Jewish movement disorders and genetics

Posted By on October 21, 2014

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As I was sitting (and standing) in a synagogue over the holidays I let my mind wander, as I often do under similar circumstances, and tried to answer the eternal question: If God designated the Jews as the Chosen People why did he/she also referred to them as the stiff-necked people? (Exodus 32:9: I have seen this people, and behold, it is a stiff-necked people). Was God making an analogy between hard-to-control oxens and the stubborn and obstinate Isrealites who used them to plow the fields? While this is one explanation offered by Jewish scholars for the term stiff-necked people, as an academic neurologist, specializing in movement disorders, and someone who likes to challenge an established dogma, I raise the possibility that the stiff-necked Jews had a neurologic condition that caused neck spasms and/or neck stiffness. After all, it is well-accepted that Moses had a neurological condition that apparently caused a speech impediment (stuttering). Indeed, it is also well known that over the centuries and possibly millennia, Jews have had an increased risk for a variety of neurologic conditions, called movement disorders. This important association of neurologic movement disorders in people of Jewish ancestry has been recently described in a scientific article in JAMA Neurol (1), but I thought it would be important bring this to the attention of readers of this journal.

Recent analysis of DNA sequences shared by Ashkenazi Jewish (AJ) individuals has provided insight into early Ashkenazi history. This research suggests that the world AJ population shrunk to only 350 as recently as 700 years ago (bottleneck) and that subsequent AJ generations, now totaling in millions, were a mixture of European and Middle Eastern ancestry (2). Because of intermarriages various genetic metabolic and neurologic diseases, such as Tay-Sachs, Niemann-Pick disease, mucolipidosis type IV, and Gaucher disease, became more common in the AJ population. In this review we wish to focus on neurologic diseases,categorized as movement disorders, that are being increasingly recognized to be relatively more frequent in people of Jewish ancestry compared to general population.

What are movement disorders? Movement disorders is a group of neurologic conditions that can be divided into slow movements (hypokinetic disorders) or abnormal involuntary movements (hyperkinesias). The best example of a hypokinetic movement disorder is Parkinsons disease. Hyperkinetic movement disorders are subdivided into tremors, dystonia, tics, chorea, athetosis, ballism, stereotypy, and akathisia. The latter term, akathisia, refers to motor restlessness, known in Yiddish as the shpilke. Restless legs syndrome, another movement disorder that could be described as the shpilke, refers to restlessness that occurs chiefly at night and predominantly involves the legs. Furthermore, incoordination, gait and balance disorders, and abnormalities in muscle tone (such as spasticity and rigidity) are also included among movement disorders (3,4). While the basal ganglia, the deep part of the brain that is involved in the fine controls of body movements, have been implicated in most of the movement disorders, there are many other parts of the central and peripheral nervous system that may be involved. Since the diagnosis of a movement disorder is based on accurate recognition of specific phenomenological features, clinicians who encounter patients with movement disorders must use their powers of observation to carefully characterize the disorder. Therefore, the phenomenological categorization of the movement disorder is absolutely critical in formulating the differential diagnosis, finding the cause, and selecting the most appropriate treatment (5).

The following is a brief summary of the most common movement disorders highlighting those that are particularly common in people of AJ ancestry.

Parkinsons Disease

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Jewish movement disorders and genetics

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