2005 Exam I Case 02

Chief Complaint:
A 62-year-old right-handed male art professor presents to your office reporting difficulty holding his pencil and an inability to write legibly.  He notes that his arms and legs have been getting weaker and states that he has muscle cramping in all extremities, especially early in the morning.   

History of Chief Complaint:
The patient reports an active lifestyle up until 12 months ago when he noticed cramping and weakness in his right hand that limited his ability to demonstrate painting techniques to his art classes.  Six months ago, the patient noted muscle wasting in both hands and weakness in all four extremities.  He admits to changing his diet because of difficulty swallowing.  His wife mentions that she often has to ask him to speak louder.  He states that his fear of receiving bad news has delayed his current visit because he is worried that his symptoms may be related to prior exposure to lead-based paints.  

Social History:
He is a faculty member at a local art college and owns a gallery in Kennebunkport.  He is married with two grown children.  He admits to consuming 1-2 glasses of wine per week.  He denies a history of tobacco or drug use.  

Physical Exam:
Patient is awake and resting in supine.  Heart rate is 74, blood pressure is 128/84, temperature is 98.7°F, and respirations are 19.  Peripheral pulses are intact at the wrists and ankles.  Abdomen is soft with no masses; normal bowel sounds are present.  Significant muscle atrophy is noted at the shoulders, hands, and legs.   

Neurologic Exam:    

Mental Status: Patient is alert and oriented to person, time, and place.  His speech is slightly dysarthric, slowed, and very quiet.  Speech patterns and content are meaningful.   

Cranial Nerves:  A full range of eye movements is present.  Pupillary reflexes are intact to both direct and consensual light.  Hearing is intact to finger rub at both ears.  Facial expressions are intact and bilaterally symmetrical.  Corneal and jaw-jerk reflexes are intact, gag reflex is present, but sluggish.  A pin prick response is present throughout face.  Tongue protrudes slowly at midline and has fasciculations.

Motor:  Strength is 3/5 at the shoulders and 2/5 at the elbows bilaterally.  Right grip strength is 2/5.  Left grip strength is 3/5.  Bilateral lower extremity strength is 4/5 throughout.    Upper extremity deep tendon reflexes are depressed bilaterally at 1/4 throughout.  Both knee reflexes are 3/4 and ankles were 4/4.  Fasciculations are present at rest in all four extremities. 

Sensory:  Pain, temperature, vibratory sense, and proprioception sensations are intact throughout his body.

Follow-Up:
Four months later, the patient demonstrates increased weakness in all four extremities.  Deep tendon reflexes remain at 1/4 throughout upper extremities.  Lower extremity deep tendon reflexes are 1/4 throughout.  He can eat only blended foods, no solids, without choking.  He also requires assistance with all activities of daily living (ADLs) and is asking for authorization for a wheelchair purchase.

Questions

INSTRUCTIONS: Provide the BEST or MOST LIKELY answer to the following multiple choice questions.