HISTORY OF PRESENT ILLNESS:

This 41-year-old right-handed white female housewife was admitted to the hospital for evaluation of weakness.  In October of 1978, the patient noted the onset of predominantly proximal muscle weakness in all 4 limbs, although the right side seemed worse than the left.  This weakness progressed over the subsequent 5 months in both the upper and lower limbs, and, at the time of admission, the patient was having much difficulty walking.  Her right upper limb was weaker than the right lower limb.  Furthermore, the patient noted that her muscles were twitching beneath her skin in all 4 limbs, particularly in the deltoid muscles.  This began approximately 3 to 4 months prior to admission and progressed slowly along with the weakness.  

NEUROLOGICAL EXAMINATION:

Mental Status Exam:  Intact.

Cranial Nerves:  Cranial nerve examination was entirely normal, except that the tongue muscles were mildly atrophic and exhibited fasciculations bilaterally.

Motor System:  There were no areas of focal muscular atrophy.  Fasciculations were observed in the deltoid muscles bilaterally and in the quadriceps muscles bilaterally.  Muscle tone was increased in both upper and lower limbs in the form of spasticity.  Muscle power was essentially normal (5/5) distally in the left upper and lower limbs, but was diminished proximally and rated at 4/5.  On the right side, muscle power was rated at 4-/5 throughout, although the distal muscles appeared slightly stronger than the proximal muscles.

Reflexes:  Deep tendon reflexes were hyperactive bilaterally (4/4).  There was unsustained ankle clonus bilaterally.  The jaw jerk was brisk.  Plantar reflexes were extensor on the right and equivocal on the left.

Sensory System:  Intact.  

Cerebellar Function:  Intact.

Gait and Stance:  Regular gait was performed on a widened base and exhibited spasticity in both lower limbs.  Tandem gait could not be performed.  The Romberg test was negative.

1.  Can this patient’s signs and symptoms be localized to one single area of the central nervous system?  If so, where is the neurologic lesion located, and which side of the central nervous system is involved?

2.  What regions, levels, and/or structures of the central nervous system are involved with signs or symptoms of lower motor neuron (LMN) disease?
 
3.  What regions, levels, and/or structures of the central nervous system are involved with signs or symptoms of upper motor neuron (UMN) disease?
 
4.  The findings in this case represent a classic neurologic syndrome.  What is the name of this syndrome?

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

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