Movement disorders and demyelinating diseases

Question 1

Parkinsons: What kind of neurons are you losing and in what two locations?

Answer 1

Dopamine-containing neurons

1. ) Substantia nigra
2. ) Locus ceruleus (midbrain)

Question 2

Is Parkinsons a clinical diagnosis?

Answer 2

Yes

Question 3

Six main symptoms of Parkinsons disease

Answer 3

1. ) Pill rolling tremor at rest (worsens with stress)
2. ) Bradykinesia - slowing of movements
3. ) Cogwheel rigidity
4. ) Difficulty initiating first step, walking with shuffling
5. ) Autonomic dysfunction
6. ) Micrographia, dysarthria, and dysphagia

Question 4

What neurotransmitters are treatments for Parkinsons directed towards

Answer 4

Either increasing dopamine or decreasing acetylcholine

Question 5

What is the main drug of choice for Parkinsons and its main side effect, and what do you expect to see throughout the day with someone on this drug

Answer 5

Carbidopa-levodopa (Sinemet) - most effective

Side effect: Dyskinesia - 5 to 7 years after therapy

Fluctuating symptoms throughout day

Question 6

Besides Sinemet, what are the other drugs used for Parkinsons

Answer 6

1. ) Dopamine receptor agonists (bromocriptine, pramipexole, ropinirole) - initiate at beginning or adjunct
2. ) Selegiline - Inhibits MAO-B, increasing dopamine and levodopa indirectly
3. ) Amantadine (antiviral) - early and mild disease
4. ) Anticholinergics - trihexyphenidyl and benztropine, for tremors but not for elderly
5. ) Amitryptline - anticholinergic and anti-depressant
6. ) Surgery - if disease before 40 or not responding to medications - deep brain stimulation

Question 7

Which drug can be used for sudden freezing in Parkinsons

Answer 7

Dopamine receptor agonists

Question 8

What is the age of onset of Huntingtons

Answer 8

Between 30 and 50

Question 9

What are the genetics of Huntington’s

Answer 9

Autosomal dominant, chrom 4 (CAG repeats), causing loss of GABA producing neurons in striatum

Question 10

Five main symptoms of Huntington’s

Answer 10

1. ) Chorea
2. ) Altered behavior
3. ) Impaired mentation
4. ) Gait unsteady and irregular
5. ) Urinary incontinence

Question 11

What would you see in the MRI of a patient with Huntington’s

Answer 11

Atrophy of head of caudate nucleus

Question 12

How do you confirm the diagnosis of Huntington’s

Answer 12

DNA testing

Question 13

What are some drug causes of physiologic tremor

Answer 13

Alcohol withdrawal, valproic acid, lithium, caffeine and theophylline

Question 14

Does essential tremor have genetics involved?

Answer 14

Yes - autosomal dominant in 1/3rd of patients

Question 15

What induces and what relieves essential tremor

Answer 15

Induction: Intentional activity i.e. using stuff
Relief: Alcohol

Question 16

What is shy-drager syndrome

Answer 16

Parkinsons and autonomic insufficiency

Question 17

What do you see in the brain of someone with Parkinsons disease

Answer 17

Lewy bodies (hyalin inclusion bodies)

Question 18

What is the exact description of the tremor in essential tremor

Answer 18

Fine and with certain postures vs. pill rolling (Parkinsons) or coarse (cerebellar)

Question 19

What other tremors do you see with essential tremor

Answer 19

Head tremor and vocal tremor

Question 20

What is the treatment for essential tremor

Answer 20

Propanolol

Question 21

What are two inherited causes of ataxia

Answer 21

1. ) Fredreich’s ataxia - Autosomal recessive with ataxia, nystagmus, impaired MVP
2. ) Ataxia telangiectasia - Autosomal recessive, Freidrich’s + telangiectases + increased cancer risk + happens earlier

Question 22

What are some general characteristics of Tourette’s syndrome

Answer 22

Associated with obsessive compulsive disorder, before age 21, some have caprolalia (involuntary swearing), maybe autosomal dominant

Question 23

Clinical features of Tourette’s

Answer 23

Motor tics (multiple)Phonic tics (at least one kind)

Question 24

When do you treat Tourette’s syndrome and what do you give

Answer 24

Only when it is symptomatic - 3 drugs

1. ) Clonidine
2. ) Pimozide
3. ) Haloperidol

Question 25

What happens to the brain in multiple sclerosis

Answer 25

Selective demyelination of CNS, forming plaques in white matter, commonly at angles of lateral ventricles

Question 26

What specific brain tracts are commonly involved in multiple sclerosis

Answer 26

1. ) Pyramidal and cerebellar 2. ) Medial longitudinal fasciculus 3. ) optic nerve 4. ) Posterior columns

Question 27

Nine clinical features of multiple sclerosis

Answer 27

1. ) Transient sensory deficits 2. ) Fatigue 3. ) Motor symptoms - weakness or spasticity 4. ) Visual disturbances - optic neuritis and internuclear opthalmoplegia 5. ) Cerebellar involvement - ataxia and intention tremor 6. ) Loss of bladdar control - UMN injury 7. ) Autonomic involvement - impotence and constipation 8. ) Cerebral involvement - personality change 9. ) Neuropathic pain

Question 28

What are the consequences of the motor symptoms in multiple sclerosis

Answer 28

Spacisity (leg stiffness) impairing balance and walking, and weakness leading to paraparesis, hemiparesis, or quadriparesis

Question 29

Consequences of optic neuritis in multiple sclerosis

Answer 29

1. ) Monocular visual loss 2. ) Pain with movement of eye 3. ) Central scotoma (black spot in center of vision) 4. ) Decreased pupillary reflex to light

Question 30

What is internuclear ophthalmoplegia (in MS)

Answer 30

Ipsilateral medial rectus palsy on attempted lateral gaze and horiztonal nystagmus of abducting eye, can cause diplopia

Question 31

What are the variants of multiple sclerosis

Answer 31

1. ) Clinically silent 2. ) Relapsing/remitting (most common) 3. ) Secondary progressive - relapsing remitting but getting worse each time 4. ) Primary progressive - less visual but more axonal involvement, after age 40

Question 32

Prognosis of multiple sclerosis

Answer 32

Normal life expectancy but diminished quality of life

Question 33

Clues to diagnosis and what to order

Answer 33

Young adults with relapsing focal neurological signs Order MRI (gold standard) and get LP (oligoclonal bands) Number of lesions on MRI doesn't indicate severity. Can measure speed of neurons, new neurons conduct sensory impulses more slowly

Question 34

What is the treatment of acute attacks in multiple sclerosis

Answer 34

High dose IV corticosteroids can shorten acute attack unlike oral, do therapeutic plasma exchange if it doesn't work No improvement in long-term outcomes

Question 35

What are disease modifying therapies (reduces relapses) for multiple sclerosis

Answer 35

1.) Inteferon b1a and b1b and glatiramer acetate Will get flu like symptoms

Question 36

What should be used for the treatment of muscle spasticity, neuropathic pain, and depression

Answer 36

1. ) Spacisity: Baclofen or dantrolene 2. ) Neuropathic pain: Carbamazepine and gabapentin 3. ) Depression: Antidepressants

Question 37

What are the general characteristics of guillain-barre syndrome

Answer 37

Inflammatory demyelinating polyneuropathy affecting motor nerves and preceeded by viral or mycoplasmal infection of upper respiratory or GI

Question 38

What are common bugs that precede guillain-barre syndrome

Answer 38

Campylobacter jejuni, CMV, hepatitis, and HIV

Question 39

What other conditions can cause guillain barre syndrome

Answer 39

Hodgkin's disease, lupus, after surgery

Question 40

What are the clinical features of guillain-barre syndrome

Answer 40

Abrupt onset, rapidly ascending weakness/parlysis of all four extremities, eventually involves respiratory, facial, and bulbar muscles Painful extremities but not sensory loss with sphincter control and mentation spared (unlike multiple sclerosis)

Question 41

What are dangerous complications of guillain barre syndrome

Answer 41

Generalized paralysis leading to respiratory arrest, arhythmias, tachycardia, postural hypotension

Question 42

What three things must you use in combination to diagnose guillain barre syndrome

Answer 42

CSF analysis showing elevated protein, nerve conduction studies showing decreased motor nerve activity, and clinical findings

Question 43

What is used in the treatment of guillain barre syndrome

Answer 43

1. ) Admit to hospital immediatley to monitor pulmonary function, may use mechanical ventilation 2. ) IV immunogluboulin or plasmapharesis

Question 44

What must you NOT give in the treatment of guillain barre syndrome

Answer 44

Steroids (unlike multiple sclerosis)

Question 45

What is a favorable prognosis of guillain barre syndrome

Answer 45

Signs of recover 1 to 3 weeks after onset

Question 46

The treatment of essential tremor is similar to what other disease

Answer 46

Migraines

Question 47

What other medication can you give besides propanolol for treatment of essential tremors, and whats the side effect

Answer 47

Primidone - intermittent porphyria side effect (abdominal pain, neuro and psych problems, dx this in urine)