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Tourette Syndrome
Introduction
-Gilles de la Tourette syndrome is a chronic neuropsychiatric disorder characterized by multiple motor and phonic tics.
-Tics are sudden, involuntary, rapid, uncontrollable, repetitive, nonrhythmic, stereotyped movements with no purpose.
-Symptoms begin before 21 years of age, most often by the age of 11
-the course is one of remission and relapse.
-the disorder is more common in males than females
Symptoms & Signs
Tics typically start early, at 3–5 years of age, and peak around 9–12 years
Phonic Tics: Throat-clearing,gruting, barking, sniffing, hissing
Coprolalia: Vulgar or obscene speech
Echolalia: Parroting the speech of others
Echopraxia: Imitation of others’ movements
Palilalia: Repetition of words or phrases
Motor tics: Eye Blinking, facial grimacing, sniffing, hopping, jumping, and kicking, body gyrations, complex obscene gestures, neck jerking, shoulder shrugging
Sensory tics: Tics consisting of pressure, tickling, and warm or cold sensations
Behavioral disorders: Anxiety, obsessive-compulsive disorder, attention deficit disorder, depression
Diagnosis
Diagnosis is made based on history and physical examination
Laboratory tests are normal
Treatment
Behavioral therapy: Cognitive behavioral therapy, Habit reversal training
Pharmacotherapy:
α-Adrenergic agonists: the first-line therapies
Clonidine: Most frequent side-effects are sedation, orthostatic hypotension, constipation
Guanfacine
Antipsychotics: Risperidone, Aripiprazole,olanzapine, ziprasidone, Haloperidol, Pimozide
Botulinum toxin A injections
Tetrabenazine
Prognosis
-the disorder is chronic, with waxing and waning
-Majority will experience significant improvement by the end of adolescence (80%).
Restless Legs Syndrome
Introduction
-Restless legs syndrome is a chronic, neurological movement disorder characterized by a feeling of needing to move the legs (dysesthesia), associated with abnormal sensations like unpleasant creeping, crawling, tingling, and itching.
-Cause is unknown in most cases.
-Known causes of RLS: iron-deficiency anemia, renal failure, alcohol before sleep, thyroid disease, pregnancy, and certain medications
-Druginduced RLS
Anti-nausea drugs: Metoclopramide, Prochlorperazine
Anticonvulsants: Phenytoin, Droperidol
Antipsychotic drugs: Haloperidol
Antidepressants: Sertraline
-The disorder seems especially common among pregnant women (1 in 5)
Symptoms & Signs
-Irresistible urge to move the limbs with creeping or crawling sensations
-Symptoms usually occur when patient is lying down or relaxing
-Abnormal sensations are temporarily alleviated by movement, stretching, or massage
-Symptoms are worse in the evening and first half of night
Diagnosis
Diagnosis is usually made on clinical grounds alone
Search for iron deficiency: Ferritin levels should always be measured
Treatment
Non-Pharmacotherapy
– Stop aggravating medications, smoking, excessive alcohol consumption, coffee
-Daily moderate exercise, massage
-cognitive behavioral therapy
Pharmacotherapy
-RLS is treated by addressing the underlying cause
-Oral iron sulfate in patients with levels ≤ 75 mcg/L (13.4 mcmol/L)
-Other medications: Pramipexole, ropinirole, rotigotine, gabapentin, Pregabalin, Levodopa, clonazepam, temazepam, opiates
Dopaminergic therapy is the treatment of choice for severe cases but carries the risk of augmentation
Augmentation: the earlier onset or enhancement of symptoms; earlier onset of symptoms at rest; and a briefer response to medication.
RLS vs PLMS (Periodic Limb Movements of Sleep):
-RLS tends to occur during waking and at sleep onset, whereas PLMs occur during sleep.
-Patients with RLS sometimes also have PLMs, but patients with PLMs often do not have RLS.
Prognosis
Prognosis depends on the underlying cause
Q.Most common treatable cause of RLS: Iron deficiency
Q.What is the most common complication of long-term dopaminergic therapy? Augmentation