Weakness COPY Flashcards

1
Q

Describe broadly the areas where neuromuscular weakness can originate

A

1) CNS
2) PNS
3) Neuromusuclar junction
4) myofibers

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2
Q

Discuss causes of nonneurological generalized weakness

A

Alteration in plasma volume (dehydration)
Alteration in plasma composition (blood glucose, electrolytes)
Derangement in circulating red blood cells (anemia or polycythemia)
Decrease in cardiac pump function (MI)
Decrease in systemic vascular resistance (vasodilatory shock)
increased metabolic demand (local or systemic infection, endocrinopathy, toxin)
Mitochondrial dysfucntion (severe sepsis or toxin mediated)
global depression of the CNS (sedatives, stimulant withdrawals)

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3
Q

Where are the anatomical origins of lower motor neurons

A

Peripherally the spinal nerves extend from the anterior horn of the spinal cord

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4
Q

Describe symptoms of UMN lesions

A

Spaciticity to extension in the upper extremities and to flexion in the lower extremities
Hyperreflexia
Hoffmans sign – middle finger is held loosely then flicked down, positive if the thumb flexes and adducts
Babinskis

UMN signs signify lesions within the cerebral cortex or corticospinal tract (CST)

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5
Q

Describe lower motor neuron symptoms

A

Flaccidity
decreased reflexes
faciculations or muscle cramps

Conditions that have only peripheral effects at the NMJ and muscle have preserved reflexes

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6
Q

Discuss a broad approach to generalised weakness

A

In the generally weak patient rule out non neurological causes first – check circulating volume and composition
Check cardiac fucntion, red cellfucntion, systemic vasuclar tone and oxygenation.

If substrate delivery and plasma composition appear sufficient consider disturbance of cellular metbaolic machinery secondary to an endocrinopathy, toxin or mitochondrial dysfunction.

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7
Q

List a differential for combination of arm hand or leg weakness with ipsilateral facial involvement

A

This presentation is generally caused by a lesion in the contralateral cerebral cortex or the CST

Patient with equal loss of strength to the face hand and leg are more likely to have subcortical lesions disrupting all of these lesions as they funnel close together in the internal capsule

Sudden onset of weakness pattern implies haemorrhage or mass lesion
Gradual onset may be seen in demylination (e.g MS, acute demyelinating encephalomyelitis) or neoplasm (metastasis, astrocytoma, oligodendroglioma, ependymoma)

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8
Q

Discuss a differential for a combination of arm, hand or leg weakness with contralateral facial invovlement

A

This pattern indicates a brainstem lesion. – do a cranial nerve exam to identify the level of the lesion
Depressed concious state can be present if the brainstem reticular activating system is involved.

The two main broad resions for this presentation are vertebrobasilar insufficiency and demyelinating disease.

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9
Q

Discuss broad differential for limb weakness without facial involvement

A

A lesions in the medial contralateral cerebral homunculus (over the area where the lower extremity is represented)
A discrete internal capsule or brainstem lesion involving only the CST rather the the coticobulbar tracts
Brown sequard internal capsulr or brains stem lesion

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10
Q

Discuss brown sequard lesions

A

Caused by hemisection of the spinal cord involving all three major spinal tracts

1: corticospinal tract - causing ipsilateral flacid paralysis - from the level of the lesion
2: lesions of one or both DCML
- -lesions to fasciculus gracilis or fasciculus cuneatus result in ipsilateral proprioception loss as well as loss of all fine touch sensation
3: loss of the spinothalmic causes loss of contralateral temperature and pain sensation beginning one or two levels below that of the lesion

Causes:

  • Trauma
  • Demylination
  • Ischaemia
  • TB
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11
Q

Discuss differential for weakness in only one limb

A

Isolated weakness of one extremity is usually caused by a spinal cord or peripheral nerve lesion

Can include

  • connective tissue disorder
  • external compression (entrapment syndrome, compressive plexopathy)
  • endocinopathy
  • paraneoplastic syndrome
  • toxins
  • trauma
  • vitamin D

Emergent

  • iscahemai or compression of spinal cord
  • acute demylination such as guillaine barre
  • NMJ dysfunction – myasthenia or cholinergic crisis, botulism
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12
Q

Discuss differentials for weakness confined to lower extremities

A

Often will be accompanied by loss of pain and temperature as the spinothamlic tract runs close to the CST

DDX include

  • Cauda equina
  • anterior cord syndrome
  • GBS
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13
Q

Discuss anterior cord syndrome

A

Caused by interruption of blood supply via the anterior spinal artery affected the CST and spinothalmic tract

Characterised by

  • complete motor paralysis below the level of the lesion
  • loss of pain and temperature
  • preservation of proprioception and touch
  • autnomic dysfunction with variation in BP and bowel and bladder dysfunction
  • Flaccid internal and external anal sphincter, bladder retention

The most common cause is insufficienies in the aorta ie, dissection or AAA

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14
Q

Briefly discuss cauda equina syndrome

A

Caused by damage to the cauda equina the bundle of nerves which continues from the spinal cord - L4-5 - lower motor lesion

Incomplete
-loss of urgency or decreased sensation wihtout incontinence or retention

Comeplte
-urinary and or bowel retention or incontience

Signs and symtpoms incldue

  • back pain
  • saddle anaethesia
  • Bladder and bowel dysfunction, decreased tone in urinary and anal sphincters, weakness of detruser leading to retention and post void residual incontinence
  • sciatica
  • sexual dysfucntion
  • absent anal reflex
Causes
1) degenerative 
-lumbar disc herniation most common at L4/5 and L5/s1 
-Lumbar spinal canal stenosis 
2) infalammatory 
-ank spond 
3)  trauma 
-fracture dislocation 
4) infection 
-arachnoiditis 
-epidural abscess
5) tumor 
-pirmary 
mets
6) vascular 
-aortic dissection 
-AVN
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15
Q

Discuss differential for weakness isolated to the upper limbs

A

This central cord pattern loaclizes to the central portion of the cervical spinal cord where CST fibers for hand and arm strength are located.

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16
Q

Discuss central cord disease

A

The most common form of cervical cord injury - characterised by loss of motion and sensation in arms and hand

Caused by trauma , hyperextension injuries
Syringomyelia

17
Q

Discuss DDX of all four limb weakness without facial involvment

A

Cord lesion complete

18
Q

Discuss DDX of proximal portion extremities only

A

Issues with myofiber

19
Q

Discuss DDX of distal portion of extremies onlyu

A

Almost always peripheral neuropathy

Can include

  • connective tissue disorder
  • external compression (entrapment syndrome, compressive plexopathy)
  • endocinopathy
  • paraneoplastic syndrome
  • toxins
  • trauma
  • vitamin D
20
Q

Facial weakness without extremity involvement

A

Two broad groups

Limited to facial nerve weakness – nil sparing of forehead
-caused be Bells, parotitis or mastoiditis

Not limited to cranial nerve 7

  • this will include some combination of ptosis, binocular diplopia, dysarthrai or dysphagia
  • dysfucntion of one or more ocular, facial or pharyngeal muscles will be the most common presentation for NMJ pathology
  • patient with an abnormality of the presynaptic release of acetylcholine (eg botulsim, eaton lambert syndrome , organophosphate poisoning) can have autonomic ganglia involvement and hence abnormal pupillary repsonse to light, dry mouth, fluctuation in heart rate and blood pressure, anhidrosis or GIT and bladder dysmotility.
21
Q

Discuss clinical signs specific for origin of weakness

A

1) UMN
- Atrophy: none
- Fasic: non
- Deep tendon: hyperreflexia
- Distribution: pyramidal/regional
- Tone: spastic
- Plantar upgoing

2) LMN
- Atrophy: severe
- Fasic: common
- Deep tendon: areflexia
- Distribution: Distal segmental
- Tone: spastic
- Plantar downgoing or absent

3) NMJ
- Atrophy: mild
- Fasic: none
- Deep tendon: normal
- Distribution: variable fatigueable
- Tone: decreased flaccid
- Plantar downgoing or absent

4) Myopathy
-Atrophy: mild
-Fasic: none
-Deep tendon: normal
-Distribution: proximal>distal
-Tone: normal
-Plantar downgoing or absent
t

22
Q

Discuss causes of non neuromuscular weakness

A

1) anaemia
2) cardiac failure ‘
3) malginancy
4) psych
5) malnutrition
6) chronic fatigue
7) medications - steroids, statins, antiretrovirals, alcohol, colchicine
8) acute electrolyte derangement (hypo and hyperkalaemia, hypocalcaemia)
9) sepsis
10) dehydration
11) hypothyroidism
12) chronic dsiease

23
Q

Discuss DDX of primary neurological causes of weakness

A

1) GBS
- Immune mediate polyradiculopathy
- post infective (15-40%) espicially due to campylobacter or viral infection
- >50% idiopathic
- Symmetrical ascending flaccid weakness, loss of deep tendon reflexes (DTRs)
- CSF high with normal glucose and cell count

2) Myasthenia gravis
- immune mediated Ach receptor dysfunction may be precipitated by thymic disorders
- fluctuating fatigable weakness of voluntary muscles especially ocular or proximal limbs
- CN involvement with ptosis in >25% of casus
- dysphagia weakness of masticatory muscles
- improves with rest
- serial resp assessment if severe
- ice pack test if there is ptosis

3) MS
- immune mediate scattered neuron demyelination; affects motor sensory visual and cerebellar function
- classically >2 separate episodes of neuo dysufnction indicating white matter or spinal cord lesions at distinct locations.

4) cord compression
- spinal stenosis +- malignancy or infeciton

24
Q

Discuss DDX of myopathies causing weakness

A

1) Congenital (duchenne muscular dysptrophy, becker muscular dystrophy)
- x linked dystrophin gene
- males effected more severely by DMD life expectancy to early 20s
- Generalised weakness
- usual ED presentation is acute deterioration with respiratory compromise
- spirometry/respiratory assessment

2) acquired
- metabolic
- hypokalaemia periodic paralysis
- endocrine
- cushing
- addisons
- thyrotoxicosis
- toxin
- statin corticosteeroids

25
Q

Discuss DDX of wekaness from intoxications

A

1) botulism due to c botulinum
- derangement of neurotransmission
- ingested botulinum toxin prevent Ach release at the NMJ
- descending flaccid paralysis
2) tetanus due to clostridium tetani
- impaired inhibitory neurotransmission causing skeletal muscle spasm and rigidity
3) tick paralysis
4) marine intoxications
- ciquatera - parasthesias, electrical sensation in response to hot and cold
- blue ringed octopus - tetrodotoxin
- puffer fish

26
Q

Describe Myasethenia gravis

A

Caused by an idiopathic autoimmune attack of post synaptic Ach receptors leading to weakness of the muscle response to stimulus
The weakness tends to be fatigable and improve with rest

Most patients experience facial and bulbar muscle weakness therefore dysphagia and dysarthria are common symptoms

Mayasethinc crisis which occurs in 15-20% of patients refers to generalized weakness with resp failure requiring intubation and mechanical ventilation. Crisis can be precipitated by

  • disease progression
  • intercurrent infection
  • pregnancy
  • surgery
  • high dose steroid treatment.
27
Q

Discuss the diagnosis of MG

A

Clinical and the presence of antibodies to the Ach receptor which are found in 85% of cases.

Clinical suspicion of MG can be supported by a positive bedside ice pack test in patients with ptosis where the eyelides are covered with an ice pack for 2 minutes bring about immediate improvement in ptosis

28
Q

Discuss treatment and disposition of myasthenia

A

Pyridostigmine
Resp failure is the main lef threat - serial PEF/FVC and paco2 are recommended

Plasmapheresis and IVIG have also been shown to be effective in myasthenic crisis

29
Q

Describe Amyotrophic lateral sclerosis

A

Is rapidly progressive muscle atrophy and weakness caused by degeneration of both the UMN adn LMN. IT causes a variable picture of spasticity hyperreflexia and muscle weakness with an inexorable decline to respiratory failure and dependence on a ventilator within 2-4 years.

30
Q

Describe Eaton Lambert syndrome

A

Rare autoimmun paraneoplastic conditions that is usually associated with small cell lung cancer.

The major clinical feature is severe limb weakness as a result of autoimmune destruction of voltage gated calcium channels in the presynaptic membrane at the NMJ which in turn inhibits the relese of presynaptic ach vesciles.

It often improves with exercise which distinguishes it from MG

EMG required for diagnosis

Management involves treatment of the underlying cancer so prognosis is linked to this

31
Q

Describe polymyositis and dermatomyositis

A

Idioapthic inflammatory myopathies that mostly affect women over 30 years of age.

Dermatomyosistis is associated with a heliotrope rash (reddish purple rash on or around the eyelids) erythroderma and other skin changes and is more commonly associated with acncer. Patients present with proximal symmetrical wekaness associated with muscle pain and tenderness. DTRs are intact unless weakness is severe. THere are no sensory or autonomic findings

32
Q

Describe acute periodic paralysis

A

They can be associated with normal low or elevated serum potassium. Patients are usually well between attacks.

Acute hypokalaemic periodic paralysis may be primary (familial) or secondary to excessive renal or GI loss or endocrinoapthy.

Familial periodic paralysis usually occurs in caucasian males is autosomal dominant and may last as long as 36 hours. Attacks usually occur at night or in the early morning upon awakening and can be precipirated by a diet high in carbs, rest following exercise or glucose and insulin given IV. Supportive care and replenishment of serum K are the main management priorities.

Thyrotoxic periodic paralysis is associated with hypokalaemai and more commonin in Asian males.

33
Q

Describe conos medullaris

A

Spinal injury syndrome at the level of t12-l2
Similar symptoms to cauda equina
-saddle anaethesa
-loss of bladder reflex - urinary retneion
-loss of bowel refelx - incontience
-lower limb motor weakness paraesthesia and numbness (mixed upper and lower)