MJD and the Sephardic Connection

by Sheri Rogers Bashor

Personal Thoughts

A question that continues to arise in my mind is the origin of MJD (Machado Joseph Disease). A disease that once was thought to be contained in my own family and thought of as our "family secret" is now known as a "common ataxia" through out the world.

In twenty-five short years a disease that was hidden for over "500 or more years" has now sparked the interest of medical professionals world wide.

I am not, nor do I claim to be a medical professional but I would like to present some facts that I believe have a definite connection between a genetic disease (which has been in my family for centuries) and many Sephardic Jews.

Machado Joseph Disease (MJD) is an autosomal dominant neurodegenerative disorder. In layman terms a genetic neurological disease or stated simply an inherited disease of the central nervous system. It was first thought that this disease originated in the Azores Islands. If your parent has or had the disease you have a 50/50 chance of getting the disease. This disease does not skip generations nor is it a respecter of persons.

This disease progresses year after year, the affected person becomes wheel chair bound, their body becomes stiff, they must depend on others for their primary care, and they are unable to communicate or be understood. The intellect is usually intact which means "one is trapped in a body that does not work; a virtual prison." My father had this disease (he died at 39 yrs. of age), as did his father, generation, after generation. My sister now has this disease.

As I was looking on the Internet for up-dates on MJD. I was amazed to see a connection materialize as to the origin of MJD.

I present to you the following information.

From a medical paper (OMIM ENTRY 109150) it was written: "The possibility that the Joseph family was originally Sephardic Jews" was raised by Sequeiros & Coutinho (1981) Mainland families originated in a mountainous and relatively inaccessible region of northeastern Portugal where large communities of Sephardic Jews settled at one time. The Joseph family was from this area.

Another medical paper "Origin of the Portuguese Mutation" written about MJD expounds on this. From unpublished data, from Sequeiros and Sa Miranda "there is some preliminary evidence that the frequency of the Tay-Sachs disease gene is increased among Portuguese patients with MJD and their relatives. Tay- Sachs has a high frequency particularly among Ashkenazi, and well as Sephardic Jews."

It has been reported that MJD is found in Portugal, with the largest of these families along the Douro River, as far north as Freixo-de-Espada-a-Cinta to the Atlantic coast in Porto. MJD has been found in families in Brazil, Angola, France, and Germany. One Brazilian patient claims to be of Jewish descent from Amsterdam (Coutinho, unpublished observation). Other reported families are from Japan, India, China, Australia, the Netherlands, and Israel!

I found a document written in 1994 with the caption "Machado-Joseph (Azorean) disease in a Yemenite Jewish family in Israel." Goldberg-Stern H, D'jaldetti R, Melamed E, Gadoth N ..."we report the first Israeli Jewish family with MJD, originating from a remote village in Yemen."

From OMIM ENTRY 109150 Goldberg-Stern (1994) ~ Machado Joseph disease in a Yemenite Jewish kindred that originated from a remote village named Ta'izz. This family, incidentally named Yoseph, had no documentation of Portuguese ancestry. Portuguese trade connections with the Yemenites most likely did not reach Ta'izz which is far from the coast and is almost inaccessible because of a wall of high mountains.

The Sackler Faculty of Medicine in Tel Aviv, Israel reports a RESEARCH PROJECT titled "The Israeli Pocket of Machado-Joseph Disease." Natan Gadoth, Professor.

I found medical research articles written about MJD in 1996 and 1997 from the Department of Human Genetics, Hadassah Medical Center, Hebrew University Medical School, Jerusalem, Israel.

There is one hospital in Israel which provides care for patients with Machado-Joseph disease.

What does all this information mean? I believe there is a definite connection between MJD and Israel.

Why is this important? Over the past year I have discovered a group of people known as the 'anusim', Conversos, Marranos, 'New Christians'. These people were Jewish and lived in Iberia (Spain & Portugal) over 500 years ago. Their descendents are scattered throughout the world. These people were and still are a stigmatized people.

I believe MJD will be shown to be a genetic marker for these people and be used to give credence to their true heritage.