Neuology Usmle

Front 1

AN ACUTE NEUROLOGIC DYSFUNCTION OCCURRING AS A RESULT OF A VASCULAR PROCESS

Back 1

stroke

Front 2

Type of stroke?

focal ischemic necrosis of brain tissue due to occlusive vascular disease or other forms of vascular insufficiency

Back 2

cerebral infact or ischemic stroke

Front 3

Type of stroke?

BRAIN DAMAGE DUE TO SPONTANEOUS RUPTURE OF
BLOOD VESSEL

Back 3

HEMORRHAGIC STROKE

Front 4

cause of ischemic stroke

Back 4

occlusion of an artery, usually by:
1. THROMBOEMBOLUS
2. ATHEROSCLEROTIC PLAQUE (stenosis, sudden occlusion)
extracranial vs. intracranial vascular lesions

Front 5

type of hemmorage?

site: basal ganglia, thalamus, pons, deep
cerebellum

usual cause: hypertensive vascular disease

outcome: often lethal

Back 5

INTRAPARENCHYMAL HEMORRHAGES (IPH): ganglionic

Front 6

Site: cerebral lobes

usual cause: various (malformation, coagulopathy. etc.)

outcome: variable

Back 6

INTRAPARENCHYMAL HEMORRHAGES (IPH) : lobar

Front 7

site: base of brain,
convexities

usual cause: berry aneurysm, AVM

outcome: often lethal, acute
vasospasm, chronic
hydrocephalus

Back 7

SUBARACHNOID HEMORRHAGES (SAH)

Front 8

hippocampus: Sommer's sector (area CA1)

cerebral cortex: laminar necrosis watershed zones

cerebellum: Purkinje cells

Back 8

pattern of DIFFUSE HYPOXIC/ISCHEMIC ENCEPHALOPATHY

Front 9

– acceleration of large-vessel atherosclerosis

– arteriosclerosis, especially in basal ganglia, thalamus, pons,
cerebellum, deep white matter

– formation of Charcot-Bouchard aneurysms

– arteriolarsclerosis

Back 9

CHRONIC HYPERTENSIVE CEREBROVASCULAR PATHOLOGY

Front 10

– hyperplastic arteriolosclerosis ("onion skinning")

– necrotizing arteriolitis (fibrinoid necrosis)

Back 10

ACUTE EFFECTS OF SEVERE HYPERTENSION

Front 11

Hematoma?

source: bridging veins

clinical feature: acute subacute chronic

causes: trauma can be mild;
brain atrophy, coagulopathy

Back 11

subdural hematoma

Front 12

source: dural arteries

Hematoma?

clinical feature: LOC, then “silent (lucid)” interval

causes: skull fracture

Back 12

epidural hematoma

Front 13

type of Blunt Head injury?

clinical features:TRANSIENT LOC (level of conciousnes)

charactieristic: ? MILD DAD = DIFFUSE AXONAL INJURY/damage = axonal spheroids (swellings) form at the torn end of the axon in white matter tracts and appear as eosinophilic globules in H&E

Back 13

CONCUSSION

Front 14

type of Blunt Head injury?

clinical features:LOC +/- DEFICITS

charactieristic: INTACT PIA-ARACHNOID, FRONTAL/TEMPORAL POLES, LATERAL TEMPORAL

Back 14

CONTUSION

Front 15

AN ACUTE NEUROLOGIC DYSFUNCTION OCCURRING AS A RESULT OF A VASCULAR PROCESS

Back 15

stroke

Front 16

Type of stroke?

focal ischemic necrosis of brain tissue due to occlusive vascular disease or other forms of vascular insufficiency

Back 16

cerebral infact or ischemic stroke

Front 17

Type of stroke?

BRAIN DAMAGE DUE TO SPONTANEOUS RUPTURE OF
BLOOD VESSEL

Back 17

HEMORRHAGIC STROKE

Front 18

cause of ischemic stroke

Back 18

occlusion of an artery, usually by:
1. THROMBOEMBOLUS
2. ATHEROSCLEROTIC PLAQUE (stenosis, sudden occlusion)
extracranial vs. intracranial vascular lesions

Front 19

site: basal ganglia, thalamus, pons, deep
cerebellum

usual cause: hypertensive vascular disease

outcome: often lethal

Back 19

INTRAPARENCHYMAL HEMORRHAGES (IPH): ganglionic

Front 20

Site: cerebral lobes

usual cause: various (malformation, coagulopathy. etc.)

outcome: variable

Back 20

INTRAPARENCHYMAL HEMORRHAGES (IPH) : lobar

Front 21

site: base of brain,
convexities

usual cause: berry aneurysm, AVM

outcome: often lethal, acute
vasospasm, chronic
hydrocephalus

Back 21

SUBARACHNOID HEMORRHAGES (SAH)

Front 22

hippocampus: Sommer's sector (area CA1)

cerebral cortex: laminar necrosis watershed zones

cerebellum: Purkinje cells

Back 22

pattern of DIFFUSE HYPOXIC/ISCHEMIC ENCEPHALOPATHY

Front 23

AN ACUTE NEUROLOGIC DYSFUNCTION OCCURRING AS A RESULT OF A VASCULAR PROCESS

Back 23

stroke

Front 24

– acceleration of large-vessel atherosclerosis

– arteriosclerosis, especially in basal ganglia, thalamus, pons,
cerebellum, deep white matter

– formation of Charcot-Bouchard aneurysms

– arteriolarsclerosis

Back 24

CHRONIC HYPERTENSIVE CEREBROVASCULAR PATHOLOGY

Front 25

Type of stroke?

focal ischemic necrosis of brain tissue due to occlusive vascular disease or other forms of vascular insufficiency

Back 25

cerebral infact or ischemic stroke

Front 26

– hyperplastic arteriolosclerosis ("onion skinning")

– necrotizing arteriolitis (fibrinoid necrosis)

Back 26

ACUTE EFFECTS OF SEVERE HYPERTENSION

Front 27

Type of stroke?

BRAIN DAMAGE DUE TO SPONTANEOUS RUPTURE OF
BLOOD VESSEL

Back 27

HEMORRHAGIC STROKE

Front 28

Hematoma?

source: bridging veins

clinical feature: acute subacute chronic

causes: trauma can be mild;
brain atrophy, coagulopathy

Back 28

subdural hematoma

Front 29

source: dural arteries

Hematoma?

clinical feature: LOC, then “silent (lucid)” interval

causes: skull fracture

Back 29

epidural hematoma

Front 30

cause of ischemic stroke

Back 30

occlusion of an artery, usually by:
1. THROMBOEMBOLUS
2. ATHEROSCLEROTIC PLAQUE (stenosis, sudden occlusion)
extracranial vs. intracranial vascular lesions

Front 31

type of Blunt Head injury?

clinical features:TRANSIENT LOC

charactieristic: ? MILD DAD = DIFFUSE AXONAL INJURY = axonal spheroids (swellings) form at the torn end of the axon in white matter tracts and appear as eosinophilic globules in H&E

Back 31

CONCUSSION

Front 32

site: basal ganglia, thalamus, pons, deep
cerebellum

usual cause: hypertensive vascular disease

outcome: often lethal

Back 32

INTRAPARENCHYMAL HEMORRHAGES (IPH): ganglionic

Front 33

type of Blunt Head injury?

clinical features:LOC +/- DEFICITS

charactieristic: INTACT PIA-ARACHNOID, FRONTAL/TEMPORAL POLES, LATERAL TEMPORAL

Back 33

CONTUSION

Front 34

type of hemmorage?

Site: cerebral lobes

usual cause: various (malformation, coagulopathy. etc.)

outcome: variable

Back 34

INTRAPARENCHYMAL HEMORRHAGES (IPH) : lobar

Front 35

type of hemmorage?

site: base of brain,
convexities

usual cause: berry aneurysm, AVM

outcome: often lethal, acute
vasospasm, chronic
hydrocephalus

Back 35

SUBARACHNOID HEMORRHAGES (SAH)

Front 36

type of Blunt Head injury?

clinical features:LOC + DEFICITS

charactieristic: TORN PIA-ARACHNOID

Back 36

LACERATION

Front 37

hippocampus: Sommer's sector (area CA1)

cerebral cortex: laminar necrosis watershed zones

cerebellum: Purkinje cells

Back 37

pattern of DIFFUSE HYPOXIC/ISCHEMIC ENCEPHALOPATHY

Front 38

– acceleration of large-vessel atherosclerosis

– arteriosclerosis, especially in basal ganglia, thalamus, pons,
cerebellum, deep white matter

– formation of Charcot-Bouchard aneurysms

– arteriolarsclerosis

Back 38

CHRONIC HYPERTENSIVE CEREBROVASCULAR PATHOLOGY

Front 39

– hyperplastic arteriolosclerosis ("onion skinning")

– necrotizing arteriolitis (fibrinoid necrosis)

Back 39

ACUTE EFFECTS OF SEVERE HYPERTENSION

Front 40

Hematoma?

source: bridging veins

clinical feature: acute subacute chronic

causes: trauma can be mild;
brain atrophy, coagulopathy

Back 40

subdural hematoma

Front 41

source: dural arteries

Hematoma?

clinical feature: LOC, then “silent (lucid)” interval

causes: skull fracture

Back 41

epidural hematoma

Front 42

type of Blunt Head injury?

clinical features:TRANSIENT LOC

charactieristic: ? MILD DAD = DIFFUSE AXONAL INJURY = axonal spheroids (swellings) form at the torn end of the axon in white matter tracts and appear as eosinophilic globules in H&E

Back 42

CONCUSSION

Front 43

type of Blunt Head injury?

clinical features:LOC +/- DEFICITS

charactieristic: INTACT PIA-ARACHNOID, FRONTAL/TEMPORAL POLES, LATERAL TEMPORAL

Back 43

CONTUSION

Front 44

type of Blunt Head injury?

clinical features:LOC + DEFICITS

charactieristic: TORN PIA-ARACHNOID

Back 44

LACERATION

Front 45

type of blunt head injury?

clinical feature: PROLONGED LOC

characteristic: TEARING OF AXONS (SPHEROIDS) IN CORPUS
CALLOSUM, LONG TRACTS

Back 45

Diffuse axonal damage

Front 46

PENETRATION OF BRAIN OR SPINAL CORD BY PROJECTILE OR OTHER MOVING OBJECT
(bullet, knife blade, etc.)

AMOUNT OF DAMAGE DEPENDS ON MASS, SHAPE, AND VELOCITY OF MISSILE

Back 46

MISSILE HEAD INJURY

Front 47

type of MISSILE HEAD INJURY?

missile produces depressed fracture but does not enter skull

Back 47

DEPRESSED

Front 48

type of MISSILE HEAD INJURY?

missile enters cranial cavity

Back 48

PENETRATING

Front 49

type of MISSILE HEAD INJURY?

missile enters and exits cranial cavity

Back 49

PERFORATING

Front 50

type of stroke?

obstruction of a feeding blood vessel or a significant reduction in blood flow

Back 50

Ischemic

Front 51

type of stroke?

rupture of a blood vessel into or around the brain

Back 51

Hemorrhagic

Front 52

• Sudden onset (usually maximum within 10
seconds)

• Weakness of one side of the body / face

• Numbness / tingling of one side of the body or face

• Incoordination / clumsiness of one side of the body,
falling to one side, spinning sensation

• Sudden change in speech or language

• Loss of vision, especially in one eye on or one side

• Double vision

• Acute confusion

• Sudden, severe headache

Back 52

8 signs and symptoms of stroke

Front 53

• Evaluation and stabilization of patient
– Vitals, cardiovascular, neurological
assessment, O2 NC, HLIV x2

• Diagnostic work-up begun
– Labs, brain imaging (CT, MRI)

• Consider “clot-buster”: t-PA therapy (call Stroke
Team)

**must tx pt with in 60 min arrive

Back 53

acute stroke management

Front 54

– High blood pressure
(hypertension)

– "High sugar" (diabetes)

– Heart disease, heart
attacks, irregular
heart beats

– High cholesterol

– Smoking, alcohol use,
illegal drugs

– No regular physical
exercise

– Family history of stroke

– Previous stroke

– Sickle-cell disease

Back 54

focused stroke history, 11 risk factors

Front 55

– Change or loss of vision (one eye? both eyes?)

– Numbness, tingling, weakness, clumsiness,
heaviness of one side of the body

– Change or loss of language (understanding,
comprehension, reading, writing)

– Change or loss of steady walking, imbalance,
falling

– Headache (location, type, severity, duration)

Back 55

5 focal symptoms for focused stroke history

Front 56

– Level of consciousness (alert, lethargic / drowsy,
stupor, coma)

– Language (comprehension, fluency, repetition,
naming, reading, writing)

– Visual fields and eye movements (other CN)

– Strength (face, arms, legs, drift) and reflexes

– Coordination (gait, finger to nose, heel to shin)

– Sensation (pinprick, temperature, joint position
sense / vibration)

Back 56

focused neurological findings in focused stroke history and physical

Front 57

• Inclusion criteria
– Age ≥ 18
– Ischemic stroke with measurable deficit
– < 3 hr since symptom onset (time of onset
is critical)

• Clock starts when patient last seen normal

• Many exclusions, but patients meeting the
above criteria should be considered for thrombolytic treatment

Back 57

t-PA criteria

Front 58

These are complications for what neurologic condition?

• Seizures
• Cardiovascular (MI, arrhythmia, sudden death)
• Respiratory (aspiration pneumonia, DVT / PE)
• Endocrine (hyperglycemia, hyponatremia)
• Urinary tract infection
• Decubitus ulcers
• Psychological / Psychiatric
– Depression (40%)
– Confusion / combativeness / hallucinations
– Abulia

Back 58

stroke

Front 59

• Symptomatic: NNT = 6
• Asymptomatic: NNT = 100
• 90% > 80% > 70% > 60% stenosis
• Benefit regardless of age, but older > younger
• Ulcerated plaque
• Hemispheric TIA / stroke (vs. retinal)
• Men> women
• Associated stroke risk factors
– Hypertension, diabetes, tobacco, lipids

Back 59

Carotid Endarterectomy

Front 60

• Increasingly used, with only one RCT showing
efficacy / equivalence to CEA (3 stopped early)
• Multiple trials underway (carotid disease)
• FDA approved only for high-risk patients with
symptomatic high-grade stenosis
• With few exceptions, vertebrobasilar and
intracranial stenting should be limited to RCTs
• As with CEA, ask about complication rates

Back 60

Carotid Angioplasty and Stenting

Front 61

txmt of choice for stroke

Back 61

aspirin

Front 62

what agents are used to dec ldl and overall risk of stroke?

Back 62

HMG-CoA reductase inhibitors: statins

Front 63

stage of neuro development?

gest age: 3-4 wk

ex of disorder: Spina bifida

Back 63

Neural tube closure (dorsal induction)

Front 64

stage of neuro development?

gest age: 5-6 wk

ex of disorder: Holoprosencephalies

Back 64

Ventral induction

Front 65

stage of neuro development?

gest age: 8-16 weeks (2 to 4 months)

ex of disorder: Microcephaly or macroencephaly

Back 65

Neuronal proliferation

Front 66

stage of neuro development?

gest age:10-15 weeks (3 to 5 months)

ex of disorder: (agyria - smooth cortex) lissencephaly/heterotopia (aberrant gyration)

Back 66

Neuronal migration

Front 67

stage of neuro development?

gest age: 6 mo-yrs post natal

ex of disorder: Mental retardation

Back 67

Synaptic organization And differentiation

Front 68

stage of neuro development?

gest age: 7 mo-yrs post natal

ex of disorder: leukodystrophies

Back 68

Myelination

Front 69

when do the following cns malformations occur?

INITIAL OCCURRENCE OF MALFORMATIONS

Back 69

EMBRYONIC PERIOD

Front 70

when do the following cns malformations occur?

-CLINICAL RECOGNITION OF MALFORMATIONS
FUNCTIONAL/BEHAVIORAL

-CONSEQUENCES OF
INTRAUTERINE OR PERINATAL DISEASE

-BECOME APPARENT
CONTINUED EFFECTS ON LATER GROWTH & DEVELOPMENT

Back 70

POSTNATAL PERIOD

Front 71

when do the following cns malformations occur?

-DISTURBANCE OF SUBSEQUENT DEVELOPMENTAL EVENTS

-ABNORMALITIES OF GROWTH

-DEFORMATIONS

-DISRUPTIONS

Back 71

FETAL PERIOD

Front 72

NEURONS CAN ARISE FROM:

-Ependymal zone

-Subependymal zone (germinal matrix)

Back 72

Neuronal proliferation

2-4 mo

Front 73

age of neuro development?

Back 73

physial exam at birth

Front 74

age of neuro development?

Back 74

development at 2 mo

Front 75

age of neuro development?

Back 75

development at 6 mo

Front 76

age of neuro development?

Back 76

development at 10 mo

Front 77

age of neuro development?

Back 77

development at 1 year

Front 78

age of neuro development?

Back 78

development at 2 years

Front 79

reflex?

onset: birth

disapperance: 3 mo

Back 79

rooting

Front 80

reflex?

onset: birth

disapperance: 5 to 6 mo

Back 80

moro

Front 81

reflex?

onset: birth

disapperance: 6 mo

(hand)

Back 81

palmar grasp

Front 82

reflex?

onset: birth

disapperance: 9 - 10 mo

Back 82

planter grasp

Front 83

reflex?

onset: birth

disapperance: 1 to 2 mo

Back 83

galant (truncal incurvation)

Front 84

reflex?
onset: birth

disapperance: 6 mo
(neck)

Back 84

tonic neck

Front 85

reflex?

onset: 3 - 5 mo

disapperance: 2 yr

Back 85

landau

Front 86

reflex?

onset: 6 - 9 mo

disapperance: persists

Back 86

parachute

Front 87

A disturbance in the acquisition of cognitive,
motor, language or social skills which has a
significant and continuing impact on the developmental progress of a child before 5 or 6 yo

Back 87

global developmental delay

Front 88

A disturbance in the acquisition of cognitive,
motor, language or social skills which has a
significant and continuing impact on the developmental progress of a child after 5 or 6 yo

Back 88

mental retardation

Front 89

Disordered motor function evident in early
infancy with changes in muscle tone, involuntary
movements, ataxia or a combination

Back 89

cerebral palsy

Front 90

type of cp?

-Hemiplegic

-Quadriplegic

-Diplegic

Back 90

spastic

Front 91

type of cp?

-Choreoathetotic

-Dystonic

-Atonic/hypotonic (rare)

-Ataxic

-Mixed

Back 91

Extrapyramidal

Front 92

type of neuro disorder in child?

-developmental speech delay

-congenital deafness or word deafness or word blindness

-stuttering and stammering

Back 92

Disorders of speech and language

Front 93

what is this condition?

Back 93

intraventricular hemmorage in premature infants

Front 94

chromosomal abn?

-Most common and widely recognized autosomal trisomy

-1 in 600 live births

-Increasing frequency with advanced maternal age

-Mental retardation, hypotonia at birth, microcephaly

-Increased incidence of Alzheimer disease with increasing age

Back 94

TRISOMY 21 (Down syndrome)

Front 95

chromosomal abn?

-Most common cause of mental retardation in males

-1 in 2000-3000 live births

-Affects both males and females

-CGG triplet of bases repeated > 200 times

-Features include motor and language delay, shyness, aboveaverage
height and head circumference, dysmorphisms

Back 95

FRAGILE X SYNDROME

Front 96

chromosomal abn?

Back 96

Prader Willi syndrome

Front 97

chromosomal abn?

Back 97

Angelman's syndrome

Front 98

chromosomal abn?

Back 98

Rett syndrome

Front 99

neurocutaneous disorder?

def: autosomal dominant disorder caused by
TSC gene mutation (chromosome 9: TSC1, hamartin;
chromosome 16: TSC2, tuberin)

Back 99

tuberous sclerosis

Front 100

neurocutanous syndrome?

Back 100

neurofibromatosis

Front 101

neurocutaneous syndrome?

Back 101

Sturge Weber Syndrome

Front 102

type of inborn error of metabolism?

Back 102

mucopolysaccharidosis (mps)

Front 103

type of inborn error of metabolism?

Sphingolipid pathway eg. Metachromatic
leukodystrophy

Back 103

lysosomal storage diseases

Front 104

type of inborn error of metabolism?

Back 104

pompe's disease

Front 105

TORCH group of infections can cause brain malformations, hydrocephalus, blindness, hearing deficits and multiple organ involvement

Back 105

intrauterine infections

Back 106

fetal alcohol syndrome

Front 107

what disease has the following clinical features?

Back 107

fetal alcohol syndrome

Front 108

parasagittal, ulegyria (selective loss of cortex in depth of sulci),
thalamus, BG and brainstem

Back 108

areas that are senstive to hypoxic ischemic encephalopathy in a term infant

Front 109

periventricular infarction

Back 109

areas that are senstive to hypoxic ischemic encephalopathy in a pre-term infant

Back 110

lab manifestations commonly associated with metabolic diseases

Back 111

developmental language disorders

Front 112

AN INFILTRATIVE NEOPLASM OF ASTROCYTES OF VARIABLE MALIGNANCY MOST COMMONLY ARISING IN ADULTS

Back 112

Diffuse astrocytomas

Front 113

orgin of these tummors?

-gliomas (astrocytoma, oligodendroglioma, ependymoma,
glioblastoma multiforme, choroid plexus tumor, others)

-primitive neuroectodermal tumors (PNET) (medulloblastoma,
others)

Back 113

Tumors of neuroectoderm

Front 114

orgin of these tummors?

tumors of brain coverings (meningiomas)
tumors of nerve roots/peripheral nerves (Schwannomas,
neurofibromas, others)

Back 114

Mesenchymal/neural crest

Front 115

brain tumor type?

TUMOR NOMENCLATURE:
-astrocytoma (WHO grade 2)

-anaplastic astrocytoma (WHO grade 3)

-glioblastoma multiforme (WHO grade 4)

CHARACTERISTICS:

-cells infiltrate brain

-spectrum of anaplasia/malignancy

-progress in malignancy over time/age

Back 115

diffuse astrocytomas

Front 116

where does glioblastoma multiforme arise in adult man?

Back 116

basal ganglia

Front 117

what are 2 histological characteristics seen in glioblastoma multiforme?

Back 117

anaplasia and necrosis with pseudoplasisading

Front 118

resulting brain tummor?

genetic event:
-p53 mutation/17p
-PDGF overexpression
-loss of 22q

path event:
-increased proliferation
-decreased apoptosis

Back 118

astrocytoma

Front 119

type of cp?

-Hemiplegic

-Quadriplegic

-Diplegic

Back 119

spastic

Front 120

type of cp?

-Choreoathetotic

-Dystonic

-Atonic/hypotonic (rare)

-Ataxic

-Mixed

Back 120

Extrapyramidal

Front 121

type of neuro disorder in child?

-developmental speech delay

-congenital deafness or word deafness or word blindness

-stuttering and stammering

Back 121

Disorders of speech and language

Front 122

what is this condition?

Back 122

intraventricular hemmorage in premature infants

Front 123

chromosomal abn?

-Most common and widely recognized autosomal trisomy

-1 in 600 live births

-Increasing frequency with advanced maternal age

-Mental retardation, hypotonia at birth, microcephaly

-Increased incidence of Alzheimer disease with increasing age

Back 123

TRISOMY 21 (Down syndrome)

Front 124

chromosomal abn?

-Most common cause of mental retardation in males

-1 in 2000-3000 live births

-Affects both males and females

-CGG triplet of bases repeated > 200 times

-Features include motor and language delay, shyness, aboveaverage
height and head circumference, dysmorphisms

Back 124

FRAGILE X SYNDROME

Front 125

chromosomal abn?

Back 125

Prader Willi syndrome

Front 126

chromosomal abn?

Back 126

Angelman's syndrome

Front 127

chromosomal abn?

Back 127

Rett syndrome

Front 128

neurocutaneous disorder?

def: autosomal dominant disorder caused by
TSC gene mutation (chromosome 9: TSC1, hamartin;
chromosome 16: TSC2, tuberin)

Back 128

tuberous sclerosis

Front 129

neurocutanous syndrome?

Back 129

neurofibromatosis

Front 130

neurocutaneous syndrome?

Back 130

Sturge Weber Syndrome

Front 131

type of inborn error of metabolism?

Back 131

mucopolysaccharidosis (mps)

Front 132

type of inborn error of metabolism?

Sphingolipid pathway eg. Metachromatic
leukodystrophy

Back 132

lysosomal storage diseases

Front 133

type of inborn error of metabolism?

Back 133

pompe's disease

Front 134

what brain tumor do these events lead to?

genetic event?
inactivation of various suppressor genes (9p,11p,13q,19q)

-CDK4 amplification

path event?
-loss of cell cycle control

Back 134

anaplastic astrocytoma

Front 135

what brain tumor do these events lead to?

genetic event?
-loss of chr 10
-EGFR amplification/
rearrangement
-P16/RB/CDK4 alteration

path event?
-necrosis
-angiogenesis
-clonal selection

Back 135

globlastoma

Front 136

where are the following tumors located and what age group are they associated with?

-astrocytomas / glioblatomas

-metastatic carcinomas

-menigiomas

-lymphomas

-pituitary adenomas

Back 136

location: cerebrum

age group : adults

Front 137

where are the following tumors located and what age group are they associated with?

-metastaic carcinomas

-menigiomas

-schwannomas (cn VIII)

Back 137

location: posterior fosssa

age group: adults

Front 138

where are the following tumors located and what age group are they associated with?

-PNET

-ependymonas

-choroid plexus tumors

-optic nerve gliomas

-germ cell tumors

-craniopharyngiomas

Back 138

location: cerebrum

age group: children

Front 139

where are the following tumors located and what age group are they associated with?

-PNET (cerebellar medulloblastoma)

-pilocytic astrocytomas

-ependymomas

-brainstem gliomas

Back 139

location: posterior fossa

age group: children

Front 140

type of brain tumor?

A SLOWLY GROWING NEOPLASM OF ASTROCYTES TYPICALLY ARISING IN THE CEREBELLUM OF CHILDREN

Back 140

pilocytic astrocytoma, who grade 1

Front 141

what are the locations for pilocytic astrocytoma, who grade 1 in children?

Back 141

cerebellum, optic nerve,
hypothalamus

Front 142

what are the locations for pilocytic astrocytoma, who grade 1 in adults?

Back 142

adults: less common but usually occur in
cerebrum, spinal cord

Front 143

what brain tummor has the following features?

-mostly solid growth pattern, often cystic

-slowly growing

-indolent clinical behavior

-do not progress in grade over time

Back 143

pilocytic astrocytoma, who grade 1

Front 144

tumor composed of cells resembling oligodendrocytes

Back 144

OLIGODENDROGLIOMA, WHO GRADE 2

Front 145

location of OLIGODENDROGLIOMA, WHO GRADE 2

Back 145

cerebral hemispheres

Front 146

what brain tumor has the following features?

-often slow-growing

-infiltrative or solid

-often contain calcifications

-often associated with epilepsy

-may have anaplastic form (WHO grade 3)

Back 146

OLIGODENDROGLIOMA, WHO GRADE 2

Front 147

NEOPLASMS DERIVED FROM VENTRICULAR LINING CELLS

Back 147

ependymomas adn choroid plexus tumors

Front 148

what brain tumor presents as follows?

MASS EFFECT, OBSTRUCTIVE HYDROCEPHALUS

Back 148

ependymomas adn choroid plexus tumors

Front 149

for ependymomas (grade 2 or 3), what is the location for:

1. children

2. adults

Back 149

1. lateral and fourth ventricles

2. fourth ventricle, spinal cord

Front 150

for papillomas(grade 1), what is the location for:

1. children

2. adults

Back 150

1. lateral or fourth ventricles

2. fourth ventricle

Front 151

fxn: Low grade (2) glial tumor
More common in cerebral hemisphere in adults
Hits the brainstem of kids

special:Infiltrative
Progress to malignancy increases with age.
5 year survival rate
Astrocytomas can be stained with GFAP

Back 151

Astrocytoma

Front 152

fxn:High grade glial tumor
They are more invasive and divide more rapidly than astrocytomas

special: Necrosis with
Pseudopalisading
Survival is less than 1 year

Most common primary brain tumor.

Back 152

Glioblastoma

Front 153

fxn: Grade 1
Slow growing, benign glial tumor of kids Does not progress to a later grade

special: Optic Nerve, cerebellum and hypothamus are affected

Back 153

Pilocytic Astrocytoma

Front 154

fxn: Grade 2
Tumor of Oligodendrocytes
Affects Adults only

Milk and Eggs-fried egg appearance with calcifications.

special:
-Calcifications

-Epilepsy

-Rare and slow growing

Back 154

Oligodendroglioma

Front 155

fxn: Neoplasm derived from lining of the ventricles.
Low grade (II)

Kids-lateral and 4th ventricles

special:
May lead to hydrocephalus and has a poor prognosis.

Characteristic perivascular pseudorosettes.


Adults-4th and spinal cord

Back 155

Ependymoma

Front 156

fxn:
Papillomas are the benign form

Carcinomas are the malignant form

Tumors mimic normal choroid and may overproduce CSF.

special:
Papillomas in kids-lateral and 4th
Adults-4th

Carcinomas are only in kids.
Lateral ventricle.

Rosettes

Back 156

Choroid Plexus tumors

Front 157

fxn:
-Embryonic Tumor
-Retinoblastoma

-Medulloblastoma-affects cerebellum.

PNET-Cerebral Hemispheres

special:
These tumors are highly metastatic

May disseminate thru the CSF

Often associated with gentic defects

Rosettes

Back 157

Embryonal Tumors
Primitive Neuroectodermal Tumors

Front 158

fxn:
Most common Embryonic tumor.

Arises in the cerebellum

Dissemination into the subarachnoid space is common.

special:
These are very fast growing tumors, folks.

Five year survival rate is 50%.

Symptoms typically result from blockage of the 4th ventricle which leads to hydrocephalus.

Back 158

Medulloblastoma

Front 159

fxn:
Non neuroectodermal tumor
Second most common brain tumor

Adults:
-Arises from arachnoid cells

-Benign but mass effect causes troubles.

Special:
Resectable

-Psammoma Bodies-calcifications commonly found in meningiomas

Back 159

Meningioma

Front 160

fxn:
Arises from Schwann Cell

-Often localized to the 8th nerve, causing an acoustic schwannoma-leads to unilateral hearing loss and vertigo

Adults

special:
Bilateral form found in Neurofibromatosis 2

Back 160

Schwannoma

Front 161

Damage to the brain due the bulk of a tumor, the blockage of fluid, or excess accumulation of fluid within the skull. Mass effect often presents as seizures and dementia.

Back 161

Mass effect

Front 162

most likely brain tumor in children

Back 162

cerebellar tumors of the posterior fossa

Front 163

most likely brain tumors in adults

Back 163

Adults are more likely to get tumors of the cerebral hemispheres. These include astrocytomas, meningiomas and metastases

Front 164

Low grade, may cause endocrine dysfunction.
Derived from Rathkes pouch

Back 164

Pituitary Adenoma - a sellar tumor

Front 165

Low-grade but locally aggressive epithelial tumor
Derived from Rathke’s pouch

Back 165

Craniopharyngioma

Front 166

~ 20-30% of all brain tumors, with greater incidence in older people, and about 85% are multiple within the CNS.

~Sources: lung, breast, malignant melanoma, kidney, GI tract, others

~Path: solid or cystic circumscribed mass with central necrosis and prominent vasogenic edema

Back 166

Secondary Tumors – Metastatic cancer

Front 167

Diffuse tumor cell spread through the CSF pathways, often associated with high CSF protein, hydrocephalus, involvement of multiple cranial and spinal nerve roots

Back 167

Leptomeningeal carcinomatosis

Front 168

Acute infection in Subarachnoid space caused by virulent bac, fungi, some protozoans

Back 168

Acute Purulent Leptomeningitis
(pyogenic, bacterial)

Front 169

Acute inflam in SAS: examine CSF
-↑ pressure (200-500 mmH2O), ↑ protein (>50mg/dl), ↓ glucose (often<40mg/dl)
leukoctosis (mostly PMNs)
-organism may be detectable by Gram stain, culture

-spectrum of organisms varies w/age & clinical circumstances, commonly includes:
bac: strep, staph, hemophilus, neisseria, gram (–) rods, listeria, anaerobes
fungi: immunosuppresed: aspergillus, candida, mucor
environmental exposure: coccidoides, histoplasma
other: toxoplasma, ameba

pathogenic sequence: 10 colonization/infection in body

dissemination to CNS (usu hematogenous)
infect meninges
acute inflammatory response in SAS
brain swelling, damage, disseminationoutcome

-inflam/overall response to infection may be modified by pt’s immune status, virulence of organism, effects of tx

Back 169

Acute Purulent Leptomeningitis
(pyogenic, bacterial)

Front 170

cns infection?

Systemic: fever, n/v, irritability, lethargy

Meningismus: nuchal rigidity, Brudzinski sign (passive flexion neckflexion hip, knee), Kernig sign (starting from flexed knee/thigh, extension of knee resisted)

Direct CNS involvement: heachache, photophobia, altered consciousness, seizures, focal localizing signs

Back 170

Acute Purulent Leptomeningitis
(pyogenic, bacterial)

Front 171

cns infection?

CSF findings: ↑ protein (mild), normal glucose, leukocytosis (very early: few PMNs,

later: mostly lymphocytes)

-viral: common, includes common viruses causing URI or upper GI infections

-some less virulent bacteria/other agents

-non-infectious agents (cancer cells=leptomeningeal carcinomatosis,
chemicals/drugs=chemical meningitis) can produce similar syndrome

Back 171

Acute Lymphocytic Meningitis
(viral, “aseptic”)

Front 172

Acute, usually self-limiting viral infection in SAS, characterized by lymphocytic inflammation

Back 172

Acute Lymphocytic Meningitis
(viral, “aseptic”)

Front 173

cns infection?

Fever, headache, signs of meningeal irritation, lethary, rash (milder than acute purulent leptomeningitis, and usu does not significantly alter consciousness)

Back 173

Acute Lymphocytic Meningitis
(viral, “aseptic”)

Front 174

cns infection?

CSF findings:

-↑ pressure or CSF blockage, ↑ protein (100-200mg/dl, higher w/CSF blockage), ↓ glucose (less than in acute purulent), leukoctosis (lymphocytes, monocytes)

-agent (organism, tumor cell) may be detectable

-commonly “basal meningitis” w/mononuclear inflammation, fibrosis, +/- granulomas (depends on agent), most prominent at base of brain

-chronic inflammation in SASprogressive meningeal, fibrosis, vasculitis, root/parenchymal involvementhydrocephalus (non-obstructive), ↑ ICP, infarcts, focal deficits, cognitive decline

Back 174

Chronic Meningitis

Front 175

Chronic inflammation in leptomeninges due to relatively indolent, persistent agent (TB, syphilis, sarcoidosis, some low-grade tumors, foreign substances)

Back 175

Chronic Meningitis

Front 176

cns infection?

-often non-specific/non-localizing, slowly evolving (+/- heachache, +/- stiff neck, low grade fever, seizures, cognitive dysfunction)

Back 176

Chronic Meningitis

Front 177

cns infection?

-predisposing factors: infection elsewhere (osteomyelitis, dental, sinusitis, otitis media, endocarditis, congenital heart disease), trauma skull/spine (including surgery), foreign body (catheter, gauze, bullet)
-bacteria, fungi, others

-localized area of tissue damage & acute/chronic inflam surrounded by reactive layer; brain parenchyma may show prominent edema

Complications:
-mass effect(↑ ICP, herniation
-abscess rupture into ventricles or disseminate in SAS as acute purulent leptomen.
-infection spread to other organs or systemic (sepsis)
-focal permanent neurologic deficits

Back 177

Empyema & abscesses

1. subdural/epidural empyema
2. brain parenchymal abscess
(“cerebritis”)

Front 178

Acute/chronic localized purulent or necrotizing infections due to destructive bacteria, fungi, or other organisms

Back 178

Empyema & abscesses

1. subdural/epidural empyema
2. brain parenchymal abscess
(“cerebritis”)

Front 179

cns infection?

Infec/inflam: Fever, malaise

Mass effects: headache, ↑ICP

Localizing signs, if brain parenchyma is involved: ex. seizures, focal deficits

Back 179

Empyema & abscesses

1. subdural/epidural empyema
2. brain parenchymal abscess
(“cerebritis”)

Front 180

cns infection?

Acute viral infection of brain parenchyma +/- meninges

Back 180

Acute Viral Encephalitis

Front 181

cns infection?

agent: HSV 1

site of involvement: -medial temporal lobes (Cowdry type A nuclear viral inclusions)

Back 181

Acute (meningo)encephalitis

Acute necrotizing

Front 182

cns infection?

Progressive viral infection, w/prolonged incubation following initial exposure, insidious onset, slow progression of sx, due to conventional viral agents

*prions excluded here



Sx: slowly progressive dementia or loss of particular neurologic functions

Back 182

Chronic (slow) viral encephalitis

Front 183

1) Acute Lymphocytic meningitis – often during 10 infection

2)Chronic HIV encephalitis w/ progressive dementia (AIDS dementia)

3)Vacuolar myelopathy – uncommon degeneration of posterior & lateral columns in spinal cord in chronic AIDS pt, resembles subacute combined degeneration seen in vitamin B12 deficiency

4)peripheral neuropathy (various types)

5)inflammatory myopathy (polymyositis-like)

Back 183

HIV: syndromes

Front 184

cns infection?

agent: CMV

site of involvement:
-periventricular tissues (cytomegalic cells); occurs as congenital infection (TORCH) & immunocompromised host

Back 184

Acute (meningo)encephalitis

Acute necrotizing

Front 185

cns infection?

agent?
HIV

involved site: brain atrophy, demyelination, encephalitis w/monocytes & giant cells

Back 185

AIDS Dementia

Front 186

cns infection?

agent:
papovirus JC strain)

involved site:
infection, destruction oligodendrocytes -> progressive demyelination, usu in immunocompromised host

Back 186

PML(progressive multifocal leukoencephalopathy)

Front 187

the removal, treatment, and return of (components of) blood plasma from blood circulation.

-separates things based on molecular weight.

Back 187

Plasmapheresis

Front 188

autoimmune cns disease?

auto-antibodies attack AchR

Weakness and Muscle Fatigue

Distribution of affected muscles is variable.

**Eye muscles are the 1st thing involved.

Reflexes and sensation is unaffected

Back 188

Myasthenia Gravis

Front 189

Lab Tests for MG

Back 189

Anti AchR antibody is 85-90% positive.

Front 190

can induce Myasthenia and antibodies to AchR

Back 190

Thymoma and Penicillamine can induce Myasthenia and antibodies to AchR.

FYI: A thymoma is a tumor of the thymus gland that leads to an autoimmune response.

Front 191

Treatment Myasthenia Gravis

Back 191

 Plasmapheresis

 Pyridostigmine blocks Ach Esterase which makes more Ach available.

 Thymectomy

Front 192

Autoantibodies against the presynaptic voltage-gated calcium channels

-caused by circulating antibodies to Voltage gated Calcium Channels which block Ach release.

Clinical Features:

• Weakness and fatigability

• Autonomic Dysfunction-sexual dysfunction, dry mouth,

• Reflexes are depressed

• 60% of cases are associated with small cell lung cancer

Back 192

Lambert-Eaton Syndrome

Front 193

Lab Tests for Lambert Eaton

Back 193

Antibody to Voltage Gated Calcium Channels

Front 194

txmt for Lambert-Eaton Syndrome

Back 194

Search for and treat the tumor if it’s present (often paraneoplastic syndrome of small cell lung cancer)

Pyridostigmine works, but is less effective than in Myasthenia gravis (MG).

Plasmaphereisis works well too.

Front 195

cns autoimmune disease?

-degenerations are disorders of the cerebellum, the part of the brain responsible for coordination, which are associated with tumors (neoplasms).

-They arise when tumors express proteins that are normally found only in neurons, and it is believed that the immune system, in its attempt to kill the tumor, also damages the cerebellum.

-current thought is that "killer T-cells", or cytotoxic CD8+ T lymphocytes, are the most likely mediator of neuronal injury.

Back 195

Paraneoplastic Syndromes

Front 196

Examination of patients with anti-Purkinje cell antibodies, especially anti-Yo, is usually dominated by a rapidly progressive unsteadiness, and downbeating nystagmus.

Back 196

Paraneoplastic Syndromes

Front 197

rare complication of cancer characterised by chaotic, synchronous eye movements (opsoclonus), spontaneous muscle jerks (myoclonus), and ataxia.

Back 197

Paraneoplastic opsoclonus-myoclonus syndrome (OMS)

Front 198

autoimmune disease in cns?

almost exclusively associated with neuroblastoma

Back 198

children OMS

Paraneoplastic opsoclonus-myoclonus syndrome (OMS)

Front 199

autoimmune disease in cns?

associated with small cell lung cancer (SCLC) and breast cancer are the most frequent tumors associated

It’s also known as dancing eye, dancing feet syndrome.

Back 199

adult OMS

Paraneoplastic opsoclonus-myoclonus syndrome (OMS)

Front 200

• Difficulty in finding works (dysnomia)

• Impaired visuospatial memory (misplace things ,not finding their way home)

• Impaired recent memory.

• Delusion ,insomnia, depression

Back 200

early sx of alzheimer's disease

Front 201

• Increasingly impaired remote memory

• Non confluent speech

• Agitation, aggression

Back 201

later sx of alzheimer's disease

Front 202

• Recent and remote memory obliterated

• Mute

• Severe rigidity

Back 202

final stage of alzheimer's disease

Front 203

What disease has the following MRI features?

• Marked reduction in brain volume

• Cortical atrophy, frontal ,parietal

• Ventricular dilatation (hydroceplus ex vacuo )

Back 203

alzheimer's disease

Front 204

what disease is associated with the following?

Positron emission tomography (PET) : (fluorodeoxyglucose)

Early changes: impaired glucose utilization in posterior cingulate gyrus and parietal cortex (cf.

DLBD :anterior cingulate gyrus )

Back 204

alzheimer's disease

Front 205

What disease has the following?

Key Pathological makers:

1. Neurofibrillary tangles
Flame shaped ; globose

2. Senile plaques
diffuse, primitive, mature and burnt-out

Back 205

alzheimer's disease

Front 206

what is the AD pathlogic criteria for the following ages?

< 50 years

52-65 years

66-75 years

75 years & over

Back 206

< 50 years = 5 or more senile plaques per 200 x field.

52-65 years = 8 or more

66-75 years = 10 or more

75 years & over = 15 or more

Front 207

what disease has the following pathogenic mechanism?

- B-secretase and G-secretase cleave APP (amyloid protien) to form A-beta with 1-40 (accum. in vessel walls) or 1-42 (accum. in neurons)

-change in amyloid protien leads to alteration in 2nd and 3rd structure of normal protien

Back 207

Alzheimer's disease

Front 208

amyloid-beta activates varies caspases, which capsases?

These are responsible for splicing tau protiens resulting in tau protiens mor succeptible to phosphorlaton

Back 208

caspase 3, 8 and 9.

Front 209

what chromosomes are assoc with the following AD genetic cases?

- presenilin 1:

- presenilin 2:

- amyloid precursor protein (APP);

- associated with late onset AD (sporadic).

Back 209

- presenilin 1: Chromosome 14

- presenilin 2: Chromosome 1

- amyloid precursor protein (APP); Chromosome 21

- Apo-E ε 4 allele associated with late onset AD (sporadic).

Front 210

a chronic, inflammatory disease that affects the central nervous system (CNS).

Back 210

MS

Front 211

what disease has the following sx?

changes in sensation, visual problems, muscle weakness, depression, difficulties with coordination and speech, severe fatigue, cognitive impairment, problems with balance, overheating, and pain.

Back 211

MS

Front 212

What disease requires the following for dx?

• Clinical history and examination
– Multiple CNS lesions in time and space

• CSF examination
– mild pleocytosis (5-50 lymphs) during acute attack

– increased protein with high IgG

– oligoclonal bands (persistent)

• MRI shows multiple plaques, active plaques
may enhance

Back 212

MS

Front 213

what disease has the following common manifestations?

• Optic neuritis: visual loss


• Limb weakness

• Sensory symptoms (paresthesias, hypesthesia)

• Cerebellar ataxia

• Nystagmus

• Diplopia (CN VI palsy or INO)

Back 213

MS

Front 214

what disease presents as follows?

 Age 15-50, but most cases start in the 20’s

 Women are effected 2 times more than men

 Optic Neuritis

 Limb weakness which often presents as difficulty walking down stairs

 Imbalance

Back 214

MS

Front 215

what disease has the following?

CSF examination:
 Mild pleocytosis (increase in cell count) during an acute attack

 Increased IgG

 Oligoclonal Bands-due to high Ig levels on electrophoresis.

Back 215

MS

Front 216

what drug used to tx MS are thought to interfere with INF Gamma which enhances attacks?

Back 216

INTERFERONs

Front 217

what drug used to tx MS is a synthetic medication made of four amino acids that are found in myelin? This drug stimulates T cells in the body's immune system to change from harmful, pro-inflammatory agents to beneficial, anti-inflammatory agents that work to reduce inflammation at lesion sites.

Back 217

GLATIRAMER ACETATE: (trade name Copaxone)

Front 218

what drug has been approved by the USA´s FDA for secondary progressive, progressive-relapsing, and worsening relapsing-remitting MS?

Back 218

MITOXANTRONE: (trade name Novantrone)

Front 219

what drug used for MS is used to reduce the occurance of clinical exacerbations. It is recombinant IgG directed against Alpha Integrin. Alpha Integrin inhibition prevents the inflammatory response?

Back 219

NATALIZUMAB: (trade name Tysabri)

Front 220

what are 2 epidemiologic factors associated with MS

Back 220

-MS incidence increases with latitude.

-Prior to age 15, risk is correlated with geographic location.

Front 221

cns disease?

characterized by a brief but intense attack (punctate) of inflammation in the brain and spinal cord that damages myelin – the protective covering of nerve fibers.

It often follows viral infection, or less often, vaccination for measles, mumps, or rubella.

The symptoms come on quickly, beginning with encephalitis-like symptoms such as fever, fatigue, headache, nausea and vomiting, and in severe cases, seizures and coma.

Back 221

Acute disseminated encephalomyelitis (ADEM)

Front 222

what cns disease has the following pathology?

Multiple focal areas of small perivascular areas of demyelination and inflammation in white matter

Back 222

Acute disseminated encephalomyelitis (ADEM)

**be able to distiguish from Leukodystrophy and MS

Front 223

cns disease?

Rare inherited disease which manifest as damage to myelin.
Most have an early onset, are progressive and lethal.
The majority are Autosomal Recessive.

-bilateral, symetric, and diffuse

Back 223

Leukodystrophy

**be able to distiguish from ADME and MS

Front 224

cns disease?

Caused by mutations or conformational changes of the prion-protein (PrPSC) or glycated PrPSC

Back 224

prion disorders

Front 225

Formation of vacuoles in neural cytoplasm and nuclei. The size of the vacuole varies in the different sporadic forms. The vacuoles harbor the abnormal PrPSC-protein.

Back 225

Spongiform Degeneration:

Front 226

type of amyloid plaque?

characteristic of the
cerebellar form of sporadic CJD

Back 226

Kuru-plaques

Front 227

type of amyloid plaque?

seen in the human form of bovine spongiform encephalopathy.

Back 227

Cortical amyloid plaques

Front 228

prion disorder?

-85% of all cases (annually 1 per 1 milj) . Peak age of onset 60 – 65 years

-Usually rapidly progressive dementia 4 -12 mo . With myoclonus, periodic sharp-wave EEG.

Back 228

Sporadic Creutzfeldt-Jakob disease

Front 229

type of prion disease?

all defined by genotype at codon 129 of the prion gene. Differences in the methionine / valine polymorphism of this site and degree of protease resistance of conformationally changed protein do determine the type ,i.e., clinical symptoms and distribution of
pathology.

Back 229

Sporadic Creutzfeldt-Jakob disease

Front 230

all cases occur with an autosomal dominant mode of inheritance. Twenty different mutations of the PrP have been identified .Each type shows a relatively uniform clinical and pathological picture.

Back 230

familial Creutzfeldt-Jakob disease

Front 231

prion disease?

pathological picture is characterized by
spongiform degeneration, excessive gliosis and sometimes deposition of ß-pleated amyloid plaques which are made up of insoluable protease-resistant accumulation of the altered PrPSC.

Back 231

Creutzfeldt-Jakob disease

Front 232

TYPES OF SPORADIC CJD?

clinical sign: Dementia,
ataxia

EEG: Sharp waves

Pathology: Occipital-parietal
cortex

Back 232

M/M type 1

Front 233

TYPES OF SPORADIC CJD?

clinical sign: Dementia

EEG: Diffuse slowing

Pathology: Cerebral cortex

Back 233

M/M type 2

Front 234

TYPES OF SPORADIC CJD?

clinical sign: Ataxia, Dementia

EEG: Diffuse slowing

Pathology: Cerebellum
(Kuru plaques)

Back 234

M/V type 2

Front 235

TYPES OF SPORADIC CJD?

clinical sign: Dysarthria,
dysphagia, dementia

EEG: Periodic sharp waves

Pathology: Subcortical
Involvement (thalamus)

Back 235

V/V type 2

Front 236

what effect does Pathological PrPSC have on Schematic flow of pathogenetic mechanisms in prion disorders?

Back 236

∗ Β-sheet conformation/ plaques

∗ Protease resistant

∗ disease

also, Variations due to
polymorphism and
glycation of PrPSC give
rise to specific disease
entities.

Front 237

inability to produce and/or comprehend language b/c brain damage

Back 237

Aphasia

Front 238

inability to name, .e., “word finding” difficulty

Back 238

Anomia

Front 239

acquired impairment in reading

Back 239

Alexia

Front 240

impairment in writing

Back 240

Agraphia

Front 241

inability to do arithmetic

Back 241

Acalculia

Front 242

impairment in the execution of a motor act in the absence of weakness, sensory loss, or
incoordination

Back 242

Apraxia

Front 243

type of aphasia?

speech: poor

comprehension: intact

repetition: poor

Back 243

broca's aphasia

Front 244

type of aphasia?

speech: fluent

comprehension: poor

repetition: poor

Back 244

wernicke's aphasia

Front 245

type of aphasia?

speech: intact

comprehension: intact

repetition: poor

Back 245

conducion aphasia

Front 246

type of aphasia?

speech: Poor

comprehension: poor

repetition: poor

Back 246

global aphasia

Front 247

• Dominant parietal lobe lesion (angular
gyrus = area 39)

• Finger agnosia
• Left-right disorientation
• Acalculia
• Alexia with Agraphia
• Usually not all 4 are present

Back 247

Gerstmann's syndrome

Front 248

• Deficits due to inability to transmit
information from one area of cortex to another

• After a complete callosotomy the right hand
does not know what the left is doing, or what is in the left visual field

Back 248

Disconnection syndromes

Front 249

type of amnesia?

• Inability to form new memories

• Korsakoff's syndrome

Back 249

Anterograde

Front 250

type of amnesia?

• Loss of premorbid memories

• Ribot's law: more recent memories are most affected

Back 250

Retrograde

Front 251

• Bilateral limbic system lesions

• Medial temporal lobes

• Medial thalamic nuclei

• Usually a mixture of anterograde and retrograde amnesia

• Common after traumatic brain injury

Back 251

Amnestic Syndrome

Front 252

type of amnesia?

• Acute onset profound transient anterograde
amnesia

• Benign usually (migraine, seizure, TIA)

• Leaves a permanent memory gap

• Full recovery of anterograde memory

Back 252

Transient Global Amnesia

Front 253

type of amnesia?

• Loss of personal identity and past

• Intact memory for recent events

• Occurs most often in fiction

Back 253

Psychogenic Amnesia

(psychogenic fugue)

Front 254

Loss of multiple cognitive abilities in a person with a clear sensorium (no delerium)

Back 254

Dementia

Front 255

DSMIV-R requires memory impairment plus impairment in one of:

• language
• judgement
• abstract thinking
• praxis
• constructional abilities
• visual recognition

Back 255

Dementia

Front 256

• Memory complaint, preferably corroborated
by an informant

• Objective memory impairment

• Normal general cognitive function

• Intact activities of daily living

• Not demented

Back 256

Mild Cognitive Impairment Criteria

Front 257

lesion: Left occipital and
splenium of CC

associated deficits: Right hemianopia, color anomia or
achromatopsia

Back 257

Alexia without Agraphia

Front 258

lesion: Left hemisphere
angular gyrus

associated deficits: Right hemisensory, hemiparesis, and
aphasia

Back 258

Alexia with Agraphia

Front 259

Blind but preserved PLR

Back 259

Cortical Blindness

Front 260

cortical blindness with
anosognosia

Back 260

Anton’s Syndrome

Front 261

type of syndrome?

• Oculomotor apraxia—saccades

• Optic ataxia—visually guided limb mvts

• Simultanagnosia—foreground/background

• Sometimes prosopagnosia—face recognition

Back 261

Balint’s syndrome

Front 262

drug class used to tx AD?

Back 262

cholinesterase inhibitors should be considered for mild to moderate AD

-donepezil (aracept)
-rivastigmine (exelon)
-galantamine (renimyl)

Front 263

What disease do the following relate to?

• Memory loss that affects job skills
• Difficulty performing familiar tasks
• Problems with language
• Disorientation to time and place
• Poor or decreased judgment
• Problems with abstract thinking
• Misplacing things
• Changes in mood or behavior
• Changes in personality
• Loss of initiative

Back 263

Ten Warning Signs of AD

Front 264

What disease requires the following evaluation?

• Complete blood cell count
• Serum electrolytes
• Glucose
• BUN/creatinine
• Serum B12 levels
• Liver function tests
• Thyroid function tests
• Depression screening

Back 264

Routine Evaluation of the
Demented Patient

Front 265

drug?

-indicated for the treatment of moderate to severe dementia of the Alzheimer's type (FDA)

-a low-affinity N-methyl-D-aspartate (NMDA) receptor antagonist

Back 265

Memantine (Namenda)

Front 266

T or F?

Estrogen should be prescribed to treat AD

Back 266

False

Front 267

type of headache?

Recurring headache disorder manifesting in attacks lasting 4-72 hours. Typical characteristics of headache are unilateral location, pulsating quality, moderate or
severe intensity, aggravation by routine physical activity, and association with nausea, photo- and phonophobia

Back 267

Migraine without aura

Front 268

type of headache?

Headache has at least two of :
• Unilateral location
• Pulsating quality
• Moderate or severe intensity
• Aggravation by or causing avoidance of routine
physical activity (eg, walking or climbing stairs)

During headache at least one of :
• Nausea and/or vomitng
• Photophobia and phonophobia

Back 268

Migraine without aura

Front 269

type of headache:

least one of the following, but no motor weakness:
• 1. fully reversible visual symptoms including positive features (eg, flickering lights, spots or lines) and/or negative features (ie, loss of vision)
• 2. fully reversible sensory symptoms including positive features (ie, pins and needles) and/or negative features (ie, numbness)
• 3. fully reversible dysphasic speech disturbance

At least two of the following:
• 1. homonymous visual symptoms and/or unilateral sensory symptoms
• 2. at least one aura symptom develops gradually over ≥5 minutes
and/or different aura symptoms occur in succession over ≥5 minutes
• 3. each symptom lasts ≥5 and ≤60 minutes

Headache fulfilling criteria B–D for 1.1 Migraine without aura begins during the aura or follows aura within 60 minutes

Back 269

Typical aura with migraine headache

Front 270

Warning signs in headache

Back 270

• First & worst headache comes to the ER: needs a
CT & LP to rule out subarachnoid hemorrhage
(SAH)

• Headaches that awaken a patient at night or occur
first thing in the morning suggest increased ICP

• Palpable tender temporal arteries suggest GCA:
check ESR

• Cherry-red headache patients in winter: think CO

• Virtually all headaches can cause nausea & vomiting

Front 271

Headaches that awaken a patient at night or occur
first thing in the morning is suggestive of what?

Back 271

suggest increased ICP

Front 272

Palpable tender temporal arteries suggest GCA, check for what?

Back 272

ESR

Front 273

clinical characteristic of virtually all headaches?

Back 273

Virtually all headaches can cause nausea & vomiting

Front 274

Cherry-red headache patients in winter

Back 274

think CO

Front 275

type of headache?

• Excrutiating, penetrating, non-throbbing pain
unilaterally, usually in the trigeminal distribution

• Associated conjunctival injection, lacrimation,
nasal congestion, rhinorrhea, forehead and facial
sweating, miosis, & ptosis

• Peaks in 10-15 minutes, lasts 45-60 minutes

• Occurs 1-3 times per day during cluster, often
nocturnal

• Typical cluster lasts 2-3 months and occurs every
year or two

• Affect men more often than women, 4-7:1

Back 275

Cluster Headache

Front 276

type of headache?

• Diffuse, bilateral, pressing or “tightening”
quality, “like a band around the head”

• Mild to moderate in severity

• Usually episodic, may become chronic

• Phonophobia, photophobia, or mild nausea occur rarely

• Not really due to muscle tightness

• Prevalence is 69% for men, 88% for women

Back 276

Tension-type Headache

Front 277

Migraine is a _______ disorder

Back 277

neurovascular

Front 278

what system is activated when Local vasodilatation of intracranial extracerebral
blood vessels occurs

-causes the release of vasoactive sensory
neuropeptides, especially CGRP, that increase the
pain response

Back 278

'trigeminovascular system'

The activated trigeminal nerves convey nociceptive information to central neurons in the brain stem trigeminal sensory nuclei

Front 279

drugs to tx migrane?

-cause vasoconstriction through 5-HT1B
receptors that are expressed in human intracranial
arteries

-inhibit nociceptive transmission through an
action at 5-HT1D receptors on peripheral trigeminal sensory nerve terminals in the
meninges and central terminals in brain stem
sensory nuclei

Back 279

The 'triptan' anti-migraine agents (e.g. sumatriptan, rizatriptan, zolmitriptan, naratriptan) are serotonergic agonists

Front 280

What should be avoided to abort a migraine except in the ER?

Back 280

Avoid narcotics except in ER

Front 281

what is the specific tx for cluster headaches?

Back 281

100% oxygen, ipsilateral intranasal 4% lidocaine for cluster headache

Front 282

prophylaxis for migraine therapy

Back 282

• Dietary: find precipitants
• Caffeine, chocolate, nuts, aged cheeses, processed meats, alcohol (especially red wine)

• Beta blockers (especially propranolol)

• Daily naproxen

• Calcium channel blockers

• Amitriptyline and other tricyclics

• Valproic acid (Depakote)

• Prednisone (short course) to break status migrainosus or cluster

• Lithium for cluster headaches

Front 283

type of headache?

• Signs and symptoms of increased intracranial pressure without hydrocephalus

• Usually small, slit-like ventricles

• Mean age at onset 30 years, F:M 9:1

• 90% of patients are obese, also associated with endocrinopathies, pregnancy, oral contraceptives, steroids, lithium, tetracycline, vitamin A intoxication

Back 283

Pseudotumor cerebri

Front 284

What type of headache presents 100% of the time with papilledema?

Back 284

Pseudotumor cerebri

Front 285

What type of headache requires the following txmt?

• Weight loss

• Acetazolamide

• If there is progressive visual loss:
• Optic nerve sheath fenestration
• Lumbar-peritoneal shunting

Back 285

Pseudotumor cerebri

Front 286

type of headache?

• Paroxysmal, severe lancinating unilateral
pain in the distribution of one or more branches of the trigeminal nerve

• Episodes last <30-60 seconds evoked by
stimulation of trigger points by touch, chewing or talking

• Age is >40 years in 90%, F:M 3:2

• Examination is normal

Back 286

Trigeminal Neuralgia or Tic Doloreux

Front 287

what drugs are effective in 70-80% of cases in treating trigeminal neuralgia?

Back 287

Carbemazepine and phenytoin

Front 288

what headache type can be treated in one of the following ways?

• Carbemazepine and phenytoin each are effective in 70-80% of cases

• Tricyclics, baclofen, clonazepam, valproic
acid, gabapentin can also be effective

• Surgical treatments include microvascular decompression, percutaneous radiofrequency ablation of the trigeminal ganglion, local neurolysis and trigeminal rhizotomy

Back 288

Trigeminal Neuralgia

Front 289

type of headache?

Patients are usually >60 years old

Symptoms:
• headache, often in temporal or occipital areas
• fever, malaise, weight loss
• jaw claudication
• sudden vision loss

Signs and findings
• tenderness over the temporal arteries
• elevated ESR • vasculitis on biopsy

Treatment: oral prednisone

Back 289

Giant Cell or Temporal Arteritis

Front 290

location of diffuse astrocytoma in adults?

Back 290

AGE/LOCATION:

adults: most common in cerebral hemispheres

Front 291

location of diffuse astrocytoma in children?

Back 291

children: most common in brainstem, diencephalon (thalamus, hypothalamus)