Question 1.
An 8-year-old girl is brought to the pediatric clinic by her parents because of a 6 month history of hair-pulling which has left a baldspot on her head. Her parents deny that she is eating her hair. They state that she appears to be pulling on her hair compulsively. Which of the following is the most appropriate first-step for treatment?
Clomipramine
Clomipramine is a tricyclic anti-depressant. Clomipramine is useful in the treatment of major depressive disorder and obsessive-compulsive disorder. While it can be used to treat trichotillomania, clomipramine is a second-line therapy for trichotillomania due to its side effect profile. Side effects include anti-cholinergic syndrome.
Phenelzine
Phenelzine is a monoamine oxidase inhibitor and is used to treat major depressive disorder and anxiety. Patients on monoamine oxidase inhibitors need to carefully monitor their diet. Consumption of tyramine rich foods such as cheese, cured meats, and red wine can induce a hypertensive crisis.
Cognitive behavioral therapy
Major takeaway
Trichotillomania is an impulsive disorder in which patients experience anxiety which is relieved by the pulling of their own hair. First line therapy of choice is cognitive behavorial therapy.
Main explanation
Trichotillomania is an impulsive disorder in which patients experience anxiety which is relieved by the pulling of their own hair. This can lead to bald spots on their scalp. While the scalp is the most visible, patients with trichotillomania may also pluck hairs from their eyebrows and pubic regions. Trichotillomania occurs in 1-3% of the population and is more common in females than in males. Trichotillomania mainly affects the pediatric and adolescent population and occurs as a result of a stressful event. These patients should be screened for other comorbid conditions such as obsessive-compulsive personality disorder, mood disorders, and borderline personality disorder. This is a egodystonic disorder and patients with trichotillomania experience distress with their condition.
First-line therapy for trichotillomania is cognitive behavioral therapy. Cognitive behavioral therapy teaches the patient to be aware of the patient’s condition, understand the trigger and stimulus behind the need to pull their hair, and learn relaxation behavior to reduce hair pulling. Other medications used for trichotillomania include selective serotonin re-uptake inhibitors, tricyclic antidepressants, anti-psychotics, and lithium. Studies done show that selective serotonin re-uptake inhibitors are no more efficacious compared to placebo. Clomipramine has been shown to be effective but side effects include anti-cholinergicsyndrome.
Fluvoxamine
Fluvoxamine is a selective serotonin re-uptake inhibitor (SSRI). SSRI function by binding to the serotonin re-uptake transporter and inhibiting the re-uptake of serotonin. This increases the concentration of serotonin within the pre-synaptic cleft. Fluvoxamine can be used to treat obsessive-compulsive disorder, major depressive disorder, and anxiety.
Clozapine
Clozapine is a powerful atypical anti-psychotic used to treat patients with medication-refractory schizophrenia. Clozapine, however, can cause metabolic syndrome and life-threatening agranulocytosis. For this reason, patients on clozapine should have regular complete blood count tests done. Other side effects include extrapyramidal symptoms and anti-cholinergic syndrome.
Question 2.
A 55-year-old man comes to the clinic because of muscle weakness for the past two months. His temperature is 37.8°C (100°F), pulse is 72/min, respirations are 18/min, and blood pressure is 110/82 mm Hg. Physical examination shows wasting of the muscles in his hands and a positive Babinski sign. Which of the following is the most appropriate next step in management?
Albuterol
Salbutamol, also known as albuterol, shifts potassium back into cells and is used after the risk for arrhythmia. It is also used to treat high blood potassium levels, asthma, exercise-induced bronchospasm, and chronic obstructive pulmonary disease (COPD).
Bicarbonate
Bicarbonate is an intermediate form in the deprotonation of carbonic acid. Bicarbonate helps shift potassium back into cells and is used for the risk of arrhythmia, and for the use of calcium gluconate to stabilize the membrane.
Dantrolene
Dantrolene sodium is a postsynaptic muscle relaxant that lessens excitation-contraction coupling in muscle cells. It is the primary drug used for the treatment and prevention of malignant hyperthermia, neuroleptic malignant syndrome, muscle spasticity, and 2,4-dinitrophenol poisoning.
Riluzole Show explanation
Major takeaway
Riluzole is a drug used to treat amyotrophic lateral sclerosis (ALS) and it works by decreasing presynaptic glutamate release, thereby limiting cytotoxic effects of this neurotransmitter. ALS is a disorder that involves the death of neurons that control voluntary muscles and is characterized by stiff muscles, muscle twitching, and gradually worsening weakness.
Main explanation
Amyotrophic lateral sclerosis (ALS or Lou Gehrig disease) is a disorder that involves the death of neurons that control voluntary muscles. The cause is not known in 90%-95% of cases and about 5–10% of cases are inherited from a person’s parents. A defect on chromosome 21, which codes for superoxide dismutase 1 (SOD1), is associated with about 20% of familial cases of ALS, or about 2% of ALS cases overall. Diagnosis is made with clinical reference, with a nerve conduction study, and electromyography. ALS is characterized by stiff muscles, muscle twitching, and gradually worsening weakness due to muscle atrophy and wasting. This results in difficulty speaking, swallowing, and breathing. No cure for ALS is known but it is often treated with riluzole in attempts to slow the progression of the disease. Riluzole works by decreasing presynaptic glutamate release, thereby limiting cytotoxic effects of this neurotransmitter. Non-invasive ventilation results in improved quality and length of life. ALS starts around the age of 60 and in inherited cases around the age of 50. The average survival from onset to death is 3-4 years. About 10% survive >10 years and most die from respiratory failure.
Succinylcholine
Succinylcholine is a medication used to induce muscle relaxation and short-term paralysis, usually to help with tracheal intubation. It is used in combination with pain medications and sedatives for euthanasia.
Question 3.
A 22-year-old woman comes to the gynecology clinic because of an increase in vaginal discharge. She describes the discharge as thin and grayish-white, with a strong fishy odor. She has been in a monogamous relationship for the last several years. Which of the following findings would most likely be expected on exam?
Negative whiff test
A positive whiff test, performed by adding potassium hydroxide to a vaginal prep, is considered to be pathognomonic of bacterial vaginosis.
Potassium hydroxide preparation with branching hyphae and spores
Infection with Candida albicans can give rise to symptoms of pruritus, burning, dysuria, dyspareunia, and “cottage cheese” discharge. Diagnosis is usually made by microscopic examination of a potassium hydroxide preparation of the vaginal discharge in which branching hyphae and spores are visualized.
Strawberry cervix
Cervical erythematous punctate epithelial papillae, or “strawberry” cervix, is characteristic of infection with Trichomonas vaginalis. This unicellular, anaerobic flagellated protozoan is transmitted sexually and gives rise to a yellow, gray, or green discharge that may be frothy in appearance, and gives off an unpleasant odor.
Wet prep with clue cells
Major takeaway
Bacterial vaginosis develops when there is disruption in the normal balance of bacterial florain the vagina. Diagnosis can be made with a wet prep of a vaginal swab that reveals clue cells, or vaginal epithelial cells that are diffusely covered with bacteria.
Main explanation
The clinical picture described is classic for bacterial vaginosis. Bacterial vaginosis develops when there is disruption in the normal balance of bacterial flora in the vagina. Although bacterial vaginosis is thought to be polymicrobial, Gardnerella vaginalis is commonly present in cultures. Many patients with bacterial vaginosis are asymptomatic; however, those that are symptomatic often complain of a profuse, nonirritating discharge that is characteristically thin, grayish-white, and carries a fishy amine odor. Diagnosis can be made with a wet prep of a vaginal swab that reveals clue cells, or vaginal epithelial cells that are diffusely covered with bacteria. Bacterial vaginosis is typically treated with metronidazole or clindamycin.
Wet prep with flagellated protozoa
Trichomonas vaginalis is a flagellated protozoan is transmitted sexually and gives rise to a yellow, gray, or green discharge that may be frothy in appearance and gives off an unpleasant odor. Diagnosis is made via microscopic examination of wet preps of vaginal swabs in which the flagellated protozoa are visualized.
Question 4.
A 46 year old man presents with left-sided “weakness.” He first noticed symptoms one month ago after he went Bungee jumping and “tweaked his back.” It started with flaccid paresis of the left side of his body, below L1. Now it has become a spastic paresis and it is accompanied by hyperreflexia, and a positive Babinski sign on his left foot. He has also lost the left side position sense, vibrationsense, and tactile discrimination below L1. Pain and temperature sensation are spared on the left side, but are lost on the right side.
Which is the most likely diagnosis?
Brown-Séquard syndrome
Main explanation
Brown-Séquard syndrome or spinal cord hemisection syndrome is a rare and usually incomplete syndrome. Its most common causes are spinal trauma or disk herniation. It is the hemisection of the spinal cord. The interruption of the descending motor pathways on one side of the spinal cord causes an initially flaccid, ipsilateral paresis below the level of the lesion, which later becomes spastic and is accompanied by hyperreflexia, Babinski signs, and vasomotor disturbances. Meanwhile, the interruption of the posterior columns of one side of the spinal cord causes ipsilateral loss of vibration and position sense, and tactile discrimination below the level of the lesion. On the contralateral side there is a segmental loss of pain and temperature sensations, one to two segments below the level of the hemisection; this is due to interruption of the spinothalamic fibers that cross on the anterior white commissure. Simple tactile sensation is not impaired.
Ch 01 05 Brown Sequard Syndrome
by duc nguyen
2:52
YouTube
Major Takeaway:
Brown-Séquard syndrome, or spinal cord hemisection syndrome, is mostly due to trauma to one side of the spinal cord, causing:
• ipsilateral loss of vibration and spastic paresis below the lesion
• ipsilateral flaccid paralysis at the level of the lesion
• contralateral loss of pain and temperature sensation below the lesion
Wallenberg syndrome
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Dejerine syndrome
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Horner syndrome
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Question 5.
At a psychiatric hospital, a resident first meets with an anxious patient that gasps before talking then talks at a hurried pace with elevated speech. Then, she meets with a depressed patient who gasps, then with resignation speaks slowly and in a low register. Then, she startles a schizoaffective patient with a history or anger and violences, who lashes back in a forceful and loud voice. Finally, she meets a patient with avoidant personality disorder who speaks in a very soft voice about her fears of rejection. What is the name for these non-verbal signs the resident has observed from this variety of patients with different diagnoses?
Paralanguage
Major takeaway
Paralanguage is the use of non-verbal communication to fill spaces in communication e.g. gasping.
Main explanation
Paralanguage is the use of non-verbal communication to fill spaces in communication. Kinesics is the use of “body language” writ large and not necessarily just to fill spaces in communication (e.g. like punctuation).
Social history
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Question 6.
A 58-year-old man comes to the clinic because of an intermittent headache for the past six months. He says the pain has steadily worsened and is unresponsive to over-the-counter pain medication. He denies any lateralization of the pain or any specific localization. “My head just hurts,” he exclaims. Physical examination shows oversized hands and jaw. His lateral vision is quite restricted bilaterally. Which of the following findings is most likely in this patient?
Acromegaly
Major takeaway
Pituitary tumors tend to be characterized by a new-onset headache and bitemporal visual field loss. They can also secrete hormones including growth hormone, which could causeacromegaly in adults.
Main explanation
A new onset headache in a person over 50 is usually quite worrisome. If such a headache is lateralized, it suggests the possibility of temporal arteritis. If such a headache has little localization, a neoplastic process should rise on the differential diagnosis. Loss of peripheral visual fields on both the right and left is called bitemporal hemianopsia. Bitemporal hemianopsia results from an injury to the crossing fibers at the optic chiasm. A crush injury to the optic chiasm typically results from the presence of a pituitary tumor. Pituitary adenomas can secrete their hormones at supraphysiologic concentrations. When a growth hormone secreting tumor is present in adults, it can present as acromegaly. Acromegaly involves an overgrowth of various tissues, most notably the hands, brow, and mandible. The hands particularly have a “doughy” quality from soft tissue overgrowth. Thus, it has been said that you can “diagnose acromegaly by shaking hands.” The hand on the right is from a patient with acromegaly.
Paget disease of bone
Paget disease of bone is characterized by an accelerated rate of bone remodeling and overgrowth or deformity of the affected bone. Pain, fractures, and arthritis are the most common clinical findings.
Primary hyperparathyroidism
Primary hyperparathyroidism leads to chronic hypercalcemia that is usually asymptomatic. “Bones, stones, abdominal moans, and psychic groans” are an uncommon, but classically described manifestation.
Pseudogout
Pseudogout is also called acute calcium pyrophosphate crystal arthritis. Crystal formation may reflect elevated concentrations of either calcium or inorganic pyrophosphate and is initiated near the cartilage.
Thenar pseudohypertrophy
Pseudohypertrophy refers to muscular enlargement through deposition of fat instead of muscle fibre, as occurs in muscular dystrophy. Thenar pseudohypertrophy is associated with X-linked Becker muscular dystrophy.
Question 7.
An 85-year-old man comes to the outpatient clinic because of bad breath, regurgitation of food overnight, and trouble swallowingsolids. For several months he has had these symptoms. He denies fever, chills, nausea, vomiting, or weight loss. The patient does not smoke cigarettes and does not drink alcohol. Examination shows sensation of fullness in the neck and halitosis. His BMI is 40 kg/m2. Which of the following is the most appropriate next step in management?
Barium swallow with fluoroscopy
Major takeaway
Zenker diverticulum is a herniation of mucosal tissue at the Killian triangle. It is best diagnosed on barium swallow fluoroscopy.
Main explanation
Zenker diverticulum is an out-pouching of pharyngeal mucosa through an acquired defect in the muscular wall (false diverticulum). Zenker diverticulum is due to the herniation of mucosal tissue at the Killian triangle between the thyropharyngeal and cricopharyngeal parts of the inferior pharyngeal constrictor. Patients can be asymptomatic or characterized by dysphagia, obstruction, and halitosis. This condition most common in elderly males, although the reasonfor this epidemiological finding is unclear.
If the patient does have Zenker diverticulum, the barium swallow fluoroscopy will show an (intermittent) out-pouching arising from the midline of the posterior wall of the distal pharynxnear the pharyngoesophageal junction. The pouch is best identified during swallowing and is best seen on the lateral view, on which the diverticulum is typically noted at the C5-6 level.
CT of head and neck
While CT head and neck may be helpful in ruling out malignancies, this patient is more likely to have a Zenker diverticulum than esophageal cancer because he does not smoke, drink, or have weight loss.
Exploratory laparotomy
Exploratory laparotomy is invasive, expensive, and not the best next step in management. While surgery is the treatment for this condition, Zenker diverticulum (ZD) should be diagnosed prior to any invasive treatment. ZD is usually diagnosed with a barium swallow combined with a dynamic continuous fluoroscopy during the swallowing process.
MRI of head and neck
MRI of the head and neck is excessive and expensive for the diagnosis of a Zenker diverticulum (ZD). ZD is usually diagnosed with a barium swallow combined with a dynamic continuous fluoroscopy during the swallowing process.
Ultrasound of the neck
While ultrasound of the neck may help diagnose a Zenker diverticulum, it is not the preferred method of diagnosis. It is a good alternative in patients who experience difficulty swallowing barium.
Question 8.
A 7-year-old boy comes to the emergency department because the teacher noticed he was having “staring spells” and was not paying attention well in school. His mother says he is otherwise healthy, but has been struggling in school recently. An electroencephalogram shows spike-and-slow wave complexes. Other laboratory tests and imaging show no abnormalities. Which of the following is the most appropriate first pharmacologic treatment for this patient?
Ethosuxamide
Major takeaway
Absence seizures are typified by staring spells which the patient is not aware of, and no signs of other seizure like activity. Treatment is with ethosuxamide.
Main explanation
Absence seizures are characterized by a brief loss and return of consciousness with not period of lethargy following. Staring spells with no post-ictal state are most consistent with absence seizures. These seizures are diagnosed with an electroencephalogram. Absence seizures may be treated with ethosuxamide if present in isolation. Ethosuxamide acts on the T-type calcium channels that are involved in this specific type of seizure. Ethosuxamide is not effective in the treatment of other types of seizures. Valproic acid is recommended for use if a patient exhibits multiple seizure types. Lamotrigine may also be used for patients who suffer from multiple types of seizures.
Amphetamine salts
Amphetamine salts are commonly used for the treatment of attention deficit hyperactivity disorder, and are not used for the treatment of absence seizures.
Gabapentin
Gabapentin is used to treat neuropathic pain, hot flashes, restless leg syndrome, and epilepsy, specifically partial seizures. It is not indicated in the treatment of absence seizures.
Methylphenidate
Methylphenidate, a stimulant medication like amphetamine salts, are used to treat attention deficit hyperactivity disorder. They are not useful in the treatment of absence seizures.
Phenytoin
The medications phenytoin, carbamazepine, and gabapentin have been shown to worsen absence seizures, and should not be used in this patient.
Question 9.
A 65-year-old man comes to the urgent care clinic with his wife who describes dramatic changes in the personality of her husband over the past two years. Specifically, she mentions that he has become increasingly aggressive, hypersexual, and easily agitated. As part of the initial workup, a non-contrast head CT is ordered which shows focal, severe atrophy most predominantly affecting the frontal and temporal lobes. The clinical and imaging findings most likely represent which of the following diagnosis?
Alzheimer's disease
Alzheimer’s disease is the most common cause of dementia, yet remains a diagnosis of exclusion. The disease is characterized predominantly by amnesia, and progresses insidiously over time. Positron emission tomography (PET) of the brain may demonstrate bilateral temporoparietal hypometabolism, but these findings are nonspecific.
Creutzfeldt-Jakob disease (CJD)
Creutzfeldt-Jakob disease is an extremely rare but progressive prion disease that causes a subacute dementia combined with ataxia and myoclonic jerks. MRI may demonstrate increased T2/FLAIR signal within the putamen and caudate head, but definitive diagnosis can only be made at autopsy. There is currently no effective treatment.
Normal Pressure Hydrocephalus (NPH)
Normal pressure hydrocephalus is a potentially treatable form of dementia theorized to be caused by impaired cerebrospinal fluid(CSF) outflow from the brain. Patients present with incontinence, ataxia, and dementia (wet, wild, and wacky). CT or MRI of the brain demonstrate ventricular enlargement out of proportion with global atrophic changes.
Pick's Disease
Major takeaway
Frontotemporal dementia is a rare, progressive form of dementia with no cure. The most common variant is behavioral which is characterized by loss of social inhibition, ritualized behaviors, apathy, aggression, and lack of insight. CT and MRI of the head will show focal atrophy in the frontal and temporal lobes.
Main explanation
Frontotemporal dementia, also known as “Pick’s disease”, is a relatively rare but progressive form of dementia that is characterized by significant changes in patient behavior and personality, typically within a period of months to years. There are behavioral and language variants of this dementia, with behavioral variant the most common. Neuroimaging, including CT and/or MRI examinations of the brain, will demonstrate severe atrophy within the frontal and temporal lobes, bilaterally. On autopsy, pathologic examination may reveal round intraneuronal inclusion bodies (also called “Pick bodies”), which are diagnostic. There is no curative therapy for frontotemporal dementia, therefore, supportive treatment by a multidisciplinary team, and family education, are central to management.
Vascular Dementia
Vascular dementia is caused by repeated micro- or macro-infarctions, leading to a stepwise increase in cognitive impairment. Patients will often have history of stroke, hypertension, diabetes, and hyperlipidemia. CT or MRI of the brain will demonstrate evidence of chronic small vessel ischemic and potentially multiple prior infarctions.
Question 10.
A 64-year-old woman comes to her primary care provider’s office because of a droopy left eyelid for the past 2 weeks. The patient’s husband states that he has noticed that her left pupil is smaller than her right pupil. The patient also reports of an inability to sweat on the left side of the face. On physical examination, her right pupil dilates when the lights are turned off but the left pupil does not. Which of the following is the most likely diagnosis?
Bell palsy
Bell palsy is a term used to describe a facial nerve (cranial nerve VII) dysfunction of unknown etiology. Patients with Bell palsy will experience facial drooping and an inability to raise their eyebrow.
Horner's syndrome
Major takeaway
Horner syndrome is caused by a disruption of the cervical or thoracic sympathetic chain. The classic triad of Horner syndrome is ptosis, anhidrosis, and miosis.
Main explanation
Horner syndrome is a constellation symptoms due to a lesion or compression of one side of the cervical or thoracic sympathetic chain which innervates the face. The first neuron of this sympathetic chain originates from the hypothalamus and travels down the lateral aspect of the brain stem and spinal cord until it synapses at the lateral horn at the level of C8/T1. The second neuron travels along the carotid artery until it synapses at the superior cervical ganglion deep to the internal carotid artery. The third neuron then carries the sympatheticfibers to the rest of the face. Any disruption to this sympathetic chain will result in Horner syndrome. Patient’s with Horner syndrome will have ipsilateral ptosis, miosis, and anhidrosis. Etiologies of Horner syndrome include superior sulcus lung tumors, syringomyelia, and multiple sclerosis.
Dorsal midbrain syndrome
Dorsal midbrain syndrome, or Parinaud syndrome, is a condition in which a patient loses their upward gaze. This classically seen in patients with pineal gland tumors.
Tonic Pupil
Tonic pupil, or Adie syndrome, is a disease which classically affects middle aged women. The etiology of the condition is unknown but is thought to be related to a bacterial or viral infection. Patients with a tonic pupil will have mydriasis with decreased deep tendon reflexes.
CN III palsy
Cranial nerve III, or the oculomotor nerve, is responsible for most of the extrinsic muscles of the eyes except for the superior oblique (cranial nerve IV) and the lateral rectus (cranial nerve VI) muscles. A cranial nerve palsy will present as a pupilwhich is “down and out.”