Other Neuro Topics Flashcards

1
Q

What age does Tourette’s Syndrome symptoms typically start?

A

Most commonly 6 years of age (5-10 years)

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2
Q

How long does it typically take (duration) for Tourette’s Syndrome to be diagnosed?

A

About 2 years from onset of symptoms

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3
Q

When do tics increase in severity or frequency?

A

Under stress (new school, move)

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4
Q

What are the three tic disorders?

A

Tourette’s
Persistent
Provisional

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5
Q

How is Tourette’s defined?

A

Both motor and vocal tics persistent for at least 1 year

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6
Q

How is Persistent tic disorder defined?

A

Motor or vocal tics persistent for at least 1 year

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7
Q

How is Provisional tic disorder defined?

A

Motor or vocal or both
Present for at least 1 year

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8
Q

How is Tourette’s diagnosed?

A

Clinical

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9
Q

What is the diagnostic requirements for Tourette’s?

A

-2+ motor ticks
-1+ vocal tics
-Tics present for at least 1 year
-Tics onset prior to age 18
-Symptoms are not related to medication or other medical condition

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10
Q

What are the other top two diagnoses that Tourette’s presents with?

A

ADHD and Anxiety

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11
Q

What is the goal of Tourette’s treatment?

A

Tic suppression

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12
Q

What drug class is the only approved for Tourette’s syndrome?

A

Neuroleptics
(haloperidol, pimozidole, aripiprazole)

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13
Q

What are two off label drug classes used to treat Tourette’s?

A

Alpha adrenergic’s and stimulants

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14
Q

Why would you not prescribe a stimulant to a child with Tourette’s?

A

Although shown helpful at reducing tic frequency they are contraindicated for Tourette’s due to MOA

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15
Q

Do you typically treat Tourette’s with pharmacologic therapy?

A

No, behavioral therapy is more appropriate as a first line. Only severe tics are treated with neuroleptics.

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16
Q

What is the disease trajectory of Tourette’s Syndrome?

A

Typically tics decrease during adolescence/early adulthood, and sometimes disappear completely. Rarely worsens with age (10-15% of cases).

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17
Q

What does ALS stand for?

A

Amyotrophic Lateral Sclerosis

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18
Q

What is the Epidemiology of ALS?

A

Rare progressive neurological disease

Most common in men than women, diagnosed most commonly between 55-75 years of age (can strike at any age)

More in caucasians and non-Hispanics

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19
Q

What occupation is highly associated with ALS?

A

Military service

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20
Q

What is the life expectancy after diagnosis with ALS?

A

Most die from respiratory failure usually 3-5 years from symptom onset

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21
Q

What is the pathology of ALS?

A

Not well understood, thought to have a genetic predisposition with environmental triggers.

Degeneration of the upper and lower motor neurons

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22
Q

What are the two types of ALS?

A

Sporadic and Familial

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23
Q

What is the sporadic type of ALS?

A

90% of cases, diagnoses at random with no clear associated risk factors or family history

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24
Q

What is the familial (genetic) type of ALS?

A

5-10% of cases, inherited from one autosomal dominant parent

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25
Q

What is the initial presentation of ALS?

A

Early muscle weakness and stiffness (generally painless), gradual in onset

Begins with neck/head muscles, limb, respiratory muscles

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26
Q

What are the later symptoms of ALS?

A

Fasciculations in arm, leg, shoulder or tongue
Muscle cramps and spacicity
Muscle weakness (eventually diaphragm)
Slurred and nasal speech, uncontrollable laughing/crying
Difficulty chewing or swallowing

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27
Q

Why is nutrition status critically important in ALS management?

A

Patients burn calories at a faster rate, concern for weight loss and malnourishment

28
Q

Are sensory or motor neurons impaired in ALS?

A

Only motor neurons affected

29
Q

What is the diagnosis and work-up for ALS?

A

Detailed Hx and PE (presence of upper and lower motor neuron involvement)

Other testing to rule out concerns (test for polio and HIV)

30
Q

What is the treatment for ALS?

A

No treatment, treatment geared towards controlling symptoms (constipation, pain, sleep) or preventing complications

31
Q

What are some non-pharmacological treatment for ALS?

A

PT, OT, Speech therapy and nutritional support

32
Q

What is “breath stacking”

A

series of small breaths without exhaling, hold then cough

Used in ALS because exhalation doesn’t require a lot of work/energy

33
Q

What is the Epidemiology of MS?

A

Most often appears in young adulthood, with incidence peaking at 30 years of age (more common in females, 3:1)

MS cases on the rise

34
Q

What is the pathophysiology of MS?

A

T-cell mediated autoimmune disease - Cytotoxic T-cells target myelin and myelin producing cells that activate B-cells producing auto-antibodies against self (CNS damage)

Viral precursor theory?

35
Q

What is a geographical (environmental) factor of MS?

A

Living further away from the equator

36
Q

What are some risk factors of MS?

A

Age, gender, smoking, low vitamin D, and obesity, family history (first degree relative)

37
Q

What are the most common symptoms of MS?

A

Fatigue, weakness, parasthesias, vision problems (retinal detachments, pain with occular movement), bladder problems, cognitive changes (brain fog, difficulty focusing, emotional changes)

38
Q

What are the four types of MS

A
  1. Clinically Isolated Syndrome (CIS)
  2. Relapsing-remitting MS (RRMS)
  3. Secondary Progressive MS (SPMS)
  4. Primary Progressive MS (PPMS)
39
Q

What is the most commonly diagnosed type of MS?

A

Relapsing-remitting MS (RRMS)

40
Q

What is Clinically Isolated Syndrome (CIS)?

A

First episode of symptoms caused by inflammation, unsure diagnosis of MS. Seizure mimicking.

If brain lesions (white matter lesion) on MRI, 2nd episode highly likely to be diagnosed as RRMS - if no brain lesions then unlikely to develop

41
Q

What is RRMS?

A

Relapses of new or increasing neuro sx then remission (where all symptoms are gone). No progression during remission.

42
Q

What does RRMS progress to?

A

SPMS, progressive worsening of neuro function. Unique to every patient but progression is occuring even when in remission stages.

43
Q

What is the worst stage of MS?

A

PPMS, worsening accumulation of disability from onset of symptoms early relapse or remission (persistently getting worse)

44
Q

How is MS diagnosed?

A

Clinical diagnosis of history and PE

McDonald Criteria
- Damage in 2 areas of the CNS that date at different times
- CSF + oligoclonal bands (Spinal Tap)

45
Q

What is an MRI finding of MS?

A

White matter lesions (dating can be helpful in diagnosis for different attacks)

46
Q

What is the treatment for MS?

A

Immunotherapy agents (injectables, oral, and infused therapies)

Symptom management - fatigue is most common (stimulants)

47
Q

What is the relapse management for MS?

A

Severe symptoms (vision loss, severe weakness) high dose steroids (prednisone or methylprednisolone) or ACTH (inflammatory process)

48
Q

What is the most common cause of death in MS patients?

A

Secondary complications caused by immobility, chronic UTI, compromised swallowing and/or breathing

49
Q

How is normal pressure hydrocephalus (NPH) simply defined?

A

Too much CSF in the ventricles

50
Q

When does NPH usually occur?

A

Older adults (> 60 years of age), developing slowly over time

51
Q

What is the presentation in NPH?

A

Dementia, difficulty walking, urinary incontinence, headache, nausea, difficulty with vision focus (subtle sx that gradually get worse)

Symptoms usually attributed to “getting older”

52
Q

What is the diagnosis of NPH?

A

History and PE with mental status and gait evaluation

Labs, CT, MRI, or LP to rule out other cause

LP can demonstrate need for shunt placement

53
Q

What is the treatment of NPH?

A

No cure or definitive management

Surgical shunt vs. non-surgical

54
Q

What are the three W’s of NPH

A

Weird, wet, and wobbly (all are reversible)

55
Q

What is the epidemiology of Myasthenia Gravis

A

Affects M:W equally (earlier in females and later in men)

56
Q

What is neonatal myasthenia?

A

Mom with M. gravis pushes antibodies to baby in-utero and when born babies demonstrates symptoms (weakness). Typically resolves 4-6 weeks after birth.

57
Q

What is the pathophysiology of M. gravis?

A

Interruption at the neuromuscular junction in that antibodies block acetylcholine receptors, impacts skeletal muscle fibers

58
Q

What is the role of the thymus in M. gravis?

A

Thymus remains large (lymphoid hyperplasia), but the function is not fully understood (maybe incorrect instructions in developing T-cells)

59
Q

What is the first noticeable symptom in M. gravis?

A

Weakness of eye muscles

60
Q

What are the symptoms of M. gravis?

A

Ptosis (drooping of eyelids)
Diplopia
Facial expression changes
Dysphagia
Shortness of breath
Dysarthria (impaired speech)
Weakness in arms, hands, fingers, legs and neck

61
Q

What is the most sensitive test for diagnosing MG?

A

Single fiber EMG - detects impaired nerve to muscle signal transmission

62
Q

How is MG diagnosed?

A

Edrophonium test
History and PE
Blood work- Ach receptor antibodies and anti-MuSK antibodies
Single fiber EMG (not done by all neurologists)
CT/MRI of thymus (thymoma)
PFT’s

63
Q

What is the treatment of MG?

A

Thymectomy (maybe a cure in up to half of patients)
Anti-cholinesterase medications (Pyridostigmine)
IVIG

64
Q

What is a common complication of MG?

A

Weakness of respiratory muscles (require intubation, IV immunoglobulin, plasmapheresis)

65
Q

What can trigger a MG crisis?

A

Infection
Stress
Surgery
Adverse reaction to medication

66
Q

What is the prognosis of MG?

A

With treatment, most severe weakness will improve and patients can live full lives