21 October 2005 Biomed 262: Neurologic Pathophysiology Mid-Term EXAMINATION 1. A patient presents with a complaint of weakness. His neurologic examination reveals symmetric bilateral weakness of all four limbs with distal greater than proximal weakness. His sensory examination reveals decreased sensation of all modalities in the distal limbs in a glove-stocking distribution. Reflex examination reveals that all deep tendon reflexes are absent. His illness likely is localized to A. Neuromuscular junction disease B. Cervical spinal cord lesion C. Polyneuropathy D. Muscle disease E. Bifrontal cerebral lobe lesions 2. A patient presents with severe, progressive, bilateral upper and lower extremity weakness. Recently he has also noted urinary incontinence. Neurologic examination reveals normal mental status and cranial nerve examination. Motor examination in the upper extremities reveals 4/5 weakness in all muscles of the upper extremities. Both legs are weak: hip and knee flexors 4- to 4/5, knee extensors 2 to 3/5, ankle extensors and flexors 1-2/5. All deep tendon reflexes are brisk. There are bilateral extensor plantar responses (Babinski signs). Sensation is reduced in all areas below a horizontal line that can be drawn at the patient’s upper thorax. His problem is most likely attributed to A. Cauda equina lesion B. Polyneuropathy C. Muscle disease D. Cervical spinal cord lesion E. Brainstem lesion 3. A patient presents with gait ataxia. His examination reveals normal mental status and cranial nerve examination (no nystagmus), normal strength and sensation in his limbs and normal reflexes. Gait is wide based and unsteady. His problem is most likely attributed to A. Cerebellar stroke B. Vestibular neuronitis C. Posterior column dysfunction due to B12 deficit D. Sensory neuropathy E. Muscle disease 4. A patient presents with left hemiparesis of one day’s duration. The finding on examination that MOST specifically suggests a brainstem lesion is A. Increased tone of the left arm and leg B. Right gaze preference C. Left spatial neglect D. Right facial muscle weakness E. Positive Babinski response on the left 5. Which of the following would NOT explain a patient being unresponsive to deep pain (in coma)? A. Acute renal failure with extremely high BUN and creatinine B. Right internal capsule lacunar infarction C. Large midline pontine hemorrhage D. Acute herpes encephalitis with destruction of both temporal lobes E. Prolonged cardiac arrest followed by successful restoration of cardiac function 6. Which of the following is NOT consistent with the diagnosis of brain death? A. No response to deep pain B. Absence of all brainstem reflexes C. Cause of coma is not known D. There is no possibility of recovery E. Cessation of brain function has been documented for 24 hours 7-8. A 78 year old man with no prior medical history develops sudden aphasia, right hemiplegia, nausea and vomiting. Over the next 20 minutes he becomes drowsy, CT scan reveals a large hyperdense lesion of the left frontal lobe with mass effect producing left to right shift. Blood pressure and ekg are normal. Which of the following pathologies is the most likely cause of this hemorrhage? A. Amyloid angiopathy B. Hypertensive small artery disease C. Arteriovenous malformation D. Aneurysm E. Cocaine abuse 8. The wife of the patient in question #7 asks about his likelihood of survival. You respond that this most depends on: A. His age and cardiac stability B. His intracranial pressure C. The size of the hemorrhage D. His blood pressure E. The results of surgery for the hemorrhage 9. A 55 year old woman with a long history of hypertension develops sudden hemiparesis. Ct scan reveals a large basal ganglia hemorrhage. She becomes comatose within a matter of hours. Her family asks whether you plan to perform a conventional catheter angiogram. Your best response would be: A. Yes, because an aneurysm is likely to be found and could be treated. B. Yes, because an AVM is likely to be found and could be treated. C. Yes, because the information could be used to treat impending brain herniation. D. No, because the angiogram most likely will reveal no abnormality. E. No, because the patient has too severe a grade of subarachnoid hemorrhage to benefit from treatment. 10-12. A 69 year old woman suffers sudden headache followed by loss of consciousness, In the ER, she is drowsy, with stable vital signs, normal cranial nerves and a normal motor and sensory exam. CT scan reveals diffuse subarachnoid blood. The most likely finding on catheter angiogram would be: A. Normal B. AVM C. Saccular aneurysm D. Brain tumor E. Basilar artery thrombosis 11. The patient’s family asks you about her prognosis for survival. You would tell them that her prognosis is related to A. the degree of bleeding seen on CT B. her age C. whether the angiogram shows vasospasm D. the size and location of the hemorrhage E. her level of consciousness 12. Treatment options for this patient include all of the following EXCEPT: A. Inserting coils into the aneurysm B. Clipping the neck of the aneurysm C. Operating just to remove the blood D. Giving calcium channel blockers and hydration to avoid vasospasm E. Placing a ventricular shunt if hydrocephalus occurs 13-17. Match each of the following clinical presentations (13-17) with the single arterial site of atherosclerosis (A-E) with which it is most likely associated: (An answer may be used once, more than once, or not at all) 13. Transient bilateral (both eyes) visual loss e A. Posterior Communicating Artery 14. Hemianopic visual field cut, no other symptoms b B. Posterior Cerebral Artery 15. Transient aphasia and right hemiparesis c C. Middle Cerebral Artery 16. Coma, ophthalmoplegia and quadriparesis e D. Anterior Cerebral Artery 17. Unilateral leg weakness d E. Basilar Artery 18-19. Ms. A is a 52 year old woman sent to a neurologist for severe headaches. She had headaches intermittently for 38 years which had not increased in frequency and still occurred weekly. The headaches usually came on during the day, heralded by an odd feeling of malaise, and were accompanied by nausea, vomiting, and noise and light sensitivity. The headaches were often left sided but occasionally were right sided or bilateral. She described the pain as severe and throbbing. She frequently had to stop whatever she was doing and lie down in a dark room with curtains drawn. The headaches lasted for several hours and occasionally, for days. Often, the headaches were preceded by numbness of her right side which started on her hand and spread to her arm, followed by difficulty speaking and understanding others. This lasted 20 -30 minutes and subsided as the headache worsened. Her mother and an aunt had severe headaches as well. She worked as an accountant, smoked 2 packs of cigarettes per day and drank alcohol rarely because it brought on headaches. Her only other medical problem was longstanding hypertension. Her physical exam was normal, including fundoscopic exam. You order a head CT scan and it is normal. Upon follow up visit, the patient tells you she has had three spells of a different type involving right eye blindness, unassociated with headache and lasting only minutes at a time. You tell her that A. her headaches and neurological symptoms are all due to migraine and she requires no further testing B. her headaches and neurological symptoms are all due to temporal arteritis and she needs further tests C. she likely has a brain tumor and needs more tests D. her chronic headaches and neurological symptoms may be due to migraine but the newer neurological symptoms require additional evaluation E. her new neurological symptoms may be due to a meningeal process and she needs further tests 19. The patient agrees with you and your next step is to A. prescribe antimigraine therapy B. order a temporal artery biopsy C. consult a neurosurgeon about brain surgery D. order a carotid ultrasound and start aspirin therapy E. perform a lumbar puncture 20. A patient with no symptoms is found to have a carotid artery bruit. His doctor orders a carotid ultrasound which reveals a 70% diameter stenosis of the right internal carotid artery. Which of the following statements are true? A. The patient’s stroke risk from this lesion is about 2% per year B. Surgery is the best treatment for this lesion. C. Surgery cuts the stroke risk from this lesion in half. D. Surgery increases the patient’s chances of living the next year without a stroke from this lesion from 98% to 99%. E. All the statements are true. 21. Drop question A 35 year old man presents with new onset of seizures. MRI reveals a non-enhancing lesion in the left frontal lobe. Biopsy is performed. Cytogenetic analysis of tumor tissue reveals a deletion of the short arm of chromosome 1 and a deletion of the long arm of chromosome 19 (1-p:19-q deletion). What is the most likely diagnosis in this case? A. astrocytoma B. oligodendroglioma C. glioblastoma multiforme D. meningioma E. primary central nervous system lymphoma 22. A 55 year old woman underwent complete resection of a T2N0M0, stage IB adenocarcinoma of the lung one year ago. She now presents with one-month history of gradually increasing headaches and mild left sided weakness. An MRI of the brain reveals a solitary contrast enhanced mass in the right frontal lobe. CT scan of the chest and abdomen reveal no evidence of recurrent disease. The presumptive diagnosis is a solitary brain recurrence of her lung cancer. Which of the following therapeutic approaches are most likely to extend her life and reduce the risk of neurologic deterioration? A. High dose steroids and observation B. Whole brain radiation therapy C. Surgical resection alone D. Surgical resection followed by whole brain radiation E. Surgical resection and adjuvant chemotherapy 23. Which of the following statements is true with regard to astrocytoma? A. Surgical resection can cure the majority of patients with low grade (diffusely infiltrating) astrocytoma. B. In adults, it is most often located in the posterior fossa. C. The prognosis of astrocytoma is superior to that of oligodendroglioma. D. Most diffusely infiltrating (low grade) astrocytomas and anaplastic astrocytomas will eventually transform into glioblastoma multiforme. E. Astrocytoma represents an uncommon primary brain tumor in adults. 24. A 50 year-old man presents with a one-week history of new onset of tingling on the right side of his face and right upper extremity associated with slurred speech. Physical examination reveals dysarthria and mild weakness on the right side. A T-1 weighted MRI of the brain performed with gadolinium reveals a ring enhancing mass with surrounding edema. The presumptive diagnosis is glioblastoma multiforme (GBM). Based upon this diagnosis, which would be the most appropriate treatment option: A. Stereotactic biopsy followed by radiation therapy B. Stereotactic biopsy followed by radiation and corticosteroids C. Stereotactic biopsy followed by radiation and chemotherapy D. Surgical debulking followed by radiation therapy E. Surgical debulking followed by radiation therapy and chemotherapy 25. With respect to MR imaging of the adult brain, A. On a T1-weighted MR image, fat and subacute blood (methemoglobin) are both relatively lower in signal intensity than cerebral cortex. B. On a T1-weighted MR image, cerebrospinal fluid is higher in signal intensity than normal white matter. C. On a T2-weighted MR image, cerebrospinal fluid is relatively lower in signal intensity than deep grey matter. D. Compared to normal white matter, most focal lesions in the brain are hypointense on a T1weighted MR image, and hyperintense on a T2-weighted MR image. E. The contrast agent most often used in MR imaging (chelate of gadolinium) is contraindicated in patients with renal failure on hemodialysis. 26. Which one of the following correctly lists the following tissues from most radiodense to least radiodense on a noncontrast head CT: A. bone>acute hemorrhage>deep grey matter>air>CSF. B. bone>acute hemorrhage>cerebral white matter>CSF>air. C. acute hemorrhage>bone>cerebral white matter>CSF>fat. D. bone>acute hemorrhage>CSF>cerebral white matter>air. E. acute hemorrhage>bone>deep grey matter>fat>CSF. For questions #27 to #31 select the neuroimaging test that may provide the most specific answer to the clinical question indicated. 27. Whether or not subarachnoid hemorrhage is the cause of severe headache in a 38 year old woman. A. lateral skull radiograph B. transcranial Doppler ultrasound C. SPECT scan D. noncontrast CT scan E. contrast-enhanced MR imaging 28. The cause of cervical myelopathy in a 69 year old man. A. lateral skull radiograph B. transcranial Doppler sonogram C. SPECT scan D. contrast-enhanced CT scan E. MR imaging of the cervical spine 29. Whether or not small-vessel vasculitis is the cause of ischemic infarction in a 32 year old man with history of amphetamine abuse. A. lateral skull radiograph B. duplex sonogram C. SPECT scan D. contrast-enhanced CT scan E. conventional catheter angiogram 30. Whether or not there is focal high-grade stenosis in the proximal internal carotid artery. A. CT angiography B. MR angiography C. conventional catheter angiography D. duplex sonography E. all of the above 31. Whether or not there is thoracic cord compression in a 66 year old woman with carcinoma of the breast. A. plain film radiographs of the spine B. radionuclide bone scan C. MR imaging of the spine D. CT scan of the spine E. FDG-PET scan 32. With respect to imaging tests used in neurodiagnosis, A. the pharmacokinetics of high-frequency sound waves is the basis of image contrast in ultrasound. B. the complex interactions and relaxation of tissue protons in water and fat, placed in a strong external magnetic field, is the basis for image contrast in electroencephalography. C. computed tomography (CT) is based on the differential absorption of x-rays as they are attenuated during their transmission through the body part of interest. D. positron-emission is the basis for localization of the radiotracer in single-photon emission computed tomography (SPECT). E. gadolinium-chelate contrast agents cause prolongation of the T2 relaxation time in tissues with a deficient blood brain barrier rendering these tissues hyperintense on conventional T2-weighted imaging. 33-35. A 49 year old man with no past history of any significance presents with 2 weeks of excruciating, periorbital, left sided head/face pain. The pain lasts 45 minutes and is succeeded by a dull ache that lasts hours. He admits to tearing and redness of the left eye and his wife says that his eye droops when he is in pain. The pain usually occurs twice a night at about the same time. He is afebrile with a normal exam when in the office. What is the most likely diagnosis? A. Acute sinusitis B. Cluster headache C. Migraine D. Temporal arteritis E. Orbital pseudotumor 34. What is the first study would you do? A. CT of the sinuses and brain B. Cathether carotid angiogram C. MRI of the brain with contrast D. Sedimentation rate E. None of the above 35. If he also complained of a purulent nasal discharge and had a mild fever, and his exam showed periorbital edema, which of the above would you do first? A. CT of the sinuses and brain B. Catheter carotid angiogram C. MRI of the brain with contrast D. Sedimentation rate E. None of the above 36. A patient reports chronic neck pain and slowly increasing weakness of both upper arms. He mentions that he recently cut his finger while cooking but did not notice it until he saw blood covering the cutting board. He denied having problems handling the vegetables he was cutting. On exam he had mild atrophy of the upper arms with diminished deep tendon reflexes but his legs were strong but mildly spastic. The most likely localization for this lesion is: A. Cervical polyradiculopathy (multiple nerve root process) B. Central cord around C 5 C. Posterior cervical cord compression D. Anterior cervical cord tumor E. Cauda equina lesion 37. drop question A 20 year old man with a severe anxiety disorder reports the sudden onset of back pain with paralysis and numbness of the left leg. His exam reveals paralysis of all leg muscles on the left, normal tone, and numbness to all modalities below the left groin. Deep tendon reflexes are normal and symmetric. The Babinski reflex is absent. This is likely to be a psychiatric problem rather than a cord lesion because: A. To have a numb and weak leg, the lesion should be in the brain, not the spine so that the back pain is physiologically unrelated. B. A spinal cord lesion causing weakness ipsilaterally should cause numbness contralaterally. C. A central cord lesion would cause dissociated sensory loss on both sides D. There are no reflex abnormalities and the numbness is in a non-physiological distribution. E. All of the above statements are true. 38. Poliomyelitis is a viral meningitis that affects anterior horn cells only. It therefore produces a pure lower motor neuron syndrome. Which one of the following would be expected? A. Bladder incontinence B. A sensory level C. Reduced or absent deep tendon reflexes D. Positive Babinski reflex E. Reduced anal sphincter tone 39. A lesion of the thoracic spinal cord would be likely to cause which of the following bladder problems? A. Reduced urgency but increased frequency B. Increased urgency but reduced frequency C. Reduced urgency and reduced frequency D. Increased urgency and increased frequency E. Constant leakage (dribbling) 40. All of the following are example of secondary brain injuries in a head trauma victim except A. Hypoxia B. Hypotension C. Depressed skull fracture D. Intracranial hypertension 41. A skull fracture overlying the middle meningeal groove raises the suspicion of A. Subdural hematoma B. Epidural hematoma C. Raised intracranial pressure D. Transtentorial herniation E. Diffuse Axonal Injury 42. Signs of transtentorial herniation include all of the following except: A. Unilateral third nerve palsy B. Contralateral abnormal motor movements C. Altered consciousness D. Battle’s sign (ecchymosis behind the ear) 43. Subdural hematoma is typically secondary to: A. Ruptured aneurysm B. Tearing of superficial veins C. Laceration of the middle meningeal artery D. Basal skull fracture E. Diffuse Axonal Injury 44. A 25 year old man presents to the ER with a witnessed generalized tonic-clonic seizure. After recovering a bit, he describes a type of warning that preceded his convulsion: a feeling of rising abdominal distress, which moved from his gut to his chest and into his head, after which he is amnestic. In hindsight, he had been experiencing identical odd gastric spells of rising abdominal distress over the preceding year that lasted 10-60 seconds each. He had even been to his doctor who prescribed antacids for presumed reflux. He has complete recall of these gastric spells; they did not attenuate his level of consciousness. Which of the answers below best fits the isolated gastric spells? A. Absence seizure B. Atonic seizure C. Simple Partial seizure D. Complex Partial Seizure E. Non-epileptic seizure 45. A 55-year old woman presents to the ER with a seizure in which she first noted repetitive twitching of her left hand and left side of her mouth. Over 30 seconds, this twitching proceeded to extend to the left shoulder and left thigh twitching followed. Based upon the above, which of the choices below is the most apt description: A. Right occipital seizure B. Left temporal seizure C. Mesial temporal lobe seizure, laterality uncertain D. Right frontal lobe seizure E. Hypothalamic seizure 46. drop question All of the following are well known to be associated with seizures, except A. Hypoglycemia B. Hyperglycemia C. Strobe lights D. Pungent odors E. Binge ethanol consumption 47. drop question Convulsive syncope can mimic a seizure. All of the following features serve to differentiate convulsive syncope from seizure, except A. Occurring while standing in line on a hot day for hours B. Little to no confusion following the spell C. Symmetric twitching of the limbs D. Swooning toward the ground at first E. Occurring while donating blood 48. All of the following are considered mechanisms that are used by existent anti-epileptic drugs, except: A. Acetylcholinesterase inhibition B. Voltage-gated sodium channel blockade C. Augmentation of GABA neurotransmission D. Calcium channel modulation E. Blunting glutamate-mediated neurotransmission 49. drop question Each of the following modulates GABA-A receptor function, except: A. Diazepam B. Phenytoin C. GABA D. Phenobarbital E. Transmembrane concentration gradient of Cl50. The following are all considered broad-spectrum anti-epileptic drugs except: A. Valproate B. Lamotrigine C. Phenytoin D. Topiramate E. Zonisamide 51. drop question Many of the older anti-epileptic drugs, e.g., phenytoin, carbamazepine, valproate and phenobarbital, are associated with excessive drug interactions. All of the following are reasons for this propensity except: A. Autoinduction with carbamazepine B. Predominantly cleared by renal route C. Cytochrome P-450 enzyme induction with several D. Cytochrome P-450 enzyme inhibition with valproate E. High protein binding in phenytoin and valproate 52. drop question Which one of the following would NOT present with the acute onset of vertigo, ataxia, nausea and vomiting lasting two hours before spontaneously remitting? A. Meniere’s Disease B. Vertebral-basilar TIA C. Migraine D. Viral Neurolabyrinthitis E. Perilymphatic fistula 53. Which one of the following would present with brief paroxysms of positional vertigo and would likely respond to Epley (particle repositioning) Maneuvers? A. Herpes zoster oticus (Ramsay Hunt Syndrome) B. Bacterial labyrinthitis. C. Canalolithiasis D. Perilymphatic fistula E. Luetic (syphilitic)labyrinthis 54. Which of the following symptoms would likely be present in the chronic stage of viral neurolabyrinthitis A. Severe persistent vertigo B. Spatial disorientation C. Nausea and vomiting D. Recurrent episodes of vertigo E. Severe ataxia 55. Which of the following would present with paroxysms of vertigo lasting hours, unilateral hearing loss, tinnitus and fullness in the ear? A. Cupulolithiasis B. Vertebral-basilar TIA C. Meniere’s Disease D. Viral Neurolabyrinthitis E. Migraine