Lhermitte-Duclos Disease
An Issue Facing Some Who Have Multiple Hamaratoma Syndrome, as Known as Cowden's Disease
Signs of a Lhermitte-Duclos brain tumor are: headache, movement disorders, tremor, visual disturbances, an abnormal EEG, and diplopia (double vision). These signs are often found with other types of brain tumors. Because Multiple Hamaratoma Syndrome has been known to cause vascular abnormalities allowing for these same symptoms, it is important to differentiate between the two. Lhermitte-Duclos Disease may be missed on a ct-scan. MRI's are needed for definitive purposes and even then the MRI needs to scan the cerebellum very carefully.
Lhermitte -Duclos Disease is a rare entity among those who have Multiple Hamaratoma Syndrome. The brain tumor consistent with this disease is composed of both malformation and benign neoplasm. The Lhermitte-Duclos brain tumor involved in this disease usually has a "tiger-striped" appearance on MRI. Because Lhermitte-Duclos brain tumors tend to grow slowly, but do grow, they often require surgery. The risk of recurrence of these brain tumors is very high as is the risk of hamaratomas occurring in other parts of the body for patients who have Multiple Hamaratoma Syndrome.
The presence of Lhermitte-Duclos disease is a direct indicator the person has Multiple Hamaratoma Syndrome. However, some cases of Lhermitte -Duclos Disease have existed in patients who do not have any additional signs of the genetic condition. Prior to 1997 when the test for the gene mutation involved in Multiple Hamaratoma Syndrome became available, Lhermitte-Duclos was one of the ways Multiple Hamaratoma Syndrome was diagnosed. There are other signs of Multiple Hamaratoma Syndrome that, combined with Lhermitte Duclos Disease, allowed for diagnosis prior to 1997. Now, 80 percent of those who have Multiple Hamaratoma Syndrome will show the PTEN mutation on a blood test.
Lhermitte-Duclos Disease is most common when someone who has Multiple Hamaratoma Syndrome is in his or her 30's and 40's.
Email correspondence with Dr. Charis Eng who mapped the PTEN mutation and helped develop the test from 1995-1997.
Published by Andrea Rowe
Born in NE Arkansas six miles from where my dad s family lived as long ago as 1820. College grad in psychology field. My children and I have a very rare genetic disease that seriously impacts our lives. I... View profile
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- Most people who have Cowden Syndrome or MHS will not have Lhermitte-Duclos though some will.
- It's important to rule out a regular brain tumor or vascular abnormality before diagnosis.
- Lhermitte-Duclos brain tumors can sometimes be missed unless screening is very focused.