1. In HIV infection all are affected except:
a. Anterior Cingulate cortex
b. Caudate nucleus
c. Globus pallidus
d. cerebral white matter
Answer is A. Anterior cingulate cortex
Reference: Harrison text book chapter 189
· HIV causes subcortical dementia and therefore you can see that
basal ganglia involvement is there. Cerebral white matter
involvement is mentioned in Harrison. Hence by exclusion the
answer is A.
· AIDS related dementia causes psychomotor slowing, apathy,
bradykinesia and altered posture and gait similar to those observed
in advanced Parkinson's disease. The dementia has the hallmarks
attributed to subcortical dementia. The exquisite sensitivity of many
of these patients to dopamine receptor blockade suggested a
profound and, perhaps, selective abnormality of striatal
dopaminergic systems.
· In contrast to "cortical" dementia (such as Alzheimer's disease),
aphasia, apraxia, and agnosia are uncommon, leading some
investigators to classify HIV encephalopathy as a "subcortical
dementia" characterized by defects in short-term memory and
executive function. In addition to dementia, patients with HIV
encephalopathy may also have motor and behavioral abnormalities.
Among the motor problems are unsteady gait, poor balance,
tremor, and difficulty with rapid alternating movements.
MEDICINE 2013
Increased tone and deep tendon reflexes may be found in
patients with spinal cord involvement. Late stages may be
complicated by bowel and/or bladder incontinence. Behavioral
problems include apathy, irritability, and lack of initiative, with
progression to a vegetative state in some instances. Some patients
develop a state of agitation or mild mania. These changes usually
occur without significant changes in level of alertness. This is in
contrast to the finding of somnolence in patients with dementia due
to toxic/metabolic encephalopathies.
· HIV-associated dementia is the initial AIDS-defining illness in 3% of
patients with HIV infection and thus only rarely precedes clinical
evidence of immunodeficiency. Clinically significant encephalopathy
eventually develops in 25% of untreated patients with AIDS.
2. A patient has developed confusion and keeps bumping into objects. He
can speak fluently and on examination patient has inability to differentiate
between fingers, cannot write. On MRI T2 images show cortical and sub cortical
lesions.
a. Gerstman syndrome
b. Anton syndrome
c. Millard Gubler syndrome
d. Locked in syndrome
Gerstmann syndrome is characterized by four primary
symptoms:
1. Dysgraphia/agraphia: deficiency in the ability to
write.
2. Dyscalculia/acalculia: difficulty in learning or
comprehending mathematics.
3. Finger agnosia: inability to distinguish the fingers
on the hand.
4. Left-right disorientation.
3. Which can differentiate between seizures and syncope
a. Unconsciousness
b. Injury due to fall
c. Urinary incontinence
d. Tongue bite
Answer D
Reference : Harrrison 18th edition table 399-7.
Harrison mentions clearly that urinary incontinence which is the first thing that
comes to mind is present with both of these conditions. Injury due to fall can
occur in both . Unconsciousness will occur in both though the transition to
unconsciousness varies and so does the recovery.
4. All are true about iron deficiency anaemia?
a. increased RDW > 14.5%
b. decreased serum ferritin
c. decreased Total iron binding capacity( < 480ng/dl)
d. decreased serum iron( less than 15ng/dl)
Reference: chapter 103 Harrison 18th edition
Red cell distribution width is a measure of dimorphic cell population in patients in
early stages of IRON deficiency anemia as only a part of total cell population would be
microcytic hypochromic and the rest of the cells normal. Thus it is a good screening
test for evaluation of patients of iron deficiency Anemia.
In Iron deficiency obviously iron and ferritin would be lesser and this would send the
Total iron binding systems to work harder to get iron into the system. Thus TIBC is
always increased in iron deficiency Anemia. Remember TIBC value is always inverse of
serum ferritin value.
The following table is a must know for initial MCQ of haematology
5. most common genetic defect in M.O.D.Y
a. Glucokinase
b. Hepatocyte nuclear factor 1 alpha
c. Hepatocyte nuclear factor 4 alpha
d. Insulin promoter 1
Reference : CMDT page no 1194
Maturity onset dibetes of the young (MODY)
· This subgroup is a relatively rare monogenic disorder characterized by
non – insulin dependent diabetes with autosomal dominant inheritance
and an age at onset of 25 yrs or younger.
· Patients are nonobese, and their hyperglycemia is due to impaired glucose
induced secretion of insulin. Six types of mody have been described.
Except for MODY 2 , in which a glucokinase gene is defective, all other
types involve mutations of a nuclear transcription factor that regulates
islet gene expression.
· The enzyme glucokinase is a rate limiting step in glycolysis and
determines the rate of adenosine triphosphate (ATP) production from
glucose and the insulin secretory response in the beta cell. MODY 2, due to
glucokinase mutations, is usually quite mild, associated with only slight
fasting hyperglycemia and few if any microvascular diabetic
complications. It generally responds well to hygienic measures or low
doses of oral hypoglycemic agents.
· MODY 3, due to mutations is hepatic nuclear factor 1 α is the most
common form, accounting for two birds of all MODY cases. The clinical
course is of progressive beta cell failure and need for insulin therapy.
Mutations in both alleles of glucokinase present with more severe
neonatal diabetes. Mutation in one allele of the pancreatic duodenal
homeobox 1 (PDX1) causes diabetes usually at a later age (~ 35 years)
than other forms of MODY; mutations in both alleles of PDX1 causes
pancreatic agenesis.
a. Anterior Cingulate cortex
b. Caudate nucleus
c. Globus pallidus
d. cerebral white matter
Answer is A. Anterior cingulate cortex
Reference: Harrison text book chapter 189
· HIV causes subcortical dementia and therefore you can see that
basal ganglia involvement is there. Cerebral white matter
involvement is mentioned in Harrison. Hence by exclusion the
answer is A.
· AIDS related dementia causes psychomotor slowing, apathy,
bradykinesia and altered posture and gait similar to those observed
in advanced Parkinson's disease. The dementia has the hallmarks
attributed to subcortical dementia. The exquisite sensitivity of many
of these patients to dopamine receptor blockade suggested a
profound and, perhaps, selective abnormality of striatal
dopaminergic systems.
· In contrast to "cortical" dementia (such as Alzheimer's disease),
aphasia, apraxia, and agnosia are uncommon, leading some
investigators to classify HIV encephalopathy as a "subcortical
dementia" characterized by defects in short-term memory and
executive function. In addition to dementia, patients with HIV
encephalopathy may also have motor and behavioral abnormalities.
Among the motor problems are unsteady gait, poor balance,
tremor, and difficulty with rapid alternating movements.
MEDICINE 2013
Increased tone and deep tendon reflexes may be found in
patients with spinal cord involvement. Late stages may be
complicated by bowel and/or bladder incontinence. Behavioral
problems include apathy, irritability, and lack of initiative, with
progression to a vegetative state in some instances. Some patients
develop a state of agitation or mild mania. These changes usually
occur without significant changes in level of alertness. This is in
contrast to the finding of somnolence in patients with dementia due
to toxic/metabolic encephalopathies.
· HIV-associated dementia is the initial AIDS-defining illness in 3% of
patients with HIV infection and thus only rarely precedes clinical
evidence of immunodeficiency. Clinically significant encephalopathy
eventually develops in 25% of untreated patients with AIDS.
2. A patient has developed confusion and keeps bumping into objects. He
can speak fluently and on examination patient has inability to differentiate
between fingers, cannot write. On MRI T2 images show cortical and sub cortical
lesions.
a. Gerstman syndrome
b. Anton syndrome
c. Millard Gubler syndrome
d. Locked in syndrome
Gerstmann syndrome is characterized by four primary
symptoms:
1. Dysgraphia/agraphia: deficiency in the ability to
write.
2. Dyscalculia/acalculia: difficulty in learning or
comprehending mathematics.
3. Finger agnosia: inability to distinguish the fingers
on the hand.
4. Left-right disorientation.
3. Which can differentiate between seizures and syncope
a. Unconsciousness
b. Injury due to fall
c. Urinary incontinence
d. Tongue bite
Answer D
Reference : Harrrison 18th edition table 399-7.
Harrison mentions clearly that urinary incontinence which is the first thing that
comes to mind is present with both of these conditions. Injury due to fall can
occur in both . Unconsciousness will occur in both though the transition to
unconsciousness varies and so does the recovery.
4. All are true about iron deficiency anaemia?
a. increased RDW > 14.5%
b. decreased serum ferritin
c. decreased Total iron binding capacity( < 480ng/dl)
d. decreased serum iron( less than 15ng/dl)
Reference: chapter 103 Harrison 18th edition
Red cell distribution width is a measure of dimorphic cell population in patients in
early stages of IRON deficiency anemia as only a part of total cell population would be
microcytic hypochromic and the rest of the cells normal. Thus it is a good screening
test for evaluation of patients of iron deficiency Anemia.
In Iron deficiency obviously iron and ferritin would be lesser and this would send the
Total iron binding systems to work harder to get iron into the system. Thus TIBC is
always increased in iron deficiency Anemia. Remember TIBC value is always inverse of
serum ferritin value.
The following table is a must know for initial MCQ of haematology
5. most common genetic defect in M.O.D.Y
a. Glucokinase
b. Hepatocyte nuclear factor 1 alpha
c. Hepatocyte nuclear factor 4 alpha
d. Insulin promoter 1
Reference : CMDT page no 1194
Maturity onset dibetes of the young (MODY)
· This subgroup is a relatively rare monogenic disorder characterized by
non – insulin dependent diabetes with autosomal dominant inheritance
and an age at onset of 25 yrs or younger.
· Patients are nonobese, and their hyperglycemia is due to impaired glucose
induced secretion of insulin. Six types of mody have been described.
Except for MODY 2 , in which a glucokinase gene is defective, all other
types involve mutations of a nuclear transcription factor that regulates
islet gene expression.
· The enzyme glucokinase is a rate limiting step in glycolysis and
determines the rate of adenosine triphosphate (ATP) production from
glucose and the insulin secretory response in the beta cell. MODY 2, due to
glucokinase mutations, is usually quite mild, associated with only slight
fasting hyperglycemia and few if any microvascular diabetic
complications. It generally responds well to hygienic measures or low
doses of oral hypoglycemic agents.
· MODY 3, due to mutations is hepatic nuclear factor 1 α is the most
common form, accounting for two birds of all MODY cases. The clinical
course is of progressive beta cell failure and need for insulin therapy.
Mutations in both alleles of glucokinase present with more severe
neonatal diabetes. Mutation in one allele of the pancreatic duodenal
homeobox 1 (PDX1) causes diabetes usually at a later age (~ 35 years)
than other forms of MODY; mutations in both alleles of PDX1 causes
pancreatic agenesis.
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