This 37-year-old white female school teacher was referred for
neurological consultation to evaluate multiple neurologic
symptoms. Approximately 2 to 3 weeks prior to consultation, the
patient noted difficulty in typing with the left hand and also noted
that when she reached for things with the left hand she would miss
them. Soon thereafter, she noticed that fine coordinated
movements involving the left hand, such as buttoning her clothes, also
were deteriorating. These symptoms of incoordination continued to
progress up to the time of consultation. Approximately 2 weeks
prior to consultation, she also noted numbness, tingling, and a cold,
heavy feeling involving the left hand and much of the left upper
limb. Within a few days, the sensation of coldness and heaviness
began to spread onto the left side of the trunk, both anteriorly and
posteriorly, and by the time of consultation extended from about the
3rd rib down to the groin but not really into the left lower
limb. During this period of time, the left upper limb also felt
somewhat weak to the patient.
PAST NEUROLOGICAL HISTORY:
The patient gave a history of prior neurologic problems. In 1976,
she noted the gradual onset of decreased vision in the right eye that
stabilized for a short time and then completely resolved within 3
weeks. The right eye had been asymptomatic since. In March
of 1979, the patient noticed the gradual onset of decreased vision in
the left eye, which progressed to perception of hand motion only over a
period of 10 days. Associated with this decreased vision, she
noticed pain in the eye with extraocular movements. An
ophthalmologist treated her with Prednisone, and her visual acuity
improved to 20/300 within 2 weeks. The medication was
discontinued after a 10 day taper, and the vision in the left eye again
deteriorated until she could see hand motion only. Prednisone was
started again, and her visual acuity improved back to 20/200. By
the first of June 1979, visual acuity was 20/40 in the left eye and
remained stable since that time. In August of 1979, the patient
noted transient numbness and tingling of the right hand after being out
in the sun and overheating. She jumped into a swimming pool, and
the symptoms completely resolved. Since that time, she had
experienced transient recurrences of right upper limb numbness and
tingling, but these always spontaneously resolved after a few days to a
few weeks.
NEUROLOGICAL
EXAMINATION:
Mental Status Exam: Intact.
Cranial Nerves: Cranial nerve
examination was normal except for the following. Visual acuity
was 20/20 in the right eye and 20/100 in the left eye.
Funduscopic examination revealed moderate pallor of the right optic
disc and marked optic atrophy of the left optic disc. Visual
field testing revealed a dense central scotoma in the left eye but no
abnormality in the right eye. There was a 3+ afferent pupillary
defect (APD, Marcus Gunn pupil) present in the left eye, and there was
80 per cent color desaturation in the left eye. There was a mild
decreased appreciation of pinprick on the left side of the face.
Motor System: There were no
areas of focal muscular atrophy. There were no
fasciculations. Muscle tone was normal in all 4 limbs.
Muscle power was normal on the right side. On the left side,
there was a mild to moderate weakness (4/5) with the upper limb being
worse than the lower limb.
Reflexes:
Deep tendon reflexes were brisk bilaterally in all 4 limbs but were
somewhat brisker on the left side than the right. Plantar
reflexes were flexor bilaterally.
Sensory System: Sensory
examination was normal except for the following. In the upper
limbs, pinprick was perceived much better on the left than the
right. Joint position sense was intact in the right upper limb
and totally absent on the left. In the lower limbs, pinprick,
vibration, and light touch were equal bilaterally. Joint position
sense was mildly impaired in the left foot but was normal in the
right.
Cerebellar Function: The patient
performed all cerebellar tests normally on the right. On the
left, there was dysmetria, dyssynergia, and difficulty with rapid
alternating movements, with the upper limb being much more involved
than the lower limb.
Gait and Stance: Intact.
Questions
1. Considering only the
patient's acute neurologic problem in the 2 to 3 weeks prior to
consultation, what specific structures are involved by the pathologic
process to produce each of the findings listed below? Indicate
the side of the lesion.
a. Ataxia (dysmetria, dyssynergia, dysdiadochokinesia)
on the left side, with the upper limb more involved than the lower limb
b. Numbness and impaired joint position sense
(conscious proprioception) in the left upper limb more than the left
lower limb
c. Weakness and hyperreflexia on the left side, with
the upper limb more involved than the lower limb
d. Diminished pain perception in the right upper limb
e. Diminished pain perception on the left side of the face
2. Where is the lesion producing the above findings located?
3. These findings
constitute a partial example of a classic neurological syndrome.
What is the name of this syndrome?
4. Considering her entire neurologic history, have multiple areas
of the central nervous system been involved, or is a single localized
site involved throughout the entire course of her illness?
5. If multiple areas have been involved, list them and indicate
which structures are involved by the pathologic process. Indicate
the side of the lesion(s).
6. In general, what type of pathologic process do you think is
involved in this case?
7. What diagnostic procedure(s) would you undertake at this
point?
