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