Chap 40 - Acquired Metabolic Disorders Flashcards
1
Q
Main features of reversible metabolic encephalopathies:
A
Confusion, Asterexis, Myoclonus, Absent focal signs
2
Q
Formula of serum osmolality
A
OSM = 2 x Na + Glu/18 +BUN/3
3
Q
Normal serum osmolality
A
270-290 mOsm/L
4
Q
Discrepancy of 10 mOsm/L of calculated & actual means:
A
additional circulating ions present
5
Q
Medical conditions causing HIE
A
Global reduction of cerebral blood flow, Hypoxia from suffocation, Respiratory drive problem, Non-ischemic hypoxia
6
Q
Oxygen saturation curve is
A
Not Linear
7
Q
Compensatory dilation of resistant vessels in response to reduciton of cerebral perfusion then maintains blood flow at constant rate
A
Autoregulation
8
Q
In total cerebral ischemia, tissue depletes sources of energy in how many minutes:
A
5 min
9
Q
T/F. Pathologic effects of ischemia differs from pure anoxia
A
TRUE
10
Q
Brain areas vulnerable for anoxia
A
Hippocampus & deep folia of cerebellum; brainstem & spinal cord resistant to anoxia & hypotension
11
Q
“No reflow” phenomenon
A
irreversibilty of the ischemic lesion due to swelling of endothelium, blockage of circulation in to ischemic tissue
12
Q
Mild degree of hypoxia w/o LOC
A
Inattentiveness, poor judgement, incoordination. Visual and verbal long term memory & mild aphasia
13
Q
T/F. Degree of hypoxia that at no time abolishes conciousness rarely cause permanent damage
A
TRUE
14
Q
Poor prognostic sign in HIE / Grave sign
A
Myoclonus
15
Q
Most common early pathologic changes in anoxic encephalopathy
A
Loss of grey-white matter interface
16
Q
Represents most severe degree of hypoxia, w/ comatose and isoelectric EEG
A
Brain Death Syndrome
17
Q
Conditions that may look like Brain Death Syndrome
A
Anesthesia, Drug intoxication, Hypothermia
18
Q
Freqent post-hypoxic syndromes
A
Persistent coma/stupor, Dementia, Extrapyramidal signs w/ cognitive impairment, Choreoathetosis, Cerebellar ataxia, Lance-Adams Syndrome, Amnesic state
19
Q
Watershed syndromes between PCA & MCA
A
Balint syndrome & Anton Syndrome
20
Q
Watershed syndrome between MCA & ACA
A
Man-in-the-barrel syndrome; w/ weakness of the hip
21
Q
Initial improvement after anoxic event, then relapse. Imaging shows white matter disorder, where mitochondrial disorder suggested underlying mechanism
A
Delayed Postanoxic Leukoencephalopathy
22
Q
5 clinical signs at 1 day after that may predict poor neurologic outcome /death
A
Absent corneal reflex & pupillar reaction, NO WTP, No motor response
23
Q
Drug of choice for polymyoclonus post-anoxic
A
Clonazepam (8-10mg/day)
24
Q
Affinity of CO for Hgb
A
200x of Oxygen
25
Level of carboxyHgb where headache, nausea, dyspnea, confusion, dizziness, clumsiness occurs
20-30%
26
Level of carboxyHgb where comatose occurs
50-60%
27
Clinical feature seen in delayed neurologic detoriation after chronic CO exposure
Parkinsonia gait & bradykinesia
28
Characteristic lesion of CO poisoning that produced coma
discrete lesions in bilateral globus pallidus & inner putamen
29
Half-life of CO
5 hours
30
Indication of administration of hyperbaric oxygen of 2-3 atm. Reduces cognitive sequelae from 45 to 25%. 3 hyperbaric sessions in the first 24 hrs.
carboxyHgb level 40% or if coma or w/ seizures
31
This is implicated as cause of cerebral edema in High-altitude sickness
Over-expression of VEGF
32
Most effective preventive measure for High-altitude sickness
Acclimatization of 2-4 days in intermediate altitudes
33
Drugs that may be used for High-altitude sickness
Dexamethasone & Acetozolamide
34
Chronic High Altitude Sickness seen among Peruvians is also known as
Monge Disease
35
Hypercapnic Pulmonary disease is described by Adams as:
headache, papilledema, mental dullness, drowsiness, confusion, stupor, coma, asterixis
36
T/F. In CO2 narcosis, Normally the CSF is basic compare to blood and PC02 is less than blood by 10mmHg
FALSE. CSF is acidic & has more pCO2
37
Important diagnostic features in Hypercapnic Pulmonary Disease
Papilledema, myoclonus, asterexis
38
Levels of blood glucose that will manifest the ff: 1) confusional state or seizures related to adrenal overacitvity 2) irreversible damage
1) 30 mg/dL 2) 10mg/dL
39
Factor in both depression of conciousness & residual dementia
RATE of blood glucose decline; same goes for most metabolic encephalopathies
40
Normal glucose reseve in the brain is in the form of:
Glycogen
41
Glucose reserve may sustain brain activity for
30 min
42
Convulsion in hypoglycemia is attributed to:
altered integrity of neuronal membrane, elevated NH3, & depressed GABA & lactate levels
43
Most common causes of Hypoglycemic encephalopathy
Overdose of anti-diabetic meds, Islet cell insulin secreting tumor, Depletion of liver glycogen, glycogen storage disease of infancy, idiopathic hypoglycemia in neonatal, subacute & chronic hypoglkycemia islet cell dysfunction
44
Sensitive and reliable indicator of impending coma in Hepatic encephalopathy
EEG
45
Repeated attacks of hepatic coma resulting to irreversible dementia, & disorder or posture & movement
Chronic acquired hepatocerebral degeneration
46
MRI in Portal-systemic encephalopathy____ / result to deposition of what mineral _____
High signal intensity of globus pallidus ; Manganese
47
T/F. Concentration of NH3 levels in excess of 200mg/dL, parallels neurologic & EEG findings
TRUE
48
In Hepatic Enecphalopathy: this cells are diffuse increase in number & size of protoplasmic astrocytes in deep layers of brain, w/ PAS staining of glycogen inclusions
Alzheimers type II astrocytes
49
Chronic cases of Hep encephalopathy results to this neuropathologic changes
neuronal loss in deep layers of cerebral & cerebellar cortex, lenticular nuclei, & vacuolization of tissue
50
More or less specific to Hep Enceph
EEG features, & astrocytic hyperplasia
51
T/F. The brain has urea cycle enzymes to eliminate ammonia
FALSE. Absent, hence hypertrophy of astrocytes to compensate
52
Removal of ammonia depends on the formation of _____, a reaction catalyzed by ATP -dependent enzyme _______, compartmentalized in astrocytes
glutamate, glutamate synthase
53
in Hepatic enceph, this neurotransmitter increases in activity, as a result inhibits binding of endogenous benzodiazepines. Clinical effect is transient arousal.
GABA; GABA-benzodiazepine theory
54
Concept that unifies hyperammonemia & neurotransmitters
Ammonia inhibits metabolism of GABA by the astrocytes & enhances GABAnergic transmission
55
Treatment of hepatic encpehalopathy
Protein restriction, reduction of bowel flora, enemas
56
Mainstay treatment for hepatic encephalopathy, that produces hydrogen ion and shift ammonia to ammonium, nontoxic product
Lactulose
57
Drug that may have transient beneficial effect as well as diagnostic for hepatic encephalopathy
Flumazenil
58
Prominent finding of fulminant hepatic failure & main cause of death in patients awaiting liver transplant
Cerebral edema
59
Individuals w/ Hep C treated w/ ____ develops subtle cognitive impairment, worsening of headache, vomiting, altered conciousness
Interferon-alpha
60
Occuring in children & adolescents, acute brain swelling w/ fatty infiltration of viscera esp liver, due to aspirin given during infection.
Reye Syndrome
61
Improtant diagnostic clue as to the cause of the cerebral changes in Reye Syndrome
Liver englargement
62
EEG characterized by arrhythmic delta, progressing to electrocerebral silence for patient who failed to survive
Reye syndrome
63
Major pathologic findings of Reye syndrome
Cerebral edema w/ cerebellar herniation, & infiltration of hepatocytes w/ fine droplets of fats, mitochondrial dysfunction is implicated
64
Initial symptoms of uremic encephalopathy
apathy, fatigue, inattentiveness & irritability
65
Usually occur early in course of uremic enceph, where px begins to twitch & jerk & convulse, incessant during wakefulness & sleep
uremic twitch-convulsive syndrome
66
Reason why in uremic px, low levels of AEDs may suppress seizures:
Serum albumin is depressed in uremia hence more unbound AEDs
67
Shift of water into the brain is akin to water intoxication & results to inappropriate secretion of ADH; may present w/ headache resembling migraine, muscle cramps, agitation, drowsiness, seizures.
Dysequilibrium syndrome
68
Symptoms of dysequilibrium syndrome occurs in ____ of dialysis and may reach up to ____ after dialysis
3rd-4th hr of dialysis; 48 hours
69
Hepatic paraplegia is attributed to
loss of Betz cell in frontal cortex
70
Neuropathologic changes of mild degree of microcavitation of superficial layer of cerebral cortex more severe in ____ hence distinctive of its disorder of speech & language
Dialysis Dementia
71
Subacutely progressing syndrome w/ speech and language disorder, follwoed seizures, personality changes & intellectual changes in HD patients
Dialysis Dementia
72
Plausible view of pathogenesis of dialysis encephalopathy
Aluminum intoxication
73
Possible complications of Renal transplant patient.
Primary lymphoma, Progressive multifocal leukoencephalopathy, PRES, Fungal CNSi, CMV infection
74
In how many percent do patients become disoriented within hours of onset of systemic infection
70%
75
Hypotonic isovolemic hypernatremia, results to excretion of urine that is hypertonic relative to plasma
SIADH
76
SIADH repsond to restriction fluid intake by how many in Na level 120meqs/L & 130meqs/L in 24 hrs
120 meqs/L: 500 mL; 130 meq/L 1000mL
77
Formula for sodium correction
(target Na- actual Na) x 0.6 x weight in kg
78
Administration of hypertonic solution should include this to decrease intravascular volume
Furosemide
79
To avoid Osmotic demyelination correction of Na should not exceed
10mmol/L in first 24 hrs or 21 mmol/L in 48 hours
80
Sodium loss is attributable to production of natriuretic polypeptide factor from heart & brain
Cerebral salt wasting
81
Serum level for calcium to produce neurologic symptoms w/ normal albumin; & low albumin
12mg/dL; 10mg/dL
82
Most common cause of hypercalcemia in young persons; older persons
hyperparathyroidism; osteolytic bone tunmors
83
Usual manifestation of hypocalcemia
parasthesia, tetatny & convulsions
84
Pathologic feature of pontine myelinosis
destruction of myelinated sheaths w/ sparing of axons & neurons
85
ODS is commonly seen In half of this pateints w/
Chronic alcoholism
86
In pontine lesions in ODS, MRI discloses a characteristic appearance
Batwing lesion
87
Neurologic abnormalities observed in acute hepatic encephalopathy is similar in chronic hepatocerebral degeneration EXCEPT
Irreversible & progressive in chronic type
88
Pathologic changes in myxedema patients that may distinguish from malnutrition & alcholoisms
loss of Purkinje celss & gliosis of molecular layer pronounced in vermis
89
Damaging effects of hyperthermia is disproportionately seen in
Cerebellum
90
Antibody that correspond to neurologic manifestation in celiac disease
antigliadin antibody
91
Psychic effect seen among 10-15 % of patients taking steroids
High dose (60-100mg/d equivalent of prednisone)
92
Encephalopathy consisting of confusion, altered conciousness, & prominent myoclonus, high titers of antibodies of _____ & ______, which is responsive to steroids
Hashimoto encephalopathy
93
Most frequent & potentially & correctable metabolic mental defect in the world
Cretinism or Neonatal Myxedema
94
Clinical manifestation usually seen in 6th -12th month of life, manifesting as
prolonged physiologic jaundice, widening of posterior fontanelle, mottling of skin, psychomotor retardation
95
Two types of early life hypothyroidism:
Sporadic & endemic
96
Sporadic type of cretenism is characterize by
Consequence of congenital metabolic or anatomic defect of thyroid gland, stunting of growth, delay in psychomotor devt, preventable by treatment of thyroid hormone
97
Endemic cretenism is characterize by
occurrence is due to iodine deficiency; two types neurologic cretenism & myxedematous endemic cretenism
98
Difference of neurologic & myxedematous endemic cretenism
Neurologic form: deaf-mutism, rigid spastic motor disorder & mental deficiency; lack of available iodine at 2nd or 3rd trimester; prevented by giving iodine in 1st trimester::::: Myxedematous form: short stature, microcephaly, coarse facial features, psyhomotor retardation
99
T/F. Thyroid hormone is essential for neuronal formation & migration
FALSE. It is for dendritic-axonal development & organization
100
Pathologic findings of cretenism
decrease interneuronal distances, packing density in increased in immature cortex, deficiency of neuropil; poverty of dendritic branching & synapses
