1. What is the definition of dementia?

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a. Loss of i_____ abilities

intellectual

b. Severe enough to interfere with_____

social

c. or o_____ functioning

occupational

d. Cardinal feature is m_____ d_____

memory deficit

e. plus at least one additional i_____

impairment

f. Affects_____% of persons over 65

3 to 11%

2. Risk factors for dementia include

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Hint: afA

a. a_____ a_____

advanced age

b. f_____ h_____

family history

c. A_____ _____ _____ a_____

Apoli protein E4 allele

3. True or False. Because delirium is distinct from dementia, patients with dementia are not at increased risk of developing delirium.

false (Patients with dementia are at increased risk of developing delirium.)

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4. True or False. Fifty percent of patients with delirium die within 2 years.

true

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5. In regard to a unilateral headache, if it persists

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a. for > a year an_____ _____ is recommended

MRI scan

b. because this is_____ for migraine

atypical

c. and may be a hint of an underlying_____

AVM

6. Matching. Match symptoms with category of migraine.

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Symptoms:

Episodic H/A; N/V; Photophobia; Aura; Focal neurological deficit (a) that resolves within 24 hrs; (b) slow march—like progression of deficit; (c) that resolves within 30 days; No headache; Mostly seen in children; Hemiplegia; Mostly seen in adolescents; Vertigo, ataxia, dysarthria, severe HA Category of migraine:

a. Common migraine

––

b. Classic migraine

–––––a-b

c. Complicated migraine

–C-

d. Migraine equivalent

––

e. Hemiplegic migraine

–

f. Basilar artery migraine

–

7. True or False. Neurological deficits seen in classic migraine typically resolve within

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a. 1 hour

false

b. 1 day

true

c. 1 week

false

d. 1 month

false

e. They are permanent.

false

8. True or False. Regarding cluster headaches:

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a. may include partial Horner and autonomic symptoms

true (ptosis, miosis, tearing, nasal stuffiness)

b. are more common in women

false (5 men to 1 woman)

c. occur almost daily

true

d. last 30 to 90 minutes

true

e. continue for a 6- to 9-month period

false (1 to 3 months)

f. may have a period of remission for ^˜1 year

true

9. List the drugs for treatment of migraine headaches.

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a. M_____

Midrin (isometheptane mucate, methysergide)

b. l_____

Inderal

c. F_____

Fiorinal Fioricet

d. r_____

rizatriptan (Maxalt)

e. a_____

aspirin, amitriptyline (Elavil)

f. l_____

(Imitrex) sumatripan (Inderal) propranolol

g. n_____

nonsteroidal antiinflammatory drugs, naproxen (Anaprox)

h. e_____

ergotamine tartrate (Cafergot)

     i. S_____

(Sansert) methysergide, serotonin antagonists, steroids

10. True or False. Basilar artery migraines are essentially restricted to

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a. geriatric patients

false

b. postmenopausal women

false

c. adolescents

true

d. men

false

11. True or False. Patients suffering basilar artery migraine attacks usually have a family history of migraine.

true (86%)

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12. Most postlumbar puncture headaches occur within_____ after the lumbar puncture.

3 days

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13. The incidence of postpuncture headaches is_____%.

2 to 40%, typically 20%

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14. A treatment for post puncture headache that is effective in 90% of cases is_____ _____ _____.

epidural blood patch

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15. Matching. Match the symptoms with type of parkinsonism.

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Symptoms:

Gradual onset of bradykinesia; Asymmetric tremor; Responds well to levodopa; Rapid progression of symptoms; Equivocal response to levodopa; Early midline symptoms (i.e., ataxia, gait, balance); Early dementia; Orthostatic hypotension; Extraocular movement abnormalities Types of parkinsonism:

a. Primary idiopathic paralysis agitans (IPA)

––

b. Secondary parkinsonism

–––––

16. In parkinsonism, degeneration of substantia nigra cells (pars compacta) results in

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a. _____ D2 dopamine receptors projecting to the globus pallidus interna (GPi)

↓

b. _____ D1 receptors projecting to globus pallidus externa (GPe) and subthalamic nucleus (STN)

↑

17. This results in increased activity by

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a. _____, causing

GPi

b. _____ of the thalamus, which then suppresses activity in the

inhibition

c. _____ _____ _____.

supplemental motor cortex

18. Provide parkinsonism pathophysiology.

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a. Degeneration of pigmented_____ neurons

dopaminergic

b. Of the pars compacta of the_____ _____

substantia nigra

c. This reduces the levels of_____ in the

dopamine

d. neostriatum; that is, the:

     i. c_____

caudate

     ii. p_____

putamen

     iii. g_____ p_____

globus pallidum

e. This reduces inhibitory D2 receptors to_____

GPi

f. and causes the loss of inhibitory D1 receptors to_____

GPe

g. and the s_____ n_____.

subthalamic nucleus

h. The net result is an_____ in activity

increase

i. of_____.

GPi

j. GPi has inhibitory projections to the t_____.

thalamus

k. Inhibiting the thalamus also suppresses the s_____ m_____ c_____.

supplemental motor cortex

19. A hallmark of Parkinson disease

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a. is_____ _____,

Lewy bodies

b. which are

     i. e_____ i_____

eosinophilic intraneuronal

     ii. h_____ i_____

hyaline inclusions

20. List secondary parkinsonism examples.

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Hint: P4 secondary

a. P

phenothiazine antiemetics, Compazine

b. P

progressive supra nuclear palsy

c. P

poisoning CO₂, manganese

d. P

Parkinson dementia complex of Guam

e. S

strial nigral degeneration, Shy-Drager

f. E

(post)-encephaletic parkinsonism

g. C

Compazine (phenothiazine antiemetics) carbon monoxide

h. O

olivo-ponto-cerebellar degeneration

i. N

neoplasms near substantia nigra

j. D

dementia pugilistica (boxing—post traumatic parkinsonism)

k. A

anti psychotic drugs

l. R

Reglan reserpine, Riley Day (familial dysautonomia)

m. Y

21. Multisystem atrophy (MSA) (i.e., Shy-Drager syndrome) is parkinsonism plus

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a. _____ _____ _____ dysfunction

autonomic nervous system (ANS)

b. plus_____ hypotension.

orthostatic

c. Most don’t respond to_____ _____.

drug therapy

22. List the distinguishing features of the progressive supranuclear palsy (PSP) triad.

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a. _____ (vertical gaze)

ophthalmoplegia

b. _____ dystonia

axial

c. _____ palsy

pseudobulbar

23. Characteristics of the early stage of progressive supranuclear palsy (PSP) (i.e., Steele-Richardson-Olszewski) include

a. falling due to_____ _____ palsy (can’t see floor)

downward gaze

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b. difficulty eating due to_____ _____ palsy (can’t see plate)

downward gaze (supranuclear ophthalmoplegia), vertical gaze

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24. Fill in the blank to summarize surgical treatment for Parkinson disease.

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a. The target site was_____ _____.

ventrolateral nucleus

b. True or False. The surgery worked best for which of the following symptoms:

     i. bradykinesia

false

     ii. tremor

true

c. Which is the more disabling symptom?

     i. bradykinesia

true

     ii. tremor

false

d. The operation cannot be done bilaterally because of risk to_____ _____.

speech function

e. Current treatment site is the p_____ p_____.

posteroventral pallidum

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25. Study Chart.

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a. M

(de) myelinating

b. U

urinary symptoms

c. L

latitudes (northern latitudes affected)

d. T

time and space dissemination

e. I

inter-nuclear ophthalmoplegia (INO)

f. P

paresthesias, peri-ventricular plaques

g. L

lymphocytes

h. E

enhancing lesions on MRI

i. S

scars of the glia

j. C

cortico spinal tracts involved

k. L

la belle indifference (euphoria)

l. E

equator spared

m. R

remissions

n. O

optic atrophy

o. S

sensory loss

p. I

inflammatory response, IgG elevated

q. S

shower test (hot causes exacerbation)

26. Prevalence of multiple sclerosis (MS) per 100,000 is variable.

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a. Near the equator it is_____ per 100,000.

1

b. In Canada and the northern United States it is_____ per 100,000.

30 to 80

27. The most common category is r_____ -r_____.

relapsing-remitting (Acute course with recovery, but 50% become secondarily progressive.)

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28. Name the clinical categories of MS corresponding to their definition.

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a. r_____ -r_____ (acute episodes with recovery)

relapsing-remitting recovery

b. s_____ -p_____ (gradual deterioration)

secondary-progressive

c. p_____ -p_____ (continuous deterioration)

primary-progressive

d. p_____ -r_____ (gradual deterioration with superimposed relapses)

progressive-relapsing

e. Deficits persist if they remain >_____ _____

6 months

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29. Conditions found in the differential diagnosis for multiple sclerosis include

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a. _____, generally monophasic

ADEM (acute disseminated encephalomyelitis)

b. CNS_____

lymphoma

30. Matching. Match multiple sclerosis signs and symptoms with anatomic location.

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Symptoms:

visual acuity; diplopia; extremity weakness; quadriplegia; spasticity; scanning speech; loss of proprioception

Anatomic location:

a. optic nerve

b. retro-bulbar region

c. MLF

d. pyramidal tract

––

e. cerebellum

f. posterior columns

31. Matching. Match anatomic location with multiple sclerosis signs and symptoms.

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Anatomic location:

optic nerve; retro bulbar region; MLF; pyramidal tract; cerebellum; posterior columns

Symptom:

a. visual acuity

–

b. diplopia

c. extremity weakness

d. quadriplegia

e. spasticity

f. scanning speech

g. loss of proprioception

32. Provide the frequency of multiple sclerosis signs and symptoms.

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a. Visual symptoms are among the presenting symptoms of multiple sclerosis in_____%

15%

b. and occur in multiple sclerosis patients during their course of illness in approximately_____%.

50%

c. In addition, abdominal cutaneous reflexes are lost in_____%.

70 to 80%

33. A multiple sclerosis plaque in the medial longitudinal fasciculus (MLF) will cause

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a. _____ _____, which will result in

internuclear ophthalmoplegia

b. _____.

diplopia

c. This is important because_____ rarely occurs in other diseases.

INO

34. Indicate the presence or absence of the following reflexes in MS:

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a. hyperactive muscle stretch reflexes

present

b. Babinski

present

c. abdominal cutaneous reflexes

absent

35. True or False. In multiple sclerosis the more MRI lesions, the higher the likelihood of the diagnosis of MS.

true (MRI is very specific for MS plaques; specificity is 94%.)

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36. Provide MRI criteria for MS.

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a. gadolinium: acute lesions_____

enhance

b. size: at least_____ in diameter

3 mm

c. white matter abnormalities:_____%

80%

d. T2-weighted image_____ _____ _____ _____

lesions are high signal

e. periventricular lesions best seen on_____ _____ images

proton density

f. criterion for dissemination is a_____ _____ _____

new enhancing lesion

g. or a_____ _____ _____

new T2WI lesion

37. True or False. Focal tumefactive demyelinating lesions (TDL) can be mistaken for neoplasms because they

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a. Enhance

true

b. Show perilesional edema

true

c. Can be solitary

true

d. Can be in patients known to have MS

true

e. Can be distinguished from MS

false

f. Biopsy may be necessary

true

g. Biopsy results may be confusing

true

38. What is CNS analysis for MS?

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a. It should include q_____ _____ testing.

qualitative IgG

b. In 90% of MS patients the CSF_____ is high.

IgG

39. Complete the following regarding amyotrophic lateral sclerosis:

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a. aka m_____ n_____ disease

motor neuron

b. aka L_____ G_____ disease

Lou Gehrig

c. A mixed_____ and_____

upper and lower

d. m_____ n_____ disease

motor neuron

e. Degeneration of alpha motor neurons in brain stem

     i. Therefore_____ m_____ neuron disease

upper motor

     ii. and in spinal cord, therefore_____ m_____ neuron disease

lower motor

40. True or False. In ALS, there is no cognitive, sensory, or autonomic dysfunction.

true

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41. True or False. ALS spares voluntary eye muscles and urinary sphincter.

true

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42. The common condition that must be distinguished from ALS is_____ _____.

cervical myelopathy

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43. In ALS, two causes of major disability include

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a. a_____

aspiration

b. s_____

spasticity

44. True or False. Guillain-Barré involves areflexia and progressive muscle weakness

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a. proximally

true (more severely)

b. distally

false

45. True or False. Guillain-Barré shows

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a. albuminocytologic dissociation

true (↑ prot > 55 mg/dL, < 10 cells)

b. little or no sensory involvement

true (but paresthesias are not uncommon)

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46. In Guillain-Barré, what infectious organism is involved?

Campylobacter jejuni

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47. True or False. In Guillain-Barré, there is progressive motor weakness that is relatively symmetric.

true

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48. Features casting doubt on the diagnosis

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a. asymmetry of_____

weakness

b. dysfunction of_____

bladder

c. more than 50_____ in CSF

monocytes

d. any_____ in CSF

PMNs

e. sharp_____ level

sensory

49. Complete the following about CIDP:

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a. True or False. CIDP is also known as chronic relapsing Guillain-Barré syndrome.

true

b. CIDP stands for c_____ i_____ d_____ p_____.

chronic immune

demyelinating

polyradituloneropathy

c. For CIDP, symptoms must be present for more than_____.

2 months

d. CSF findings are similar to_____ – _____.

Guillain-Barré

50. The Miller-Fisher variant of Guillain-Barré syndrome includes

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a. a_____

ataxia

b. a_____

areflexia

c. o_____

ophthalmoplegia

51. True or False. In Guillain-Barré, plasmapheresis hastens recovery and reduces residual deficit.

true

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52. True or False. In acute transverse myelitis (ATM), the animal model is EAE.

true

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53. Complete the following:

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a. EAE stands for_____ _____ _____.

experimental allergic

encephalomyelitis

b. It requires central or peripheral MBP?

central NS MBP (myelin basic protein, not peripheral MBP)

54. True or False. The most common sensory level in acute transverse myelitis is thoracic.

true (68% thoracic sensory level in ATM)

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55. True or False. Acute transverse myelitis progresses rapidly.

true (66% reach maximal deficit by 24 hours)

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56. True or False. In acute transverse myelitis, MRI/CT/myelography is often performed to rule out compressive lesions.

true (no characteristic imaging findings in ATM)

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57. True or False. In acute transverse myelitis, symptoms include

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a. paresthesia

true (paresthesia 100%)

b. weakness

true (weakness 97%)

c. sphincter disturbance

true (sphincter disturbance 94%— hesitancy, retention, overflow)

58. Characterize myelitis.

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a. Diagnose with_____.

MRI

b. If not available use_____.

myelogram

c. And treat with_____.

steroids

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59. True or False. Regarding acute transverse myelitis:

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a. There is 15% mortality.

true (15% mortality by 4 months)

b. 62% of survivors are ambulatory.

true (62% ambulatory by 3 to 6 months)

c. Recovery occurs between 1 month to 2 years.

false (1 to 3 months)

d. No improvement occurs after 3 months.

true

60. The most common neurologic

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a. manifestation is_____ _____.

diabetes insipidus

b. Treat with_____.

corticosteroids

61. Complete the following statements about neurosarcoidosis:

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a. Pathology characteristic: m_____ – e_____ of the s_____ a_____ of the t_____ v_____ and hy_____. May produce d_____ i_____.

meningo-encephalitis of the subependymal area of the third ventricle and hypothalamus diabetes insipidus

b. Serum test that is positive in 83% of cases is_____.

ACE

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c. Cerebrospinal fluid (CSF) test that is helpful is_____.

ACE

d. How frequently positive?

55%

e. CSF analysis suggests_____.

meningitis

f. ACE stands for_____ _____ _____.

angiotensin converting enzyme

62. Complete the following regarding neurosarcoidosis:

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a. Microscopically we see features of n_____g_____

non-caseating granulomas

b. Clinical findings include:

     i. c_____n_____palsies

cranial nerve

     ii. p_____n_____

peripheral neuropathy

     iii. m_____

myopathy

     iv. h_____

hydrocephalus

c. Diabetes insipidus from involvement of the_____

hypothalamus

63. List the test performed with the results in sarcoidosis.

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a. Chest x-ray

     i. h_____a_____

hilar adenopathy

     ii. m_____l_____n_____

mediastinal lymph nodes

b. MRI

     i. enhancement of l_____

leptomeninges

     ii. enhancement of o_____n_____

optic nerve flair

     iii. best seen on_____ sequence

flair

c. Gallium scan (nuclear medicine). Useful in neurosurgery for:

     i. s_____

sarcoidosis

     ii. c_____v_____o_____

chronic vertebral osteomyelitis

64. You have been called in consultation to see a 6 days post-partum woman complaining of headaches, seizures, and blindness. CT shows occipital intracerebral hemorrhage and bilateral vasogenic edema.

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a. Diagnosis: stands for p_____r_____e_____s_____

PRES, posterior reversible encephalopathy syndrome

b. Blood pressure will show_____

hypertension

c. True or False. Other causes beside pregnancy could be

     i. malignant hypertension

true

     ii. eclampsia

true

     iii. infection

true

     iv. auto-immune disease

true

     v. chemotherapy

true

     vi. transplantation

true

65. A patient develops blindness. Imaging studies reveal infarctions of both occipital lobes. You should consider

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a. the diagnosis of v_____d_____r_____e_____.

vascular dysauto regulatory encephalopathy

b. The blindness may be_____.

temporary

c. Treat with_____ _____ pressure control.

tight blood

d. Without control of BP_____-_____ _____ could occur

intra-cerebral hemorrhage (ICH)

e. In a non-pregnant patient this syndrome could occur due to a drug toxicity with_____.

cyclosporine

66. The treatment of vascular dysautoregulatory encephalopathy is to

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a. treat b_____p_____,

blood pressure

b. hold_____ _____, and

immune suppressives

c. remove the p_____.

placenta

67. Uremic encephalopathy

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a. has a site of characteristic edema in the b_____g_____.

basal ganglia

b. In severe cases, it can develop f_____ i_____.

focal infarcts

68. Crossed cerebellar diaschisis

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a. is h_____ of the cerebellum

hypometabolism

b. due to a

     i. c_____

contralateral

     ii. c_____h_____ lesion

cerebral hemisphere

c. Theory is that this occurs because the

     i. c_____-p_____-c_____ pathway

cerebro-ponto-cerebellar

     ii. becomes_____

disconnected

     iii. resulting in reduced: o_____and g_____consumption.

oxygen and glucose

     iv. Decreased_____production causes

CO₂

     v. local arterial_____

constriction

     vi. and reduced_____blood flow.

cerebellar