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Buddhism and the Rite
Resident Friday AM Lecture
What is Buddhism
• A religion (ish) that dates back 2,500 years and
has approximately 300 million followers around
the world
• Founded by Siddhartha Gotama upon his
enlightenment under a fig tree at the age of 35
• Buddhism teaches that the solution to our
problems can only be found within ourselves
• When attachment to all worldly desires including
the idea of self is relinquished Nirvana can be
attain
• The core of Buddha's teachings are encompassed
in the four noble truths and the eightfold path
The Four Noble Truths
1. Dukkha – life is suffering, impermanent and
fleeting
2. Samudaya – the arising of dukkha is
contained in thirst, carving, desire and
attachment
3. Nirodha – the cessation of dukkha is nirvana
4. Magga – the eightfold path towards
cessation of dukkha
The Eightfold Path
1. Right Understanding
2. Right Thought
3. Right Speech
4. Right Action
5. Right Livelihood
6. Right Effort
7. Right Mindfulness
8. Right Concentration
The Four Truths of the RITE
1. The RITE is suffering
2. The RITE is predictable
3. You have to take the RITE
4. Yojana (skrt) – the plan to do well on the RITE
The Eightfold Plan
1. RITE Attitude - you’re the smartest!
2. RITE Approach – start studying early
3. RITE Material – prior answers keys are gold
4. RITE Practice – go through old images
5. RITE Bathroom breaks – don’t break the seal!
6. RITE Fuel – nuts, berries, grains, water, eggs and
greens
7. RITE Energy - bring your stamina
8. RITE celebration (when it’s over)
A patient underwent surgery to remove a colloid
cyst which was causing obstructive
hydrocephalus. Following the procedure she
was unable to create new memories. Her
ability to name objects and perform tasks was
unaffected. Which part of the brain was likely
damaged during the surgery?
1. Anterior temporal lobe
2. Basal ganglia
3. Fornix
4. Orbito-frontal lobe
A patient underwent surgery to remove a colloid
cyst which was causing obstructive
hydrocephalus. Following the procedure she
was unable to create new memories. Her
ability to name objects and perform tasks was
unaffected. Which part of the brain was likely
damaged during the surgery?
1. Anterior temporal lobe
2. Basal ganglia
3. Fornix
4. Orbito-frontal lobe
Damage to the fornix can occur with transcallosal surgery to remove a colloid cyst of the
third ventricle, which interrupts the Papez circuit and results in loss of the ability to form
new memories. The other structures, while important for memory, would not be affected
by a transcallosal procedure.
This individual has problems with procedural memory, that is the ability to acquire
(with practice) cognitive and behavioral skills that subsequently operate automatically.
Procedural memory is associated with lesions of the basal ganglia-cerebellum
supplementary motor area system. Procedural memory problems are commonly
reported in patients with Parkinson disease (PD)and affect tasks such as playing a
musical instrument or golfing. Episodic memory localizes to the medial temporal
lobe-Papez circuit system, semantic memory to the anterior and inferior temporal
lobes, and working memory to the prefrontal cortex subcortex-parietal-association
cortex system. Balint syndrome localizes to the posterior parietal regions and consists
classically of the triad of optic ataxia, ocular dysmetria and asimultagnosia. Episodic
memory deficits are typical of Alzheimer disease (AD), as is Balint syndrome. Semantic
memory deficits are typical of Alzheimer disease and frontotemporal lobar
degeneration (FTD). Working memory deficits can result from multiple cerebrovascular
events, AD, FTD, PD, dementia with Lewy Bodies, and traumatic brain injury.
Papez Circuit
- Part of the limbic system
- Important for control of emotion and episodic memory
A lesion at what level of the brainstem can cause decerebrate
posturing?
1.
2.
3.
A lesion at what level of the brainstem can cause decerebrate
posturing?
1.
2.
3.
The lateral vestibulospinal tract
facilitates extensor muscle tone,
whereas the rubrospinal tract facilitates
flexor tone. A lesion between the red
nucleus and vestibular nuclei can result
in decerebrate posturing.
A 55-year-old male is recovering from a left ACA
ischemic infarct. He has severe RLE weakness
and his family notes that the he is quite
apathetic and not engaged in rehab. Which of
the following medications can be used to treat
his behavioral changes.
1. Quetiapine
2. Methylphenidate
3. Sertraline
4. Mirtazapine
A 55-year-old male is recovering from a left ACA
ischemic infarct. He has severe RLE weakness
and his family notes that the he is quite
apathetic and not engaged in rehab. Which of
the following medications can be used to treat
his behavioral changes.
1. Quetiapine
2. Methylphenidate
3. Sertraline
4. Mirtazapine
Dopaminergic agonists and inhibitors of
dopamine reuptake have been used to treat
the behavioral traits associated with medial
frontal syndrome. Methylphenidate is such a
drug. This syndrome may be seen after
ischemia in the distribution of the anterior
cerebral artery.
A 75 year old man suffers are large L MCA
stroke. PET scan reveals hypometabolism in
the left MCA territory. A PET scan would also
be expected to show hypometabolism in what
structure?
1. Contralateral frontal eye field
2. Contralateral cerebellum
3. Contralateral midbrain
4. Contralateral thalamus
A 75 year old man suffers are large L MCA
stroke. PET scan reveals hypometabolism in
the left MCA territory. A PET scan would also
be expected to show hypometabolism in what
structure?
1. Contralateral frontal eye field
2. Contralateral cerebellum
3. Contralateral midbrain
4. Contralateral thalamus
The contralateral cerebellar hemisphere (in this case, the left
cerebellar hemisphere) is engaged with motor tasks planned and
executed from the opposite cerebral hemisphere. Activation of a
cerebral cortical motor strip is accompanied by activation of the
contralateral cerebellar hemisphere. Conversely, if the motor areas of
a cerebral hemisphere are damaged, the contralateral cerebellar
hemisphere shows a reduction in activity. This is also called
cerebellar diaschesis.
The synapse for deep tendon reflexes is located
where in the nervous system?
1. Intermediate column of the spinal cord
2. Substantia gelatinosa
3. Doral root ganglion
4. Ventral horn of the spinal cord
The synapse for deep tendon reflexes is located
where in the nervous system?
1. Intermediate column of the spinal cord
2. Substantia gelatinosa
3. Doral root ganglion
4. Ventral horn of the spinal cord
The synapse of the afferent axons for the muscle stretch reflex is located in the anterior
(ventral) horn. The muscle stretch reflex is monosynaptic directly from sensory neuron to
an alpha motor neuron. The dorsal root ganglion contains cell bodies of the pseudounipolar sensory neurons. The intermediate column contains synapses related to
sympathetic function while the substantia gelatinosa is the site of synapse for afferent
fibers which will form the spinothalamic tract. The substantia gelatinosa sits within the
zone of Lissauer.
A 75 year old women is brought to your office by her
husband for evaluation of memory changes. On
your exam you notice that the relaxation phase of
her reflexes is prolonged. Which additional lab
finding could suggest an etiology to explain her exam
and subjective memory concerns?
1. Elevated 24 hour urine copper
2. Elevated TSH
3. Anemia
4. Elevated MCV
A 75 year old women is brought to your office by her
husband for evaluation of memory changes. On
your exam you notice that the relaxation phase of
her reflexes is prolonged. Which additional lab
finding could suggest an etiology to explain her exam
and subjective memory concerns?
1. Elevated 24 hour urine copper
2. Elevated TSH
3. Anemia
4. Elevated MCV
Decreased hemoglobin and elevated mean corpuscular volume are
compatible with a macrocytic anemia that may be secondary to
vitamin B12 deficiency. Vitamin B12 deficiency, in turn may cause
combined system degeneration (affecting posterior columns and
lateral corticospinal tracts). Dementia and peripheral neuropathy
may also be seen. Copper metabolism is altered in Wilson's disease,
which is accompanied by a movement disorder. In hypothyroidsim,
the relaxation phase of reflexes is prolonged, the reflexes are not
hyperactive.
This patient most likely has hypothyroidism, which may present with a
dementing illness and systemic symptoms including weight gain,
hoarseness, lethargy, and cold intolerance. Screening for
hypothyroidism is considered standard for patients presenting with
dementia.
An 8 year boy is brought to your office for evaluation of
abnormal movements. 9 months ago the patient started
snapping followed by jerking of his head. Later in the
course he was noted to make inappropriate squeeking
sounds. The child stated that if he did not perform these
movements and make these sounds he would feel very
uncomfortable until he finally perform the action. Which of
the following is the correct diagnosis?
1. Tourette’s syndrome
2. Motor stereotopy
3. Transient Tic Disorder
4. complex motor and vocal tic disorder
An 8 year boy is brought to your office for evaluation of
abnormal movements. 9 months ago the patient started
snapping followed by jerking of his head. Later in the
course he was noted to make inappropriate squeeking
sounds. The child stated that if he did not perform these
movements and make these sounds he would feel very
uncomfortable until he finally perform the action. Which of
the following is the correct diagnosis?
1. Tourette’s syndrome
2. Motor stereotopy
3. Transient Tic Disorder
4. complex motor and vocal tic disorder
A diagnosis of Tourette's disorder is made when a
patient has a history of tics for over year and at
some point in the course has had the presence of
motor tics and vocal tics. The onset of symptoms
has to be before the age of 18. Regardless of
whether or not there are motor or vocal tics (or
both), if symptoms have been under a year, the
diagnosis of Transient Tic Disorder is given.
There is no diagnosis of "Complex motor and
vocal tic disorder," but the tics in the patient are
all simple tics anyway.
A 65-year-old male with an extensive psychiatric history is
seen in your clinic for evaluation of memory loss. On
exam you notice that he has acne and a very fine
resting tremor, intention tremor and action tremor.
Which of the following medications can produce side
effects to explain his symptoms?
1. Clonazepam
2. Buproprion
3. Lamotrigine
4. Lithium
A 65-year-old male with an extensive psychiatric history is
seen in your clinic for evaluation of memory loss. On
exam you notice that he has acne and a very fine
resting tremor, intention tremor and action tremor.
Which of the following medications can produce side
effects to explain his symptoms?
1. Clonazepam
2. Buproprion
3. Lamotrigine
4. Lithium
Well-known side effects of lithium therapy include
dyspepsia, nausea, vomiting, diarrhea, hair loss,
acne, tremor, decreased cognition, and
incoordination.
You are seeing a 55 year old patient in clinic referred by her
PCP with specific concern for dementia. You are struck by
her language difficulties without deficits in any other
cognitive domain. On exam she can name single objects.
Her speech is generally very fluent, but she will occasionally
pause to find a word. Repetition of multi-clause sentences
is quite impaired. Post-mortem pathology is most likely to
show?
1. NF tangles and amyloid plaques
2. TDP-43 inclusions
3. Lewy bodies
4. Negri bodies
You are seeing a 55 year old patient in clinic referred by her
PCP with specific concern for dementia. You are struck by
her language difficulties without deficits in any other
cognitive domain. On exam she can name single objects.
Her speech is generally very fluent, but she will occasionally
pause to find a word. Repetition of multi-clause sentences
is quite impaired. Post-mortem pathology is most likely to
show?
1. NF tangles and amyloid plaques
2. TDP-43 inclusions
3. Lewy bodies
4. Negri bodies
Logopenic progressive aphasia (LPA) is one of several different
progressive language disorders (semantic dementia, progressive
anarthria, progressive nonfluent aphasia are some of the others).
The pathologic findings in LPA are generally the same of patients
with Alzheimer's disease. Hypometabolism is seen in the left
temporal/parietal regions. Bilateral anterior temporal
hypometabolism is more common in patients with semantic
dementia, whereas the left frontal opercular region is affected in
patients with progressive nonfluent aphasia.
- Semantic variant is most commonly TDP-43 pathology.
- Non-fluent variant is usually FTD-tau pathology
- Logopenic is usually AD pathology
A 59 year old male presents to clinic with his wife with
behavior changes. He is sleeping all day, not changing his
clothes and has become very impulsive. On exam you
notice wasting of his intrinsic hand muscles and
fasciculations throughout the muscles of his arms and legs.
His reflexes are quite brisk. Post-mortum pathology is most
likely to show inclusions composed primarily of what?
1. Tau
2. beta-amyloid
3. TDP-43
4. alpha-synuclein
A 59 year old male presents to clinic with his wife with
behavior changes. He is sleeping all day, not changing his
clothes and has become very impulsive. On exam you
notice wasting of his intrinsic hand muscles and
fasciculations throughout the muscles of his arms and legs.
His reflexes are quite brisk. Post-mortum pathology is most
likely to show inclusions composed primarily of what?
1. Tau
2. beta-amyloid
3. TDP-43
4. alpha-synuclein
This man best fits the diagnosis of frontotemporal dementia
(FTD) with motor neuron disease. This condition is
characterized by personality changes prior to memory
disturbances typically before age 65 and development of
upper and lower motor neuron deficits (amyotrophic lateral
sclerosis). Frontotemporal dementias are heterogeneous
with the two most common protein accumulations in these
cases consisting of tau or TDP-43. Nearly all of the cases of
FTD with motor neuron disease have TAR DNA-binding
protein 43 (TDP-43) protein accumulations. Alphasynuclein inclusions are mostly seen in Parkinsonian
dementias. Amyloid is seen in Alzheimer disease cases.
Vascular dementia will show ischemic changes.
Which of following tests is recommend for all
children with autism spectrum disorder?
1. CSF profile
2. MRI brain
3. 2 hour sleep deprived EEG
4. Genetic testing
Which of following tests is recommend for all
children with autism spectrum disorder?
1. CSF profile
2. MRI brain
3. 2 hour sleep deprived EEG
4. Genetic testing
Routine genetic testing is recommended for all children with
autism spectrum disorder. Brain imaging, EEG and CSF
analysis are not routinely indicated unless there is a clear
history of regression, or there are focal deficits or possible
seizure like episodes.
The most common cause of inherited mental retardation is
fragile X syndrome. Nearly all affected boys manifest
attention deficit disorder and have learning disabilities. The
most frequent neurocognitive symptoms are difficulty with
abstract reasoning, complex problem solving, and
expressive language. Many will also show manifestations
of autism, with 33% meeting criteria for autism. Female
carriers can have a milder form of the disease, with
learning disabilities; about 50% of them will manifest
attention deficit disorder. Characteristic physical features
include a long thin face, prominent forehead and jaw,
protuberant ears, hip dislocation, and club feet.
A 70 year old male presents with falls. On exam you notice
mild bradykinesia. He has a wide based ataxic gait. He
scores a 24/30 on MMSE. Family history is notable for
two grandsons with mental retardation. His MRI is show.
Which of the following is the most likely diagnosis?
1. Freidriech ataxia
2. Charcot Marie Tooth
3. FAXTAS
4. Spinocerebellar ataxia
5. Dentatorubral-pallidoluysian atrophy
A 70 year old male presents with falls. On exam you notice
mild bradykinesia. He has a wide based ataxic gait. He
scores a 24/30 on MMSE. Family history is notable for
two grandsons with mental retardation. His MRI is show.
Which of the following is the most likely diagnosis?
1. Freidriech ataxia
2. Charcot Marie Tooth
3. FAXTAS
4. Spinocerebellar ataxia
5. Dentatorubral-pallidoluysian atrophy
This patient most likely has fragile X-associated
tremor ataxia syndrome (FXTAS). He is not likely
to have Friedreich ataxia, dentatorubralpallidoluysian atrophy, or spinocerebellar ataxia
type 3 given his age, absence of other clinical
features, and the history of mental retardation
in his daughter's son. Patients with CharcotMarie-Tooth disease may have postural and
action tremor but should not have cerebellar
ataxia.
FAXTAS
- FMR1 gene CGG repeats 55-200 can present with
FAXTAS
- > 200 repeats = MR
- Can present in males or females usually > 55 years of
age
- Classic features: kinetic tremor, cerebellar ataxia,
cognitive decline, parkinsonism, psychiatric symptoms
(anxiety, mood lability, apathy)
- Other less common features: impotence and
autonomic dysfunction, neuropathy
- “MCP” sign : bilateral T2 middle cerebellar peduncle
hyperintensities, present in ~ 60% cases
- Another clue in women is premature ovarian failure
Of the following anti-psychotics, which of the
following is associated with the most weight
gain?
1. Olanzapine
2. Quetiapine
3. Haldol
4. Clozapine
Of the following anti-psychotics, which of the
following is associated with the most weight
gain?
1. Olanzapine
2. Quetiapine
3. Haldol
4. Clozapine
Although all atypical antipsychotics and many
typicals are known to cause weight gain and
diabetes mellitus, numerous studies in both
dementia and schizophrenia have shown that
olanzapine causes the most weight gain.
Which of the following antipsychotics is most
commonly associated with seizures?
1. Olanzapine
2. Quetiapine
3. Haldol
4. Clozapine
Which of the following antipsychotics is most
commonly associated with seizures?
1. Olanzapine
2. Quetiapine
3. Haldol
4. Clozapine
Although much emphasis is placed on its causing
agranulocytosis, seizures occur more frequently
with the use of clozapine than with other
atypical antipsychotics.
An 80 year old female was referred to your clinic for
hallucinations. She has a long history of macular
degeneration and poor vision at baseline. With minimal
assistance, however she is able to live mostly independently.
She has no history of any cognitive issues. Her neurologic
exam other than poor vision is normal and her MMSE score is
28/30. Which of the following is the most likely diagnosis for
her hallucinations?
1. Charles bonnet syndrome
2. Anton syndrome
3. Peduncular hallucinosis
4. Diffuse Lewy body disease
An 80 year old female was referred to your clinic for
hallucinations. She has a long history of macular
degeneration and poor vision at baseline. With minimal
assistance, however she is able to live mostly independently.
She has no history of any cognitive issues. Her neurologic
exam other than poor vision is normal and her MMSE score is
28/30. Which of the following is the most likely diagnosis for
her hallucinations?
1. Charles bonnet syndrome
2. Anton syndrome
3. Peduncular hallucinosis
4. Diffuse Lewy body disease
Charles Bonnet syndrome involves visual hallucinations
without other psychopathology. It is seen in the elderly and is
due to visual loss, with the most common pathology being
macular degeneration. The other clinical exam findings are
not usually identified with Charles Bonnet syndrome.
- Anton syndrome: cortical blindness but agnostic to the fact
that they are blind
- Peduncular hallucinosis: damage to the midbrain which
causes well formed hullicinations. Usually patient knows they
are hallucinations (similar to CBS). Thought to be due to
release of reticular activating system control.
A 43 year old women is recovering from HSV encephalitis. While in
acute rehab her providers notice that she has an excessive fascination
with objects and has to touch them and often puts them in her mouth.
Prior to her hospitalization she was a very strong willed CEO of a major
company and prided herself on her physical appearance. She is now
very docile, eating an excessive amount and gaining weight. Her
symptoms are best described by which
of the following?
1. Foix-Chavany-Marie
2. Foster-Kennedy
3. Klein-Levin
4. Kluver-Bucy syndrome
A 43 year old women is recovering from HSV encephalitis. While in
acute rehab her providers notice that she has an excessive fascination
with objects and has to touch them and often puts them in her mouth.
Prior to her hospitalization she was a very strong willed CEO of a major
company and prided herself on her physical appearance. She is now
very docile, eating an excessive amount and gaining weight. Her
symptoms are best described by which
of the following?
1. Foix-Chavany-Marie
2. Foster-Kennedy
3. Klein-Levin
4. Kluver-Bucy syndrome
This syndrome is known as Klüver-Bucy syndrome and
occurs as a result of bilateral temporal lesions involving
amygdalae. Hypermetamorphosis, a common feature,
involves excessive fascination with objects in the patient's
environment and the urge to touch them all.
-Foster-kennedy : optic atrophy and anosmia due to
sphenoid or parasellar tumor
-Foix-chavany-Marie: bilateral opercular damage which
causes facial diplegia and drooling
- Klein-Levin: sleeping beauty syndrome
A 41 year old female was hospitalized for the treatment of paranoia
and personality changes. She had a history of medically refractory
epilepsy that came under very good control with lacosamide. There
was no prior psychiatric history. A routine EEG was obtained which had
a normal background rhythm and no epileptiform activity. A thorough
medical work up was negative for any toxic/metabolic/infectious cause
of her symptoms. Structural brain imaging was unchanged from
previous evaluations. Which of the following could be an explanation
for her symptoms.
1. Ictal psychosis
2. Forced normalization
3. Limbic encephalitis
4. medication induced psychosis
A 41 year old female was hospitalized for the treatment of paranoia
and personality changes. She had a history of medically refractory
epilepsy that came under very good control with lacosamide. There
was no prior psychiatric history. A routine EEG was obtained which had
a normal background rhythm and no epileptiform activity. A thorough
medical work up was negative for any toxic/metabolic/infectious cause
of her symptoms. Structural brain imaging was unchanged from
previous evaluations. Which of the following could be an explanation
for her symptoms.
1. Ictal psychosis
2. Forced normalization
3. Limbic encephalitis
4. medication induced psychosis
Forced normalization refers to a psychosis
occurring after achievement of good clinical
seizure control, or resolution of interictal
epileptiform discharges.
Seizures are basically ECT?
An 18 year old female presents with ptosis,
difficulty breathing, and poor pupillary light
response that progressed over the course of 24-48
hours. Her symptoms are most concerning for
which of the following etiologies?
1. Botulism
2. Myasthenia-gravis
3. Guillain-Barre
4. Diptheria
An 18 year old female presents with ptosis,
difficulty breathing, and poor pupillary light
response that progressed over the course of 24-48
hours. Her symptoms are most concerning for
which of the following etiologies?
1. Botulism
2. Myasthenia-gravis
3. Guillain-Barre
4. Diptheria
Botulism causes pupil dilation due to its effect on both muscarinic
and nicotinic cholinergic nerve terminals. Other disorders that may
acutely produce this constellation of symptoms, for example
myasthenia gravis, Guillain Barre syndrome and diptheria, are typically
not associated with pupillary abnormalities. Autonomic dysfunction
can be seen with Guilain Barre syndrome and diptheria but usually not
early on.
The same 18 year old patient with botulism is
admitted to the hospital and you were rightly
concerned about her ability to ventilate. Which of
the following is the best predictor of the need for
intubation?
1. Hypoxia on blood gas
2. Hypercarbia on blood gas
3. FVC
4. Drop in O2 Sat
The same 18 year old patient with botulism is
admitted to the hospital and you were rightly
concerned about her ability to ventilate. Which of
the following is the best predictor of the need for
intubation?
1. Hypoxia on blood gas
2. Hypercarbia on blood gas
3. FVC
4. Drop in O2 Sat
The best predictor to assess the need for intubation in
patients with neuromuscular respiratory failure (NMRF)
is the forced vital capacity (FVC). It accurately predicts
diaphragmatic dysfunction and the development of
atelectasis due to diminished cough. A drop in FVC is an
early indicator of respiratory failure and the need for
possible intubation. Drops in arterial oxygen saturation
and hypercarbia occur late in the setting of NMRF.
Although anti-MAG antibodies and repetitive nerve
stimulation studies may assist with the diagnosis of
demyelinating polyneuropathy and myasthenia gravis,
neither predicts the clinical severity of disease or the
need for intubation.
A 25 year old male with a history of IV drug use and a prior head injury
presents for evaluation of abnormal pupils which he noticed several
months ago. On exam he has anisicoria L>R with a great difference in
ambient light. The left pupil reacts poorly to light, but reacts very slowly
to accommodation. Instillation of dilute pilocarpine does not affect the
right eye but causes constriction of the left eye. Which of the following is
the most likely diagnosis?
1. Ocular trauma
2. Horner’s syndrome
3. Neurosyphillis
4. Tonic pupil
A 25 year old male with a history of IV drug use and a prior head injury
presents for evaluation of abnormal pupils which he noticed several
months ago. On exam he has anisicoria L>R with a great difference in
ambient light. The left pupil reacts poorly to light, but reacts very slowly
to accommodation. Instillation of dilute pilocarpine does not affect the
right eye but causes constriction of the left eye. Which of the following is
the most likely diagnosis?
1. Ocular trauma
2. Horner’s syndrome
3. Neurosyphillis
4. Tonic pupil
This patient's history is most compatible with a unilateral
Adie's tonic pupil. An Adie's pupil is typically initially large
although becomes smaller over time. It shows minimal
reaction to light but normal constriction to near, ie, lightnear dissociation. Unlike normal pupils, which will show no
response to instillation of dilute pilocarpine, Adie's pupils will
constrict to this agent due to denervation super sensitivity
from ciliary ganglion dysfunction. Unlike Adie's pupils, ArgyllRobertson pupils due to neurosyphilis are typically bilaterally
small. Adie's pupils are often associated with impaired muscle
stretch reflexes; this combination of findings is referred to as
the Holmes-Adie syndrome. Apraclonidine is used to detect a
Horner pupil which should dilate in response to this agent.
0.125%
Pilocarpine
•Normal pupil will constrict to 1%
pilocarpine, but not to dilute
pilocarpine
•A pupil that has lost parasympathetic
denervation for any reason will
become hypersensitive to dilute
pilocarpine
Tonic Pupil
• One large poorly reactive pupil
• Caused by damage to ciliary ganglion or short ciliary nerve
• Can see near light dissociation and pilocarpine hypersensitivity but these
take weeks to develop
• When associated with hypoactive deep tendon reflexes: Adie-Holms
syndrome
• Differential includes:
- idiopathic
- trauma
- infection (syphilis)
- Sjogren’s
- space occupying lesion
- migraine (transient tonic pupil)
• For an uncomplicated tonic pupil, neuro-imaging is not warranted
The most likely cause of death in acute liver
failure is what?
1. GI hemorrhage
2. Diffuse ischemic brain injury
3. Cerebral edema
4. Intraparenchymal brain hemorrhage
The most likely cause of death in acute liver
failure is what?
1. GI hemorrhage
2. Diffuse ischemic brain injury
3. Cerebral edema
4. Intraparenchymal brain hemorrhage
Patients who develop liver failure acutely are more
prone to development of hepatic encephalopathy.
In patients with hepatic encephalopathy-induced
coma, up to 80% develop cerebral edema, which is
the leading cause of death in these patients.
Which of the following tests would be most
useful in identifying frontal lobe pathology?
1. Wisconsin Card Sort Test
2. Facial Recognition Test
3. Letter fluency
4. Clock drawing
Which of the following tests would be most
useful in identifying frontal lobe pathology?
1. Wisconsin Card Sort Test
2. Facial Recognition Test
3. Letter fluency
4. Clock drawing
The Wisconsin Card Sort Test (WCST), which challenges a patient to
change cognitive sets without warning, is particularly sensitive to
frontal damage. Language skills may be unaffected, and vocabulary is
often spared. Face recognition and visual perception abnormalities
such as hemi-inattention syndromes are most often associated with
damage to the parietal, temporal, or occipital lobes, rather than the
frontal lobes. Therefore, the best answer is the WCST.
Inhibition of unwanted or inappropriate responses falls under the
rubric of executive function. In the widely-used Stroop test, subjects
are presented with names of colors written in colors other than the
one spelled out. After reading the words, subjects then must report
the color of the word seen, rather than its meaning (e.g., the word red
written in green ink), necessitating inhibition of the immediate
response to read the word as written. Trail making tests the ability to
shift sets, while letter fluency primarily tests generation of new items.
Digit span subtest tests working memory, while the CVLT (California
verbal learning test) is a test of episodic memory
Wisconsin Card Sort Test
Stroop Test
Which of the following lesions is most likely to
produce a congruous homonymous field defect?
1.
2.
3.
4.
5.
Which of the following lesions is most likely to
produce a congruous homonymous field defect?
1.
2.
3.
4.
5.
In the retrochiasmatic visual pathways,
homonymous hemianopias are most likely to be
incongruous when caused by lesions of
the optic tracts or lateral geniculate body, and
more congruous in the optic radiations and
occipital lobe. A lesion in an optic
chiasm would cause a bitemporal or junctional
visual field defect.
A patient on long term isoniazid develops diarrhea
and profound memory difficulties. Which of the
following vitamin deficiencies may be to blame?
1. Vitamin D
2. Niacin
3. vitamin E
4. B12
A patient on long term isoniazid develops diarrhea
and profound memory difficulties. Which of the
following vitamin deficiencies may be to blame?
1. Vitamin D
2. Niacin
3. vitamin E
4. B12
Niacin deficiency, commonly found in
malnourished individuals and in those taking
isoniazid, classically presents with the 3 D's:
dementia, dermatitis, and diarrhea. Other
neurologic manifestations include peripheral
neuropathy and myelopathy. Vitamin E deficiency
typically presents with cognitive dysfunction,
ataxia, and neuropathy. Can also get a B6
deficiency with INH.
Pellagra
You are seeing a 30 year old operation Iraqi
Freedom male verteran at the VA in evaluation for
TBI and headaches. When discussing sleep he
endorses that it is very interrupted because he
wakes up in the middle of the night multiple times
with vivid nightmares. Which of the following
medications may be effective in reducing this
symptoms?
1. Quetiapine
2. Prazosin
3. Zolpidem
4. Clonazepam
You are seeing a 30 year old operation Iraqi
Freedom male verteran at the VA in evaluation for
TBI and headaches. When discussing sleep he
endorses that it is very interrupted because he
wakes up in the middle of the night multiple times
with vivid nightmares. Which of the following
medications may be effective in reducing this
symptoms?
1. Quetiapine
2. Prazosin
3. Zolpidem
4. Clonazepam
This individual has traumatic brain injury (TBI) along
with flashbacks, nightmares and social avoidance
characteristic of posttraumatic stress disorder
(PTSD). Prazosin, an alpha-adrenergic blocking
agent which penetrates the blood-brain barrier, is
the medication of choice to improve sleep and to
block nightmares associated with TBI and PTSD. It
may also help other adrenergic symptoms of PTSD
as well. The other medications listed are not as
useful for this purpose and may produce cognitive
slowing and worsening daytime sleepiness.
You are seeing an 8 year old boy in clinic for
difficulty walking. On exam you notice four limb
and truncal ataxia. He has weakness in his legs and
absent deep tendon reflexes throughout. An EMG
reveals absent sensory response in the arms and
legs. His brother had a similar condition. Which of
the following will be the most likely cause of death
in this child?
1. Cardiomyopathy
2. Non-ketotic hyperosmolar hyperglycemia
3. Aspiration
4. Respiratory failure
You are seeing an 8 year old boy in clinic for difficulty
walking. On exam you notice four limb and truncal
ataxia. He has weakness in his legs and absent deep
tendon reflexes throughout. An EMG reveals absent
sensory response in the arms and legs. His brother had a
similar condition. Which of the following will be the most
likely cause of death in this child?
1. Cardiomyopathy
2. Non-ketotic hyperosmolar hyperglycemia
3. Aspiration
4. Respiratory failure
5. Immunodeficiency
Hypertrophic obstructive cardiomyopathy is the
characteristic cause of death in Friedreich ataxia.
Smaller numbers of patients may succumb to
complications of diabetes. Lymphoma and other
consequences of immunodeficiency occur at
increased rates in ataxia-telangiectasia, another
recessively inherited progressive ataxic
syndrome.
Friedreich Ataxia
• Loss of function mutation in Frataxin gene, usually GAA
repeats in both alleles
• Protein normally functions in iron trafficking around the
cell, expressed in high levels in brain, heart and pancreas
• Age of onset usually adolescence 2-70
• Most common hereditary ataxia
• Clinical features: ataxia (limb and truncal), sensory
neuronopathy (absent reflexes and sensory loss), posterior
column disease (upgoing toes and sensory loss), LE
weakness, dysarthria/dysphagia, degeneration of betz cells
(UMN weakness)
• Aside from neurologic problems also have diabetes and
cardiomyopathy (leading cause of death)
Which of the following EEG patterns portends
the worse prognosis after cardiac arrest with
resulting hypoxic ischemic encephalopathy?
1. TIRDA
2. Burst suppression
3. Reactivity
4. Diffuse beta
Which of the following EEG patterns portends
the worse prognosis after cardiac arrest with
resulting hypoxic ischemic encephalopathy?
1. TIRDA
2. Burst suppression
3. Reactivity
4. Diffuse beta
Burst suppression pattern in hypoxic ischemic coma carries an extremely poor
prognosis. Diffuse beta activity is most often associated with drugs/medications, most
commonly benzodiazepines, the effects of which are reversible. Diffuse polymorphic
delta is a nonspecific finding indicative of generalized cerebral
dysfunction/encephalopathy and IRDA (intermittent rhythmic delta activity) does not
clearly portend poor prognosis. EEG reactivity following cardiac arrest is a relatively
favorable prognostic sign compared with the absence of reactivity, which typically
carries a poor prognosis.
Excess Beta (13-30 Hz)
Burst Suppression
A neonate presents clinically with meningitis.
Their imaging is shown. What is the most
likely organism?
1. Citrobacter
2. Pseudomonas
3. E. Coli
4. Group B strep
A neonate presents clinically with meningitis.
Their imaging is shown. What is the most
likely organism?
1. Citrobacter
2. Pseudomonas
3. E. Coli
4. Group B strep
Several microorganisms including Citrobacter
species, Serratia marcescens, Proteus,
Pesudomaonas and Enterobacter can result in
neonatal central nervous infection complicated
by necrosis and abscess formation. Of these, the
Citrobacter species are the most common
microorganism.
A 45 year old male presents with facial droop upon
awakening in the morning. On exam he has
weakness of lower face and forehead. There is
no visible rash nor vesicles. He has no prior
history of rash and lives in New York City and has
never left. What is the most appropriate
treatment?
1. Prednisone
2. Valacyclovir
3. Doxycycline
4. Acyclovir
A 45 year old male presents with facial droop upon
awakening in the morning. On exam he has
weakness of lower face and forehead. There is
no visible rash nor vesicles. He has no prior
history of rash and lives in New York City and has
never left. What is the most appropriate
treatment?
1. Prednisone
2. Valacyclovir
3. Doxycycline
4. Acyclovir
This patient has signs and symptoms of an idiopathic (Bell) facial palsy.
The 2001 AAN Practice Parameter on treatment of Bell palsy
concluded that, based on the available evidence, steroids are
probably effective for improving outcome. Subsequent class I and
II studies have not shown clear additional benefit from either
acyclovir or valacyclovir for this condition, as underscored by the
updated 2012 AAN Practice Parameter on the treatment of Bell
Palsy. However, some experts believe that the addition of antivirals
to steroids provide a marginal increased benefit. Even in Lyme
endemic regions idiopathic facial palsy is more common than Lyme
related facial palsy. In the absence of a tick bite, erythema migrans
rash, or meningitis-like prodrome, Lyme neuroborreliosis is unlikely
and doxycycline is therefore not warranted unless Lyme serologies
are positive
A marathon runner is brought to the ED at the end of a
race with confusion and then has a seizure. She ate a
normal meal prior to the event and then stayed well
hydrated throughout the race by drinking water. What
is the most likely etiology the seizure?
1. Hyponatremia
2. Hyperkalemia
3. Hyperuremia
4. Hypocalcemia
A marathon runner is brought to the ED at the end of a
race with confusion and then has a seizure. She ate a
normal meal prior to the event and then stayed well
hydrated throughout the race by drinking water. What
is the most likely etiology the seizure?
1. Hyponatremia
2. Hyperkalemia
3. Hyperuremia
4. Hypocalcemia
Hyponatremia has been documented in 13% of
marathon runners. Patients can have both
systemic and neurologic symptoms related to
hyponatremia including delirium, weakness and
seizures. While hypocalcemia and hypoglycemia
could also cause altered mental status they are
less likely in this setting. Increased or decreased
potassium, while concerning from a cardiac
standpoint, would not be expected to cause
prominent mental status alterations.
A 5 year old boy presents with progressive behavioral
disturbances and cognitive decline. His parents bring
him to the ED after a tonic clonic seizure. His imaging
is show. What additional work up should be obtained
at this time?
1. ECHO
2. Audiogram
3. TSH
4. Serum cortisol
A 5 year old boy presents with progressive behavioral
disturbances and cognitive decline. His parents bring
him to the ED after a tonic clonic seizure. His imaging
is show. What additional work up should be obtained
at this time?
1. ECHO
2. Audiogram
3. TSH
4. Serum cortisol
Hypoadrenalism (Addison's) is a common feature of
adrenoleukodystrophy. This is a typical history of the progression
of adrenoleukodystrophy, once the cortical form becomes manifest.
The MRI typically shows posterior to anterior progression of
leukodystrophy, with enhancement of the advancing rim. In
contrast, PRES due to hypertension typically is acute, involves both
posterior gray and white matter, and has no enhancement of the
rim. Cardiomyopathy is associated with several neurodegenerative
conditions, most notably several mitochondrial cytopathies, but this
is not consistent either clinically or radiographically. Hashimoto's
encephalopathy may be associated with abnormal thyroid function
tests but this would not be either the clinical or radiographic
presentation.
But whosoever in this world overcomes this
wretched craving so difficult to overcome, his
sorrows fall away from him like water-drops
from a lotus leaf.
Calm is the thought, calm the word and deed of
him who, rightly knowing, is wholly freed,
perfectly peaceful and equipoised.