injury at the plexus

nerve root/nerve injury

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What is ALS?

Does it have UMN or LMN degeneration?

Who is more commonly affected?

Progressive fatal disease that has degen of both UMN and LMN

More men than women

10% familial

Average life expectancy after diagnosis: 3 years

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What is a common way of diagnosing ALS?

You see atrophy of tongue muscles, fasciulations of tongue muscles

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T or F: ALS also causes cognitive problems

F, no cognitive probems

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C9orf72 ALS vs SOD1 ALS

SOD1 is a rare type that is actually treatable

C9orf72 is the common form that is not treatable

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What are the most common symptoms of ALS?

Fatigue

Muscle stiffness

SOB

muscle cramps

increased saliva

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How is the west nile virus transmitted

Mosquito bites

note: peak transmission between july-october

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T or F: most cases of west nile virus are asymptomatic

T, 80% asymptomatic

20% have a fever

Less than 1% are neuroinvasive

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What is acute flaccid paralysis

Usually after viral illness

acute onset and rapid progression of asymmetric flaccid weakness and hypoactive/absent reflexes, respiratory insufficiency, bowel and bladder problems

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What is poliomyelitis

Virus that is usually aymptomatic

minor disease- flu like symptoms

major disease- CNS invasion (less than 1% fo cases)

Has been eradicated in north america

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What is the diagnostic criteria for post-polio syndrome?

History of paralytic poliomyelitis

period of partial or complete recovery

gradual onset of progressive and presistent muscle weakness

symptoms for +1 year

exclude other causes

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Radiculopathy is most common in what nerve root?

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Symptoms of radiculopathy

Pain, numbness, tingling in dermatome

weakness in myotome

reduced reflexes

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__% of cervical radiculopathies due to herniated disc improve without surgical intervention

note: surgery is need if: Not responding to PT, significant weakness

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How do you differentiate between radiculopathy and lower motor neuron lesion?

Radiculopathy is in a dermatomal distribution AND shooting pain down neck

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If you’ve got more than one nerve distribution affected by radiculopathy in one arm or leg, what is a possible cause?

Plexopathy

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What are the signs and symptoms of plexopathy? What is plexopathy most commonly caused by?

typically painful, restricted to single limb, more widespread than single nerve root

most commonly casued by trauma, tumor, or thoracic outlet syndrome

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What is mononeuropathy

what are the signs and symptoms

what is it caused by?

Neuropathy of single peripheral nerve

Signs and symptoms: restricted to anatomic distribution of one nerve

etiology: usually compression, entrapment, trauma

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What is polyneuropathy?

generalized process affecting (many) peripheral nerves

signs and symptoms: depending on which nerves are involved

commonly isometric and length dependent weakness, sensory loss, loss of DTR

stocking/glove distribution

hundreds of reasons you could have this

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How do you characterize polyneuropathy?

Is it sensory, motor, or autonomic?

is it large or small fibers?

demyelinating or axonal?

hereditary or accquired?

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What nerves are myelinated?

Which ones arent?

Myelinated: Motor, sensory A-Beta A-Delta, Some autonomic

Unmyelinated: Sensory C fibers, some autonomic

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Most myelinated fibers are _____ diameter and mainly __________

large

mainly motor

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What is the main kind of neuropathy we can treat w/ medication (neurologists can)

demyelinating

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What are the suggestive features that someone has demyelinating neuropathy (polyneuropathy)

**weakness without atrophy** (at first) length independent patchy asymmetric

What are the suggestive features that tell you it's an axonal problem vs a demyelinating problem?

Suggestive features of an **axonal** problem: Distal > proximal Legs > arms (longer axons more susceptible) muscle wasting early (demyelinating doesnt have early muscle wasting)

What are two common causes of axonal problems?

Diabetes (most common) Alcohol (second most common)

What are 2 examples of "small" nerve fibers

Sensory nerves (specifically pain and temp) Autonomic nerves

Patient's with small fiber neuropathy will experience what symptoms?

Autonomic dysfunction allodynia neuropathic pain discoloration of skin, reduced sweating, hairloss reduced ability to feel pain, hot/cold

what are examples of large diameter nerve fibers?

Motor Nerves Sensory nerves that carry pressure/vibration/proprioception

Patients that have "large fiber neuropathy" will present w/.....

Numbness, tightness, balance problems "walking on foam/cobblestone" (no proprioception)

Orthostatic hypotension arrythmias severe constipation, ED, Urinary Retention abnormal sweating early satiety, light headedness w/ meals These are signs of what kind of problem?

Autonomic neuropathy

What are 2 of the most common causes of polyneuropathy?

1. Diabetes 2. Alcohol Others: Thyroid disease, vitamin deficiency, systemic problems, infections

What is an example of a **rapidly progressing** inflammatory demyelinating polyneuropathy? What is an example of a **slowily progressive** inflammatory demyelinating polyneuropathy

**Rapid:** Acute inflammatory demyelinating polyneuropathy (AIDP) or Guillain Barre syndrome. -progression ends in majority by 4 weeks **Slow:** Chronic inflammatory demyelinating polyneuropathy (CIDP) -Weakness progresses for over 2 months

What are common causes of Acute inflammatory demyelinating polyneuropathy (AIDP)

preceding illness, surgery, vaccination in some

How does Acute inflammatory demyelinating polyneuropathy (AIDP) progress?

1. Tingling paresthesia w/ little sensory loss 2. severe radicular back pain 3. Weakness (mild to complete quadriplegia)

How is Acute inflammatory demyelinating polyneuropathy (AIDP) usually diagnosed with an exam?

Exam: -symmetric weakness, -facial weakness -sensory loss variable -areflexia in most after 1 week note: also diagnosed w/ CSF and EMG

What is the progression of Guillain Barre syndrome

Monophasic

How do you treat Guillain Barre syndrome?

Supportive care: monitor/treat respiratory failure, autonomic dysfunction -plasma exchange and IVIG (anti immune system drug) NO STEROIDS **Rehab starts early**

at the start of demyelinating conditions you do not see muscle __________, but you do see muscle ___________

atrophy weakness

Who is normally affected by Chronic inflammatory demyelinating polyneuropathy (CIDP)?

Male > female Average onset 50+ Usually relapsing-remittent or can be progressive

How does Chronic inflammatory demyelinating polyneuropathy (CIDP) progress?

slowily over months **Motor** > sensory symmetric **proximal and distal weakness** which progresses to atrophy hypo or areflexive sensory loss mainly in legs, rarely disabling

What condition is slowily progressive with symmetric weakness in proximal and distal muscles hypo/areflexive

CIDP

What is the normal length dependent pattern in polyneuropathy

stocking/glove

How does diabetic neuropathy usually present?

Distal symmetric sensory polyneuropathy "stocking/glove" usually affects LE/UE slowily progressive no weakness autonomic problems

diabetic neuropathy affects what % of diabetics

65%

What condition is associated w/ **large weight loss** begins **abruptly** begins w/ sharp aching pain asymmetrically in hip and thigh, spreads to leg and foot **pain** -> progressive **weakness** of thigh and knee -> weakness progresses distally often becomes **bilateral**

Diabetic **lumbosacral** radiculoplexus neuropathy AKA **diabetic amyotrophy** note: usually affects DM2 in middle or older age

Hereditary neuropathies: Charcot-marie-tooth disease Hereditary sensory autonomic neuropathy amyloid HNPP These commonly have an insidious onset when??

Under 20 years old

What condition presents w/ distal weakness and atrophy length dependent sensory loss foot deformities: pes cavus, pes plannus, hammer toe abscent or decreased reflexes chronic slow progression

Charcot-Marie-Tooth-disease

What can clue you in that a demyelinating condition is hereditary

delayed early milestones slow progression foot deformities, injuries, surgeries family history hip problems/socliosis abscence of positive sensory symptoms but prominent sensory loss on exam

Charcot Marie Tooth disease clinical phenotypes: Early onset: Childhood onset: Late onset:

Early onset: delayed onset of walking (>15 months) Childhood onset: Normal onset of walking slow runner as a child develops symptoms between 5-20 most common presentation late onset group: 20+

Supportive treatment for Charcot Marie Tooth disease

PT and OT orthotics screen for scoliosis and hip dysplasia surgery for skeletal and soft tissues abnormalities

Lambert eaton myasthenic syndrome and botulism are forms of __________ disorders myasthenia gravis is a ___________ disorder

Presynaptic Post synaptic

How can you differentiate myasthenia gravis from MS?

Pt improves w/ rest

What disease presents as: Flucuating/fatiguable (can test fatigue w/ eye muscles) asymmetric ptosis/diplopia difficulty chewing dysarthra,dysphasia, nasal speech hoarseness head drop limb weakness

Myasthenia gravis

Who does Myasthenia gravis mainly affect?

Younger woman and older men

What are common triggers for Myasthenia gravis

Emotional Upset Viral Illness/systemic illness heat or hyperthyroidism surgery menstral cycle drugs

What drugs make Myasthenia gravis worse?

D-Penicillamine Botox Magnesium Salts IFN Succinylcholine

How to diagnose Myasthenia gravis?

**Tensilon test**- The test involves injecting the drug Tensilon (edrophonium chloride) intravenously into a patient's arm or hand, and then observing their response to repetitive physical movements: **Ocular cooling -** Their eye works better after u put a cold pack on it because cold makes the NM junction work better antibody panel serum EMG

Symptoms of Myasthenia gravis

fatigue exercise intolerance weakness myalgia cramps stiffness atrophy

How do you identify myopathy on a physical exam

Usually proximal weakness -identify w/ GOWER'S SIGN (they have to get up from the ground using their hands to post) \ DTR can be normal or slightly reduced

Polymyositis, dermatomyositis, and inclusion body myositis are all examples of...

inflammatory myopathies

How do polymyositis and dermatomyositis usually present?

Subacute/chronic onset of **proximal weakness** variable pulmonary and cardiac involvement usually involve a malignancy (cancer) autoimmune disorder HIV Dermatomyositis: Inflammation of the skin aswell, theyre all red and inflamed in different places

What supplementation is important for bonehealth for patients w/ dermatomyositis

Calcium and vitamin D

Who most commonly gets inclusion body myositis?

50+ men

How does inclusion body myositis usually present?

Proximal leg and distal arm weakness (finger flexors and quad) asymmetric muscle atrophy dysphagia sometimes associated w/ autoimmune disease note: 56% need an AD after 7 years (20% WC)