Chorea and Ballismus in Neurology

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Questions and Answers

What is the typical age of onset for early onset focal dystonia?

20 years

30 years

35 years

26 years (correct)

What is the primary treatment for dystonia?

Medications

Botulinum toxin (correct)

Physical therapy

Surgery

What is the characteristic of UMN weakness?

Wasting of muscles

Reduced or absent reflexes

Increased tone (correct)

Reduced tone

What is the term for inflammation of the grey matter of the spinal cord?

Poliomyelitis (C) Signup and view all the answers

What is the term for the pattern of weakness that defines the type of muscular dystrophy?

Pattern of weakness (B) Signup and view all the answers

What is the primary cause of polio?

Viral infection (A) Signup and view all the answers

What is the term for acute inflammatory demyelinating polyneuropathy?

AIDP (D) Signup and view all the answers

What is the typical treatment for polio?

Supportive care (A) Signup and view all the answers

What is the characteristic of chorea in terms of movement?

Irregular, rapid, non-stereotyped, and random (D) Signup and view all the answers

What is the term for proximal chorea resulting in large amplitude movements?

Ballismus (A) Signup and view all the answers

Which of the following is a therapy for chorea?

Butyrophenones such as haloperidol (C) Signup and view all the answers

Which of the following is a type of chorea that usually occurs in childhood?

Sydenham's chorea (C) Signup and view all the answers

What is the result of dysfunction within the complex neuronal network that interconnects motor cortical areas and subcortical nuclei?

Chorea (D) Signup and view all the answers

What is the term for the involuntary sustained contraction of muscles or groups of muscles resulting in an abnormal posture?

Dystonia (D) Signup and view all the answers

What is the usual age of onset for Huntington's disease?

Adolescence (B) Signup and view all the answers

What is the associated condition with Sydenham's chorea?

Rheumatic fever (C) Signup and view all the answers

Which of the following is a characteristic of Parkinson's disease?

Lewy body accumulation in areas of the body, including the CNS and outside the CNS (C) Signup and view all the answers

What is a common symptom of cerebellar ataxia?

Upper-limb dysmetria (C) Signup and view all the answers

What is a possible treatment for ataxia in patients with multiple sclerosis?

Deep brain stimulation (C) Signup and view all the answers

What is a characteristic of Parkinson's disease diagnosis?

Bradykinesia and at least one of the following: muscular rigidity, 4-6 Hz resting tremor, or postural instability (C) Signup and view all the answers

What is a type of movement disorder?

All of the above (D) Signup and view all the answers

What is the meaning of the term 'ataxia'?

Incoordination of movement (B) Signup and view all the answers

What is a cause of epilepsy?

All of the above (D) Signup and view all the answers

What is a characteristic of cerebellar ataxia?

Variability in amplitude (C) Signup and view all the answers

Study Notes

Chorea/Ballismus

Chorea: irregular, rapid, non-stereotyped, random, involuntary movement
May manifest as fidgetiness or restlessness in children
Ballismus: proximal chorea resulting in large amplitude movements
Usually seen with lesion of the subthalamic nucleus, but can occur from lesions along all its afferent or efferent paths
Classified according to aetiology
Therapy relates to aetiology and symptomatic therapy usually with butoryphenones (e.g. haloperidol)

Neuroanatomy and Neurophysiology of Chorea

Chorea results from dysfunction within a complex neuronal network interconnecting motor cortical areas and the basal ganglia
Basal ganglia includes the caudate nucleus, putamen, external and internal segments of the globus pallidus (GPe and GPi), subthalamic nucleus, and substantia nigra

Chorea: Disease

Huntington's disease: AD, 10/100000, Chr 4, codes for huntingtin, neurotoxic gain of function, may have psychiatric or movement disorder, associated with caudate atrophy, depression is common
Sydenham's chorea: usually in childhood, major criterion for rheumatic fever, increased Group A beta hemolytic streptococcus titer, usually remits within 15 weeks but can recur, treat symptomatically with haloperidol and provide prophylaxis for rheumatic fever

Dystonia

Dystonia: involuntary sustained contraction of muscles or groups of muscles resulting in an abnormal posture
Can be caused by trauma, altered blood supply, altered blood content, metabolic abnormal brain chemistry, acidosis, hypoglycemia, hypoxia, electrolyte abnormalities, toxins

Threats to the System

Ischemia
Tumours in the brain or spinal cord
Metabolic abnormal brain chemistry
Acidosis, hypoglycemia, hypoxia
Electrolyte abnormalities
Toxins
Abnormal circuits resulting in epilepsy, movement disorders, psychosis
Neurodegenerative disease

Parkinson's Disease

Neurodegenerative disease characterized by Lewy body accumulation in areas of the body resulting in dysfunction
Recent data suggest that abnormality also occurs outside the CNS (e.g. the myenteric plexus)
Classical degeneration in Substantia nigra pars compacta

Diagnosis of Parkinsonism

Bradykinesia and at least one of the following:
- Muscular rigidity
- 4-6 Hz resting tremor
- Postural instability not caused by primary visual, vestibular, cerebellar or proprioceptive dysfunction

Cerebellar Ataxia

Characterized by an incoordination of movement and unsteadiness due to cerebellar dysfunction
Clinical examination reveals:
- Gait disorder with imbalance, staggering
- Difficulties with tandem walking
- Upper-limb and lower-limb dysmetria, dysdiadochokinesia
- Hypotonia, cerebellar dysarthria, and saccadic ocular pursuit

Ataxia: Management

If etiology found, treat
Otherwise, symptomatic therapy:
- Difficult to treat
- Physical measures such as walkers, weights, etc.
- In MS patients, DBS has some benefit

UMN and LMN

UMN:
- Weakness
- No wasting
- Increased tone (spastic)
- Brisk reflexes
- Extensor plantar responses
LMN:
- Weakness
- Reduced tone
- Reduced or absent reflexes
- Wasting of muscles

UMN & LMN Causes

UMN:
- Brain lesions (stroke, tumour, haemorrhage, demyelination, ADEM)
- Spine (fracture with spine impression, tumour, demyelination, viral myelitis, B12 deficiency)
LMN:
- AHC (polio, SMA, AVM)
- Root (disc disease, arachnoiditis, AIDP, CIDP)
- Plexopathies
- Peripheral neuropathy
- Myopathies
- Myoneural junction (myasthenia gravis)

Muscular Dystrophies

Many causes of muscular dystrophy
Pattern of weakness defines type
Examples:
- Duchenne's muscular dystrophy (dystrophinopathy)
- Limb girdle muscular dystrophy (many types)
- Congenital myopathy (many types)
- Myotonic dystrophies (dystrophia myotonica, schwartz Jampel)
Most are genetically determined
Therapy usually supportive: OT, physiotherapy

Infections

Polio
GBS (AIDP)
Abscess
Meningitis
Encephalitis

Polio

Poliomyelitis is inflammation of the grey matter usually of the spinal cord where the AHC resides
Caused by polio or other viruses (CMV, herpes, entero-viruses)
Characterized by LMN features and no sensory abnormality
Therapy supportive

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Description

This quiz covers the definitions and characteristics of chorea and ballismus, including their causes, symptoms, and treatment options. Test your knowledge of these neurological movement disorders.