Berkowitz’s – Section X: Neurologic Disorders

Hypotonia

Kenneth R. Huff, MD
CASE STUDY
A 6-month-old girl is brought to the office because she does not reach for her toys anymore. The pregnancy was full term, but the mother remembers that the fetal kicking was less than with an older brother. Delivery was uncomplicated, and the newborn fed well from birth. The girl began to show visual attention at 2 to 3 weeks, smiled socially at 1 month, and pushed up while prone at 2 months. Although she turned over at 4 months, she has not done this in the last month. She no longer reaches up to the mobile over her crib. On examination, the girl lies quietly on the table and watches the examiner intently. Her growth parameters, including head circumference, are normal. After she has been undressed, it is apparent that she “seesaw” breathes (ie, abdomen rises with inspiration) and has a “frog-leg” posture (batrachian position). Her cranial nerve examination is normal except for head-turning strength. When she is pulled to a sitting position, her head lags far behind and her arms are straight at the elbows. She cannot raise her arms off the table. When a rattle is placed in her hands, she manipulates the toy, which she regards from the corner of her eye. Her deep tendon reflexes are absent, but her pain sensation is intact.
Questions
1. How is the level of nervous system involvement determined in infants with hypotonia?
2. What is the significance of a loss of developmental milestones or abilities?
3. When are genetic tests appropriate for children with hypotonia?
4. How are clinical management issues related to prognosis?

Headaches

Kenneth R. Huff, MD
CASE STUDY
A 12-year-old girl is brought in with a history of headaches. Although she has been sent home from school twice in the last 6 weeks, she has experienced headaches for at least a year. The last episode, 1 week ago, was typical. The headache began as a dull feeling over both eyes, radiated up to the top of her head, and eventually became pounding. She had no preliminary visual symptoms or other warning signs prior to the head pain. The episode began during an afternoon class after she had been outside on a hot, sunny day for physical education. The headache worsened after she walked home from school. Once she got home, she went to her room, drew the curtains, and lay down on her bed. She experienced some nausea and loss of appetite but no vomiting. She did not get up for dinner. She denied diplopia, vertigo, ataxia, or limb weakness, and her speech was observed to be articulate and coherent. She took two 80-mg children’s acetaminophen tablets without significant relief but eventually fell asleep. The following morning she felt fine. Between headaches, her behavior has not changed, and she has continued to make above-average grades.
She has not experienced any major changes in her home environment. When initially questioned, her mother denied having migraines, but she admits to needing to lie down because of headaches about once a month. A detailed neurologic examination of the girl is completely normal.
Questions
1. What are the major classes of headache?
2. How do the symptoms help differentiate the classes?
3. How does family history influence the etiology and help in management of the headache?
4. What is the appropriate treatment for the problem headache?

Tics

Kenneth R. Huff, MD
CASE STUDY
An 8-year-old boy has unusual recurring behaviors that began 2 to 3 months ago. He stretches his neck or raises his eyebrows suddenly several times a day. Sometimes he is able to suppress these actions. The boy’s parents report that in the last 2 years he has displayed several repetitive behaviors, including blinking, grimacing, rubbing his chin on his left shoulder, making a “gulping” sound, and sniffing. Originally they thought the sniffing was related to hay fever, but the boy has no other allergic symptoms. He does not use profane words. In conversations, he sometimes repeats the last phrase of a sentence that was just uttered by himself or someone else. In addition, he must touch each light switch in the hallway every time he leaves his room, and he must retie his shoelaces several times until they are exactly the same length. Although his schoolwork has not deteriorated, he has always had trouble completing tasks and finishing homework. His teacher and his best friend have asked about his strange behavior. His mother has a “psychological” problem with her son’s gulping sounds (ie, they recur in her own mind), and she recalls that her father had a habit of frequently looking over one shoulder for no apparent reason. Although during examination the boy does not exhibit any unusual behaviors, he raises his eyebrows twice and places his hand over his crotch several times while his parents are interviewed. Except for mild fine motor incoordination, the neurologic examination is normal.
Questions
1. What are the characteristics of tics?
2. What are the social implications of tics?
3. Should pharmacologic treatments be part of the management of tic disorders?
4. What other problems are associated with the tic disorder that also should receive intervention?