Case History #26

Date of Consultation: 10-25-1987

This 70-year-old right-handed white male retired salesman was referred for neurological consultation for evaluation of weakness and other neurologic symptoms.  Approximately one to two years prior to consultation, the patient and his wife noted the gradual, insidious onset of a number of neurologic symptoms.  These included a generalized slowing of many motor activities.  Especially prominent in this was a tendency to walk with a stooped posture and with diminished arm swing.  However, almost all motor activities were performed more slowly and deliberately than before.  The patient's voice became lower in volume and more monotonous.  A mild tremor or "nervousness" of the right upper limb was noted.  When writing, he noted a tendency for the words to become smaller and smaller (micrographia).  The patient denied any festination associated with his gait and also denied any knowledge of muscle rigidity.  He noted a mild tendency to lose his balance, but he had not actually fallen.

The patient denied any exposure to medications such as Aldomet, antipsychotic preparations, or antihistamines.  He denied prior exposure to carbon monoxide or manganese.  He did not contract encephalitis during the epidemic after World War I. 

NEUROLOGICAL EXAMINATION: 

Mental Status Exam:  Intact.  The patient's speech was of low volume and somewhat monotonous and dysarthric in character. 

Cranial Nerves:  Intact.

Motor System:  There were no areas of focal muscular atrophy.  There were no fasciculations.  Muscle power was normal in all 4 limbs.  Muscle tone was increased to a moderate degree in all 4 limbs with the upper limbs being worse than the lower.  There was cogwheel rigidity in both upper limbs with the right being worse than the left.  There was also a mild tremor present in both upper limbs, which was worse on the right than the left.  This was most prominent when the patient was at rest and tended to dampen as he initiated voluntary movement.  In general, all types of movements were performed slowly, although accurately.

Reflexes:  Deep tendon reflexes were equal and symmetric bilaterally.  The plantar reflexes were flexor bilaterally.  There was a positive glabellar tap and positive snout reflex. 

Sensory System:  Intact.

Cerebellar Function:  Intact.

Gait and Stance:  Regular gait was performed fairly briskly but did have a slight shuffling character to it.  The patient walked with a stooped posture, and there was a decrease in arm swing.  Tandem gait was performed adequately, although the patient did exhibit a slight tendency to lose his balance (postural instability).  The Romberg test was negative.

Head and Neck Exam:  The patient exhibited a lack of normal facial expressions and had a somewhat blank look on his face (masked facies).

Questions:

1.  This patient's signs and symptoms constitute a classical neurologic syndrome.  What is the name of that syndrome?

2.  What is the anatomical location of the basic pathologic process leading to this patient's condition?  What anatomical pathway(s) is involved in this patient's condition? 
    
3.  What neurotransmitter systems are involved in the manifestations of this syndrome, and which manifestations are associated with which neurotransmitters?
   
4.  In general, what type of pathologic process is involved in this syndrome? 
   
5.  What diagnostic procedure(s) would you undertake at this point?