1. Which of the following is not a feature of Parkinson’s disease?a) Resting tremor of hands
b) Bradykinesia
c) Resting tremor of the feet
d) Paralysis of lower extremities
e) Improvement with levodopa
2. Huntington’s disease is characterized by a) autosomal recessive inheritance
b) chorea and dementia
c) atrophy of the putamen on CT / MRI scan
d) an abnormal decrease in the number of CTG repeats
e) Low serum ceruloplasmin levels
3. Which of the following is not true about the substantia nigra?a) It degenerates in Parkinson’s disease
b) The pars reticulata and pars compacta are functionally different
c) 90 % of the D1 and D2 dopamine receptors in the brain are located here
d) It is pigmented because of the presence of neuromelanin
e) It is located in the midbrain
4. Idiopathic Parkinson’s diseasea) May present with depression and fatigue
b) Can be caused by exposure to neuroleptic medications
c) Usually affects the age-group 40-50 years
d) Is usually diagnosed by an MRI head scan
e) Can be diagnosed by testing for the parkin gene
5. Which of the following is true about the basal ganglia?a) The putamen efferents project chiefly to the red nucleus
b) The globus pallidus interna and externa are functionally alike
c) The caudate head degenerates in Parkinson’s disease
d) The putamen receives nigrostriatal dopaminergic fibers
e) The tail of the caudate nucleus degenerates in Huntington’s disease
Answers: 1d, 2b, 3c, 4a, 5d
For the past 5 weeks a 35-year-old woman has had episodes of intense vertigo lasting several hours. Each episode is associated with tinnitus and a sense of fullness in the right ear; during the attacks, she prefers to lie on her left side. Examination during an attack shows that she has fine rotary nystagmus, which is maximal on gaze to the left. There are no ocular palsies, cranial-nerve signs, or long-tract signs. An audiogram shows high-tone hearing loss in the right ear, with recruitment but no tone decay. The most likely diagnosis in this case is A labyrinthitis
B Ménière's disease
C vertebral-basilar insufficiency
D acoustic neuroma
E multiple sclerosis
The answer is B (review chapter chapter 21).
Explanation:
The symptoms and signs described in the question are most consistent with Ménière's disease. In this disorder, paroxysmal vertigo resulting from labyrinthine lesions is associated with nausea, vomiting, rotary nystagmus, tinnitus, high- tone hearing loss with recruitment, and, most characteristically, fullness in the ear. Labyrinthitis would be an unlikely diagnosis in this case because of the hearing loss and multiple episodes. Vertebral-basilar insufficiency and multiple sclerosis typically are associated with brainstem signs. Acoustic neuroma only rarely causes vertigo as its initial symptom, and the vertigo it does cause is mild and intermittent.
A 25-year-old woman presents to the emergency department with a severe, throbbing headache of the right supraorbital area for the past hour. She also complains of nausea and photophobia. She has had similar attacks in the past, often brought on by menstruation. About 45 min ago she took 400 mg of ibuprofen. Which of the following would be the best therapeutic choice at this time? A Meperidine, 50 mg intramuscularly
B Codeine, 60 mg orally
C Naproxen, 750 mg orally
D Sumatriptan, 6 mg subcutaneously
E Verapamil, 300 mg orally
The answer is D (review chapter chapter 15, Welch, N Engl J Med 329:1476-1483, 1993).
Explanation:
While the pathophysiology of migraine remains unclear, electrical stimulation of midline dorsal raphe in the brainstem leads to characteristic pain. Pharmacologically, serotonin-mediated neurotransmission appears to be critical in the generation of migrainous pain. Sumatriptan and dihydroergotamine both work by blocking 5-hydroxytryptamine receptors (type I, especially the D subtype). While nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen and naproxen are helpful in patients with mild to moderate migraine, presumably by reducing inflammatory stimuli from cyclooxygenase inhibition leading to reduced prostaglandin generation, the patient in the question has too severe an attack to benefit from the additional use of this class of agents. Also, the use of narcotic analgesics as a primary therapy is no longer recommended; sumatriptan will relieve a migraine headache in ~75% of patients within 1 h of treatment. Unfortunately, because of its short half-life (with either oral or subcutaneous administration), headache recurs in up to one-third of patients. Sumatriptan-associated side effects are usually mild to moderate and highly reversible; they include reactions at the injection site, flushing sensations, and neck pain or stiffness. Although up to 5% of patients treated with sumatriptan experience chest tightness or pressure, myocardial ischemia is exceedingly rare. Nonetheless, this drug should not be given to those with a history of myocardial infarction, ischemic heart disease, or Prinzmetal's angina. Both -adrenergic antagonists and calcium channel blocking drugs are effective prophylactic agents in patients with frequent migraines.
A 29-year-old woman who uses oral contraceptives comes to the emergency room because when she looked in the mirror this morning, her face was twisted. It felt numb and swollen. While eating breakfast, she found that her food tasted different and she drooled out of the right side of her mouth when swallowing. Neurologic examination discloses only a dense right facial paresis equally involving the frontalis, orbicularis oculi, and orbicularis oris. Finger rubbing is appreciated as louder in the right ear than in the left. The physician should A instruct the patient in using a patch over the right eye during sleep
B recommend that she discontinue the use of oral contraceptives
C order brainstem auditory evoked potentials to assess her hearing asymmetry
D inform her that her chances of substantial improvement within several weeks are only about 40%
E order an echocardiogram to rule out mitral valve prolapse as a source of emboli
The answer is A (review chapter chapter 367).
Explanation:
The abrupt appearance of an isolated peripheral facial palsy, which may include ipsilateral hyperacusis resulting from involvement of fibers to the stapedius and loss of taste on the anterior two-thirds of the tongue resulting from involvement of the fibers of the chorda tympani, is most often idiopathic, as in Bell's palsy. If the patient is unable to close the eye, artificial tears may be helpful during the day to prevent drying, and the eye should be patched at night to prevent corneal abrasion. Excellent recovery occurs in 80% of these cases. Oral contraceptives and mitral valve prolapse are not associated with the causes of this clinical picture. Evoked potentials are not helpful diagnostically.
A 78-year-old woman with metastatic breast cancer arrives in your clinic for evaluation. She is currently receiving palliative chemotherapy. The patient lives with her husband and her 40-year-old son. Over the past several months she has fractured two vertebral bodies and has been hospitalized for a deep venous thrombosis. Both she and her family have sought aggressive treatment. Her husband is ill from advanced coronary artery disease, and the patient is his primary caregiver. Unfortunately, as her disease has progressed she has become too sick to care for her husband. Her pain remains poorly controlled, and she is receiving naproxen and morphine, in both the long- acting as well as immediate-release form. She has lost ~11.5 kg (25 lb) over the past 3 months, and her appetite is described as poor. On further questioning the patient volunteers that she feels her future is over and that only pain and suffering lie ahead. The patient is gravely worried about the care her husband will receive in the future with her loss. She feels she is no longer able to serve as his caregiver and feels saddened by this. She is focused on the suffering she is putting both her husband and friends and family through with her physical and emotional decline. She states that she is sad and nervous about the future and has been unable to focus on anything else. Which of the following diagnoses best describes the mental status of this patient? A Grief
B Adjustment disorder
C Depression
D Posttraumatic stress disorder (PTSD)
E Dysthymic disorder
The answer is C (review chapter chapter 385, Block, Ann Intern Med 132:209-218, 2000).
Explanation:
Several factors indicate the patient is depressed. Physicians caring for terminally ill patients should always consider the diagnosis of depression. The diagnosis of depression should be considered in any geriatric patient who complains about increasing levels of somatic concern. The fact that the patient is unable to imagine anything positive in her future and the fact that she feels inability to contribute and believes her presence is only a burden to others suggests underlying depression. An important point is that although many patients and clinicians believe that depression is a normal feature of a terminal illness, most terminally ill patients do not become depressed. The differential diagnosis includes anxiety. Anxiety may coexist with depression and in some patients may be manifested as an anxious depression. In addition, organic mental disorders such as delirium may be caused by metastatic disease or paraneoplastic syndrome.
A 45-year-old man presents with a daily headache. He describes two attacks per day over the past 3 weeks. Each attack lasts about an hour and awakens the patient from sleep. The patient has noted associated tearing and reddening of his right eye as well as nasal stuffiness. The pain is deep, excruciating, and limited to the right side of the head. The neurologic examination is nonfocal. The most likely diagnosis of this patient's headache is A migraine headache
B cluster headache
C tension headache
D brain tumor
E giant cell arteritis
The answer is B (review chapter chapter 15).
Explanation:
Cluster headaches, which can cause excruciating hemicranial pain, are notable for their occurrence during characteristic episodes. Usually attacks occur during a 4- to 8-week period in which the patient experiences one to three severe brief headaches daily. There may then be a prolonged pain-free interval before the next episode. Men between ages 20 and 50 are most commonly affected. The unilateral pain is usually associated with lacrimation, eye reddening, nasal stuffiness, ptosis, and nausea. During episodes alcohol may provoke the attacks. Even though the pain caused by brain tumors may awaken a patient from sleep, the typical history and normal neurologic examination do not mandate evaluation for a neoplasm of the central nervous system. Acute therapy for a cluster headache attack consists of oxygen inhalation, although intranasal lidocaine and subcutaneous sumatriptan may also be effective. Prophylactic therapy with prednisone, lithium, methysergide, ergotamine, or verapamil can be administered during an episode to prevent further cluster headache attacks.
A 25-year-old woman who was the driver of a car struck in the rear by another car while she was stopped at a red light presents to the emergency department with neck pain as well as discomfort in the axilla, upper arm, elbow, dorsal forearm, and index and middle fingers. Coughing exacerbates the pain. Neurologic examination reveals weakness in the right second and third fingers, forearm, and wrist. The right triceps reflex is diminished. The most likely diagnosis in this case is A syringomyelia
B cervical sprain
C thoracic outlet syndrome
D cervical disk herniation
E brachial plexopathy
The answer is D (review chapter chapter 16).
Explanation:
Herniation of a lower cervical disk may be due to trauma, especially in the setting of neck hyperextension. If the disk herniates laterally, it will generally compress the nerve route exiting the lower of the two vertebrae that account for the intervertebral space. For example, if the disk between the fifth and sixth cervical vertebrae herniates, the full syndrome will be characteristic of a C6 radiculopathy: pain in the trapezius, shoulder, radial forearm, and thumb; absent biceps reflex; and preserved triceps reflex. A C7 radiculopathy caused by a disk protruding between the sixth and seventh cervical vertebrae will produce the following: pain in the shoulder blade, pectoral and medial axillary region, upper arm, elbow, dorsal forearm, and index and middle fingers; paresthesia and sensory loss in the second and third fingers or the tips of all the fingers; weakness in forearm and wrist extension as well as hand grip; and a preserved biceps reflex but a diminished triceps reflex. Coughing and sneezing often exacerbate the pain caused by a herniated cervical disk. Unlike the lateral disk syndromes mentioned above, a disk that herniates centrally may be painless but cause symptoms in the lower extremities.
A patient with previous spells of diplopia, ataxia, dysarthria, and dizziness becomes acutely comatose. The most likely cause is A basilar artery thrombosis
B subarachnoid hemorrhage
C carotid occlusion
D cerebellar hemorrhage
E pontine hemorrhage
The answer is A (review chapter chapter 24, chapter 361).
Explanation:
Patients with basilar artery stenosis frequently have spells of ischemic brainstem dysfunction before a catastrophic stroke caused by arterial thrombosis. Timely anticoagulation and allowing a higher blood pressure can arrest the progression of this potentially fatal stroke. Acute coma can occur in association with each of the cerebrovascular accidents mentioned in the question except carotid occlusion. Subarachnoid hemorrhage causes an acute increase in intracranial pressure that reduces blood flow to the brain. Unilateral cortical infarction does not cause coma, but damage to brainstem structures via infarction or compression will cause coma.
A 75-year-old woman complains of dizziness and lightheadedness while walking. The patient has had long- standing diabetes and is taking an oral hypoglycemic agent. She has no other medical problems and lives alone. Physical examination reveals visual acuity of 20/80 in both eyes and sensory neuropathy in a stocking-glove distribution. On close questioning, she denies any symptoms of "herself spinning or the world spinning." She has no apparent anxiety or depression. Orthostatic vital signs are normal. A head-tilt maneuver reveals no nystagmus. The most likely diagnosis in this case is A dysequilibrium of aging
B benign positional vertigo
C Ménière's disease
D brainstem stroke
E neoplasm of the central nervous system
The answer is A (review chapter chapter 21, chapter 22, Froehling, JAMA 271:385-388, 1994).
Explanation:
The evaluation of a "dizzy" patient relies on a combination of careful history taking and neurologic examination. It is important to get a sense of whether the patient has true vertigo, which is usually manifest as the sensation that either the world or the patient is spinning. Some elderly patients complain of dizziness while ambulating or standing without true vertigo, although they may have mild lightheadedness. Typically these patients have peripheral neuropathy, myelopathy, parkinsonian rigidity, cerebellar ataxia, or poor vision. Such patients actually have multiple sensory-defect dizziness, also known as benign dysequilibrium of aging. Unlike patients with benign paroxysmal positional vertigo, they should not display excess nystagmus on head-tilt testing. Central lesions are unlikely given a neurologic examination that was normal except for the peripheral neuropathy and other sensory deficits.
A 35-year-old woman complaining of trouble with her "peripheral vision" is subjected to visual field examination. While one eye is tested at a time, she is asked to focus on a central target while the examiner's fingers are moved in from various directions. She is unable to distinguish objects brought laterally toward the midline, encompassing about half the visual field in each eye. Which of the following lesions would most likely account for these findings? A Open-angle glaucoma
B Closed-angle glaucoma
C Multiple sclerosis
D Pituitary tumor
E Embolic occlusion of the posterior cerebral artery
The answer is D (review chapter chapter 28, chapter 328).
Explanation:
Knowledge of visual pathway anatomy is necessary for an understanding of visual field defects. Monocular visual field loss often results from retinal fiber loss, corresponding to lesions visible on ophthalmoscopic examination. Retinal fibers traveling in the optic nerve change direction at the optic chiasm so that the right brain appreciates left visual space and the left brain appreciates right visual space. Therefore, a discrete vertical midline characterizes all visual pathway disorders resulting from lesions at or posterior to the chiasm. Because chiasmal lesions interrupt the central fibers that mediate temporal vision (with peripheral fibers mediating more midline vision), a pituitary tumor or craniopharyngioma (which typically impinges centrally) results in loss of visual fields in the bitemporal regions. If a lesion exists well posterior to the optic chiasm, such as loss of visual cortex in the one occipital lobe as a result of an embolism in the posterior cerebral artery, a complete loss of visual perception in one field will result. For example, destruction of the right visual cortex will lead to complete left homonymous hemianopia with loss of temporal vision in the left eye and medial vision in the right eye.
Evoked-potential testing is most useful in diagnosing A brainstem involvement in stroke
B a clinically occult lesion in multiple sclerosis
C large hemispheral strokes
D spinal cord compression
E shearing of white matter tracts after head injury
The answer is B (review chapter chapter 357).
Explanation:
The testing of evoked potentials is of the greatest utility in detecting subclinical spinal cord and optic nerve lesions. Up to two-thirds of persons with multiple sclerosis have neurologic deficits that are evident on visual or peroneal somatic evoked potentials but not on physical examination. A "second lesion" of this type frequently establishes the diagnosis of multiple sclerosis. Evoked potentials may be abnormal in the other conditions listed in the question.
Drop attacks may be exacerbated by carbamazepine in patients with Rolandic epilepsy. True
Explanation: Rolandic epilepsy is a benign epilepsy of childhood. Drop attacks in these patients may be precipitated by carbamazepine.
Which neuroleptic is least likely to exacerbate Parkinson’s disease? A. Risperidone
B. Thioridazine
C. Haloperidol
D. Clozapine
D.Clozapine is the least likely to exacerbate extrapyramidal symptoms, although it requires frequent blood monitoring.
In addition, clozapine has fairly significant anticholinergic effects which may exacerbate cognitive deficits.
A 27-year-old male presents with the abrupt onset of headache, which abates during the next 24 hours. Brain CT is normal including with contrast. CSF shows 3 WBCs/ul, no RBCs, normal glucose and protein. Which of the following statements is not true? A. Aneurysm may still be present despite these findings, although migraine is the most likely diagnosis.
B. Recurrent localized pain may be due to aneurysm despite the absence of meningeal symptoms.
C. The normal CT and CSF rule out aneurysmal rupture, securing the diagnosis of migraine.
D. Sentinel headaches occur in about half of patients with aneurysmal rupture.
C. The normal CT and CSF are reassuring but cannot completely rule out the diagnosis of intracranial aneurysm. Hemorrhage within the walls of the vessel may produce headache with acute aneurysmal enlargement without the CSF findings.
A 57-year-old female presents with decreased responsiveness, horizontal gaze palsy, pinpoint pupils, and decerebrate posturing. Which would be the most likely etiology? A. Mesencephalic hemorrhage
B. Pontine hemorrhage
C. Cerebellar hemorrhage
D. Thalamic hemorrhage
B. This clinical presentation is typical of pontine hemorrhage. Damage to pontine gaze centers, reticular formation, and corticospinal tracts will produce most of these symptoms. Cerebellar hemorrhage can produce brainstem compression and these findings but usually not as an initial presentation.
A 68-year-old man develops a rest tremor of the right hand and arm. The patient moves slowly and has a diminished range of facial expressions. He has no postural abnormalities. Which of the following drugs would be most appropriate at this time? (A) Deprenyl
(B) Levodopa
(C) Carbidopa-levodopa (Sinemet)
(D) Bromocriptine
(E) Benztropine
The answer is A. (Chapter 368. Standaert, Med Clin North Am 77:169, 1993)
The explanation for the correct response is:
Parkinson's disease is a chronic degenerative disease of middle-aged and elderly persons that is characterized pathologically by a decrease in dopaminergic transmission in the caudate nucleus and putamen. Early manifestations of the disease include a unilateral rest tumor with a frequency of 4 to 5 per second. The tremor may progress to involve structures on both sides of the body with eventual postural imbalance, profound restriction of movement, and eventual degeneration to a chair-bound existence. Total paralysis is highly uncharacteristic, and tendon reflexes as well as sensory examination are normal. The early stage of the disease can be treated with deprenyl, a monoamine oxidase inhibitor that slows disease progression. Treatment of more progressive Parkinson's disease requires dopamine replacement in the form of levodopa. Levodopa is given in combination with a dopa-decarboxylase inhibitor (carbidopa), which prevents bloodstream destruction of levodopa but is unable to pass through the blood-brain barrier. Carbidopa in combination with levodopa in a ratio of 1:4 or 1:10 (Sinemet) is available. Though costly, dopamine-receptor agonists such as bromocriptine may be used to lower the required dose of Sinemet. Anticholinergic drugs such as benztropine and trihexyphenidyl may constitute useful adjunctive therapy but must be used carefully because of the side effects of confusion, glaucoma, urinary retention, and progression of dementia. Amantadine, which causes the release of dopamine from presynaptic terminals, also may be useful early in the disease. Unfortunately, as the disease progresses, the therapeutic index of the levodopa-carbidopa combination decreases. If levodopa-induced hallucinations occur, clozapine may be helpful, although neutropenia may occur.
Which is the most likely cause of caudate hemorrhage? A. AVM
B. Aneurysm
C. Hypertension
D. Hemorrhagic transformation of infarction
C. Hypertension is the most common cause of caudate hemorrhage, and of most hemorrhages.
AVM or aneurysm is unusual in this area. Also, hemorrhagic transformation of a small vessel infarction
producing caudate infarction would not be expected.
