HISTORY OF PRESENT ILLNESS:

This 63-year-old right-handed female homemaker was referred for neurologic evaluation of her persistent headache and diplopia.  The patient has a past history of hypertension, diabetes, tobacco use, and two prior episodes of Bell’s palsy, one on the right side (1977) and one on the left side (1982).  The patient’s present headache problem began approximately two months prior to consultation (9-10-83), and there were no specific circumstances surrounding the onset of the problem.  She described a severe left periorbital and orbital head pain, which has been present continuously since that date.  Approximately one week after the onset of the headache (9-18-83), the patient had the acute onset of diplopia, which has also persisted to the present time.  Aside from the diplopia, the patient experienced absolutely no other neurologic signs or symptoms during the past two months.  Systemic and autonomic symptoms associated with the headache and diplopia include nausea and vomiting, photophobia in the left eye, sonophobia, and the desire to lie quietly in a dark room.  The diplopia had both horizontal and vertical components.  It was most pronounced with right horizontal gaze and upgaze. 

NEUROLOGICAL EXAMINATION:

Mental Status Exam:  Intact.

Cranial Nerves:  Sensation of smell was intact bilaterally.  Visual acuity was J1 in the right eye and J2 in the left eye with her glasses.  Visual field and funduscopic examinations were unremarkable.  External ocular movements were full without nystagmus in the right eye.  In the left eye, the patient had full abduction and depression of the eye when it was in the adducted position.  However, adduction and other vertical movements were restricted, although not entirely so.  The pupils were equal (4mm), round, and reactive to light and accommodation.  There was a 1 mm ptosis of the left eyelid.  Sensory and motor functions of the Vth cranial nerve were normal.  There was no facial weakness.  Hearing was intact bilaterally.  XIth cranial nerve functions were normal.  Tongue and palate appeared normal.

Motor System:  There was no evidence of focal or generalized muscular atrophy.  There were no fasciculations.  Muscle tone and power were normal in all four limbs. 

Reflexes:  Deep tendon reflexes were equal and symmetric, and the plantar reflexes were flexor bilaterally. 

Sensory System:  All modalities of sensation, including cortical sensations, were normal, except for distal blunting to pinprick and vibration in both lower limbs. 

Cerebellar Function:  Intact.

Questions

1.  Do the left eye signs and symptoms in this case indicate involvement of the sympathetic nervous system, parasympathetic nervous system, brain stem, a cranial nerve or nerves, an extraocular muscle or muscles, or a disorder of neuromuscular transmission?  Cite the evidence supporting your conclusion. 
          

2.  Do you think this patient’s headache problem is due to a serious or structural problem or a more benign process?  

3.  Indicate the specific structures involved by the pathologic process in this case and the most likely anatomical location of the lesion.
            

4.  In general, what type of pathologic process is involved in this case?
            

5.  At this point what would be your working diagnosis?  What other diagnoses would you consider? 
            

6.  What diagnostic procedure(s) would you undertake at this point?

Answers

Follow-Up Visit: 1-3-1984

HISTORY:

Since the patient’s initial visit on 11-8-83, her diplopia and left-sided headache have continued to improve and are almost completely resolved.

However, on 12-20-83, the patient noted the acute onset of difficulty seeing out of the right eye.  She described this difficulty as if she were looking through a spider web.  There was also a scintillating quality to the impaired vision.  These visual symptoms were accompanied by a left hemicranial headache, which was entirely different from her initial periorbital head pain. 

EXAMINATION:

1.  In view of the above clinical course, is there a change in your working diagnosis concerning the patient’s initial head pain and diplopia?  If so, what is your tentative diagnosis of those findings at this point?                 

2.  How would you describe this patient’s visual findings?
                       

3.  Concerning the new visual symptoms, indicate the specific structures involved in producing these signs and symptoms.  Indicate the most likely anatomical location of the lesion.  Is there only one anatomical location that can produce these visual signs and symptoms?  If so, what is that location?  If not, what are the possible locations of such a lesion?
       

4.  Concerning the patient’s new visual findings, what general type of pathologic process do you think was involved in producing these findings?
                       

5.  Are there any additional diagnostic procedures that you would like to undertake at this point? 

Follow-Up Visit: 5-22-1985

HISTORY:

Since the patient’s last clinic visit, she remained stable on treatment for her condition.  However, in March 1985, she had the acute onset of the inability to recognize familiar faces and familiar places.  At that time, she also noted that colors did not look normal to her and that she was having difficulty distinguishing between some colors.  This difficulty in recognizing familiar faces and objects and the difficulty with color recognition persisted without change for the past two months. 

EXAMINATION:

The patient’s neurological examination was essentially unchanged from 16 months ago.  She had a right superior homonymous quadrantanopsia.  The remainder of her neurologic examination was essentially unremarkable, except for her mild distal sensory polyneuropathy involving both lower limbs.  There was no evidence of any visual field defect in the left visual fields of either eye.  She indicated to me that she sort of recognized me, but not entirely so.  Also, she gave a history of not recognizing family members and familiar streets and scenes.  In testing color, she thought a yellow sheet of paper was white and thought a red object was probably red, but perhaps deep orange. 

Questions

1.  What is the term describing the patient’s inability to recognize familiar faces?
                        

2.  What is the term describing the patient’s inability to recognize familiar objects and places? 
                       

3.  What is the term describing the patient’s difficulty with color identification?
                       

4.  What is the specific anatomical location of the lesion producing the above findings?
                

5.  Are the above findings due to her most recent lesion or are they due to a combination of her present lesion plus one of her past lesions?  If so, which of her prior problems is pertinent to her present condition?
               

6.  In general, what pathologic process is involved in her most recent lesion?
              

7.  In general, what pathologic process is involved in all of the neurologic events that this patient has sustained?