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Neurology > Motor Neurone Disease > Flashcards

Motor Neurone Disease Flashcards

1
Q

what is an upper motor neurone disease?

A

damage to corticospinal tracts anywhere from motor nerve cells in primary cortex, internal capsule, brainstem, cord, to synapse, with anterior horn cells in cord

  • NEED IMAGING OF PATIENT’S HEAD ( and spine)

tone up, reflexes up, spasticity, weakness, Babinski’s = upper motor N lesion ( pseudobulbular)

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2
Q

what is a lower motor neurone

A

damage anywhere from, anterior horn cells in cord, nerve roots, plexi or peripheral nerves

tone lower, reflex down, wasting, fasciculations= lower motor neurone N ( bulbar)

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3
Q

what is the difference between spasticity and rigidity?

A

spasticity is velocity dependent unlike rigidity is not velocity dependent

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4
Q

what is the difference between UMN and LMN signs and conditions like parkinson’s?

A

UMN and LMN signs and there is no impairment of bladder, bowel or sexual function- this differentiates it form parkinson’s

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5
Q

define motor disease?

A

a progressive neurodegenerative disorder of cortical , brainstem and spinal motor neurones ( low and upper motor neuron

-> there is selective loss of neurones in motor cortex, cranial nerve nuclei and anterior horn cells

NO SENSORY LOSS

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6
Q

what are the subtypes of motor neurone disease?

A

amyotrophic lateral sclerosis

progressive muscular atrophy variant

progressive bulbar palsy variant

primary lateral sclerosis variant

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7
Q

what are the subtypes of motor neurones disease?

A

amyotrophic lateral schlerosis ( ALS)

Progressive muscular atrophy variant

progressive bulbar palsy variant

primary lateral sclerosis variant

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8
Q

describe amyotrophic lateral sclerosis?

A

Aka lou Gerhig’s disease

combined neurodegeneration of upper and lower motor neurones-> mix of LMN and UPN signs

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9
Q

describe progressive muscular atrophy variant?

A

only LMN neurone

better prognosis

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10
Q

describe bulbar palsy variant?

A

only affects CN9-12

dysarthria

dysphagia

wasting fasciculating tongue

brisk jaw jerk

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11
Q

describe primary lateral sclerosis variant?

A 
loss of Bets cells in motor cortex 
UMN pattern of weakness
Brisk reflexes 
extensor plantar response 
no LMN signs
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12
Q

what is pseudo bulbar palsy?

A
  • UMN
  • V, VII, X, XI and XII

• Stroke of internal capsule
MS
MND

  • Spasticity, hyperreflexia
  • Donald duck speech
  • Emotions labile
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13
Q

what is bulbar palsy?

A
  • LMN
  • X, XI and XII

• MND
Guillain-Barre

  • Fasciculations, wasting, hyporeflexia
  • Nasal speech
  • Emotions normal
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14
Q

outline the aetiology of motor neurone disease?

A

UNKNOWN

free radicle damage and glutamate excitoxicity have been implicated

=> progressive motor neurone degeneration and death
=> gliosis replacing normal neurones

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15
Q

what are the associations of motor neurone disease ?

A

frontotemporal lobar dementia

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16
Q

what are the risk factors of motor neurones disease?

A

genetic disposition/ FH

age more than 40 years

17
Q

summarise the epidemiology of motor neurone disease?

A

RARE

Incidence: 6/100,000

Mean age of onset: 55 yrs

5-10% have a family history with autosomal dominant inheritance

18
Q

what are the presenting symptoms of motor neurone disease?

A 
weakness of limbs- proximal myopathy 
speech disturbance ( slurring or reduction in volume) 
swallowing disturbance ( choking on food) 
behavioural changes ( disinhibition, emotional liability)
19
Q

what are the signs of motor neurone disease on physical examination?

A

combination of UMN and LMN signs

LMN features

  • muscle wasting
  • fasciculations i.e. of tongue, abdomen, back and thigh
  • flaccid weakness
  • hyporefelxia

UMN Features

  • spastic features
  • extensor plantar response
  • hyperreflexia

SENSORY EXAMINATION- should be normal

frontotemporal dementia occurs in 25%

20
Q

give examples of the causes of UMN?

A

stroke, space occupying lesion, dymyelination ( MS), spinal chord, pathology

21
Q

give examples of the causes of LMN?

A

MND, peripheral neuropathy, myasthesia Gravis, muscular dystrophy

22
Q

what are the signs of upper motor neurone disease?

A

contralateral signs

no fasciculations

no muscle wasting

spasticity, may clonus

weakness: extensors in arms, flexors in legs

hyperreflexia

upgoing plantars

pronator drift

23
Q

what are the sings of lower motor neurone disease?

A

unilateral signs

fasciculations

muscle wasting

hypotonia

weakness

hyporeflexia

normal plantar response

24
Q

what are the appropriate investigations for motor neurone disease?

A

CLINICAL DIAGNOSIS

bloods

  • Mild elevation in CK
  • ESR
  • anti-GM1 ganglioside antibodies

Electromyography

Nerve conduction studies- often common

MRI- exclude cord compression and brainstem lessons

spirometer- assess respiratory muscle weakness

LP can help exclude inflammatory causes

Neurology (29 decks)

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