1. A 50 year old man is brought in to your office by his two daughters. They have noticed an increase in apathy and depressive episodes since the patient's wife (their mother) died one year ago of metastatic lung cancer. The patient has also reduced his eating. He walks more slowly than he did, and seems to labor when getting up from a chair. At times he is unsteady. He denies other symptoms, and his review of systems is negative. He takes only atenolol (a beta- adrengeric blocker) for hypertension. On examination, he appears somewhat apathetic and is slow to carry out your requests. His pulse is 64 per minute and regular, blood pressure is 130/75 in both arms, respirations 14 per minute, and temperature is 97.8°F orally. HEENT and neck exam are normal. The chest, heart, and abdomen are normal. The rectal reveals an enlarged, non-tender, non-nodular prostate with no other masses. The occult blood is negative. The patient's gait is slow with a wide base. The finger-to-nose motion is tentative but usually on the mark. The strength is 5/5 throughout, and the sensation is intact. There is cogwheeling in both arms on elbow extension against resistance. The most likely diagnosis is [1]:

   A. Huntington's Chorea
   B. Depression
   C. Amyotrophic Lateral Sclerosis
   D. Parkinsonism
   E. Myasthenia Gravis

2. A 28 year old woman has a 30 minute episode of blindness in the right eye, followed by gradual return of her vision over the next 2 hours. This was her first episode, and she has no other medical problems or symptoms. She takes no medications, has mild hay fever, does not smoke or use illegal drugs. She drinks 1-2 glasses of wine 3 to 4 times a week with dinner. There is a strong family history of stroke and myocardial infarction. On examination, she is a well appearing young woman in no obvious distress. Her visual acuity is 20/25 in both eyes. The remainder of her ocular, retinal, and extraocular exam is normal. The ears, nose, and throat are normal. The temporal arteries are have normal, equal pulses without pain. There is an electric-shock-like sensation running down her back on full neck flexion but not extension. There is no lymphadenopathy, normal carotid pulses without bruits. Chest, cardiac, abdomen and extremity examinations are normal. The reflexes are brisk throughout, graded slightly hyperreflexic in the patellar and Achilles' tendons. Sensation, tone, gait, and strength are intact. The most likely explanation for the ocular symptoms is [2]:

   A. Acute Glaucoma
   B. Transient Ischemic Attack (TIA)
   C. Anti-Cardiolipin Syndrome
   D. Vasculitis of the Giant Cell or Takayasu Type
   E. Optic Neuritis

3. The most appropriate test to confirm the diagnosis is [3]:

   A. Magnetic Resonance Imaging for plaque-like changes
   B. Computerized Tomography for evidence of ischemic or hemorrhagic stroke
   C. Serological test for lupus anticoagulant and anti-cardiolipin antibody
   D. Temporal Artery Biopsy
   E. Doppler Ultrasound study of the carotid

4. A 68 year old woman comes in for her annual examination. Her only complaint is decreased vision in the left eye. The vision seems to be worse at night. She has no other symptoms or complaints, and takes only hydrochlorothiazide for mild systolic hypertension. Her blood pressure today is 145/82mm in both arms. A quick visual acuity exam reveals 20/30 in the right and 20/60 in the left. There seems to be a decrease in the nasal compared with the temporal field. Extraocular movements are intact. Retinal exam is unremarkable; there are normal bilateral red reflexes. The conjunctiva are normal, and there is normal tearing. The most likely diagnosis is [4]:

   A. Cataract
   B. Glaucoma
   C. Pituitary Adenoma
   D. Hypertensive Retinopathy
   E. Diabetic Retinopathy

5. A 55 year old man with a history of high cholesterol who takes only lovastatin presents with increasing double vision and difficulty swallowing. He had been very well until three months ago when he noted increasing trouble focusing on objects: he saw two closely approximated objects instead of one. When he covered either eye, the double-vision ceased. Over the past month, he developed increasing difficulty swallowing both liquids and solids. He felt that food took a long time to pass down to his "food-pipe," but that it was not really getting stuck. He never smoked tobacco, drinks only occasional alcohol, and uses no illegal drugs. On examination, his blood pressure was 115/75mm, pulse 80 per minute and regular, respirations 16 per minute, temperature 98.4° orally. Extraocular motions appeared sluggish, particularly on lateral left gaze. There were no field cuts; fundascopic exam was unremarkable. The oropharynx was normal, with apparently normal external swallowing motions. Chest, heart, abdomen, and extremity exam was normal. Neurologic examination was notable only for 5-/5 strength in both shoulders, left worse than right. Electrolyte, thyroid function, calcium, and creatine kinase levels were all normal. A radiograph of the chest showed a questionable mediastinal mass but no lung parenchymal lesions. The most likely diagnosis is [5]:

   A. Eaton-Lambert Syndrome
   B. Chronic Inflammatory Demyelinating Polyneuropathy
   C. Myasthenia Gravis
   D. Multiple Sclerosis
   E. Polymyositis

6. A 74 year old woman is admitted to the intensive care unit with pneumonia and mental status changes. She has a history of systolic hypertension and mild chronic obstructive pulmonary disease. She takes hydrochlorothiazide and a beclomethasone inhaler; albuterol inhalers are used on an as needed basis. She had a 50 pack-year history of smoking, quit 10 years ago. She does not drink alcohol or use illegal drugs. She was well until one week prior to admission when she developed a cough productive of yellowish sputum. She was seen in a community health center and erythromycin was prescribed. She became progressively worse over the past 5 days and was found in bed, sleeping but sluggishly reactive, by her family. She was taken to the emergency room. On physical examination she was responsive only to moderate pain. Her temperature was 96.2°F (tympanic), pulse 110, respirations 24, blood pressure 105/60mm in both arms. Pupils were reactive to light; bilateral cataracts were present. Oropharynx was dry. Neck was supple, nontender, without lymphadenopathy; no bruits were heard. Chest exam revealed middle and upper right sided wet rales with egophany. The remainder of the examination was normal except for the mental status; the patient was able to move all four limbs. Laboratory examination was notable for a sodium of 118mM, potassium 3.1mM, chloride 90mM, bicarbonate 25mM, BUN 40mg/dL, creatinine 2.0mg/dL, glucose 75mg/dL. The white cell count was 15.5K/µl with 80% neutrophils, 7% band forms, 8% lymphocytes, 4% monocytes and 1% eosinophils. The patient was treated with aggressive normal saline rehydration, broad spectrum antibiotics, and oxygen. Her mental status improved after 36 hours; the sodium was 145mM. However, 12 hours later she was unable to move her entire right leg, and the lower half of the left leg. Toes were upgoing (dorisflexed) bilaterally. There was a mild but significant expressive aphasia. The most likely explanation for the patient's new neurological symptoms is [6]:

   A. Stroke
   B. Guillain-Barre Syndrome
   C. Central Pontine Myelinolysis
   D. Meningitis with brain abscess
   E. Amyotrophic Lateral Sclerosis

7. Which of the following statements concerning diabetic retinopathy is NOT correct [7] ?

   A. Proliferative and Non-Proliferative forms exist
   B. Intensive control of glucose levels does not inhibit development of retinopathy
   C. Advanced glycosylation endproducts (AGEP) contribute to retinopathy development
   D. Retinopathy is more common in patients with type I diabetes than in those with type II
   E. Laser photocoagulation is the treatment of choice for proliferative retinopathy

8. A 62 year old man presents with increasing weakness and muscle spasms in his legs. He takes only propranolol for hypertension and has no other medical problems. He was well until 2 months earlier when he noticed difficulty getting up from a chair because of weakness in both legs. The weakness progressed to where he needed two canes in order to walk. He has also noticed weakness in his arms over the past two weeks. One month earlier he developed flu-like symptoms and was seen in local hospital emergency walk-in unit. He was told that the weakness was due to a virus; anti-histamines and decongestants were prescribed. The flu-like symptoms resolved but the weakness did not. On examination he is an anxious older man unable to walk without aid. Vital signs are all within normal limits. Extraocular movement are intact. Significant positive findings include 4/5 strength in both legs including flexors and extensors of the foot, ankles, knees; the hips are 5-/5 bilaterally. The shoulders have 5-/5 strength in the abductors bilaterally; remainder of the upper extremity muscles have normal strength. The lower extremity reflexes are abnormally brisk (3/3) bilaterally at the patellar and Achilles tendons. The lower extremity tone is increased, but there is muscle atrophy right greater than left. The toes are upgoing (dorsiflexion) bilaterally (positive Babinski sign). Sensation is completely intact and there is not pain at rest. Cranial nerve exam shoes mild weakness of the orbicularis oris muscle (patient is unable to completely smile on the right). The most likely diagnosis is [8]:

   A. Multiple Sclerosis
   B. Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
   C. Myasthenia Gravis
   D. Amyotrophic Lateral Sclerosis (ALS)
   E. Parkinson's Disease

9. Sinamet®, the mainstay of Parkinson's Disease therapy, is a combination of L-dopa and carbidopa. The function of the carbidopa is [9]:

A. Enhance penetration of L-dopa into the cerebrospinal fluid
B. Prevent breakdown of L-dopa in the brain by blocking cerebral dopa decarboxylase
C. Acts on remaining substantia nigra neurons to increase dopamine secretion
D. Blocks cholinergic input to the substantia nigra and globus pallidus
E. Inhibits dopa decarboxylase in the blood, thus permitting higher levels of L-dopa to reach the brain

10. A 45 year male patient is admitted to the hospital with cocaine induced schizophrenia. He is treated with haloperidol, a butyrphenone neuroleptic agent, intramuscularly, for acute psychosis. Diazepam (valium) is also administered for sedation and the patient is doing well. He receives haloperidol every four to six hours. Over the next 24 hours improves well. The haloperidol is stopped, and the patient is maintained on low doses of valium for sedation. On the following hospital day, he develops a fever to 102°F tympanic, diaphoresis, and marked muscle rigidity. The patient is having difficulty speaking. The pulse is 120 beats per minute and regular; the blood pressure is 160/90mm and equal in both arms. A set of electrolytes is normal, including the calcium, phosphate, and magnesium levels. The most appropriate therapy in addition to intravenous fluids and acetaminophen for the fever is [10]:
A. Bromocriptine
B. Benzodiazepine antagonist (flumazenil)
C. Restart haloperidol
D. Intravenous nitroglycerin
E. Supportive therapy only

Answers

1. D
2. E
3. A
4. B
5. C
6. C
7. B
8. D
9. E
10. A (Bromocriptine is strongly recommended in severe neuroleptic malignant syndrome.)