Movement disorders and demyelinating diseases Flashcards

1
Q

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

A

Dopamine-containing neurons

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

Is Parkinsons a clinical diagnosis?

A

Yes

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3
Q

Six main symptoms of Parkinsons disease

A
  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
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4
Q

What neurotransmitters are treatments for Parkinsons directed towards

A

Either increasing dopamine or decreasing acetylcholine

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5
Q

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

A

Carbidopa-levodopa (Sinemet) - most effective

Side effect: Dyskinesia - 5 to 7 years after therapy

Fluctuating symptoms throughout day

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6
Q

Besides Sinemet, what are the other drugs used for Parkinsons

A
  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
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7
Q

Which drug can be used for sudden freezing in Parkinsons

A

Dopamine receptor agonists

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8
Q

What is the age of onset of Huntingtons

A

Between 30 and 50

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9
Q

What are the genetics of Huntington’s

A

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

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10
Q

Five main symptoms of Huntington’s

A
  1. ) Chorea
  2. ) Altered behavior
  3. ) Impaired mentation
  4. ) Gait unsteady and irregular
  5. ) Urinary incontinence
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11
Q

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

A

Atrophy of head of caudate nucleus

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12
Q

How do you confirm the diagnosis of Huntington’s

A

DNA testing

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13
Q

What are some drug causes of physiologic tremor

A

Alcohol withdrawal, valproic acid, lithium, caffeine and theophylline

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14
Q

Does essential tremor have genetics involved?

A

Yes - autosomal dominant in 1/3rd of patients

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15
Q

What induces and what relieves essential tremor

A

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

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16
Q

What is shy-drager syndrome

A

Parkinsons and autonomic insufficiency

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17
Q

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

A

Lewy bodies (hyalin inclusion bodies)

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18
Q

What is the exact description of the tremor in essential tremor

A

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

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19
Q

What other tremors do you see with essential tremor

A

Head tremor and vocal tremor

20
Q

What is the treatment for essential tremor

A

Propanolol

21
Q

What are two inherited causes of ataxia

A
  1. ) Fredreich’s ataxia - Autosomal recessive with ataxia, nystagmus, impaired MVP
  2. ) Ataxia telangiectasia - Autosomal recessive, Freidrich’s + telangiectases + increased cancer risk + happens earlier
22
Q

What are some general characteristics of Tourette’s syndrome

A

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

23
Q

Clinical features of Tourette’s

A

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

24
Q

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

A

Only when it is symptomatic - 3 drugs

  1. ) Clonidine
  2. ) Pimozide
  3. ) Haloperidol
25
Q

What happens to the brain in multiple sclerosis

A

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

26
Q

What specific brain tracts are commonly involved in multiple sclerosis

A
  1. ) Pyramidal and cerebellar
  2. ) Medial longitudinal fasciculus
  3. ) optic nerve
  4. ) Posterior columns
27
Q

Nine clinical features of multiple sclerosis

A
  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
28
Q

What are the consequences of the motor symptoms in multiple sclerosis

A

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

29
Q

Consequences of optic neuritis in multiple sclerosis

A
  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
30
Q

What is internuclear ophthalmoplegia (in MS)

A

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

31
Q

What are the variants of multiple sclerosis

A
  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
32
Q

Prognosis of multiple sclerosis

A

Normal life expectancy but diminished quality of life

33
Q

Clues to diagnosis and what to order

A

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

34
Q

What is the treatment of acute attacks in multiple sclerosis

A

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

35
Q

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

A

1.) Inteferon b1a and b1b and glatiramer acetate

Will get flu like symptoms

36
Q

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

A
  1. ) Spacisity: Baclofen or dantrolene
  2. ) Neuropathic pain: Carbamazepine and gabapentin
  3. ) Depression: Antidepressants
37
Q

What are the general characteristics of guillain-barre syndrome

A

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

38
Q

What are common bugs that precede guillain-barre syndrome

A

Campylobacter jejuni, CMV, hepatitis, and HIV

39
Q

What other conditions can cause guillain barre syndrome

A

Hodgkin’s disease, lupus, after surgery

40
Q

What are the clinical features of guillain-barre syndrome

A

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)

41
Q

What are dangerous complications of guillain barre syndrome

A

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

42
Q

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

A

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

43
Q

What is used in the treatment of guillain barre syndrome

A
  1. ) Admit to hospital immediatley to monitor pulmonary function, may use mechanical ventilation
  2. ) IV immunogluboulin or plasmapharesis
44
Q

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

A

Steroids (unlike multiple sclerosis)

45
Q

What is a favorable prognosis of guillain barre syndrome

A

Signs of recover 1 to 3 weeks after onset

46
Q

The treatment of essential tremor is similar to what other disease

A

Migraines

47
Q

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

A

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