Cerebellar disorders

Question 1

Overview of cerebellum

Answer 1

• Latin for little brain
• Historically associated with motor control
• Contains 80% of the neurons in the brain
• Enlarged lateral cerebellar growth
  (cerebrocerebellum) with human evolution

Question 2

Motor role of cerebellum

Answer 2

• Coordination
• No initiation
• Comparison and correction
• Motor learning
• Ipsilateral function

Question 3

Non-motor roles of cerebellum

Answer 3

• Memory
• Cognition
• Language
• Attention
• Social behaviour.. (?link to ASD & schizophrenia)

Question 4

Cognitive, Affective, and Behavioral Changes in Cerebellar disorder

Answer 4

• Attention
• Memory
• Learning
• Executive control
• Emotion (fear, pleasure)
• Visuo-spatial function
• Language
• Verbal working memory
• Cognitive impairment in cerebellar patients is generally mild, but more prominent in those with acute cerebellar damage

Question 5

What is ataxia and how to test for it?

Answer 5

• Definition: Lack of coordination, a hallmark of cerebellar damage.

Tests:

• Finger-nose-finger test (past-pointing) due to inability to judge distances.
• Rapid alternating movements: Jerky and inaccurate (dysdiadochokinesis).
• Heel-knee-shin test (cerebellar rebound)

Question 6

Other Features of Cerebellar Disorder

Answer 6

• Decomposition of movement
• Dysmetria (inability to judge distances)
• Dysdiadochokinesis
• Rebound (abnormal muscle response)
• Hypotonia
• Tremor
• Nystagmus
• Posture and gait disturbances
• Ataxic dysarthria (speech difficulty)

Question 7

Aetiology of Cerebellar Disorders

Answer 7

• Inherited: Friedrich’s Ataxia, - Spinocerebellar Ataxias (SCA).
• Congenital: Arnold Chiari Malformation.
• Trauma.
• Infections: Abscess, post-viral.
• Inflammation: (MS).
• Vascular: Infarction, hemorrhage.
• Neoplastic: Paraneoplastic, metastases.
• Metabolic: Myxoedema (hypothyroidism).
• Toxic/Drug-induced: Alcohol, phenytoin.
• Degenerative: Multiple Systems Atrophy (MSA)

Question 8

Spinocerebellar ataxias

Answer 8

• group of neurodegenerative disorders frequently
  associated with autosomal dominant inheritance (over 30 groups)
• SCA Type 3: Develops in middle age, affects multiple systems (cerebellar, pyramidal, extrapyramidal, autonomic).
• SCA Type 6: Late-onset disease, affects mainly the cerebellum

Question 9

subtypes of cerebellar disorders

Answer 9

1. midline cerebellar disorders: primarily affect balance and truncal coordination,
2. hemispheric cerebellar disorders: primarily affect limb coordination and fine motor skills.

Question 10

Diagnosis of Cerebellar Disorders

Answer 10

• Family History
• Imaging (MRI)
• Genetic Testing

Question 11

Rating scale

Answer 11

SARA: Scale for Ax and Rating of Ataxia

Question 12

Management of cerebellar disorders

Answer 12

• Education: Important for patient and family.
• Pharmacological Treatment: Symptomatic management.
• Rehabilitation: Key in improving mobility and functional outcomes.
• Multidisciplinary Team (MDT) Input: Includes physicians, physiotherapists, occupational therapists, and speech therapists

Question 13

Ataxic dysarthria features and management

Answer 13

• Features: Slurred, slow, and irregular speech.
• Management: Speech therapy for improving articulation, Energy conservation strategies.

Question 14

Dysphagia in Cerebellar Disease

Answer 14

• Poor oral control of bolus.
• Penetration/aspiration risk.
• Weak cough response.
• Poor posture for feeding.
• Impaired feeding ability (tremor, dysmetria).
• Poor volume control.

Question 15

Friedrich’s Ataxia (FA

Answer 15

• Rare autosomal recessive disorder
• Typically presents in childhood or adolescence, causing progressive ataxia, gait disturbance, and skeletal deformities like kyphoscoliosis.
• It often leads to cardiomyopathy, which is the primary cause of early death.
• FA is caused by mutations in the frataxin gene and is managed symptomatically, focusing on mobility and addressing speech and swallowing difficulties.