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Neurology > Ataxia > Flashcards

Ataxia Flashcards

1
Q

What is ataxia?

A

Loss of order in executing movement

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2
Q

What does the term ataxia refer to? (2)

A
  1. Umbrella term for a group of neurological disorders affecting balance, co-ordination and speech
  2. A sign, not a disease and results from cerebellar dysfunction
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3
Q

Why does ataxia occur? (2)

A
  1. Can result from structural damage to the cerebellum
  2. Can be genetic or acquired
  3. Can also occur due to damage to spinal cord or other nerves
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4
Q

What is the prevalence of ataxia? (3)

A
  1. 10,000 people in the UK
  2. 500 children with progressive ataxia
  3. Acquired diseases account for the majority (80%) and genetic ataxias (20%)
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5
Q

When is ataxia most commonly diagnosed?

A
  1. At any age but certain types are more common at certain ages
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6
Q

What is the aetiology of ataxia?

A
  1. Can be genetic or acquired

2. Damage to the cerebellum but also damage to spinal cord or other nerves

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7
Q

Give 4 ways that ataxia can be inherited

A
  1. Autosomal recessive
  2. Autosomal dominant
  3. Mitochondrial
  4. X-linked
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8
Q

Give 5 ways that ataxia can be acquired

A
  1. Toxic causes
  2. Immune mediated
  3. Neurodegenerative
  4. Idiopathic sporadic
  5. Vascular/structural
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9
Q

Give 3 toxic causes of ataxia

A

Alcohol
Phenytoin
Lithium

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10
Q

Give 5 types of immune-mediated ataxia

A
  1. Paraneoplastic cerebellar degeneration
  2. Post-infectious cerebellitis
  3. Gluten ataxia (GA)
  4. Ataxia with anti-glutamic acid decarboxylase (GAD) antibodies
  5. Primary Autoimmune Cerebellar Ataxia (PACA)
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11
Q

Give 11 causes of acquired ataxia

A
  1. Severe head injury
  2. Bacterial brain infection eg. meningitis, encephalitis
  3. Viral infection eg. chickenpox, measles
  4. Stroke, haemorrhage or TIA
  5. Cerebral palsy
  6. Multiple sclerosis
  7. Sustained alcohol misuse
  8. Underactive thyroid gland
  9. Vitamin B12/E deficiency
  10. Toxic chemicals eg. mercury, solvents
  11. Gluten intolerance
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12
Q

Give 2 examples of autosomal recessive inheritance of ataxia

A
  1. Freidreich’s ataxia

2. Ataxia-telangiectasia

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13
Q

Give 2 examples of autosomal dominant ataxia

A
  1. Episodic ataxia

2. Some cases of spinocerebellar ataxia

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14
Q

Describe idiopathic sporadic ataxia (2)

A
  1. Late onset of undetermined cause (50%)

2. Proportion of patients likely to be immune-mediated

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15
Q

Describe gluten ataxia (2)

A
  1. Sensitivity to gluten

2. one of the most common forms of sporadic idiopathic ataxia

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16
Q

Describe episodic ataxia (3)

A
  1. Characterised by attacks of ataxic symptoms and no/mild symptoms for rest of the time
  2. Relatively rare group of conditions
  3. Inherited in autosomal dominant manner (50% chance of passing to children)
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17
Q

Describe spinocerebellar ataxia (3)

A
  1. Group of hereditary ataxia
  2. Don’t often begin until adulthood
  3. Caused by mutations in different genes (100 identified) Eg. SCA 6
18
Q

Describe Freidreich’s ataxia (2)

A
  1. Most common form of ataxia affecting 1/50,000

2. Inherited mutation (autosomal recessive) in the frataxin gene leading to reduced frataxin

19
Q

What is the role of frataxin?

A

A protein essential for life and cell survival

20
Q

Give 2 types of neurodegenerative ataxia

A
  1. MSA-C (multi-system atrophy - cerebellar variant)

2. PSP (progressive supernuclear palsy)

21
Q

Describe MSA-C (5)

A
  1. Autonomic symptoms
  2. Bulbar symptoms
  3. Ataxia
  4. Respiratory hypoventilation
  5. Characteristic MRI finding
22
Q

Describe PSP (3)

A
  1. Falls/loss of truncal balance
  2. Restricted eye movements
  3. Bulbar symptoms
23
Q

Describe ataxia-telangiectasia (3)

A
  1. Rare type of hereditary ataxia
  2. Symptoms usually begin in early childhood
  3. Condition rapidly deteriorates, usually live until 19-25 years (some may live into 50s)
24
Q

Give 9 symptoms of cerebellar dysfunction

A
  1. Slurring of speech, dysarthria
  2. Swallowing difficulties
  3. Oscillopsia
  4. Clumsiness (arms and legs)
  5. Tremor
  6. Loss of precision in fine motor skills
  7. Unsteadiness when walking/worse in dark
  8. Stumbles and falls
  9. Cognitive impairments
  10. Nystagmus
25
Q

What is oscillopsia?

A

Jumping of the environment due to rapid jerking movements

26
Q

Give a summary of the diagnosis process of ataxia (5)

A
  1. Patient history
  2. Brain scans (MRI)
  3. Family history
  4. Genetic tests
  5. Other: blood tests (vitamin levels), EMG, ECG, VFS
27
Q

What information is collected in the patient history? (7)

A
  1. Age of onset
  2. Course of progression
  3. Additional symptoms
  4. Pattern of involvement
  5. Family history
  6. Drug/alcohol
  7. Social history
28
Q

What will an MRI show? (2)

A
  1. Highly cerebellar atrophy and dysfunction

2. Excludes cerebrovascular damage, tumours, hydrocephalus, MS, white matter disease, cerebellar dysgenesis

29
Q

How is the clinical severity rated in ataxia? (2)

A
  1. Mild, moderate, severe

2. Scale for the Assessment and Rating of Ataxia (SARA)

30
Q

Define mild ataxia

A

Mobilising independently or with one walking aid

31
Q

Define moderate ataxia

A

Mobilising with 2 walking aids or walking frame

32
Q

Define severe ataxia

A

Predominantly wheelchair dependent

33
Q

Summarise the treatment of ataxia (3)

A
  1. MDT
  2. Medication
  3. Management of additional symptoms
34
Q

What additional symptoms may require management in ataxia? (5)

A
  1. Bladder problems
  2. Eye problems
  3. Nerve pain
  4. Depression
  5. Muscle cramps, spasms and stiffness
35
Q

What can be used to help muscle cramps, spasms and stiffness? (2)

A
  1. Medication eg. baclofen, tizanidine

2. Botox injections

36
Q

What can be used to help eye problems?

A
  1. Gabapentin to control eye muscles (also controls seizures and nerve pain)
37
Q

What is the cerebellum? (3)

A
  1. “Little brain” which controls the timing and pattern of motor activation during movement
  2. Contains complete motor ans sensory representation of the whole body
  3. Receives information from the sensory systems, spinal cord and other parts of the brain to regulate movement
38
Q

Where is the cerebellum located?

A

Behind the top part of the brainstem

39
Q

What actions does the cerebellum control? (5)

A
  1. Walking and sitting
  2. Balance
  3. Limb co-ordination
  4. Eye movements
  5. Speech
40
Q

What is dysdiadochokinesia?

A

An impaired ability to perform rapid, alternating movements

Neurology (17 decks)

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