Neurodegenerative Disorders Flashcards

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1
Q

What are the 5 areas mostly affected in motor neurone disease?

A
Motor cortex - precentral gyrus
Corticobulbar pathway
CN nuclei, particularly 7 and 9
Corticospinal tract
Anterior horn cells
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2
Q

What is found in the anterior grey matter horn?

A

Motor neurons - mostly cell bodies of alpha motor neurons

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3
Q

Give 5 diseases that preferentially effect the anterior horn cells?

A
MND
CMT disease 
Spinal muscular atrophy
Poliomyelitis
West Nile disease
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4
Q

What is the type of MND characterised by early corticospinal tract damage and thus UMNL sign predilection?

A

Primary lateral sclerosis

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5
Q

What is primary lateral sclerosis?

A

Early corticospinal tract damage in MND so prominent UMNL signs and pyramidal weakness

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6
Q

What is pyramidal weakness and what does it suggest?

A

Extensor weakness in ULs, flexor weakness in LLs

Indicates corticospinal or motor cortex damage

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7
Q

What is the ‘unit’ characteristically most affected in motor neuron disease?

A

Anterior horn

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8
Q

What type of MND is characterised by early anterior horn damage and this LMNL-sign prominence?

A

Progressive muscular atrophy

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9
Q

What characterises progressive muscular atrophy?

A

Anterior horn cell damage early and LMNL prominence

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10
Q

What type of MND has the best prognosis?

A

Progressive muscular atrophy

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11
Q

What is bulbar-onset motor neuron disease otherwise known as?

A

Progressive bulbar palsy

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12
Q

Where is the pathology in progressive bulbar palsy?

A

Corticobulbar pathway and lower CN motor nuclei involvement

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13
Q

What are the cranial nerves mostly implicated in progressive bulbar palsy?

A

CN 5, 7, 10, 11 and 12

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14
Q

What is a metabolic differential for progressive muscular atrophy?

A

Diabetic amyotrophy

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15
Q

Major common differentials for motor neuron disease that warrants spinal MRI?

A

Cervical spondylosis

Spinal rumours

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16
Q

Poisoning with what can yield an ALS-like picture?

A

Lead

17
Q

Lysosomal storage disorder that can present like MND?

A

Tay-Sachs disease

18
Q

3 neurological signs that do not occur in MND?

A

Sensory signs
Bladder disturbance
Ocular muscle involvement

19
Q

What happens to conduction velocity in MND?

A

Normal velocities

20
Q

What 2 signs does EMG show in MND?

A

Denervation

Fibrillation

21
Q

What metabolic parameters should be checked before diagnosing MND and why?

A

TFTs and PTH/Ca

Because hyperthyroidism and hyperPTH can mimic with UMNL and LMNL signs

22
Q

What is the only current disease modifying drug for MND and what properties does it have? How long does it add on to life expectancy?

A

Riluzole - anti-glutamate

Adds on maybe 2m