Perspectives - Vol. 5, No. 3 - Dead Arm de Jour
Brian C. sat nestled among the toys on the floor of my clinic while I took his history. According to his dad and step mom, Brian's biological mother was in and out of jail the first year and a half of his life and her mother had most of the responsibility for taking care of him. From what I could gather, Brian had spent much of his early life in a baby bed, watching his grandmother take care of a quadriplegic uncle. Now dad had custody.
Something tells me lawyers are going to get involved again in this case I whispered to myself as I sat down on the floor in front of Brian. "Give me five," I said, holding out my hand to the two-year old. He was unusually small for his age, but he reached out with his left hand and whap, gave me five.
"Give me five with your other hand," I instructed. Brian reached across his body with his left hand, grabbed his right arm, which was hanging limp from the shoulder, and brought it around to my waiting palm. There was no whap as he smiled and laid his lifeless right hand in mine. Left hemiplegia (paralysis) I wrote in my notes.
The following week I sat down on the floor in front of Brian again, held out my hand, and asked him to give me five. Whap with his right hand this time, and then he reached across his body grabbed his now lifeless left arm and with a smile laid his left hand in mine. Remarkable. Last week left hemiplegia and now right. I'd heard of alternating hemiplegia, but I'd never actually seen a case. And Brian's neurologist had told me this was the first case he'd diagnosed. Neuroanatomy 101.
The brain consists of a right and left hemisphere, each the mirror image of the other. Many of the nerve fibers, which control movement, cross over to the opposite side of the body at the medulla in the lower brain stem. Hemiplegia is defined by a loss of voluntary motor control on one side of the body contralateral (opposite) to the damaged side of the brain. Cerebral palsy, strokes, tumors, and head injuries are just a few of the causes of hemiplegia.
If a clinician sees paralysis on one side of the body he immediately knows the culprit is in the opposite hemisphere. Alternating hemiplegia--hemiplegia that can alternate from arm to arm sometimes in a single day seems to defy basic neuroanatomy.
Brian and his family arrived early for their next appointment. I watched them interact with each other while I finished my notes. Spring had come and dad had worn a short sleeve shirt. I had not noticed the week before but his right arm was paralyzed and hung limp from his shoulder just like Brian's.
I didn't want to embarrass dad, but the clinical relevance of his paralyzed arm was too much. "What happened to your arm?" I asked. "And when?"
"Motorcycle accident," he replied. "Before Brian was born."
I noticed that Brian handled his "paralyzed" arm exactly like dad. And when he saw his stepmother massaging dad's paralyzed arm, trying to relieve his chronic pain, he ran over and held up his arm for a massage.
Could Brian's "paralysis", his mimicking of a rare neurological disease, be simple imitation learning? And if it was copycat behavior why the alternation from arm to arm? Was he simply forgetting which arm was supposed to be "paralyzed"?
Most children are well into the monkey see monkey do stage by two. But this alternating hemiplegia of Brian's I wasn't so sure about.
That evening I went on-line and logged into the RDB (Rare Disease Database). The RDB is just what it says it is. Every disease you've never heard of with a short description, stats, etiology, and treatments. Often there is an address for a foundation supporting research in the causes and cures, and a support group.
According to the RDB, only 250 children worldwide are affected by alternating hemiplegia. (What are the odds of a case this rare popping up in my clinic I asked myself). The only minimally effective treatment is a calcium channel blocker called Flunarizine which the FDA has not approved.
Brian's medical records arrived several days later and I read through them carefully. Hospital stays, every test in the book, med trials, nurses' notes, different specialists called in to consult, different opinions and diagnoses--I got the impression no one really knew what was going on with this kid--including his neurologist.
"I would like for you to start keeping a detailed diary," I said to the parents at their next appointment. "Every time Brian is paralyzed tell me which arm and how long the paralysis last. Tell me what happened before the paralysis began and how you treat him when he's paralyzed."
At their next appointment I quickly read through step mom's notes for the week. Her notes were detailed and a pattern seemed clear. Separation--even the possibility of separation for a brief period of time from step mom always occurred before each episode of paralysis. And Brian always received a great deal of tender loving care from both parents whenever one of his arms was paralyzed.
I decided to set up a simple behavioral program to see if I could decrease the frequency of Brian's paralysis. "I want you to make some changes in the way you are handling Brian when one of his arms is paralyzed," I announced.
The parents nodded their heads. "We'll try anything."
"Whenever Brian is using both arms I want you to lavish him with attention, especially physical contact," I said slowly. And when one of his arms is paralyzed I want you to put him in time out."
They looked at each other. "Time out?"
"That's right," I confirmed. "His baby bed will do for now. He stays in time out until he starts using both arms. I don't think this is alternating hemiplegia. Brian has learned to mimic dad's paralysis to get attention. Does he like M&M's?"
"He's crazy about any kind of chocolate," step mom answered, still unsure of what I was suggesting.
I sat down on the floor and asked Brian to give me five. Whap with the right hand. No whap with the left. Again he smiled as he laid his lifeless left hand in mine.
"Our little games is about to change," I told the parents as I reached into my shirt pocket and took out a bag of M&M's. I held out my hand and let Brian pick up several M&M's with his right hand. He smiled as he put the candy in his mouth. Then I grabbed his right hand and held onto it. He would have to use his left hand if he wanted any more M&M's.
Brian whined and struggled briefly before he reached out with his left hand and picked up an M&M. His movements were jerky and uncoordinated (hysterical ataxia?) and he dropped the M&M several times before he finally got the candy to his mouth.
Step mom and dad didn't know what to say. But I had learned something. M&M's don't cure neurological disorders. Now I could treat this "paralysis" behaviorally.
Dad and step mom's faces alternated between glad and mad. "So he's faking?" dad blurted out. He picked up his paralyzed arm and laid it in his lap.
"I wouldn't call it faking," I said, noticing that Brian had just picked up his left arm, and mimicking dad, laid it in his lap. "It's more like imitation learning. By observing his paralyzed uncle and you, he's learned that paralysis is the best way to get attention and tender loving care.
The following week the parents walked in with smiles on their faces. "Time out worked," dad boasted. "We put him in several times the first day we started the program and after ten minutes or so he started using both arms."
"Last Wednesday I went to the store," step mom read from her diary. "Brian stopped using his left arm when I left. And he did the same thing on Thursday and Friday."
"I put him in time out," dad added. "And it wasn't long until he started using both his arms."
Mom got out of jail a year early and the judge said we had to take Brian to her for the weekend," dad complained the next week as he sat down. "When he came back he didn't use his right arm for three days. Time out doesn't work anymore."
Step mom handed me her diary and I read through it. "Can we hold off on these visitations for awhile?"
"The judge said he had to go," dad grumbled.
Several months passed and Brian's episodes of paralysis became very predictable. He'd go for days using both arms. Then he'd go for visitation and come home with one of his arms paralyzed. The parents would take him to time out for his paralysis, praise him when he was not paralyzed, and gradually, he'd start using both arms. Then after a few days it would be time for another visitation and the cycle would begin all over again.
I tried variations in Brian's behavioral program but nothing worked. In fact, it got worse. Biological mom and her family were all hypochondriacs, constantly complaining about their health problems. Brian homed in on their "sick" behavior and before long he started getting "sick" after visitations. He'd limp around the house, moaning and groaning about his arthritis and every other in the book ailment in the book, including menstrual cramps.
I'd had enough. I talked to the social worker who was handling Brian's case and a lawyer for Human Services. We decided to ask the court to terminate biological mom's visitation rights.
Biological mom showed up for the hearing and asked the judge for a postponement until she could afford a lawyer to represent her. The judge agreed to the postponement, but he stopped her visitation rights until she paid her past due child support payments.
Brian has not had to go to his biological mom's house for visitation for six months and he's doing great. He's attending preschool everyday, he's gaining weight, and his "paralysis has disappeared.
Biological mom finally hired a lawyer, and then, the day before the case was to be heard, grandmother decided she'd apply for custody. There have been countless continuances and motions filed since then, and thankfully, the wheels of justice are turning slowly. The longer Brian's case remains tied up in court the better he'll get. And until then, I've vowed to stop telling lawyer jokes.
Brown, Gary (2000). Dead Arm de Jour. [Online]. Perspectives. [2000, July 1].