ACQUIRED METABOLIC DSE

Question 1

The main features
of the reversible ________ are confusion,
typified by disorientation and inattentiveness and accompanied
in certain special instances by asterixis, tremor, and myoclonus,
usually without signs of focal cerebral disease.

Answer 1

metabolic encephalopathies

Question 2

At normal temperature and pH, hemoglobin
is 90 percent saturated at an oxygen partial pressure of
_______mm Hg and still 75 percent saturated at 40 mm Hg;

Answer 2

60

Question 3

Through a mechanism termed _______
there is a compensatory dilatation of resistance
vessels in response to a reduction in cerebral perfusion,
which maintains blood flow at a constant rate

Answer 3

autoregulation,

Question 4

In total cerebral ischemia, the
tissue is depleted of its sources of energy in about ________min,
although longer periods are tolerated under conditions
of_______`

Answer 4

5

hypothermia.

Question 5

Under conditions of _______, one pattern of damage takes the form of
incomplete infarctions in the border zones between major
cerebral arteries

Answer 5

transient

ischemia

Question 6

With predominant __________,
neurons in portions of the hippocampus and the deep folia
of the cerebellum are particularly vulnerable

Answer 6

anoxia

Question 7

There is experimental
evidence that certain excitatory neurotransmitters, particularly
_____, contribute to the rapid destruction of
neurons under conditions of anoxia and ischemia

Answer 7

glutamate

Question 8

The most
common early change in cases of severe injury is a
_______

Answer 8

loss of the distinction between the cerebral gray and

white matter

Question 9

This represents the most severe degree of hypoxia,
usually caused by circulatory arrest; it is manifest by a
state of complete unawareness and unresponsiveness
with abolition of all brainstem reflexes.

Answer 9

Bra i n Death Syn d ro m e

Question 10

Posthypoxic Neu rologic Syndromes

Answer 10

1. Persistent corru:l or stupor, described above
2. With lesser degrees of cerebral injury, dementia with
   or without extrapyramidal signs
3. Extrapyramidal (parkinsonian) syndrome with cognitive
   impairment (discussed in relation to CO poisoning)
4. Choreoathetosis
5. Cerebellar ataxia
6. Inten tion or action myoclon us (Lance-Adams syndrome)
7. An amnesic state

Question 11

Watershed syndromes

Answer 11

PCA-MCA:Visual agnosias including Balint syndrome and cortical
blindness (Anton Syndrome)

MCA-ACA: sometimes accompanied
by hip weakness (referred to as a “man-inthe-
barrel” syndrome),

Question 12

Initial improvement, which appears to be
complete, is followed after a variable period of time (1 to
4 weeks in most instances) by a relapse, characterized by
apathy; confusion, irritability, and occasionally agitation
or mania.

Answer 12

Del ayed Posta noxic Encep h a l opathy a n d

Leu koenceph a l o pathy

Question 13

that 5 clinical signs at 1 day after
cardiac arrest predicted a poor neurologic outcome or
death:

Answer 13

(1) absent corneal responses, (2) absent pupillary
reactivity, (3) no withdrawal to pain, and (4) the absence
of any motor response. T

Question 14

hypothermia in coma

Answer 14

They reduced the
core temperature to 33°C (91 °F) within 2 h of the arrest
and sustained this level for 12 h in the first trial, and
between 32°C and 34°C for 24 h in the second study.

Question 15

treatment of poly myoclonus

Answer 15

clonazepam, 8 to 12 mg daily
in divided doses may be useful but the commonly used
antiepileptic drugs have little effect

Question 16

Sx of CO when carboxyhemoglobin level reaches 20 to 30 percent of total hemoglobin

Answer 16

headache, nausea, dyspnea,

confusion, dizziness, and clumsiness

Question 17

CO at 50 to 60 percent are associated with :

Answer 17

with coma, decerebrate
or decorticate posturing, seizures in a few patients, and
generalized slowing of the EEG rhythms

Question 18

Discrete lesions centered in the
__________ are characteristic of CO poisoning
that had produced coma

Answer 18

globus pallidus bilaterally and sometimes the inner portion of the putamina

Question 19

the half-life of CO (normally
5 h) is greatly reduced by the administration of
_________,

this additional
treatment is recommended when the carboxyhemoglobin concentration is ____________
of coma or seizures

Answer 19

hyperbaric oxygen at 2 or 3 atmospheres

greater than 40 percent or in the presence

Question 20

Pathophysiology of mountain sickness

Answer 20

The overexpression of vascular endothelial
growth factor (VEGF), a protein originally noted for its
effects on vascular permeability, has been implicated as
the cause of cerebral edema in

Question 21

_______
and __________prevent and counteract mountain
sickness to some extent. The most effective preventive
measure is _______ at intermediate
altitudes.

Answer 21

Dexamethasone

acetazolamide

acclimatization by a 2- to 4-day stay

Question 22

At a level of about
_______ the cerebral disorder takes the form of a confusional
state and one or more seizures may occur; at a level
of _______, there is coma that may result in irreparable
injury to the brain if not corrected immediately by the
administration of glucose

Answer 22

30 mg/ dL,

10 mg/dL

Question 23

Blood glucose levels of approximately 10 mg/ dL
are associated with deep coma, dilatation of pupils, pale
skin, shallow respiration, slow pulse and hypotonia,
what had in the past been termed the _____
of hypoglycemia

Answer 23

“medullary phase”

Question 24

The major clinical differences between hypoglycemic
and hypoxic encephalopathy lie in the setting and
the mode of evolution of the neurologic disorder. The
effects of hypoglycemia usually unfold more_______, rather than in a few seconds or
minutes.

Answer 24

slowly, over a period of 30 to 60 min

Question 25

Pathophysiology of DKA in the brain

Answer 25

accumulation of fructose and sorbitol in the brain

Question 26

In hyperosmolar nonketotic hyperglycemia, the blood
glucose is extremely high, _______ but
ketoacidosis does not develop,

Answer 26

more than 600 mg/ dL,

Question 27

triggers of HHS

Answer 27

corticosteroids and

phenytoin (which inhibits insulin release),

Question 28

__________following viral infections
in children, now infrequent, was also associated with
very high levels of ammonia in the blood and encephalopathy

Answer 28

Reye syndrome

Question 29

EEG of hepatic enceph

Answer 29

EEG abnormality consisting of paroxysms
of bilaterally synchronous slow or triphasic waves
in the delta range, which at first predominate frontally
and are interspersed with alpha activity and later, as the
coma deepens, displace all normal activity

Question 30

there is a group of patients (most of whom
have experienced repeated attacks of hepatic coma) in
whom an irreversible mild dementia and a disorder of
posture and movement (grimacing, tremor, dysarthria,
ataxia of gait, choreoathetosis) gradually appear

Answer 30

chronic acq uired hepatocerebral degeneration

Question 31

Pathology of chronic acq uired hepatocerebral degeneration

Answer 31

attributed the syndrome to a loss of

Betz cells in the frontal cortex;

Question 32

MRI in PSE often demonstrates high signal intensity

in the _____, likely the result of ________

Answer 32

globus pallidus

manganese
deposition.

Question 33

astrocytes

were seen to contain glycogen inclusions.

Answer 33

Alzheimer

type II astrocytes

Question 34

Characteristics of Alzheimer

type II astrocytes

Answer 34

swelling of their terminal processes,
cytoplasmic vacuolation (distended sacs of rough endoplasmic
reticulum), formation of folds in the basement
membrane around capillaries, and an increase in both
the number of mitochondria and enzymes that catabolize
ammonia

Question 35

accumulation of this element is readily discernible as

a pallidal signal hyperintensity on Tl-weighted MRI.

Answer 35

Mn

Question 36

______ is a prominent finding in cases of
fulminant hepatic failure and is the main cause of death
in patients awaiting liver transplantation

Answer 36

Cerebral edema

Question 37

Individuals with hepatitis
C who are treated with _________ may develop
a spectrum of problems ranging from subtle cognitive
impairment to a subacutely worsening headache, vomiting,
altered consciousness, and focal neurologic findings

Answer 37

interferon-alpha

Question 38

this EEG pattern is charac of

The EEG is characterized by diffuse
arrhythmic delta activity; progressing to electrocerebral
silence in patients who fail to survive.

Answer 38

Reye

Question 39

early diagnosis
and initiation of treatment before the onset of coma have
reduced the fatality rate to

Answer 39

5 to 10 percen

Question 40

movements resemble those

of chorea or an arrhythmic tremor; asterixis among uremic pts

Answer 40

uremic twitch-convulsive syndrome .

Question 41

Subdural and intracerebral hemorrhages may complicate

uremia (and dialysis) because of _________

Answer 41

clotting defects and

hypertension

Question 42

However, some seizures may be
suppressed with relatively low plasma concentrations of
antiepileptic drugs:

Answer 42

, the reason being that serum albumin
is depressed in uremia, increasing the unbound, therapeutically
active portion of a drug.

Question 43

rapid lowering of serum urea, leaving the brain with a higher concentration of urea than the serum and resulting
in a shift of water into the brain to equalize the osmotic
gradient (reverse urea syndrome). Now it is believed that
the shift of water into the brain is akin to water intoxication
and is a result of the inappropriate secretion of
antidiuretic hormone

Answer 43

” D i a lysis D i s eq u i l i b ri u m “ Syn d ro m e

Question 44

This i s a subacutely progressive syndrome that i n the past
complicated chronic hemodialysis. Characteristically, the
condition begins with a hesitant, stuttering dysarthria
and aphasia, to which are added facial and then generalized
myoclonus, focal and generalized seizures, personality
and behavioral changes, and intellectual decline.

Answer 44

D i a lysis E n c e p h a l o pathy ( D i a lysis D e m e n t i a )

Question 45

septic enceph

____percent of
patients become disoriented and confused within hours
of the onset of severe systemic infection;

Answer 45

70

Question 46

Altered __________

metabolism and circulating cytokines

Answer 46

phenylalanine

Question 47

______ caused by
hyperlipidemia or hyperproteinemia (isot?nic), hyperg.lycemic
or mannitol-induced hyponatrerma (hypertoruc),
and also cases of water intoxication

Answer 47

pseudohyponatremia

Question 48

______is the result
of excretion of urine that is hypertonic relative to the
plasma

Answer 48

SIADH

Question 49

Most cases of
SIADH respond to the restriction of fluid intake-to
________per 24 h if the serum Na is less than 120 mEq/L
and to_________per 24 h if less than 130 mEq/L.

Answer 49

500 mL

1 ,000 mL

Question 50

3 percent (hypertonic) saline
solution is \_\_\_\_\_ mEq/L

Answer 50

513

Question 51

Sodium loss in these circurnstanc􀃨s
is attributable to the production by the heart or bram
of a potent polypeptide natriuretic factor.

Answer 51

“cerebral salt wasting”

Question 52

This is defined as an elevation of
the serum calcium concentration greater than 1 0.5 mg/
dL. If the serum protein content is normal, Ca levels
greater than ______ are required to produce neurologic
symptoms.

Answer 52

12 mg/ dL

Question 53

In young persons, the most common cause of hypercalcemia
is _______ (either primary or secondary);
in older persons, _____

Answer 53

hyperparathyroidism

osteolytic bone tumors,

Question 54

______predominates

in the EEG of pts with met avid

Answer 54

High-voltage slow activity

Question 55

extrapontine myelinolytic foci locations

Answer 55

the internal capsule, deep cerebral white matter and corpus

callosum may occur independently

Question 56

causes of CPM

Answer 56

More than half the cases
have appeared in the late stages of chronic alcoholism,
often in association with Wernicke disease and polyneuropathy.

Question 57

The MRI of CPM discloses a characteristic

______ lesion of the pons in typical cases

Answer 57

“batwing”

Question 58

_____cells usually

associated with Wilson disease.

Answer 58

Opalski

Question 59

neurologic abnormalities occur in approximately _______percent of cases of adult celiac sprue

Answer 59

10

Question 60

______
while not specific for celiac disease, do correspond to the
presence of neurologic manifestations
(ataxia and neuropathy);

Answer 60

antigliadin antibodies

Question 61

causes of acquired dementia

Answer 61

chronic hepatic encephalopathy and
the syndromes of episodic hypoglycemia, chronic hypercalcemia
(in multiple myeloma, metastatic cancer, and
sarcoidosis), hyponatremia, and hypematremia.

Question 62

At higher doses_________, approximately 10 to
15 percent of patients become overly active, emotionally
labile, and unable to sleep

Answer 62

(equivalent

to 60 to 100 mg/ d of prednisone)

Question 63

_______(ventricular
enlargement and sulcal widening) has been shown radiologically
in patients with Cushing disease and after a
prolonged period of corticosteroid therapy,

Answer 63

“Cerebral atrophy”

Question 64

encephalopathy consisting
of confusion, altered consciousness, and prominent
myoclonus in patients with _____

Answer 64

Hashimoto disease

Question 65

Hashimoto disease: high titers of several
antithyroid antibodies, particularly antibodies against
_________

Answer 65

hyroid peroxidase and thyroglobulin

Question 66

The encephalopathic symptoms and high

titers of antithyroid antibodies respond well to ____

Answer 66

steroid

therapy

Question 67

The term________ is now more often
applied to a depressive illness that seems to occur with disproportionate frequency before the symptoms of a
pancreatic cancer become apparent.

Associated with pancreatic cancer and
sequential cerebral emboli from ______

Answer 67

pancreatic encephalopathy

nonbacterial thrombotic
(marantic) endocarditis.