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NEURO > Muscle and nerve disorders > Flashcards

Muscle and nerve disorders Flashcards

1
Q

where does the UMN span

A

cortex through spinal cord and anterior horn cell

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2
Q

where does the LMN span

A

anterior horn cell to nerve ending muscle

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3
Q

what are muscles designed to do

A

designed to covert chemical energy into mechanical energy

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4
Q

what are the components of the chemical to mechanical process

A

Excitation-contraction coupling
contractile mechanism
Structural components
The energy system

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5
Q

what are the symptoms of muscle disease

A

Poor suck / feeding / failure to thrive / floppy

Weakness of skeletal muscle

Short of breath (respiratory muscles)

Poor swallow (aspiration)

Cardiomyopathy - heart failure, arrhythmias

Cramp, pain, myoglobinuria (muscle breakdown, excrete myoglobin in urine - turns urine black)

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6
Q

what are the signs of muscle disease

A

Wasting/ hypertrophy (because muscles aren’t relaxing)

Normal or reduced tone and reflexes

Motor weakness…NOT sensory

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7
Q

what investigations can be done for muscle disease

A

History and examination

CK - elevated creatinine kinase

EMG

Muscle biopsy

  • Structure
  • Biochemistry
  • Inflammation

Genetic testing

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8
Q

what are the different types of muscle disease and where can they occur

A

Muscular dystrophies

Channelopathies

Metabolic muscle disease

Inflammatory muscle disease

Congenital myopathies

Iatrogenic - medication

occur all along the sarcolemma (muscle cell membrane)

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9
Q

what do muscular dystrophies affect

A

mechanics of the muscle – structure – most genetic

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10
Q

what are the different types of muscular dystrophies

A

Duchennes MD - dystrophin protein defect

Beckers MD - dystrophin protein defect

facioscapulohumeral MD - facial weakness, scapular weakness, biceps weakness

myotonic dystrophy

limb-girdle MD - upper and lower limb proximal weakness

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11
Q

what are chanelopathies

A

disorders of Ca, Na, Cl channels

Symptoms episodic – periods of weakness – or muscles that don’t relax – due to irregular channel fluxes

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12
Q

list some chanelopathies

A

Familial hypokalemic periodic paralysis

Hyperkalemic periodic paralysis

Paramyotonia congenita

Myotonia congenita

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13
Q

what is affected in Familial hypokalemic periodic paralysis

A

K channels

Most common – intermittent symptoms – can last minutes, days – key to measure K – often associated with thyroid disease

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14
Q

what is affected in hyperkalemic periodic paralysis

A

K channels

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15
Q

what is affected in paramyotonia

A

Ca channels

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16
Q

what is affected in myotonia congenital

A

Ca channels

muscles feel stiff - constant low level contraction

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17
Q

give examples of metabolic muscle disease

A

Disorders of carbohydrate metabolism

Disorders of lipid metabolism

Mitochondrial myopathies/ cytopathies

Endocrinopathy (thyroid, Cushing)

Biochemical abnormalities

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18
Q

what do disorders of carbohydrate metabolism cause

A

low level weakness but much larger weakness on exercise

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19
Q

what do disorders of lipid metabolism cause

A

Weakness on longer periods of slower exertion

20
Q

what can biochemical abnormalities cause

A

Prolonged vomiting

some drugs – bring K down – causes weakness

21
Q

give examples of inflammatory muscle diseases

A

polymyositis - affects just the muscles

dermatomyositis - affects muscles plus skin inflammation

22
Q

how do inflammatory muscle diseases present

A 
acute or subacute 
painful weak muscles
characteristic rash of dermatomyositis 
any age
systemic upset - feel generally unwell
23
Q

what investigations can be done for inflammatory muscle disease

A

↑CK
Electromyogram, inflammation and myopathic

Biopsy

  • PM: CD8 cells
  • DM: humeral-mediated, B cells and CD4 cells
24
Q

what is the treatment for inflammatory muscle disease

A

immunosuppression

steroids

25
what is a disorder of the neuromuscular junction
myasthenia gravis
26
what is the presentation of myasthenia gravis
``` Fatiguable weakness: Limbs Eyelids (ptosis) Muscles of mastication (chewing), swallow Talking SOB diplopia ``` as you use muscles - starts out strong and gets weaker - fine in the morning, gets worse as the day progresses
27
what muscles does myasthenia gravis affect
skeletal muscle only i.e. doesn't affect the heart
28
what investigations can be done for myasthenia gravis
AChR antibody - blocks binding site for ACh Anti MuSK antibody neurophysiology - repetitive stimulation (can look for fatigue) - jitter CT chest - thymoma - cancer associated with myasthenia gravis
29
what is the treatment for myasthenia graves
Symptomatic - Acetylcholinesterase inhibitor Immunosuppression - Prednisolone - Steroid saving agent (azathioprine) Immunoglobulin - binds to antibodies Plasma exchange - gets rid of antibodies in blood Thymectomy if you can get rid of the antibody - can get person into remission
30
what does the peripheral nerve consist of
sensory axons - small fibres (pain, temp) - large fibres (joint and position sense, vibration motor axons
31
what are the three groups of nerve disease
root disease lesion of the individual peripheral nerve generalised peripheral neuropathy
32
what can cause root disease
degenerative spine disease inflammation infiltration
33
what are the symptoms and signs of root nerve damage
Myotomal wasting and weakness Reflex change Dermatomal sensory change
34
what can cause individual peripheral nerve lesions
compressive/entrapment vasculitic eg carpel tunnel
35
what are the symptoms and signs of individual nerve lesions
Wasting and weakness of innervated muscle Specific sensory change
36
what is characteristic of generalised periphery neuropathy
motor/sensory/both affected affects all nerves – affects longest nerves first as they have the highest energy demand – present first in feet, then hands, then work inwards
37
what can cause generalised peripheral neuropathy
Metabolic: Diabetes, alcohol, renal, B12 Toxic: drugs Hereditary Infectious: Lyme, HIV, leprosy Malignancy: paraneoplastic Inflammatory demyelinating - Acute = Guillain Barre syndrome - Chronic = chronic inflammatory demyelinating polyneuropathy
38
what are the triggers for inflammatory demyelinating generalised neuropathy
acute - e.g. guillain barre - triggered by infection - usually diarrhoea from campylobacter, can also be triggered by cytomegalovirus chronic - no known triggers
39
what are the symptoms and sings for generalised peripheral neuropathy
Sensory and motor symptoms starting distally and moving proximally
40
what are investigations for peripheral nerve disease
Blood tests Genetic analysis Nerve Conduction Studies Lumbar puncture (CSF analysis - not many cells but very high protein content) Nerve biopsy (nb sensory nerve)
41
what does disease of the anterior horn affect
anterior horn cell upper motor neuron corticospinal tract coticobulbar tract
42
what does disease of the anterior horn cause
motor neuron disease
43
what is the progression of motor neuron disease
usually limb onset, later bulbar and respiratory involvement
44
what are the signs for motor neurone disease
LMN - muscle fasiculations, wasting, weakness UMN - increased tone, brisk reflexes NO SENSORY INVOLVEMENT
45
how is motor neurone disease diagnosed
mostly clinical - unique combination of UMN and LMN signs - no sensory issues EMG
46
what is the treatment for motor neurone disease
supportive (PEG, NIV< physio, OT care) riluzole - glutamate antagonist – blocks action – can prolong survival by a couple months

NEURO (51 decks)

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