L14 Cerebellar Ataxia Flashcards
(56 cards)
1
Q
where does the word cerebellum come from
A
the latin for “little brain”
2
Q
what percentage of brain neurons does the cerebellum contain
A
80%
3
Q
what is the motor role of the cerebellum
A
- linked to voluntary motor coordination
- does not initiate muscular activities
- coordinate motor activities initiated elsewhere in the CNS
- partly responsible for motor learning
4
Q
how does the cerebellum coordinate movement
A
- Compares expected movement (from motor cortex) with actual movement (proprioceptive feedback).
- Corrects movement errors in real time.
5
Q
what does voluntary movement in the absence of the cerebellum look like
A
clumsy and disorganised
6
Q
does the cerebellum work ipsilaterally or contralaterally
A
ipsilaterally
7
Q
does the cerebrum work ipsilaterally or contralaterally
A
contralaterally
8
Q
what nonmotor areas does is the cerebellum involved in
A
- Memory
- Cognition
- Language
- Social behavior (Possible links to Autism Spectrum Disorder (ASD) and schizophrenia).
9
Q
is cognitive impairment in cerebellar damage mild, moderate or severe
A
- chronic cerebellar lesions reveal mild cognitive impairment
- impairment may be more prominent in acute cerebellar damage
10
Q
what is ataxia
A
lack of coordination caused by cerebellar damage
11
Q
clinical signs of ataxia
A
- Finger-nose-finger test (past-pointing): Inability to judge distances.
- Dysdiadochokinesis: Jerky, inaccurate rapid alternating movements.
- Cerebellar rebound.
- Heel-knee-shin test.
12
Q
causes of ataxia
A
- inherited
- Identified cause
- Idiopathic
13
Q
examples of inherited ataxias
A
- Friedreich’s ataxia
- spinocerebllar ataxias
14
Q
examples of identified causes on aquired ataxia
A
- stroke
- tumour
15
Q
features of cerebellar disorders
A
- Ataxia.
- Decomposition of movement.
- Dysmetria (inability to judge distances).
- Dysdiadochokinesis (difficulty with rapid alternating movements).
- Rebound phenomenon.
- Hypotonia (reduced muscle tone).
- Tremor.
- Nystagmus (involuntary eye movements).
- Disturbance of posture and gait.
- Ataxic Dysarthria.
- Scanning Speech.
16
Q
aetiology of cerebellar disorder
A
- Inherited
- Congenital
- Trauma.
- Infections
- Inflammatory conditions
- Vascular causes
- Neoplastic
- Metabolic
- Toxic/Drug-induced
- Degenerative diseases
17
Q
example of a congenital cerebellar disorder
A
Arnold-Chiari Malformation
18
Q
example of infections causing cerebellar damage
A
- abscess
- post-viral
19
Q
example of an inflammatory condition causing cerebellar damage
A
MS
20
Q
example of a vascular cause of cerebellar damage
A
- infarction
- hemorrhage
21
Q
example of neoplastic causes of cerebellar damage
A
- paraneoplastic
- metastases
22
Q
metabolic causes of cerebellar damag e
A
hypothyroidism
23
Q
examples of toxic/drug induced causes of cerebellar damage
A
- alcohol
- phenytoin
24
Q
example of a degenerative disease causing cerebellar damage
A
Multiple systems atrophy
25
what are spino-cerebellar ataxias (SCAs)
- Group of neurodegenerative disorders.
- Typically autosomal dominant inheritance.
26
how many classified types of SCAs are there
more than 30
27
what are the most two most common forms of SCA
- SCA3
- SCA6
28
what is another name for SCA3
Machado-Joseph Disease
29
onset of SCA3
middle-age onset
30
what systems does SCA3 affect
- cerebellar
- pyramidal
- extrapyramidal
- autonomic
31
onset of SCA6
late onset
32
which systems does SCA6 affect
primarily cerebellar involvement
33
two subtypes of cerebellar disorders
- midline cerebellar syndromes
- hemispheric cerebellar syndromes
34
features of midline cerebellar syndromes
- Imbalance/unsteady.
- Unable to stand in Romberg with eyes open or close.
- Trunkal ataxia → syndrome where a person cannot sit on their bed without steadying themselves.
- Titubation → bobbing motion of the head or trunk.
- Nystagmus, ocular dysmetria and poor pursuit.
35
features of hemispheric cerebellar syndromes
- Incoordination of the limbs.
- Decomposition of movement, dysmetria and rebound.
- Dysdiadochokinesis.
- Intention tremours.
- Finger to nose and heel to knee tests are classic tests of hemispheric cerebellar dysfunction.
- Speech may be dysarthric, scanning or have irregular emphasis on syllables.
36
how is cerebellar damage diagnosed
- family history
- imaging (MRI)
- genetic testing
37
what rating scale is used for ataxia
Scale for the Assessment and Rating of Ataxia (SARA)
38
what are the items on the SARA
- Gate
- Stance
- Sitting
- Speech disturbance
- Finger chase
- Nose-finger test
- Fast alternating hand movements
- Heel-shin slide
39
subsystems impacted in ataxic dysarthria
- respiration
- phonation
- articulation
- prosody
40
respiration features of ataxic dysarthria
reduced breath support for speech
41
phonation features of ataxic dysarthria
harsh voice quality.
42
articulation features of ataxic dysarthria
* imprecise consonants
* distorted vowels
* poorly coordinated and irregular articulatory movements
* improper formation and separation of individual syllables.
43
prosody features of ataxic dysarthria
* abnormal stress
* slow rate
* variable pitch
* impaired loudness control
44
respiration management in ataxic dysarthria
- Speak immediately on exhalation.
- Stop phonation early (before speaking on residual air).
- Determine optimum breath group (how many syllables to speak before taking another breath).
45
articulation management in ataxic dysarthria
- Intelligibility drills.
- Over-articulation.
- Minimal contrast drills.
46
prosody (rate) management in ataxic dysarthria
- slow rate.
- Finger or hand tapping to set the pace for syllable production.
- Cued readings (finger pointing or marking pauses).
47
prosody (intonation and stress) management in ataxic dysarthria
- Contrastive stress drills.
- Intonational drills using printed material with pitch markings.
- Chunking information into syntactic units (using marked inhalation cues in written passages).
48
swallowing difficulties in cerebellar disease
- Poor oral control of bolus.
- Penetration/aspiration risk.
- Weak cough response.
49
issues which contribute to swallow difficulties in cerebellar disease
- Poor posture for feeding.
- Impaired feeding ability (due to tremor, dysmetria).
- Poor volume control.
50
what areas of language might the cerebellum have involvement in
- Verbal fluency.
- Word retrieval.
- Syntax.
- Reading & writing.
- Metalinguistic abilities.
51
what is Freidrich's Ataxia
- rare autosomal recessive disorder
- causes progressive ataxia
52
onset in Friedrich's ataxia
usually before age 25 (often adolescence)
53
characteristics of Friedrich's ataxia
- Ataxic gait.
- Tendon areflexia.
- Upgoing plantar reflexes.
- Skeletal deformities:
- Kyphoscoliosis.
- Pes cavus (high-arched foot deformity).
- Electrocardiographic abnormalities (indicative of cardiomyopathy, common cause of early death).
- Optic atrophy.
- Nystagmus.
- Dysarthria.
- Dysphagia.
54
prevalence of freidrich's ataxia
2 per 100,000
55
progression of freidrich's ataxia
- Gait difficulties bring the patient to medical attention.
- Ambulation lost within 9-15 years of onset.
56
treatment for Freidrich's ataxia
- No cure.
- Symptomatic management.
- Multidisciplinary care (focus on mobility, dysarthria, dysphagia).
- Counseling.
