Central Nervous system - movement disorder ( ataxia, vertigo, parkinson etc)

Question 1

define ATAXIA

Answer 1

• lack of smooth and intensional movement
• symptom of particular disease process
• divided into 2 types
  1) Motor cerebellar ataxia
  2) Sensory Ataxia

Question 2

What is motor cerebellar ataxia

Answer 2

Disorder of Cerebellum
(problem with the intergration of propioception signal - NB pathways are fine)
- lesion is ipsilateral
a) lateral cerebelar lesion
- cause limb incoordination
b) Midline cerebellar lesion
- Axial co-ordination problem less common ( may affect internal capsule, thalamic nucleus, frontal lobe)

Question 3

What is sensory cerebellar ataxia

Answer 3

Failure of transmission of propioception to CNS via Peripheral Nerve
( Dorsal column or cerebellar input)

Question 4

What are the causes of Ataxia

Answer 4

• Drug intoxication - ethanol, dilantin
• Metabolic - hyponatremia
• Peripheral Nerve - Alcohol peripheral neuropathy
• Vestibulopathy - meneiere disease
• Cerebellar disorder - infarction, mass, degeneration
• Posterior Column disorder - Vit B12 deficiency

Question 5

What are the clinical presentations for ataxia

Answer 5

• difficulty ambulating - weakness and falls
• worsen with loss of visual input ( More Sensory ataxia)
• Cerebellar gait - wide space with unsteady irregular step

Question 6

What is dysmetria

Answer 6

inacurate fine movement

Question 7

What is Dysdiadokinesia

Answer 7

Clumpsy rapid movement

Question 8

Dyssynergia

Answer 8

breakdown of movements into parts( Jacksonian movement)

Question 9

Romberg test

Answer 9

• unsteady with eyes open - cerebellar pathology

- unsteady with eyes close - Sensory ataxia

Question 10

What is true vertigo

Answer 10

illusion of movemet of the environment

Question 11

What are the 3 main areas of the vestibular system that can be involved

Answer 11

1) Otological disease - of middle or inner ear
2) Vestibular nerve or end organ alone
3) Vestibular nuclei in the brainstem

Question 12

With the otological cause, vertigo is associated with what clinical features

Answer 12

• hearing loss
• tinnitus
• ear discharge
• otalgia
• fullness or blockage

Question 13

Vestibular nerve or endorgan alone

Answer 13

Present with vertigo alone

Question 14

With regard to vestibular nuclei lesion - vertigo is associated with what clinical features?

Answer 14

• loss of consciousness
• diplopia
• other visual disturbance
• paraesthesiae
• limb paresis or weakness
• dysarthria
• dysphagia

Question 15

What are important test during examination that can assist with the diagnosis of vertigo

Answer 15

1) Test of hearing
2) Webber and Rhinne test ( tuning fork)
3) Features of nystagmus
4) Examine cranial nerves and cerebellar function - Rhomberg tests

Question 16

How do you perform Rinne Test

Answer 16

Rinne Test

• The doctor strikes a tuning fork and places it near the base of your mastoid bone.
• The doctor asks you to say when you no longer hear the sound.
• The doctor notes the time and moves the tuning fork near the ear canal.
• The doctor asks you to say when you no longer hear the sound.
• The doctor compares the time intervals for the two steps

Question 17

What is the result of the Rinne Test

Answer 17

• In an ear with a hearing loss, if air conduction is better than bone, the deficit is sensorineural.
• If bone conduction is better than air, the loss is conductive in nature

Question 18

How do you perform Webber Test

Answer 18

• The doctor strikes a tuning fork and places it on the middle of your head.
• The doctor asks you where the sound is coming from: the left ear, the right ear, or both.

Question 19

What is the result of Weber tests

Answer 19

• Normal hearing will indicate sound in both ears.
• Conductive loss will indicate the sound travels towards the poor ear.
• Sensorineural loss will indicate the sound travels towards the good ear

Question 20

What are the features of the Nystagmus suggesting peripheral nystagmus vs central vertigo

Answer 20

Peripheral Vertigo

• Combined horizontal and torsional
• inhibited by fixation of eyes onto object
• fades after a few days
• does not change direction with gaze to either side

Central Vertigo

• Purely vertical, horizontal, or torsional
• not inhibited by fixation of eyes onto object
• may last weeks to months
• may change direction with gaze towards fast phase of nystagmus

Question 21

What are other features that differentiate peripheral from central vertigo

Answer 21

Peripheral Vertigo

1) Imbalance - Mild to moderate, able to walk
2) Nausea & vomiting - May be severe
3) Hearing loss or tinnitus - common
4) Non auditory Neurologic symptoms - rare
5) Latency following provocative test - longer ( up to 20 seconds)

Central Vertigo

1) Imbalance - Severe and unable to walk or stand still
2) N&V - Varies
3) Hearing loss or tinnitus - rare
4) Non auditory neurologic symptoms - common
5) Latency - short ( up to 5 seconds)

Question 22

Associated symptoms for different causes of Vertigo

Answer 22

SYMPTOM SUGGESTED DIAGNOSIS
1) Aural fullness - Acoustic neuroma; Ménière’s disease

2) Ear or mastoid pain - Acoustic neuroma; acute middle ear disease (e.g., otitis media, herpes zoster oticus)
3) Facial weakness - Acoustic neuroma; herpes zoster oticus
4) Focal neurologic findings - Cerebellopontine angle tumor , CVA ; multiple sclerosis (especially findings not explained by single neurologic lesion)
5) Headache- Acoustic neuroma; migraine
6) Hearing loss - Ménière’s disease; perilymphatic fistula; acoustic neuroma; cholesteatoma; otosclerosis; transient ischemic attack or stroke involving anterior inferior cerebellar artery; herpes zoster oticus
7) Imbalance - Acute vestibular neuronitis (usually moderate); cerebellopontine angle tumor (usually severe)
8) Nystagmus- Peripheral or central vertigo
9) Phonophobia, photophobia - Migraine
10) Tinnitus - Acute labyrinthitis; acoustic neuroma; Ménière’s disease

Question 23

What are the causes of vertigo associated with hearing loss

Answer 23

DIAGNOSIS - CHARACTERISTICS OF HEARING LOSS
a) Acoustic neuroma - Progressive, unilateral, sensorineural

b) Cholesteatoma - Progressive, unilateral, conductive
c) Herpes zoster oticus (i.e., Ramsay Hunt syndrome) - Subacute to acute onset, unilateral
d) Ménière’s disease - Sensorineural, initially fluctuating, initially affecting lower frequencies; later in course: progressive, affecting higher frequencies
e) Otosclerosis - Progressive, conductive
f) Perilymphatic fistula - Progressive, unilateral
g) Transient ischemic attack or stroke involving anterior inferior cerebellar artery or internal auditory artery - Sudden onset, unilateral

Question 24

The common readily distinguishable vestibular pathologies are

Answer 24

(1) Middle ear disease affecting the inner ear
• Acute inflammatory problems.
• Tympanic membrane perforation with infection: Check for cholesteatoma (white keratin).
• Post middle ear surgery: patients may present to ED with vertigo after mastoidectomy or stapedectomy.
• Glomus tumour: pulsatile tinnitus with a red swelling behind an intact tympanic membrane.

(2) Inner ear disease
a) BPV
b) Meniere’s disease
c) Acute Labyrinthitis / Vestibular neuronitis

Question 25

Central vertigo

Answer 25

- Cranial nerve disorder - CNS VIII, VII, V eg Herpes Zoster Oticus - CNS disease - Vascular causes - basilar migraine, MS, Toxic causes - Cervical causes

Question 26

What are the treatment for the acute vertigo

Answer 26

1) Prochlorperazine - antiemetic, CNS suppression 2) IV Diazepam - Vestibular suppresion action 3) Haloperidol - for vomiting and vertigo 4) Promethazine - anticholinergics and antihistamine 5) Vestibular rehabilitation exercises 6) Surgery

Question 27

What are the types of multi- system atrophy in Multiple system Atrophy

Answer 27

* Striatonigral degeneration - : clinical picture resembles Parkinson's disease but without tremor. These patients do not respond to anti-Parkinson medications and often develop adverse reactions to these agents. * Shy-Drager syndrome: clinical picture consists of Parkinson's disease combined with severe autonomic neuropathy (particularly postural hypotension). Other important clinical features are impotence and bladder disturbances. * Olivopontocerebellar atrophy: combination of extrapyramidal manifestations and cerebellar ataxia. Patients may also have autonomic neuropathy and anterior horn cell degeneration. * Parkinsonism and motor neuron disease: rare.

Question 28

What is the pathology in Shy-Drager syndrome?

Answer 28

In 1960, Shy and Drager described changes in the brainstem and ganglia; subsequently, loss of neurons has been shown in the autonomic nervous system and in the cells of the intermediolateral column of the spinal cord. - Positron emission tomography shows decreased uptake of dopamine in the putamen and caudate lobe

Question 29

What are the clinical picture of Multiple system atrophy?

Answer 29

``` History • Dizziness when standing up (due to postural hypotension). • Dysphagia. • Ataxia. • Symptoms of Parkinson's disease. • Impotence, bladder disturbances. • Anhidrosis. ``` Examination • Mask-like facies and other features of bradykinesia. • Increased tone (rigidity). • Cerebellar signs.

Question 30

What is Parkinson's

Answer 30

is a progressive neurodegenerative condition

Question 31

What is the cause of parkinson

Answer 31

result of insufficient quantities of the neurotransmitter dopamine in a part of the brain called the substantia nigra. - Genetic factors - Environmental toxins - Accelerated ageing - Free Radicals

Question 32

What are the symptoms and signs of parkinson disease

Answer 32

- Tremor - Stiffness - Bradykinesia - Loss of Balance ``` Other symptoms Skin sensations and pain Constipation Fatigue Depression Altered speech (may be slurred and slow) Difficulty with writing Numbness Walking difficulty Dementia (memory loss) Decreased blinking Increased saliva production Emotional changes ```

Question 33

What are the treatment for parkinsonism?

Answer 33

1) Medication - Dopamine agonist eg Levodopa 2) Physiotherapy 3) Brain Surgery