Chiari malformations are congenital brain-base abnormalities
DEAR DR. ROACH: My older daughter has had headaches most of her life. When she was young, she thought it was normal.
Severe migraines started around 1995. Many doctors later, they came up with diagnosis of a Chiari malformation. She was on oxycodone but now she is off addictive painkillers. She suffers for several days when there is a change in the weather.
When she gets the headaches, she usually stays in her bedroom and wears sunglasses when she has to take the dogs out. The last one, a few days ago, lasted about three days.
What options does she have to relieve these debilitating headaches? — Anon.
ANSWER: Chiari malformations are congenital (she was born with it) abnormalities of the base of the brain, including the brainstem and cerebellum. They are more prevalent than previously believed, and with more people getting MRI scans, it is thought to be present in 0.1 to 0.5 per cent of people. There are three classic types of Chiari malformations, but the most likely in your daughter’s case is Chiari malformation Type I, as the other two are rarer and are diagnosed at an early age. CM-1 normally is diagnosed in adolescence or young adulthood.
The symptoms of CM-1 are variable. Some people have no symptoms, but neck pain, headache and damage to various nerves are common.
It sounds to me as though her headaches remain a significant burden on her quality of life. I cannot say whether the Chiari malformation is causing the headaches. Worsening of headache with coughing or sneezing tends to support the CM as the cause. A neurosurgeon with experience in treating Chiari malformations would be worth consulting, and repeat imaging may be necessary, as the condition can worsen or, rarely, improve over time.
If surgery isn’t appropriate, I would recommend a pain-management specialist to help with the headaches. Given her history, opiate drugs are a bad choice. There are many different kinds of options.
I found much information about the disease and support groups at asap.org.