Case History #24
Date of Consultation: 6-19-1984
HISTORY OF PRESENT ILLNESS:
This 12-year-old right-handed white male junior high school student was referred for neurological consultation because of wasting and weakness in the upper limbs. Five years prior to consultation, the patient fractured his right forearm, and, as the fracture was being reduced, the physician involved commented that most children his age were in a good deal more pain and were much more uncooperative than this patient was. It was then noted on examination that he actually had decreased pain perception in the right upper limb as compared to the left. In the one to two years prior to consultation, it was noted that the patient was losing muscle strength in the right upper limb. By the date of consultation, the patient reported that he was no longer able to fully extend the fingers of his right hand and that his right hand was not as coordinated as the left.
Other neurologic symptoms noted by the parents included a tendency toward urinary urgency and an inability to compete athletically with children of his own age.
DEVELOPMENTAL HISTORY:
The patient was a product of a normal pregnancy, labor, and delivery. He met his early developmental milestones on time. Some delay in speaking properly was noted, but he did not have any difficulty with school work later on. There was some type of congenital anomaly of the fifth digits of the hands, and the patient required braces on both lower limbs in the neonatal period to keep his legs from laterally rotating. He developed scoliosis of the thoracolumbar region of the vertebral column.
NEUROLOGICAL EXAMINATION:
Mental Status Exam: Intact.
Cranial Nerves: Intact.
Motor System: There was a mild degree of atrophy of the shoulder and shoulder girdle muscles bilaterally. In addition, measurements of the circumference of the arms, forearms, and calves revealed all measurements to be 1 cm less on the right side than on the left. There were no fasciculations seen. Muscle tone was normal to perhaps slightly decreased in both upper limbs and was slightly increased in both lower limbs. Muscle power was normal in the left lower limb. In the right lower limb, muscle power was normal except for mild weakness (4+/5) of the right hip flexors. In the left upper limb, muscle power was normal distally but was slightly weak (4/5) proximally. In the right upper limb, muscle power was diffusely weak in the range of 4- to 4+/5 in all muscle groups. The shoulder girdle muscles were also weak with slight winging of the scapulae bilaterally and an inability to abduct the arms above 90 degrees on either side.
Reflexes: Deep tendon reflexes were absent in the right upper limb and 1/4 in the left upper limb. In the lower limbs, deep tendon reflexes were 4/4 on the right and 3 to 4/4 on the left. The plantar reflexes were flexor on the left and extensor on the right.
Sensory System: Sensory examination was entirely normal to all modalities of sensation in both lower limbs. In the right and left upper limbs, pain or pinprick perception was diminished throughout the entire limbs. There was a "suspended sensory level" existing from C2 or 3 above to T1 below. Above and below these segments, pinprick was normal and symmetric bilaterally. Between these levels, pinprick was diminished on both sides. Joint position sense and vibration were perceived normally in both upper limbs.
Cerebellar Function: Intact.
Gait and Stance: Intact.
Neck and Back Examination: Scoliosis was observed in the thoracolumbar region as mentioned above. The patient appeared to have a short, stocky neck with some degree of webbing and a low hairline.
Questions
1. Considering this patient’s initial history and examination five years prior to consultation, how precisely can the neurologic lesion be localized at that time? If precise localization is not possible, list as many levels and/or structures as you can that might produce the same clinical picture.
2. What is the localizing significance of the findings on the examination of the motor system and the reflexes?
3. What is the anatomical basis of the "suspended sensory level" involving pain sensation?
4. Indicate the level of the neurologic lesion in this case and the structures involved by the pathologic process. Is a single restricted level or segment of the nervous system involved or does the pathologic process span a few segments? Indicate the side(s) of the lesion and its extent. A diagram may be helpful in this instance.
Signs or Symptoms Site of Lesion
Initial decreased pain, RUE
Weakness, atrophy, decreased
DTRs, RUE>LUE
Weakness, increased tone,
increased DTRs, extensor R
plantar reflex, RLE>LLE
Suspended pain deficit, BUE
5. In general, what type of pathologic process do you think is involved in this case?
6. What diagnostic procedure(s) would you undertake at this point?