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Neurology STG > Cerebellar Disorders and Movement Disorders > Flashcards

Cerebellar Disorders and Movement Disorders Flashcards

1
Q

What is cerebellar responsible for?

A

Cerebellum modulates Coordination and Learned Movement patterns rather than speed
Cerebellum receives afferents from Proprioceptive receptors (Inferior Cerebellar
Peduncles), Vestibular Nuclei, Basal Ganglia, Corticospinal System and Olivary Nuclei; Its
Effects leave (Mostly via Superior Cerebellar
Peduncles) to Red Nuclei, Vestibular Nuclei,
Basal Ganglia and Corticospinal System

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2
Q

What does the lateral cerebellar lobe do

A

Lateral Cerebellar Lobe (Cerebrocerebellum)

coordinates movement of Ipsilateral Limbs;

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3
Q

What does the vermis do?

A

Vermis (Spinocerebellum) concerned with

Maintenance of Axial Posture and Balance;

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4
Q

What does the flocculonodular lobe do?

A 
Flocculonodular Lobe (Vestibulocerebellum)
regulates Balance and Eye Movements
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5
Q

What causes cerebellar lesions?

A

Cerebellar Lesions can be caused by Obstructive Hydrocephalus; Severe Pressure Headaches, Vomiting and Papilloedema; Alternatively, Coning of Cerebellar Tonsils leads to Respiratory Arrest due to compression of the Brainstem

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6
Q

Lateral Cerebellar Hemisphere Lesions

A

• Disruption of Ipsilateral Normal Sequence of Movements (Dyssynergia); Rebound upward
overshoot; Gait becomes Broad and Ataxic faltering towards side of lesion
• Movement is imprecise in Direction, Force and Distance (Dysmetria); Dysdiadochokinesis
occurs; Intention Tremor and Past-Pointing; Speed of fine movements is preserved
• Nystagmus – Coarse Horizontal Nystagmus; Fast component moves towards side of lesion
• Dysarthria – Halting, Jerking, “Scanning” speech
• Titubation (Rhythmic head movements); Hypotonia and Hyporeflexia

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7
Q

Midline Cerebellar Lesions

A
  • Difficulty standing and sitting unsupported (Truncal Ataxia) with a Rolling, Broad, Ataxic Gait
  • Lesions of the Flocculonodular Node can cause Vertigo and Vomiting with Gait Ataxia
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8
Q

Physiological tremors

A

Physiologically normal 8-12Hz; Increased with Anxiety, Caffeine, Hyperthyroidism and Drugs; Occurs in Mercury poisoning

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9
Q

Coarse Postural Tremor

A

seen in Benign Essential Tremor (5-8Hz) and in ETOH XS

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10
Q

Intention Tremor

A

Exacerbated by Action; Associated with Past-pointing and Dysdiadochokinesis; Occurs in Cerebellar Lobe disease and Lesions of Cerebellar Peduncles;
Titubation and Nystagmus might be present

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11
Q

Rest Tremor

A

Seen typically in Parkinson’s Disease; Worse at rest; 4-7 Hz (Pill-rolling Tremor)

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12
Q

Coarse Tremors

A

can be seen in Red Nuclei lesions and rarely Frontal Lesions

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13
Q

Who gets idiopathic parkinsons disease?

A

Prevalence 150 in 100,000; Prevalence increases sharply with Age (1 in 200 of 80+yrs); 1.5× more common in Men; Small increased risk in Rural living and Well Water consumption; Pesticide induced (i.e. MPTP related) causes severe Parkinsonism; Non-smokers have a higher
risk of developing Parkinson’s Disease

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14
Q

Genetic Aetiology of IPD

A

• Significant genetic component; Gene Loci PARK 1-11; Rare but together account for large proportion of Early Onset and Familial Parkinson’s Disease

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15
Q

Pathophysiology of Parkinson’s Disease

A

Presence of Neuronal Inclusions (Lewy Bodies) and Loss of Dopaminergic Neurones in Substantia Nigra Pars Compacta
o Lewy Bodies contain tangles of α-Synuclein and Ubiquiti; Becomes more widespread as PD progresses, spreading to Lower Brainstem, Midbrain and Cortex
o Degeneration of other Basal Ganglia Nuclei; Degree of Nigrostriatal Dopaminergic Cell Loss correlates with severity of Akinesia
o Average of 70% lost by time of first presentation

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16
Q

Presentation of Parkinson’s Disease: Motor Symptoms

A

• Tremor – Starts in Fingers and Hands; Unilateral initially, spreading to leg on same side then opposite Arm; Present at rest and reduces when hand is in motion; Pill-rolling tremor pattern;
• Rigidity – Lead Pipe Rigidity; Not dependent on speed of movement (C/f Clasp Knife); Stiffness
+ Tremor = Cogwheel Rigidity
• Bradykinesia distinguished from Slowness of movements is progressive Fatiguing and
Decrement in Amplitude of repetitive movements; Difficulty initiating movement (usually Upper Limb); Rapid Dexterous movements impaired, Impassive Face (Serpentine Stare)
• Postural Instability – Stooped posture; Gait becomes shuffling with Small Stride, Slow Turns, Freezing and Reduced Arm Swing; May lead to falls although occurs late
• Micrographia (Writing becomes progressively smaller); Speech becomes Quiet, Indistinct and
Flat; Drooling and Dysphagia might feature; Leads to Aspiration Pneumonia

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17
Q

Presentation of Parkinson’s Disease: Non Motor

A

• Anosmia (90% of patients) – Dopamine transmission required for Olfaction
• Depression/Anxiety (50%), Aches/Pains, REM Sleep Behaviour Disorder, Autonomic Features
(Urinary Urgency, Hypotension), Constipation, Restless Leg Syndrome
• Cognitive – Common in late stage PD (80%), develop into Dementia; Depression is common due to involvement of Serotonergic and Adrenergic Systems; Anxiety is also comorbid

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18
Q

Progression of Parkinson’s

A
  • PD progressively deteriorates; Most patients respond well to treatment but might become treatment unresponsive when Cognitive Impairment, Dysphagia and Postural Instability/Falls start to develop by mid-70s
  • Death commonly resulting from Bronchopneumonia (Infective, Aspiration) and Immobility
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19
Q

Diagnosis of Parkinson’s Disease

A

• Clinical Diagnosis; rule out other Parkinsonian Syndromes
• MRI Head would be normal; Dopamine Transporter (DaT)
imaging to access Nigrostriatal Cell Loss; Cannot distinguish between PD and other Akinetic-Rigid
Syndromes but can rule out Drug Induced Parkinsonism

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20
Q

Treatment of Parkinson’s Disease

A

Dopamine Replacement is not always needed in Early-stage PD; Only started in disability;
Treatment of Non-Motor Symptoms E.g. Depression, Constipation, Pain and Sleep disorders
• Levodopa – Most effective form of treatment; Combined with DOPA Decarboxylase Inhibitor (e.g. Benserazide in Co-Beneldopa or Carbidopa in Co-Careldopa) to reduce Peripheral side
effects (Nausea, Hypotension)

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21
Q

Pharmacology of Parkinson’s treatment

A

• Dopamine Agonists – Used in combination with Levodopa or Initial Monotherapy in patients
65-70; Fewer motor complications but less effective and less tolerated then Levodopa
o Non-Ergot derived e.g. Pramipexole, Ropinirole used in preference to Ergot-derived, which are associated with fibrotic reactions (e.g. Valvular Fibrosis)

• Selegiline and Rasagiline (MAOB Inhibitors), Amantadine (? MOA), Anticholinergics (Rarely
used; Causes confusion in older patients), Apomorphine (Potent short acting Dopamine Agonist used as a rescue or maintenance by infusion pump for Advanced PD)

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22
Q

How does medical therapy change as PD develops

A

• Medical therapy becomes more difficult as disease progresses; Approximately 10% of patients per year develop complications from therapy (Wearing off, Dyskinesia/Choreiform
movements, On/Off Phenomenon);

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23
Q

What are strategies for managing motor complications?

A

o Dose Fractionation of Levodopa
o COMT Inhibitors (Entacapone) to prolong duration of Levodopa
o Slow release Levodopa; Mostly used for overnight
o Avoid protein rich meals; Taking doses at least 40 minutes prior to
meals
o Apomorphine Continuous
Infusion, Deep Brain Stimulation, Levodopa intestinal gel

24
Q

Neurosurgery in Parkinson’s

A

• Deep Brain Stimulation – Stereotactic insertion of Electrodes into the Basal
Ganglia/Thalamus; Usually for patients <70yrs; Subthalamic Nucleus, Globus
Pallidus (Improves Dyskinesia only),

25
Q

Other therapies in managing Parkinson’s

A

PT and OT – Walking Aids, SALT, Falls prevention, External Cueing Techniques

26
Q

Drug Induced Parkinsonism

A

• Dopamine Blockage/Depletion by drugs e.g. Neuroleptics (except Clozapine) induce
Parkinsonism, or worsen symptoms in affected patients; May precipitate symptoms

27
Q

Atypical Parkinsonism

A

• Other Neurodegenerative Diseases which affect the Basal Ganglia; Progressive, although might respond to Levodopa; Usually causes death within a decade
o Red Flags – Symmetrical initial response, Absence of Tremor, Levodopa
unresponsiveness, Early falls (within first year) and Wheelchair use

28
Q

Progressive Supranuclear Palsy

A

(Steele-Richardson-Olszewski) – Parkinsonism, Postural Instability with Early Falls, Vertical Supranuclear Gaze Palsy, Pseudobulbar Palsy and Dementia; Associated with Tau Deposition
o Affects Basal Ganglia, Brainstem, Cerebral Cortex (Especially Frontal), Dentate Nucleus of Cerebellum and Spinal Cord (might affect Continence)

29
Q

Multiple System Atrophy

A

Parkinsonism with Autonomic Symptoms and Ataxia; Associated with α-Synuclein Glial Cytoplasmic Inclusions; Neuron loss and Gliosis

30
Q

Corticobasal Degeneration

A

Alien Limb Phenomena, Myoclonus and Dementia

31
Q

Wilson’s Disease

A

• Disorder of Copper Metabolism; Deposition into Basal Ganglia, Cornea and Liver causing Cirrhosis; Should be considered in patients <50yrs with Akinetic-Rigid Syndrome or any
Hyperkinetic Movement Disorder with Liver Cirrhosis
• Investigations include Serum Copper and Caeruloplasmin
• Intellectual Impairment develops; Neurological damage reversible with early treatment
• Treat with Chelating Agent (e.g. Penicillamine)

32
Q

Hyperkinetic Movement Disorders

A

• Tremor (Rhythmic Oscillation), Chorea (Excessive Irregular Movements Fitting from one body part to another), Myoclonus (Brief Electric shock-like Jerks), Tics (Stereotyped movements or
Vocalisations) and Dystonia (Sustained muscle spasms causing twisting movements and abnormal posturing)

33
Q

Essential Tremor

A
  • Inherited in Autosomal Dominant manner; Bilateral, Fast low amplitude tremor mainly in Upper Limbs; Head and Voice occasionally involved
  • Tremor is postural; Starts at any age but usually early in life; Slowly progressive but rarely causes severe disability, exacerbated by Anxiety
  • Treatment is unnecessary and mostly unhelpful; Small amounts of Alcohol, Propranolol, Primidone or Gabapentin might help
  • Stereotactic Thalamotomy and DBS used in severe cases
34
Q

Hemiballismus

A

• Infarction/Haemorrhage of Contralateral Subthalamic Nucleus leads to Violent Swinging Movements due to loss of Indirect Pathway (Basal Ganglia loop involved in Inhibition)
• Acute Chorea-Hemiballismus can also occur after Diabetic HONK; Osmotic shift causing
Myelinolysis seen in CT/MRI imaging of the Basal Ganglia

35
Q

Causes of Chorea

A
  • Causes include Thyrotoxicosis, SLE, Antiphospholipid Syndrome, Polycythaemia
  • Genetic causes – Huntington’s Disease, Neuroacanthocytosis, Benign Hereditary Chorea
  • Structural disorders of Basal Ganglia, Drugs (Levodopa, Oral Contraceptives), Pregnancy
  • Post-Infective – Sydenham’s Chorea Post Streptococcus or Acute Rheumatic Fever
  • Dopamine Blockade (Phenothiazines) or Depleting (Tetrabenazine) drugs
36
Q

Huntington’s Disease Presentation

A

• Usually presenting in Middle age; Subtle fidgeting followed by Progressive Psychiatric and Cognitive symptoms; Prevalence worldwide of 5 per 100,000

37
Q

Huntington’s Disease Genetics

A

• Due to CAG repeat expansion; Translation of expanded Polyglutamine repeat sequence in
Huntingtin; Thought to be toxic gain-of-function protein
• Repeat length inversely related to age at onset (Juvenile patients have over 60 repeats);
Anticipation – Successive generations have Earlier Onset and more Severe disease
o Expansion of CAG repeats occurs during Meiosis especially in Spermatogenesis

38
Q

Huntington’s Treatment

A

Autosomal Dominant with Complete Penetrance; No disease modifying treatment; Chorea improved with Dopaminergic Blockade/Depletion although Progressive Neurodegeneration
leads to Dementia and ultimately death after 10-20yrs

39
Q

Myoclonus

A
  • Cortical Myoclonus – Distal presentation (Hands and Fingers) and Stimulus Sensitive (Spontaneous or Triggered by Touch/Loud noises)
  • Spinal/Brainstem Myoclonus – Localised lesions
40
Q

Primary Myoclonus

A

Physiologically normal Nocturnal Myoclonus (Commonly feeling of falling); Startle response is
a form of Brainstem Myoclonus

41
Q

Myoclonic Dystonia

A

Rare Autosomal Dominant disorder due to mutations in ε-Sarcoglycan Gene (DYT11); Allelic with Benign Essential Myoclonus
o Sarcoglycan involved in connecting the Muscle fibre cytoskeleton with ECM

42
Q

Secondary Myoclonus

A

• Seen in wide variety of Metabolic Disorders including Hepatic/Renal Failure (Asterixis = Liver
Flap), Part of Dementias and Neurodegenerative Disorders, Encephalitis
• Post-Anoxic Myoclonus secondary to Severe Cerebral Anoxia

43
Q

Progressive Myoclonic Epilepsy Ataxia Syndromes

A
  • Rare conditions of Genetic and Metabolic Disorders; Myoclonus accompanying Progressive Epilepsy, Cognitive Decline and Ataxia
  • E.g. Lafora Body Disease, Neuronal Ceroid Lipofuscinosis, Unverricht-Lundborg disease
44
Q

Tics

A

• 15% Lifetime prevalence; Brief stereotyped movements affecting Face and Neck; Might also affect any other body part (e.g. Vocal Tics); Can be suppressed, leading to build up of Anxiety
and Overflow leading to release
• Simple transient Tics – Blinking, Sniffing, Facial Grimacing are common in childhood
• Adult Onset Tics are rare; Usually secondary cause

45
Q

Tourette’s

A

Commonest cause of Tics; Multiple Motor Tics and at least one Vocal Tic starting in Childhood and persisting longer than 1yr; 3× more common in Boys
• Coexists with Behavioural problems including ADHD, OCD; Major cause of disability
• Explosive Barking, Grunting of Obscenities (Coprolalia), Gestures (Copropraxia) and Copying what other people say (Echolalia)
• Thought to be complex problem regarding Histaminergic Neurotransmission

46
Q

Primary Dystonia

A

Only or Main Clinical Manifestation; Usually Genetic

47
Q

Secondary Dystonia

A

Due to Brain Injury, Cerebral Palsy or Drugs

48
Q

Heredo-degenerative dystonia

A

Wider Neurodegenerative Disorder

49
Q

Paroxysmal Dystonia

A

Rare, mostly genetic attacks of Chorea and Dystonia

50
Q

Young Onset Primary Dystonia

A
  • Mutations in DYT1 Gene causes Limb-Onset Dystonia in Adolescence
  • Most patients have GAG Deletion in TorsinA ER ATPase protein; Autosomal Dominant, Low Penetrance, Variable Phenotype that can progress to Generalised Dystonia
  • Cognitive function is intact; Definitive treatment of severe disease by DBS
51
Q

Adult Onset Primary Dystonia

A
  • Much more common; Onset around 55yrs; Focal; Affects Head and Neck mostly
  • Treatment by Targeted injection of Botulinum Toxin into affected muscles for all Focal Dystonias; Antimuscarinics (e.g. Trihexyphenidyl) sometimes helpful
52
Q

Torticollis

A

Dystonic spasms in Neck Muscles causing head turning or drawn backwards; Jerky Head Tremor might be present; Sensory Trick (Geste) might relieve spasm temporarily

53
Q

Writer’s Cramp/ Task Specific Dystonia

A

Specific inability to perform previously Highly-developed Repetitive Skilled Movement, instead provoking Dystonic posturing; Other functions remain normal
o Overuse may lead to Task-specific Dystonias e.g. Musicians, Typists

54
Q

Blepharospasm and Oromandibular Dystonia

A

Spasms of forced blinking, Involuntary movement of the Mouth/Tongue; Speech might be affected

55
Q

DOPA-Responsive Dystonia (DRD)

A

Typically Dystonic Walking begins in Childhood; Resemble Spastic Paraparesis/Cerebral Palsy
o Dominantly Inherited Mutations in GTP Cyclohydrolase Gene; Codes for protein needed for Dopamine Synthesis
Patients with Dystonic Gaits sometimes given Levodopa trials

56
Q

Drug Induced Dystonia

A 
Drug Induced (Neuroleptics) causing Akathisia (Restless, Repetitive, Irresistible need to move), Parkinsonism (D1 and D2 Receptor Blockade), Acute Dystonic reactions (Spasmodic Torticollis, Trismus and Oculogyric Crises (Episodic Sustained Upward gaze)
o
57
Q

Tardive Dyskinesia

A

Tardive Dyskinesia – Mouthing and Lip-Smacking Grimaces after years of Neuroleptic use; Temporarily worsens when drug stopped/reduced; Atypical Neuroleptics less
prone to cause

Neurology STG (15 decks)

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