Pathology Exam 11

D (this is tabes dorsalis, or tertiary syphilis, leading to loss of sensation in the posterior columns of the spinal cord, changes in personality and intellect, and the Argyll Robertson pupils, which constrict in response to accomodation, but not to light [also called light-near dissociation])

Which of the following conditions would lead to a selective degeneration of the shaded area of the spinal cord? A. Poliomyelitis B. Amyotropic lateral sclerosis C. Multiple sclerosis D. Neurosyphilis E. Creutzfeldt-Jakob disease

E (this is a transient ischemic attack - since it resolved on its own without lasting damage, it can be classified as a TIA, whose etiology best matches reduced cerebral perfusion, or low blood flow)

A 67-year-old male with a past medical history of hyperlipidemia presents with an episode of weakness in his right arm. The patent states that it resolved after "shaking it around for 15 minutes". Physical examination reveals upper extremity strength to be 5/5 bilaterally. The most likely associated etiology in this patient is due to A. Demylienating disease B. Emboli C. Ischemia with infarction D. Lipohyalinosis E. Low blood flow

D (this is MS once again - I just wanted to lay a trap answer as the first option - peripheral demyelination is a good description of Guillan Barre syndrome, but multiple sclerosis is central demyelination)

A 28-year old woman presents to her physician's office complaining of episodes of weakness and paresthesias for the past few years that resolve on their own but come back several weeks later. She often has trouble walking and falls easily, and she cannot always make it to the bathroom in time to urinate. Her vision has been blurry as well. Which of the following is the most likely etiology of her disease? A. Peripheral demyelination B. Congenital C. Infectious D. Autoimmune E. Traumatic

B (this is metachromic leukodystrophy - not much to say other than that it's stainable [hence "chromic"] and presents super early - oh and it's autosomal recessive)

A 3-year-old child has gait abnormalities, absent deep tendon reflexes on both legs. Labs show a increases CSF protein with normal glucose and cell count, and a urine sediment that stains positive with toluidine blue. What is the genetic inheritance of the most likely disease? A. Autosomal dominant B. Autosomal recessive C. X-linked dominant D. X-linked recessive E. Mitochondrial

A (this is a neurofibroma, which when present in the patient and their parent, reflects autosomal dominant inheritance of a chromosome 17 deletion - this syndrome is associated with more neural cell tumors and Lisch nodules)

A 1-year-old boy presents with multiple oval shaped, coffee-colored macules on his lower right abdomen. His mother also has a 7.5 cm cafe-au-lait patch on her right hip and smaller spots on her back that she refers to as freckles. What is the name for this familial syndrome? A. Neurofibromatosis type I B. Neurofibromatosis type II C. Von-Hippel-Lindau syndrome D. Li-Fraumeni syndrome E. Tuberous sclerosis

D (this is an X-linked mutation of dystrophin, causing muscle cells to be replaced by fatty tissue [this is why the calves seem enlarged - "pseudohypertrophy"] - findings also include elevated CK in the first decade of life - death occurs by age 30)

A 2-year-old boy presents with fatigue and difficulty with walking. The mother describes her child as "clumsy," as he usually falls while walking. The mother also reports difficultly with climbing the stairs, which is new. The mother recalls that a relative died in his teens and also experienced muscle weakness when he was very young. On physical exam, there is weakness in the proximal muscles of the lower extremity, bilateral enlargement of the calves, and the patient is noted to push off from the floor with his hands in order to begin walking. What is the most likely diagnosis? A. Friederich's ataxia B. Dermatomyositis C. Polymyositis D. Becker's muscular dystrophy E. Duchenne's muscular dystrophy

B (pathogens of meningitis are age-related, and a petechial rash in a college aged patient with signs of meningitis is Neisseria meningitidis - this is a preventable illness from vaccines, and complications include purpura, DIC, and Waterhouse-Friderichsen syndrome [acute adrenocortical hemorrhage])

A 20-year-old college student is brought to student health by his roommate who is concerned that his friend is not doing well. The patient is minimally responsive. You learn from the roommate that the patient has been complaining of a stiff neck and a headache recently and that he has kept to himself in his room with the lights off. On physical exam you are alarmed when you find a diffuse petechial rash and a blood pressure of 80/40 mmHg. What is the most likely pathogen of this patient's pathology? A. Listeria B. Neisseria C. Streptococcus D. Haemophilus E. Toxoplasma

B (young woman with sudden monocular blindness is immediately setting off bells for multiple sclerosis - especially since there are recurrent attacks with a wide variety of symptoms - the oligoclonal bands in CSF reflect the autoimmune antibodies that attack the body's myelin and cause a slew of neurological symptoms)

A 23-year-old female presents to the emergency department with monocular blindness. She states that early this morning she lost her vision seemingly "out of nowhere." She denies trauma or any precipitating factors. She does state though that over the past year she has had occasional episodes of weakness and even an episode of urinary incontinence, which always resolve on their own. On exam, pain is elicited with eye movement and nystagmus is appreciated. The emergency physician performs a lumbar puncture. What is most likely to be observed in the CSF of this patient? A. Increased opening pressure B. Oligoclonal bands C. Albuminocytologic dissociation D. Increased lymphocyte count E. Normal findings

E (the diagnosis is Dandy-Walker malformation, a hypoplasia of the cerebellum - as with all cerebellar lesions, the chief finding would be ataxia, in this case truncal since the vermis is affected)

A 24-year-old female at 20 weeks' gestation presents for routine prenatal ultrasound. Results of the ultrasound indicate the presence of a small cerebellar vermis and enlargement of the fourth ventricle. The expected neurologic finding for this fetus later in life is A. Decreased vibratory sensation B. Deficits in pain and temperature sensation C. Hyperreflexia D. Spasticity E. Ataxia

E (the stem describes Dandy-Walker malformation, a hypoplasia of the cerebellar vermis due to a cystic dilation of the fourth ventricle - another way to think of it is that the posterior fossa becomes a cyst of CSF, which is what option E describes) (A; this is craniosynostosis, or brachycephaly) (B; this is Chiari II malformation) (C; this is Chiari I malformation) (D; this is holoprosencephaly)

A 24-year-old female at 20 weeks' gestation presents for routine prenatal ultrasound. Results of the ultrasound indicate the presence of a small cerebellar vermis and enlargement of the fourth ventricle. The most likely additional finding includes A. Bilateral fusion of the coronal sutures B. Descent of the cerebellar tonsils, the inferior vermis, and the inferior cerebellar hemispheres into the spinal canal C. Downward displacement of the cerebellar tonsils through the foramen magnum D. Fusion of the two cerebral hemispheres at the midline E. Presence of a cyst near the internal base of the skull

D (Guillain-Barre syndrome is an immune-mediated demyelinating neuropathy that presents with ascending paralysis, areflexia and spared sensation that follows a bacterial infection, typically Campylobacter) (the CSF finding for Guillain Barre is called albuminocytologic dissociation, which just means that the protein is elevated but the white blood cells are not [the reason is because this is an inflammatory process but it is not infectious])

A 26-year-old man presents to his primary doctor with one week of increasing weakness. He reports that he first noticed difficulty walking while attending his sister's graduation last week, and yesterday he had difficulty taking his coffee cup out of the microwave. He remembers having nausea and vomiting a few weeks prior, but other than that has no significant medical history. On exam, he has decreased reflexes in his bilateral upper and lower extremities, with intact sensation. If a lumbar puncture is performed, which of the following results are most likely? A. High neutrophils, high protein, low glucose, high opening pressure B. High lymphocytes, normal protein, normal glucose, normal opening pressure C. High lymphocytes, high protein, low glucose, high opening pressure D. Normal cell count, high protein, normal glucose, normal opening pressure E. Normal cell count, normal protein, normal glucose, normal opening pressure

D (the patient is an immigrant and former prisoner with signs of meningitis and pulmonary symptoms - wham, tuberculous meningitis, caused by Mycobacterium tuberculosis, so expect acid fast staining)

A 27-year-old immigrant who was just released from prison presents with symptoms that have progressed slowly over the past few months. He currently complains of neck stiffness, photophobia and a headache that have very slowly been worsening. Past medical history is not known, though he does state that he had a cough in his home country of Uzbekistan that was succesfully treated by his grandmother with goats milk. On presentation he currently still has a cough. Upon completing his lumbar puncture, the best stain to perform on the CSF would be? A. Gram B. Giemsa C. India ink D. Acid fast E. Staining would not be appropriate

E (the CSF findings dictate this is an aseptic, or viral, meningitis - JC virus causes disease in immunocompromised patients [progressive multifocal leukoencephalopathy], leaving only HSV as the best possible pathogen - HSV does preferentially attack the temporal lobes, as shown in the image - behavior and mood are the first symptoms associated with loss of the temporal lobes)

A 32-year-old female who was previously healthy is brought to the emergency department by her concerned fiance who states that she is acting very abnormally. He states that over the past day she has developed a fever, become progressively more confused, and is "just not acting like herself." On exam, she exhibits altered mental status. Her vitals are demonstrated as: T: 39 deg C, HR: 102 bpm, BP: 130/90 mmHg, RR: 12, SaO2: 100%. The emergency physician obtains a lumbar puncture which demonstrates the following CSF analysis: normal opening pressure, elevated lymphocyte count, elevated protein, elevated RBCs, and normal glucose. An MRI is obtained to confirm the diagnosis. Which of the following organisms is most likely responsible for this patient's presentation? A. C. neoformans B. JC virus C. S. pneumonia D. N. meningitidis E. HSV-1

A (the patient has an MRI confirming a Chiari malformation [hernation of the cerebellar tonsils, kinked medulla] and has clinical symptoms reflecting the presence of a syrinx - this is definitive Arnold-Chiari malformation type I)

A 32-year-old male presents to his primary care physician with complaints of chronic headaches that have developed and increased in frequency and severity over the last several months. Additionally, the patient has noted he has been less coordinated over the last few weeks, stumbling and tripping often when he is walking. Physical examination is significant for notably reduced hand grip strength bilaterally as well as decreased pain and temperature sensation along the upper back and down both arms to the hands. A referral to the appropriate specialist is made, and an MRI of the brain and neck is obtained. Which of the following is the most likely diagnosis in this patient? A. Arnold-Chiari malformation type 1 B. Arnold-Chiari malformation type 2 C. Arnold-Chiari malformation type 3 D. Arnold-Chiari malformation type 4 E. Budd-Chiari syndrome

C (this is Huntington's - the disease is autosomal dominant and expresses anticipation, meaning earlier onset in successive affected generations - the pathology is loss of the striatum, also known as the caudate nucleus and the putamen)

A 32-year-old woman is brought to your office by her husband. The husband says that she had been acting strange lately. She has been forgetful, and she sometimes becomes angered for no reason, which is highly unusual for her. She has also been having random, uncontrollable movements, which are also new. On examination, she appears withdrawn and flat. On further questioning, she reveals that her father died at age 45 from a movement disorder. Which of the following is the pathological hallmark of the patient's condition? A. Substantia nigra pars compacta B. Alpha-synuclein intracellular inclusions C. Loss of neurons in the caudate nucleus and putamen D. Lipohyalinosis E. Beta-amyloid plaques

E (this is multiple sclerosis, evidenced by relapsing neurological symptoms, optic neuritis, and fatigue - demyelination is the hallmark of MS pathology)

A 33-year-old male presents to his primary care physician complaining of fatigue, left eye pain, and paresthesia in his feet bilaterally. The patient experienced a similar tingling sensation in his lower extremities 2 years ago, but it was self-limited. On examination of the lower extremities, you note a bilateral positive Babinski reflex. Which of the following would be expected on microscopic examination of this patient's brain? A. accumulation of neuritic plaques B. copper accumulation in the basal ganglia C. decreased pigmentation in the substantia nigra D. loss of dopaminergic neurons in the locus ceruleus E. scattered plaques of demyelination

C (the patient presents with a family history of renal cancer, which is likely genetic due to bilateralism, and a brain tumor suspicious of hemangioblastoma - these point to Von-Hippel-Lindau, caused by mutation of VHL gene on chromosome 3, with dominant inheritance - retinal angiomas can also be seen in this disease)

A 35-year-old man presents to his primary care physician complaining of blood in his urine. He has also has had changes in his vision over the last several months. He reports a family history of renal cancer. The patient undergoes an abdominal CT, which shows lesions suspicious for renal cell carcinoma on both kidneys. MRI of the brain is show. Which of the following genetic defects does this patient most likely have? A. Neurofibromatosis type I B. Neurofibromatosis type II C. Von-Hippel-Lindau syndrome D. Li-Fraumeni syndrome E. Tuberous sclerosis

E (the clinical picture appears to be acute meningitis - a lumbar puncture will provide information about bacterial vs aseptic vs other causes, and may yield culturable organisms in the event of a bacterial or fungal cause)

A 35-year-old man presents to the ED with a fever. He states that he has a severe headache, as well as that his neck is very stiff. When you examine the patient you see a young man in distress sitting in a dark room. On physical exam, flexion of the patients leg or neck induces severe discomfort and pain. What is the next best step in management? A. Chromosomal analysis B. Brain MRI C. Neck CT with contrast D. Neck CT without contrast E. Lumbar puncture

C (also called an acoustic neuroma, or schwannoma - presents with cochlear symptoms, heavily associated with NF type II and cranial nerve VIII, occurs laterally at the cerebellopontine angle)

A 35-year-old man with neurofibromatosis type II presents with nausea, vomiting, vertigo, and deafness in his right ear. Hemianesthesia is present on the right side of this face. What is the best description of the lesion indicated in her radiology? A. Cerebellar astrocytoma B. Pontine astrocytoma C. Vestibular neuroma D. Ependymoma E. Medulloblastoma

B (tumors within the 4th ventricle within the first two decades of life are often ependymomas - the histological description also indicates the presence of perivascular pseudorosettes, which make ependymoma the most likely mass)

A 4-year-old male presents with increasing lethargy, headache, nausea, and projectile vomiting. A brain CT reveals a mass lesion in the roof of the fourth ventricle. Histopathological examination reveals cells with peripherally located nuclei that form a halo around an eosinophilic lumen. These findings are consistent with A. Primary lymphoma B. Ependymoma C. Atypical teratoma D. Medulloblastoma E. Pilocytic astrocytoma

D (polymyositis is easy to remember - it's just dermatomyositis without any cutaneous symptoms) (treatment is steroids)

A 40-year-old male presents to your office complaining that he is too weak to climb stairs or brush his hair. He denies any headaches or change in vision. A muscle biopsy reveals CD8+ lymphocyte infiltration in the endomysium. Which of the following is the most likely diagnosis? A. Systemic lupus erythematosus B. Scleroderma C. Polymyalgia rheumatica D. Polymyositis E. Dermatomyositis

B (the protein in particular is amyloid precursor protein, which becomes a beta amyloid and accumulates, causing the pathophysiology of Alzheimer's disease - Alzheimer's has an earlier onset in Down syndrome individuals because of the 21 trisomy, since chromosome 21 contains the amyloid precursor protein gene)

A 40-year-old male with Down syndrome is brought to your clinic by his mother. She reports that over the past few months he has started having difficulty managing his daily routine at his assisted-living facility and no longer seems like himself. She says that last week he wandered away from the facility and was brought back by police. Additionally, he has stopped taking his regular antiepileptic medication, and she is concerned that he might have a seizure. TSH is checked and is normal. Which of the following is most likely to be responsible for this man's current presentation? A. Expansion of trinucleotide repeats B. Abnormal protein metabolism C. Hormone deficiency D. Premature degradation of a protein E. Nutritional deficiency

D (the stem is Guillan Barre - ascending paralysis with areflexia post infection - the usual culprit is Campylobacter jejuni)

A 42-year-old gentleman presents to the ED after a week of a worsening sensation of heavy lower extremities. The patient describes a few episodes of intense nausea, vomiting, and diarrhea a few days ago. Physical examination elicits bilateral 2-out-of-5 lower and upper extremity strength and absent ankle and patellar reflexes. He has most recently been complaining of shortness of breath if he attempts to have conversations with others. Routine labs, chest radiograph, and head CT are all unremarkable. His recent gastrointestinal complaints were most likely due to which of the following organisms? A. Bacillus cereus B. Staphylococcus aureus C. Streptococcus pneumoniae D. Campylobacter jejuni E. Streptococcus viridans

E (the diagnosis is dermatomyositis, and the correct answer describes Gottron papules - remember that this condition is autoimmune and manifests as symmetric weakness of proximal muscles)

A 42-year-old man presents to clinic complaining of increasing difficulty climbing stairs and standing up from sitting in his chair. On exam you perceive that his strength to be 5/5 distally, but only 3/5 in proximal muscle groups bilaterally. There is a distinctive rash on his upper eyelids and around his eyes. Examination of the fingers is most likely to reveal which of the following? A. Dactylitis B. Enlargement of the PIP C. Ulnar deviation of the fingers D. Nail pitting with oil spots E. Violaceous papules over the MCP, PIP, and DIP

E (with metastatic brain lesions, if the lesion is singular, surgical resection is a worthwhile intervention - multiple metastatic lesions are handled differently) (the most common metastatic cancers to the brain are breast, kidney, colon, lung, and melanoma)

A 45-year-old female is admitted to the hospital after worsening headaches for the past month. She has noticed that the headaches are usually generalized, and frequently occur during sleep. She does not have a history of migraines or other types of headaches. Her past medical history is significant for breast cancer, which was diagnosed a year ago and treated with mastectomy. She recovered fully and returned to work shortly thereafter. CT scan of the brain now shows a solitary cortical 5cm mass surrounded by edema in the left hemisphere of the brain at the grey-white matter junction. She is admitted to the hospital for further management. What is the most appropriate next step in management for this patient? A. Chemotherapy B. Seizure prophylaxis and palliative pain therapy C. Irradiation to the breasts D. Irradiation to the brain mass E. Surgical resection of the mass

B (the stem describes an epidural hematoma - it is lens shaped and respects suture lines, and should be suspected after blunt force trauma to the head followed by a lucid interval)

A 45-year-old male is brought to the emergency department after being struck in the head with a baseball bat. Witnesses say he initially vomited profusely and lost consciousness but then awoke and continued to converse normally before becoming less responsive. Vital signs reveal a blood pressure of 132/74 mmHg, a heart rate of 98/min, and a respiratory rate of 26/min. He is unable to follow commands for manual muscle testing.His right pupil is responding sloly to light. Computed tomography of the head would most likely reveal A. a crescent-shaped hematoma that crosses suture lines B. a lens-shaped hematoma that respects suture lines C. blood in the sylvian fissure D. blood under the galea aponeurotica E. small hematomas in the posterior internal capsule

E (the patient's symptoms are rapidly progressive dementia with myoclonus, with cerebellar ivolvement - the EEG gives us periodic spike complexes, which are the buzzword for CJD, a spongiform encephalopathy caused by misfolded proteins called prions - "parenchymal vacuolation" is just a dumb way to say spongiform changes) (A; this is multiple sclerosis) (B; this is cobalamin [B12] deficiency) (C; this is an arteriovenous malformation) (D; this is viral encephalitis)

A 46-year-old male presents to his primary care physician with a 5-week history of worsening ataxia and involuntary muscle contractions. The patient also reports progressive memory loss and difficulty concentrating. EEG reveals periodic paroxysms of sharp waves on a slow background. Which of the following is most likely responsible for this patient's presentation? A. Areas of demyelination due to lymphocyte and macrophage infiltration B. Degeneration of posterior and lateral spine columns due to vitamin deficiency C. Duplication of the internal elastic lamina due to plaque formation D. Inflammation of the white matter due to viral infection E. Parenchymal vacuoation due to misfolded protein proliferation

D (this is Lambert Eateon myasthenic syndrome, which in this case is caused by autoimmune disease, where antibodies attack the presynaptic calcium channels of the neuromuscular junction - however, Lambert Eaton is often seen as a very common paraneoplastic complication of small cell lung carcinoma, the most paraneoplastic-prone of cancers) (A; botulism is a complete neuromuscular block - it does not improve with repeated muscle stimulation like Lambert Eaton does)

A 46-year-old woman with a history of lupus presents with progressive overall weakness. She reports weakness brushing her hair every day and difficulty rising from a seated position. She has no history of smoking, but has a strong history of autoimmune disease in herself (with lupus as well as alopecia areata) and her family. She denies any new dermatological rashes. Her routine labs are all normal. On physical exam, repeated muscle movement resulted in increased strength. This phenomenon is most often caused by which of the following conditions? A. Botulism B. Type II diabetes mellitus C. Focal segmental glomerulosclerosis D. Small cell lung carcinoma E. Hyperthyroidism

D (doesn't hurt to review some good old SAH, yeah? remember that this is an intracerebral hemorrhage from nontraumatic causes, usually cerebrovascular disease - deep brain bleeds are often caused by small vessel diseases, like hypertension)

A 47-year-old male with a medical history significant for hypertension, recurrent urinary tract infections, mitral valve prolapse, and diverticulosis experiences a sudden, severe headache while watching television on his couch. He calls 911 and reports to paramedics that he feels as if "someone shot me in the back of my head." He is rushed to the emergency room. On exam, he shows no focal neurological deficits but has significant nuchal rigidity and photophobia. Of the options below, what is the most likely etiology of this man's headache? A. Migraine B. Brain Tumor C. Temporal Arteritis D. Subarachnoid Hemorrhage E. Carotid Dissection

D (this is an ependymoma, which is most common in children in the fourth ventricle - the tumor is slow growing, but symptomatic and not easy to resect, and can seed into the CSF)

A 5-year-old boy presents to pediatrics clinic complaining of headaches. His head circumference is large for his age. He has no other complaints and had been growing and developing normally prior to this visit. Vital signs are stable. Physical examination is benign with the exception of increased head circumference. Fundascopic examination revealed papilledema, and a subsequent MRI of the brain is performed, as shown. Which of the following is true regarding this patient's condition? A. These masses are associated with retinal angiomas B. This mass is derived from Rathke's pouch C. This mass is a form of primitive neuroectodermal tumors D. The most common location of this mass is the fourth ventricle E. This tumor mass is the most common type in adults and children

B (highly malignant but very radiosensitive, capable of 'drop metastases' down the neuraxis - note that the patient is a child, the lesion is large, midline, and cerebellar - histology would feature small round blue cells)

A 5-year-old child presents with an occipital headache, an ataxic gait, nystagmus, and papilledema. Her scan is as shown. What is the most likely diagnosis? A. Ependymoma B. Medulloblastoma C. Glioblastoma D. Pontine astrocytoma E. Cerebellar astrocytoma

B (this patient has AIDS dementia, which is an end-stage disease - multinucleated giant cells are diagnostic) (A; this is toxo, which AIDS patients are at increased risk for, but the symptoms do not match) (C; this is herpes encephalitis) (D; this is rabies) (E; this is Creutzfeld Jakob disease) (F; this is subacute sclerosing panencephalitis, caused by the measles virus)

A 55-year-old male presents to his primary care physician with ataxia and impaired mental status. He was diagnosed with acquired immunodeficiency syndrome fifteen years ago, but has been non-adherent with his highly active antiretroviral drug therapy. Physical examination shows spastic paresis and loss of vibratory and position sense in the lower extremities. he dies three weeks later. The most likely histological change to be found in his brain at autopsy would be the presence of A. Cerebral abscess B. Multi-nucleated giant cells C. Necrosis of the temporal lobes D. Negri bodies E. Spongiform changes F. Dawson bodies

C (this is Wernicke-Korsakoff syndrome, confabulation is by far the most entertaining of its symptoms)

A 55-year-old male presents to the emergency department with his wife with complaint of worsening memory loss. He is confused and alert and oriented x 0. His wife reports a history of chronic alcohol abuse. He is given thiamine and the confusion improves. However, he is unable to repeat a series of numbers, and cannot recall any of 3 objects that are given after 5 minutes. He is also unable to recall past evens or the name of his wife. Which of the following is another characteristic associated with this condition? A. Ataxia B. Akathisia C. Confabulation D. Frontal lobe lesions E. Nystagmus

D ("clear cytoplasm with dense nucleus" is verbally describing the fried egg cells of oligodendrocytes, and the finely branching capillaries describe the "chicken feet" sign of a well anastamosed oligodendroglioma)

A 55-year-old male presents with intractable headaches that are worst upon waking up in the morning. Magnetic resonance imaging reveals a mass lesion located in his frontal lobe. A biopsy is performed which reveals compact rounded cells with a clear cytoplasm that surrounds a dense nucleus. There is also an abundant amount of finely branching capillaries present. The most likely description of this patient's mass lesion is A. Primary lymphoma B. Glioblastoma multiforme C. Meningioma D. Oligodendroglioma E. Pilocytic astrocytoma

C (this is frontotemporal dementia, or Pick's disease - the key symptom is change in behavior or personality without awareness - loss of memory and language are also possible)

A 58-year-old male is brought to the physician by his daughter for "strange" behavior. He has been innapropriately making sexual remarks, invading people's personal space, and urinating in public. She has noticed that he has been binge eating sweets as of late. His manners and social abilities have drastically worsened. On physical exam, there is a deficit in executive functioning and working memory. Visuospatial function is intact. If you were to perform imaging, which finding would be most expected? A. Gross hemorrhage B. Ventricular shrinking C. Atrophy of the frontal lobe D. Atrophy of the parietal lobe E. Atrophy of the entire cortex

E (of the listed areas, the hippocampus is the most sensitive to hypoxia - another sensitive area is the Purkinje layer of the cerebellum)

A 58-year-old man with history of diabetes and hypertension suffers a cardiac arrest at home. The family calls 911, yet no one performs CPR. Five minutes after the arrest, EMS arrives to begin resuscitation. At this point, which region of the CNS is most likely to suffer ischemic damage? A. Thalamus B. Spinal cord C. Pons D. Medulla E. Hippocampus

D (also called Pick's disease - the hallmark is behavioral and personality change without awareness - aphasia is also a classic symptom) (this differs from Alzheimer's because in Pick's, only the frontal and temporal lobes are affected, while Alzheimer's has diffuse cortical involvement)

A 59-year-old male is brought to the physician by his wife due to "odd behavior." She reports that he would attempt to kiss strangers, or would urinate in public. At times, he would grow distant and show no interest in maintaining a conversation. She has also noticed that he has been frequently binge eating sweets. He does not have insight into his symptoms. On exam, the patient displays disinhibition. There is impairment in executive functioning with intact visuospatial functioning. What is the best diagnosis? A. Alzheimer's disease B. Vascular dementia C. Hydrocephalus D. Frontotemporal dementia E. Vascular dementia

B (this is multisystem atrophy, which can present with parkinson-like symptoms, but doesn't respond to Parkinson's treatment - the findings on autopsy are inclusions in the glial cells) (A; this is a feature of Huntington's) (D; this is Alzheimer's) (E; this is multiple sclerosis)

A 59-year-old patient presented to his family physician 8 years ago with initial complaints of increasing generalized stiffness with trouble initiating movement and worsening micrographia. He was started on levodopa after further evaluation led to a suspected diagnosis of Parkinson's disease; however, this therapy ultimately failed to improve the patient's symptoms. Additionally, over the ensuing 8 years since his initial presentation, the patient also developed symptoms including worsening balance, orthostatic hypotension, urinary incontinence, and impotence. The patient's overall condition deteriorated ever since this initial diagnosis with increasing disability from his motor symptoms, and he recently passed away at the age of 67, 8 years after his first presentation to his physician. The family requests an autopsy. Which of the following would be expected on autopsy evaluation of this patient's brain tissue? A. Astrocytosis and caudate atrophy B. Glial cytoplasmic inclusions C. Round intracellular tau protein aggregates D. Beta-amyloid plaques E. Periventricular white matter plaques

E (when alcohol toxicity and thiamine deficiency come up, you better believe the answer is mamillary body necrosis)

A 59-year-old postmenopausal female is brought to the emergency department after she was found unconscious on the street, with alcohol on her breath. Toxicology tests confirmed the presence of alcohol in her system. One hour later, she revives and is agitated and confused. Physical examination reveals ataxia and the inability to gaze laterally. Thiamine is administered. Damage has most likely occurred to the A. Amygdala B. Basal ganglia C. Caudate nucleus D. Frontal lobe E. Mamillary bodies

A (asymmetric gradual loss of upper and lower motor neuron function - classic ALS)

A 61-year-old male presents with left-sided hand weakness and trouble with walking. He is not sure why these symptoms occur. On physical exam, tongue fasciculations are appreciated. He has slow speech. The left upper extremity shows forearm atrophy and depressed reflexes. The right lower extremity is hypertonic, with 3+ reflexes and positive Babinski sign. What is the most likely diagnosis? A. Amyotrophic lateral sclerosis B. Primary lateral sclerosis C. Pick's disease D. Multiple sclerosis E. Syringomyelia

C (this is Parkinson's disease, a degeneration of dopaminergic neurons in the nigrostriatal system, creating movement disorders early in the disease and dementia later)

A 63-year-old male is brought to the physician by his wife for the evaluation of a tremor. The tremor is worse at rest, and decreases in severity with purposeful movement. The patient reports to having difficulty with initiating voluntary movement, and his wife states that the patient's movements have been slow. On exam, there is seborrheic dermatosis on the nasolabial folds. There is a "pill-rolling" resting tremor accentuated when the patient is asked to perform mental calculations. Resistance to passive movement at the elbow joint is noted. On gait testing, there was difficulty with initiating gait, as well as the patient taking short steps when walking forward. Loss of which neurons characterizes this patient's pathology? A. Adrenergic B. Noradrenergic C. Dopaminergic D. Serotonergic E. GABAergic

A (this is diabetic neuropathy - high blood glucose leads to transport of glucose into Schwann cells where it is converted to sorbitol, which is toxic to the nerves - advanced glycation end products and free radical damage cause inflammation that also contributes to the onset of this condition)

A 65-year-old obese female presents to the clinic with shock-like sensations in her lower extremities. Physical examination reveals that both feet have decreased vibratory sensation, decreased ankle jerk reflexes, ad some mild paravertebral tenderness in the lumbar vertebral segments. Laboratory studies show Sodium 146 mEq/L Chloride 102 mEq/L Potassium 3.8 mEq/L Bicarbonate 27 mEq/L Glucose 198 mEq/L The most likely etiology of her condition involves A. Accumulation of excess sorbitol in nervous tissue B. Compression of the posterior tibial nerve C. Compression of the sciatic nerve D. Herniation of the nucleus pulposus in the lumbar spine E. Immune reaction against epitopes contained in the axonal membrane

A (the patient has pneumococcal meningitis, a bacterial cause - CSF findings in a bacterial meningitis include low glucose, elevated WBC with PMN predominance, elevated protein, and elevated opening pressure - normal CSF glucose is approximately 2/3 of the serum glucose, so 40 in this patient is decreased) (B; WBCs would be greatly elevated in a bacterial meningitis) (C; protein would be greatly elevated in any inflammatory process) (D; 160 is a normal opening pressure - expect pressures over 200 in meningitis cases) (E; in a bacterial meningitis, PMNs will be the dominant leukocyte, 80% or greater)

A 66-year-old male presents to the emergency department with complaints of fevers, altered mental status, neck stiffness and headaches over the past 2 days. Vital signs are as follows: T 39.2, HR 101, BP 144/88, RR 18, O2 Sat 97% RA. Physical exam is significant for involuntary flexion of the bilateral hips and knees in response to passive neck flexion. He reports being treated with antibiotics for sinusitis 8 days ago. Other medical history includes a remote splenectomy and hypertension. A Gram stain of the patient's cerebrospinal fluid is shown. Of note, the patient's serum glucose at this visit is 120 mg/dL. Which of the following findings would be expected on further analysis of this patient's cerebrospinal fluid? A. Glucose 40 mg/dL B. WBC count 500 cells/mm^3 C. Protein 20 mg/dL D. Opening CSF pressure 160 mm H2O E. 20% polymorphonuclear leukocytes

E (the keys are age, movement disorder, and evidence of hallucinations - Lewy body dementia is the best diagnosis) (B; Parkinsonism would feature the movement disorder before the dementia symptoms, though the two diseases are on the same spectrum and share diagnostic criteria)

A 67-year-old male presents with poor attention and getting lost while driving. He is accompanied by his wife. She reports that he would have episodes of staring into space and disorganized speech. His wife also reports that he would have full conversations with himself in the living room. She says that he was talking to his brother, who is deceased. Postural instability and bradykinesia is noted on physical exam. What is the best diagnosis? A. Alzheimer's disease B. Parkinson's disease C. Huntington's disease D. Vascular dementia E. Lewy body dementia

D (this is glioblastoma multiforme ["butterfly tumor"] characterized by pseudopallisading necrosis on microscopy and being in both hemispheres of the brain on gross anatomy)

A 67-year-old man presents to general medical clinic with progressively worsening headaches. Prior to a few months ago, he had never had a headache in his life. The headaches are increasing in frequency and severity. The patient reveals that NSAIDs offer some pain relief and that his symptoms are worst in the morning. He has no past medical history. Vital signs are stable. Physical examination is within normal limits. The patient receives an MRI which shows the following. Which of the following is true regarding this patient's diagnosis? A. These tumors are associated with von Hippel-Lindau syndrome when found with retinal angiomas B. These tumors most often are found in the fourth ventricle C. This is the most common central nervous system tumor, most often occurring in the convexities of the hemispheres and the parasagittal regions D. This is the most common malignant primary brain tumor E. These tumors are rare and slow growing, most often occurring in the frontal lobe

C (this is myaesthesnia gravis - note symptoms worsen with stimulation over time, to contrast with Lambert Eaton syndrome [D] where symptoms improve with stimulation over time) (the double vision is caused by fatigue of the extraocular muscles - myasthenia gravis affects ALL the muscles)

A 69-year-old gentleman presents to his physician with a chief complaint of blurry vision. Though he currently says he feels fine, he notices that his vision at the end of the day is blurry and sometimes he experiences "double vision." The patient states that he feels weak and fatigued at the end of the day and has noticed with harder foods, weakness in his ability to chew. Which of the following is likely elevated in the patient's serum? A. Glucose B. Calcium C. Anti-acetylcholine receptor antibodies D. Anti-presynaptic calcium channel antibodies E. Anti-Smith antibodies

C (this is central pontine myelinolysis, or osmotic demyelination syndrome - as the former name says, it affects the pons, and can cause a "locked in" type of syndrome, in other words quadriplegia - occurs with sudden correction of low sodium)

A 70-year-old hospitalized patient with a serum sodium of 117 mEq/L is corrected to 140 mEq/L in the next 6 hours. In the subsequent days he becomes paralyzed. Which part of the central nervous system is most affected by this sudden infusion to correct hyponatremia? A. Spinal cord B. Medulla C. Pons D. Midbrain E. Mamillary bodies

D (the important aspects of pathologic Alzheimer's histology is that the beta amyloids are extracellular, the tau proteins are intracellular, hyperphosphorylated, and tangle the neurofilaments [also intracellularly] (A; tau proteins aren't really the aggregates in this histology, nor are they round - more flame shaped) (B, C; these are features of Lewy body dementia) (E; the amyloid of Alzheimer's is A-beta, not AL)

A 72-year-old male is brought to his primary care physician by his daughter for concerns of worsening memory. She states that he several months ago began having a hard time remembering what items to buy at the supermarket. Per his daughter, his memory has since worsened, and now he is often unable to recall the topic of television shows he watches or articles he reads in the paper. She also notes that he sometimes has a hard time finding words when speaking. She is especially concerned because last week he forgot he left the stove on after making dinner. Otherwise he has been his "usual self," and he has been enjoying spending time with his family and gardening. The patient reports that he has noted increased difficulty remembering certain things, but does not feel it is a major issue. Which of the following histologic brain findings is associated with this patient's condition? A. Round intracellular tau protein aggregates B. Intracellular alpha-synuclein aggregates C. Discrete, eosinophilic intracellular inclusions D. Intraceullar neurofibrillary tangles consisting of hyperphosphorylated tau protein E. Extracellular AL amyloid deposition

A (given the symptoms of the stroke and the findings on imaging, this is likely a lacunar infarct due to microaneurysmal rupture of the lenticulostriate arteries - the most important risk factors for these types of small vessel strokes are microangiopathies, like diabetes and hypertension)

A 74-year-old African-American woman is brought to the emergency department by her home health aid. The patient was eating breakfast this morning when she suddenly was unable to lift her spoon with her right hand. She attempted to get up from the table, but her right leg felt weak. One hour later in the emergency department, her strength is 0/5 in the right upper and right lower extremities. Strength is normal in her left upper and lower extremities. Sensation is normal bilaterally. An emergency CT of the head does not show signs of hemorrhage. Subsequent brain MRI shows an infarct involving the internal capsule. Which of the following is true about her disease process? A. The most important risk factors are hypertension and diabetes B. The most common cause is embolism originating from the left atrium C. It is caused by ischemia to watershed areas D. IV thrombolysis cannot be used E. The most important risk factors are ethnicity and sex

E (after a month has passed from a cerebrovascular accident, the most prominent feature of the affected area is a glial scar, seen grossly as a cyst formed by astrocytes)

A 75-year-old woman with a history of stroke 1 year ago was found unconscious on the floor of her home by her son. The patient was brought to the emergency department by ambulance but expired prior to arrival. An autopsy was performed and showed the cause of death to be a massive ischemic stroke. The coroner also examined sections taken from the area of her prior stroke. Which histologic finding would be prominent in the area of her stroke from one year prior? A. Red neurons B. Necrosis and neutrophils C. Macrophages D. Reactive gliosis and vascular proliferation E. Cyst formed by astrocyte processes

E (this is adrenoleukodystrophy - there isn't much to go on, admittedly, but I tried to emphasize the "early school age" - as the name says, there will be adrenal involvement, and the disease process itself involves accumulation of long chain fatty acids due to an X-linked recessive enzyme deficiency)

A 9-year-old boy presents with confusion and decreased school performance. Spastic gait, dysarthria, dysphagia, and visual loss soon develop. On MRI, there is massive demyelination of the white matter in the posterior areas of the hemispheres. Which of the following is most likely associated with this disease? A. Cardiac arrhythmia B. Type II diabetes C. Bronchopulmonary dysplasia D. Renal failure E. Adrenal insufficiency

C (this patient is 4 days removed from a large ischemic stroke - at this point in time the predominant histological finding would be macrophage infiltration and phagocytosis) (A; 12-24 hours) (B; 24-72 hours) (D; 1-2 weeks) (E; greater than 2 weeks)

A patient is transferred from an outside hospital by family request. The patient is a 76-year-old gentleman who developed acute onset left-sided weakness four days ago with the imaging findings shown. Despite aggressive care, the patient dies shortly after transfer. The family requests an autopsy. What histological finding would you expect to find on evaluation of the patient's brain? A. Red neurons B. Neutrophilic infiltration and necrosis C. Macrophage infiltration and phagocytosis D. Reactive gliosis and vascular proliferation E. Glial scarring

B (we only know one disease that presents with ataxia in the first decade of life - Friederich's ataxia is a trinucleotide repeat of GAA and presents with the constellation of symptoms shown, plus some others - inheritance is autosomal recessive)

An 8-year-old boy is brought to the pediatrician because his mother is concerned about recent behavioral changes. His mother states that she has started to notice that he is slurring his speech and seems to be falling more than normal. On exam, the pediatrician observes the boy has pes cavus, hammer toes, and kyposcoliosis. Based on these findings, the pediatrician is concerned the child has a trinucleotide repeat disease. Which of the following trinucleotide repeats is this child most likely to possess? A. CGG B. GAA C. CAG D. CTG E. GCC

B (this patient has symptoms that match the clinical picture of Alzheimer's disease - the CT scan shows hydrocephalus, seen as an enlargement of the lateral ventricles - in Alzheimer's patients, the ventricles are able to enlarge due to atrophy of the surrounding cortex - this is an example of hydrocephalus ex vacuo)

An 88-year-old male is brought to clinic by his exasperated son. The patient has been in excellent health his entire life, but in the last few years appears to have grown steadily confused. He frequently calls his son about things that they have already discussed, forgets where he has placed his keys, and recently the patient's son noticed several unpaid bills on the patient's desk at home. The patient is upset at being "dragged" into see the physician and claims that everything is fine--he is just "getting older". A complete neurologic exam is normal except for significant difficulty with recall tasks. In the course of the medical work-up, you obtain a CT scan. What is the most likely cause of this patient's CT findings? A. Blockage of the cerebral aqueduct B. Cortical atrophy C. Increased CSF production D. Congenital malformation E. Infection