Nieman: Tourettes a complex disorder with a surprising history

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Family & Child

Published Aug 26, 2023 • Last updated 21 minutes ago • 3 minute read

brain scan — A small number of people with Tourettes have mutations involving the SLITRK1 gene which affects how neurons grow and connect with each other. Other genes which regulate normal formations of nerve connections also may play a role. Photo by utah778 /Getty Images/iStockphoto

German philosopher Georg Hegel (1870-1831) famously said, “What experience and history teaches us is that people and governments have never learned anything from history, or acted on principles deduced from it.”

But sometimes we do learn much from history. Consider this story:

There is a doctor in France in the 19th century. He works at a famous hospital named Salpetriere. His name is Jean-Marin Charcot. Today, we consider Charcot to be the father of modern neurology. Charcot is a researcher, teacher and mentor to many. One of his students is named Sigmund Freud. Another student mentored by Charcot publishes a paper in the medical journal Archives de Neurologie in January 1885. This 28-year-old student describes a condition where patients are noted to have involuntary spasms of the head and shoulders, and some utter obscenities – uncontrollably.

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At the time, there is no treatment for these patients and many of them are children. Experts think the reason for this bizarre illness is due to a weakened neurological system caused by immoral behaviours of previous generations.

In 1893, the student, who became a doctor in 1886, suffers greatly: his son and his mentor die in the same year. In addition, at the age of 36, he gets shot in the head by a psychotic patient who claims she had been hypnotized at the Salpetriere hospital and is handicapped and unable to make a living. The doctor survives and becomes world famous.

His name? Georges Albert Edouard Brutus de la Tourette.

Today, when pediatricians diagnose Tourette Syndrome (TS), many may not know its fascinating history. We do not believe it is degenerative and we do not believe that immoral behaviours of previous generations caused it.

Tourettees often presents in childhood around the ages of five and 10. It is marked by sudden, unwanted and uncontrolled, rapid and repeated movements or vocal sounds called tics. These events come and go over time. It affects boys more than girls. Many TS patients get worse in their early teens and then find that things get better in their early 20s. Rarely does TS get worse in adulthood.

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Simple motor tics in TS are marked by eye blinking, facial grimacing, shoulder shrugging and head or shoulder jerking. Simple vocal tics include repetitive throat clearing, sniffing, barking and grunting.

Complex motor tics include hopping, jumping, bending and twisting. Complex vocal tics include repeating one’s own words or phrases, repeating the words and phrases of others (echolalia) and also coprolalia (utterances of involuntary obscenities).

The diagnosis is mostly made via a thorough neurological history and often Tourettes may be associated with other conditions such as ADHD, OCD, anxiety, learning disabilities, sleep disorders and sensory processing issues.

There may be a genetic component at play: a small number of people with Tourettes have mutations involving the SLITRK1 gene which affects how neurons grow and connect with each other. Other genes which regulate normal formations of nerve connections also may play a role.

The treatment involves medications which block dopamine, a neurotransmitter. Haloperidol or Pimozide falls into this class of medications. Alpha-adrenergic agonists are also used: Clonidine and Guanfacine are examples. In patients with OCD and depression, selective serotonin reuptake inhibitors (SSRIs) are used.

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The problem is that many of these medications cause side effects to the point that a great number of patients refuse to continue to take them.

Awareness training, mindfulness-based stress reduction, CBT, and other forms of psychotherapy are non-pharmaceutical options but, as we know, in Canada, we have a two-tier system of care for mental health in that not all patients can pay for non-pharmaceutical modalities.

Knowing how frustrating it can be to treat these patients, it came as no surprise when I recently received mail from researchers at the University of Calgary. They are looking for patients to enter into a trial where a newer medication, Ecopipam, is being studied. For anyone interested in this trial, the study co-ordinator, Tracy Hammer, can be reached at tahammer@ucalgary.ca

One last thing about Giles de la Tourette. Ironically, he developed severe mental issues and was asked to leave the hospital which made him famous. He died in a facility for mental illness in Switzerland in 1904. And as American broadcaster Paul Harvey used to say, “Now we know the rest of the story.”

Dr. Peter Nieman is a community-based pediatrician, a marathon runner and author of three books. For more information, visit drnieman.com

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