Transcript RITE Review - LSU Health Sciences Center

RITE Review
Sergei Kashirny, MD
02-11-2011
1. Which of the following statements about the saphenous nerve is
most accurate?
A) The saphenous nerve is a motor nerve
B) The saphenous nerve supplies sensation to the medial
aspect of the leg
C) The saphenous nerve arises from the deep peroneal nerve
D) The saphenous nerve supplies sensation to the posterior
aspect of the thigh
E) The saphenous nerve is a mixed motor and sensory nerve
B) The saphenous nerve supplies sensation to
the medial aspect of the leg
The saphenous nerve is a sensory nerve which
arises from the femoral nerve. It supplies
sensation to the medial aspect of the leg.
•
2. A 35 year-old female presents to your office accompanied by a family member,
who helps provide the history. The patient has had a 3 year history of worsening
psychiatric and neurologic issues, including odd movements and memory
problems and uncharacteristic depression. The patient is on atorvastatin for
dyslipidemia, and has no other past medical history. The patient confirms that
other family members have had similar symptoms, and some have died at an early
age. On exam, you note cognitive slowing, motor impersistence, generalized
choreiform movements, slightly impaired memory, and oculomotor dysfunction.
MRI of the brain is remarkable only for slight atrophy of the caudate bilaterally.
The patient's diagnosis was confirmed by genetic testing, which showed a CAG
trinucleotide repeat on chromosome 4.
Of the following statements, which is most accurate about this patient's disease?
•
A) The earliest cognitive change in this disorder is usually memory loss
B) Tetrabenazine can delay the progression of this disease
C) This disorder has a higher prevalence among blacks than among caucasians
or asians
D) Psychosis is common in this disease
E) The degree of chorea is a reliable marker of the severity of this disease
• D) Psychosis is common in this disease
This patient has Huntington disease (HD).
Approximately 25% of patients with HD
develop psychosis.
3.
These anterior horn cells from this 63 year old who died with ALS contain which of
the following proteins?
A) Paired helical filament Tau protein
B) TAR-DNA binding protein 43
C) Huntingtin
D) Alpha-synuclein
E) Lou Gherin
• B) TAR-DNA binding protein 43
The major component of intraneuronal
inclusions in familial and sporadic ALS
• 4. A 6-year-old male has been referred to you for further
evaluation of a possible Wilson's disease because of
dystonic gait and limbs. History taking and thorough
physical examination are carried out by you and you think
that Wilson's disease is not the correct diagnosis in this
child. Why?
•
A) Flexor planters
B) Favorable response to low dose L-dopa
C) Wing-beating tremor
D) Dysphagia
E) Cataract
• B) Favorable response to low dose L-dopa
One of the most important differential
diagnosis of Wilson's is dopa responsive
dystonia (DRD). All cases of childhood
dystonia should receive a trial of a low dose Ldopa; if they respond, the diagnosis of DRD
can be done.
• 5. Which of the following is a hallmark side effect
of valproate?
•
A) Gum hyperplasia
B) Leukopenia
C) Hair loss
D) Depression
E) Blindness
• C) hair loss
Side effects of valproate include thrombocytopenia,
weight gain, hepatotoxicity, pancreatitis,
hyperammonemia, tremor, and hair loss.
•
•
•
•
Depression is a hallmark side effect of vigabatrin
Blindness is a hallmark side effect of vigabatrin
Leukopenia is a hallmark side effect of carbamazepine
Gum hyperplasia is a hallmark side effect of phenytoin
6.
The concentric structures seen within this nerve biopsy indicate:
A) Peripheral nerve involvement in a patient with adrenomyeloneuropathy
B) Plexiform neurofibroma
C) Repeated trauma with perineurial damage
D) Repeated demyelination and remyelination
E) Hereditary neuropathy with liability to pressure palsies
• D) Repeated demyelination and
remyelination
Repeated demyelination and remyelination,
such as is seen in patients with CIDP and
HSMN1, result in these concentric structures
often referred to as "onion bulbs"
• 7. A 17-year-old high school girl has been referred from the
psychiatry department as a difficult-to-manage case of affective
psychosis. She is on chlorpromazine for the past 3 months.
Examination reveals resting tremor, rigidity, and dysarthria. Her
work-up shows ALT 61 iu/L, total serum bilirubin 2.3 mg/dl, serum
bicarbonate 14 mEq/L, blood urea nitrogen 15 mg/dl, and positive
urine glucose testing. What does the girl have?
•
A) Chlorpromazine-induced Parkinsonism
B) Wilson's disease
C) Juvenile Huntington's disease (HD)
D) Occult drug abuse
E) Chlorpromazine-induced cholestatic hepatitis
• B) Wilson's disease
Up to 20% of Wilson's disease patients may have
predominant psychiatric manifestations and
psychiatrists might be the first who see those
patients. Note the profound acidosis with normal
renal function and urine sugar (normal anion gap
metabolic acidosis of proximal renal tubular
acidosis). You may assume her liver function
abnormalities to be due to chlorpromazine
therapy, but the latter would not explain other
tests.
• 8. The damage is confined to the reticular nucleus, which is
essentially destroyed. Of the following options, what
function is most likely to be affected?
•
A) Cortical arousal
B) Vision
C) Audition
D) Emotional expression and normal memory function
E) Somatic sensation from the face
• A) cortical arousal
The reticular nucleus of the thalamus is
associated with cortical arousal
• 9. A patient with a congenital myopathy has developed
malignant hyperthermia after anesthesia. Of the
following choices, which type of congenital myopathy
is most likely to be the one that this patient has?
•
A) Central core disease
B) Fingerprint myopathy
C) Cytoplasmic body myopathy
D) Centronuclear myopathy
E) Nemaline myopathy
• A) central core disease
Malignant hyperthermia after general
anesthesia is characteristic of central core
disease. It is not characteristic of any of the
other options.
• 10. A person with a major depressive episode suffers
from depressed mood or loss of interest in pleasure for
a period of at least:
•
A) 2 months
B) 7 days
C) 3 weeks
D) 2 weeks
E) 20 days
• D) 2 weeks
The DSM-IV-TR definition of a major
depressive episode includes the specific
criterion that symptoms must be present for
at least 2 weeks.
• 11. A patient presents with a history of several months of
numbness in his fingers and toes. Prior workup, including folate,
B12, homocysteine, MHATP, RPR, and hemoglobin A1C have been
unremarkable. The patient states that he used to work for a
manufacturing company, and his job involved exposure to many
various potentially toxic compounds. Of the following choices,
which chemical is most likely to produce peripheral neuropathy in
this patient?
•
A) Aluminum
B) Bismuth
C) Cyanide
D) Manganese
E) Mercury
• E) Mercury
Mercury can produce peripheral neuropathy.
• 12. Which of the following muscles is
innervated by the median nerve?
A) Flexor digitorum profundus II
B) Infraspinatus
C) Trapezius
D) Extensor pollicis brevis
E) The palmar interossei
• A) flexor digitorum profundus II
Flexor digitorum profundus II is innervated by
the median nerve
• Infraspinatus is innervated by the suprascapular
nerve, which arises from the upper trunk of the
brachial plexus
• Trapezius is innervated by the spinal accessory nerve
• Extensor pollicis brevis is innervated by the radial
nerve
• The palmar interossei is innervated by the ulnar
nerve
13. After performing a lumbar puncture for an inpatient, the lab
calls you to tell you that the protein content of the CSF sample
is 13 mg/dl. All, but one, of the following can result in this CSF
protein value?
•
A) Removal of large volume of CSF
B) Hyperparathyroidism
C) Pseudotumor cerebri
D) CSF fistulae
E) Leukemias
• B) Hyperparathyroidism
Hyperthyroidism can result in low CSF protein,
that usually normalizes when the patient is
rendered euthyroid. Note that the definition
of low CSF protein is a protein value between
3-20 mg/dl.
14. The most common location for this tumor is:
A) Basal ganglia
B) Pineal region
C) Suprasellar
D) Cerebellum
E) Temporal lobe
• B) Pineal region
Germinoma is the most common pineal region
tumor, generally presenting in adolescent
boys.
15. Which of the following is a hallmark side effect of
oxcarbazepine?
A) Irritability
B) Hepatoxicity
C) Pancreatitis
D) Coarsening of the facies
E) Hyponatremia
E) hyponatremia
•
•
•
•
Coarsening of the facies - phenytoin
Pancreatitis - valproate
Hepatoxicity - felbamate
Irritability - vigabatrin
16. This pattern of spinal cord damage is most consistent with which of the following diseases?
A) Subacute combined degeneration
B) ALS
C) Ataxia-telangiectasia
D) Tabes dorsalis
E) Fredreich ataxia
• E) Fredreich ataxia
Combined spinocerebellar and posterior
column degeneration is characteristic of
Fredreich ataxia
• 17. A 34 male in otherwise good health is standing, telling
jokes with his roommate. In response to a humorous
statement, he begins laughing hysterically. Suddenly, he
collapses to the ground. He remains awake and alert, but is
very weak for several seconds. The most accurate term for
this phenomenon is:
A) Complex partial seizure
B) Hypnagogic paralysis
C) Cataplexy
D) Gelastic seizure
E) Non-epileptic seizure
• C) Cataplexy
This sudden loss of muscle tone, with
preserved consciousness is called cataplexy.
Cataplexy usually occurs in response to an
emotional stimulus like laughter, although less
frequently it can occur without stimulus.
• 18. You examine a child with pes cavus, diminished ankle
reflexes, scoliosis, and weakness with wasting of the calf
muscles. On nerve conduction study, you find severe slowing
of the conduction velocity. Of the following, which of the
following syndromes is the most appropriate diagnosis?
A) Charcot Marie Tooth type 2B
B) Charcot Marie Tooth type 2E
C) Charcot Marie Tooth type 1B
D) Charcot Marie Tooth type 2G
E) Charcot Marie Tooth type 2C
• C) Charcot Marie Tooth type 1B
Charcot Marie Tooth type 1 (including
subtypes A-D) is a demyelinating inherited
sensorimotor polyneuropathy. Nerve
conduction velocities are slow on NCS. It has
autosomal dominant inheritance.
Charcot Marie Tooth type 2 is a primarily axonal
sensorimotor polyneuropathy. NCS would
show normal or near-normal conduction
velocities.
19. This MRI most likely comes from a patient with which of the following findings?
A) Ataxia, nystagmus, jaundice, and insomnia
B) Confabulation, nystagmus, vertigo, and nausea
C) Ataxia, telangiactasias, dementia, and hallucinations
D) Confusion, nuchal rigidity, headache, and nausea
E) Ophthalmoparesis, nystagmus, confusion, and ataxia
E) ophthalmoparesis, nystagmus, confusion, and ataxia
This FLAIR MRI shows hyperintensity in the medial thalamic
nuclei. This finding is characteristic of both Wernicke
encephalopathy and Korsakoff dementia. The typical
symptoms of Wernicke encephalopathy include
ophthalmoparesis, nystagmus, ataxia, and a change in mental
status characterized by confusion and apathy. Wernicke
encephalopathy and Korsakoff dementia are both due to a
deficiency of thiamine, and are both more common in
alcoholics. Treatment of Wernicke encephalopathy requires
administration of Thiamine. Adams and Victor recommend
50mg IV and 50mg IM immediately, followed by 50mg IM
daily.
• 20. A patient you see in clinic is suffering from major
depression. You start him on an antidepressant. A week later,
you receive a panic-stricken phone call from him. He
complains that he has a painful erection that has persisted for
the last 24 hours. Of the following medications, which is most
likely to have caused this reaction?
A) Bupropion
B) Tranylcypromine
C) Amitriptyline
D) Trazodone
E) Duloxetine
• D) Trazodone
Priapism is a rare but well-known side effect
of trazodone.
21. "I ate his liver with fava beans and a fine chianti." - H. Lecter
Hannibal Lecter is cast in a Silence of the Lambs sitcom spinoff complaining of
a severe headache, nausea, dizziness, and blurred vision. His skin is a dark
yellow, although his sclera are anicteric. Funduscopic evaluation
demonstrates swollen, edematous optic nerves bilaterally. LP opening
pressure is elevated, but CSF composition is normal and there is no
evidence of infection. CT and MRI are both normal. Oddly, he has no
eyebrows. From what neurological or metabolic disorder does Dr. Lecter
suffer, and what unusual dietary habits may have led to this presentation?
A) Wernicke's encephalopathy / chronic abuse of a nice chianti
B) Creutzfeldt-Jakob disease / remote ingestion of infected neural tissue
C) Anemia of glucose-6-phosphate dehydrogenase deficiency / ingestion of
fava beans
D) Hypervitaminosis A / overconsumption of liver
E) Malingering in order to entrap and eat the examiner / chronic cannibalism
• D) Hypervitaminosis A / overconsumption of
liver
At very high levels of vitamin A consumption
(>25000 IU/day), vitamin A toxicity results in
decreased CSF absorption, leading to elevated
intracranial pressure and papilledema
(pseudotumor cerebri). Early symptoms of
hypervitaminosis A include sparsely
distributed, coarse hair and a specific alopecia
of the eyebrows. In this case, apparent
jaundice of the skin is due to high levels of
carotenoid pigment.
• 22. A 16-year-old male has been referred to the neurology
outpatient clinic because of "recurrent loss of consciousness."
He has reeling gait, lost ankles, and up-planters with pes
cavus. His speech is dysarthric. Which one of the following is
consistent with the diagnosis of Friedreich's ataxia?
A) Bilateral ptoses
B) Wasting of right thenar eminence and left-sided wrist
drop
C) Recurrent hypoglycemia
D) Minimental status examination score of 29
E) Retinal AV nipping and silver-wiring of retinal arterioles
D) Minimental status examination score of 29
Friedreich's ataxia is not a dementing illness;
therefore MMSE score is expected to be
normal. Note that his lapses of consciousness
are due to cardiac involvement with
dysrrhythmias (and are not due to seizures).
• 23. A 85 year-old female presents to your office accompanied by her
young boyfriend, who provides most of the history.
The boyfriend states that for quite some time, the patient has had trouble
keeping his balance, has been increasingly slow getting around, has
complained of seeing visions of odorless brown doves, has had periods
when she is lethargic and drowsy, and others when she is more alert, has
complained of a tremor in his hands, and has been increasingly forgetful.
Which of the following pathology findings is MOST characteristic of this
patient's disease?
A) Inclusions comprised primarily of Huntingtin
B) Inclusions comprised primarily of alpha-synuclein
C) Lesions in the mammillary bodies
D) Punctate hemorrhages in periaqueductal grey and in the grey
matter surrounding the 3rd and 4th ventricles
E) Neurofibrillary tangles,
B) inclusions comprised primarily of alpha-synuclein
This patient's history is consistent with dementia with Lewy
bodies (DLB). Patients with DLB develop Parkinsonian
symptoms and dementia symptoms at approximately the
same time. Other key clinical features include recurrent visual
hallucinations and fluctuations in mental status. Lewy bodies
are eosinophilic cytoplasmic inclusions which contain alphasynuclein.
• Huntingtin is a protein associated with Huntington chorea.
• Lesions in the mammillary bodies are seen in Korsakoff amnestic state.
• Punctate hemorrhages in periaqueductal grey and in the grey matter
surrounding the 3rd and 4th ventricles are seen in Wenicke
encephalopathy.
• Neurofibrillary tangels are more commonly associated with Alzheimer
disease.
24. Which of the following muscles is innervated by the
radial nerve?
A) Lumbricals III
B) Serratus anterior
C) Biceps brachii
D) Flexor digitorum profundus I
E) Extensor carpi ulnaris
E) extensor carpi ulnaris
Flexor digitorum profundus I - median nerve
Biceps brachii - musculocutaneous nerve
Serratus anterior - long thoracic nerve, which
arises from the brachial plexus
Lumbricals III - ulnar nerve
25. A 45 year-old immunocompetent female presents
with bacterial meningitis. Of the following options,
which is the most likely pathogen?
A) E. coli
B) S. pneumoniae
C) L. monocytogenes
D) S. agalactiae
E) H. influenzae
B) S. pneumoniae
S. pneumoniae and N. meningitidis are the most
common pathogens isolated from CSF in
immunocompetent patients aged 18 to 50 years who
have bacterial meningitis.
S. agalactiae, E. coli, and L. monocytogenes are the
most common pathogens in infants less than 2
months of age. L. monocytogenes is also commonly
seen in patients more than 50 years old, or patients
with impaired cellular immunity. H. influenzae is
more commonly seen in patients 4 months to 17
years of age.
END