Dr. Hanna Saadah - ‘T he Shakes’ are common, cumbersome, life-altering, and at times life-threatening.  Diagnosing them is important and can usually be made by simple observation without the need for tests or formal examinations.  They are mainly caused by degenerations of the Basal Ganglia, the parts of our brains that are responsible for the grace and music of motion and without which we would move like robots.  The primary Basal Ganglia hormone is Dopamine and medicines that enhance Dopamine help most of these disorders. The following six types are important because they are common.

Familial Essential Tremor slowly grows on us as we age and is the most common.  It is an inherited, fine action tremor, which means that our hands shake when they are trying to perform fine motor skills such as writing, holding a fork, threading a needle, or buttoning a shirt but not when we are waving our arms, driving, or shaking hands.  The tremor, which may also cause the head to shake and the voice to rattle, disappears when we are at rest i.e. not moving or not sustaining a certain position or while sleeping.  The condition advances very slowly as we get older but seldom leads to important disability.  It responds to medications that block the effects of Adrenaline such as Beta Blockers or to medicines that diminish Adrenaline such as Barbiturates, but treatments do not halt disease progression and often lose their effects as the disease worsens.

Parkinson’s Disease is a more devastating movement disorder where the tremor is only part of the syndrome.  This disease attacks the elderly and its tremor, unlike the Familial Essential Tremor, is a resting tremor that gets better with motion.  Initially, the hands tend to shake only when they sit in our laps but later they begin to shake even with motion.  All motions are slowed and become uncertain with time.  The faces lose their expressiveness, the gaits become stooped, and walking occurs with smaller steps.  Progression hastens over a few years, rendering the patient stiff, drooling, immobile, and demented.  Treatment is effective during the initial years but becomes less and less effective as the Dopamine cells of the Basal Ganglia die out.  Medicines that replenish Basal Ganglia Dopamine are the main treatments.  The disease shortens life and ruins its quality.  It totally disappears during sleep.

Restless Leg Syndrome is a more benign disorder that begins at a younger age, may slowly progress with time, but seldom leads to severe disability.  When one tries to rest or sleep, one is seized with an overwhelming urge to move the legs.  Kicking and walking appease that urge but not for long; the urge returns over and over making it harder and harder to fall asleep.  Once one falls asleep, however, the urge to move disappears only to return when one wakes up.  Luckily, the disorder responds well to many treatments and seldom progresses beyond medical control.  However, when it does progress, it may involve the entire body and become resistant to all treatments leading to sleep deprivation with attendant exhaustion, depression, and despair.

Periodic Leg Movements of Sleep—in contrast to the Restless Leg Syndrome, which is only felt when one is awake—is not felt because it only occurs during sleep.  It can affect all ages and one may never know that one has it unless one undergoes a sleep study.  Its symptoms derive from poor quality sleep because, by frequently moving the legs, one does not reach the deeper, restitutive layers of sleep.  This causes excessive morning fatigue and excessive daytime sleepiness.  One wakes up feeling that one has not had enough sleep, yawns often, and catnaps whenever situations allow.  Once diagnosed, it is treatable with medicines that enhance Dopamine and some others that promote deeper sleep.  The two conditions, Restless Leg Syndrome and Periodic Leg Movements of Sleep frequently coexist and are made worse by low iron levels.

Facial Tics present with repetitive, uncontrolled spasms of the face muscles that control the eyelids, mouth, and expressions.  They can affect any age, increase with stress, and disappear with sleep.  Treatments are numerous but Botox is by far the most effective.  Repeated injections every few months can keep the disorder under control.

Spasmodic Torticollis involves spasms of the neck muscles mainly, which cause the head to point up and out.  It may affect any age, disappears during sleep, and is also best treated with Botox.

Other movement disorders are not common enough to dwell upon.  But, as a rule, most of them have to do with diseases of Dopamine transmission and Basal Ganglia degeneration.  They tend to disappear during sleep and reappear during wakefulness, are usually progressive and life altering, and their treatments provide less and less relief with time.

 Issue # 107 - June 2011