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USMLE Neurology > Herniation syndromes, spinal cord lesions and UMN/LMN signs > Flashcards

Herniation syndromes, spinal cord lesions and UMN/LMN signs Flashcards

1
Q

How is Friedreich ataxia inherited?

A

Aut Recessive trinucleotide repeat disorder (GAA on chr 9)

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2
Q

What is the trinucleotide repeat in Friedrick ataxia?
What chromosome is it on?

A

GAA on chr 9
- ataxic GAAit

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3
Q

What does the gene code for in Friedreich ataxia?

A

Frataxin (iron binding protein)

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4
Q

What tracts / nerve structures are impaired in Friedreich ataxia?

A
  • Spinocerebellar
  • Corticospinal
  • Dorsal colomns
  • Dorsal root ganglia
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5
Q

What is the cause of death in Fiedrich ataxia?

A

Hypertrophic cardiomyopathy

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6
Q

What are the symptoms of Friedreich ataxia?

A
  • Staggering gait
  • Frequent falling
  • Nystagmus
  • Dysartthria
  • Pes cavus (high arches)
  • Hammer toes
  • DM
  • Hypertrophic cardiomyopathy
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7
Q

What feature is first identified in childhood in Friedrich ataxia?

A

Kyphoscolioisis

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8
Q

Spinal muscular atrophy is due to degeneration of what part of the spinal cord?

A

Anterior horns

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9
Q

What are the symptoms of spinal muscular atrophy?

A
  • LMN symptoms only, symmetrical
  • floppy baby with marked hypotonia (flaccid paralysis) and tongue fasiculations
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10
Q

What mutation causes spinal muscular atrophy
What protein does this affect?

A

SMN1 mutation -> defective snRNP assembly

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11
Q

How is spinal muscular atrophy (SMN1 mutation) inherited?

A

Aut Recessive

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12
Q

What is spinal muscular atrophy type 1 called?

A

Werdnig-Hoffman disease

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13
Q

A defect in superoxide dismutase 1 can cause what spinal lesion?

A

Amyotrophic lateral sclerosis

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14
Q

WHat is ALS treated with?

A

Rilouzole

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15
Q

What do those with ALS usually die of?

A

Resp failure

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16
Q

What is ALS also known as?

A

Low Gehrig disease

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17
Q

What are the symptoms of ALS?

A

LMN
- Flaccid limb weakness
- Fasiculations
- Atrophy
- Bulbar palsy (dysarthria, dysphagia, tongue atrophy)

UMN
- Spastic limb weakness
- Hyperreflexia
- Clonus
- Pseudobulbar palsy (dysarthria, dysphagia, emotional liability)

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18
Q

What kind of spinal lesion can be caused by AAA repair?

A

Complete occlusion of anterior spinal artery

19
Q

What artery supplies the Anterior spinal artery (ASA) below T8?

A

Artery of Adamkiewicz

20
Q

What are the symptoms of Complete occlusion of anterior spinal artery?

A

Below lesion
- UMN defecit -> Corticospinal tract
- Loss of pain + temp -> Spinothalamic tract

Level of lesion:
- LMN defecit (anterior horn)

21
Q

What is tabes dorsalis caused by (what disease/bacteria)?

A

Tertiary syphilis

22
Q

What part of the spinal cord does tabes dorsalis primarily affect?
What symptoms/signs does this result in?

A

Dorsal columns and roots demyelinate and degenerate
- Progressive sensory ataxia -> Impaired proprioception and poor coordination
- +ve Romberg sign
- Absent DTRs

23
Q

WHat other symptoms/signs is Tabes Dorsalis associated with?

A
  • Charcot joints
  • Shooting pain
  • Argyll Robertson pupils
24
Q

Syringomyelia causes what symptoms?

A

Loss of pain and temp (spinothalamic) in cape-like distribution

25
Q

What expands and damages the ant white commissure in syringomyelia?

A

Syrinx

26
Q

What embryological disorder is associated with Syringomyelia?

A

Chiari I malformation

27
Q

What tracts are damaged by subacute combined degeneration of the cord?

A
  • Spinocerebellar tract - ataxic gait
  • Lateral corticospinal
  • Dorsal columns - parasthesia, impaired vibration/position sense. +ve Romberg sign
  • UMN symptoms
28
Q

Caudia Equina syndrome symptoms

A
  • Radicular pain
  • Absent knee and ankle reflexes
  • Loss of anal and bladder sphincter control
  • Saddle anesthesia
29
Q

Compression of spinal roots below what level may cause cauda equina syndrome?

A

L2

30
Q

Cauda equina syndrome is due to compression via what?

A
  • Intervertebral disc herniation
  • Tumour
31
Q

What part of the spinal cord is affected in poliomyelitis?

A

Anterior horn -> LMN signs (fasiculations, atrophy etc.)

32
Q

What are the symptoms of poliomyelitis?

A
  • Asymmetric weakness
    LMN signs:
  • Hypotonia
  • Flaccid paralysis
  • Fasiculations
  • Hyporeflexia
  • Muscle atrophy

Signs of infection (malaise, fever, nausea etc.)

33
Q

What will CSF show in poliomyelitis?

A
  • Increased WBCs (lymphocytic pleocytosis)
  • Slight increase in proteins
  • No change in glucose
34
Q

Where does polio virus replicate?
Where can it be recovered from?

A

Lymphoid tissue of oropharynx and SI before spreading in bloodstream to CNS
- Virus recovered from stool or throat

35
Q

What are the signs/symptoms of Brown-Sequard syndrome?

A
  • Ipsilateral loss of all sensation at level of lesion
  • Ipsilateral LMN signs (eg flaccid paralysis) at level of lesion
  • Ipsilateral UMN signs below level of lesion (due to corticospinal tract damage)
  • Ipsilateral loss of proprioception, vibration, and light touch below level of lesion (Dorsal column)
  • Contralateral loss of pain + temperature and crude touch below lesion (Spinothalamic)
36
Q

What additional syndrome may develop if Brown-Sequard syndrome occurs above T1?

A

Ipsilateral Horner’s syndrome due to damage of oculosympathetic pathway

37
Q

What cranial nerve lesions cause ddeviation towards the side of the lesion?

A
  • CN V -> jaw deviates toward lesion
  • CN XII -> Tongue deviates toward lesion
38
Q

What cranial nerve lesion causes deviation away from the side of the lesion?

A
  • CN X -> Uvula deviates away from lesion
39
Q

Describe the weakened muscles in a CNXI lesion and how these affect movements?

A
  • SCM -> Weakness turning head to contralateral side of lesion (opposite side SCM contracts to help turn head other way)
  • Trapezius -> Shoulder droop on side of lesion
40
Q

What is the treatment of Bell palsy (from HSV commonly)?

A
  • Corticosteroids +/- acyclovir
41
Q

What are the different causes of peripheral facial palsy?

A
  • HSV
  • Lyme’s disease
  • Herpes Zoster (Ramsey Hunt)
  • Sarcoidosis
  • Tumours of parotid gland
  • DM
42
Q

Flaccid paralysis is seen in UMN or LMN lesions?

A

LMN

43
Q

Clasp knife spasticity is seen in UMN or LMN lesions?

A

UMN lesiosn

USMLE Neurology (19 decks)

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