Psychology of Tics and Tourette Syndrome

Tics and Tourette Syndrome

• Tics are stereotyped motor behaviors that are unlike stereotypies, as they are less predictable and often last only a few seconds or less.
• Tics can occur repeatedly, in clusters, or in longer patterns and tend to "wax and wane" and change over time.
• Tics are more common under circumstances of stress, anger, excitement, or anxiety.

Tourette Syndrome Criteria

• To meet the criteria for Tourette syndrome, an individual must have both vocal and motor tics, which are complex and persistent over time.
• The tics must last for at least 1 year, and the individual must not have any other underlying conditions that could be causing the tics.

Therapy for Tourette Syndrome

• The first-line therapy for Tourette syndrome when tics are the main problem includes clonidine, followed by haloperidol and pimozide if clonidine is ineffective or not well-tolerated.

Hemiplegic Cerebral Palsy

• In hemiplegic cerebral palsy, an individual may exhibit tiptoe walking, muscle hypotonia, ankle clonus and Babinski sign, and an increase in deep tendon reflexes.
• Weakness of the hand and foot dorsiflexors and equinovarus deformity of the foot may also be present.

Treatment of Spasticity

• Several drugs can be used to treat spasticity, including oral diazepam, baclofen, and dantrolene.
• Levodopa is not typically used to treat spasticity.

ADHD

• An individual with ADHD, predominantly of the hyperactivity-impulsivity type, has a hard time "putting on the brakes" before acting.
• Children with ADHD often have a smaller frontal lobe cortex.

Autism

• Autism is usually diagnosed by 18 months to 2-2.5 years of age.
• Signs of autism include:
  • Not reacting or responding when the name is called at 2 years old.
  • Not seeming interested in the reactions of others, especially parents.
  • Not seeming aware of the feelings of others.

Causes of Autism

• There is no known single cause of autism, and it is not caused by cold parenting or poor prenatal nutrition.

Neuromuscular Disorders

• Spastic paralysis is characterized by an increase in deep tendon reflexes and muscle tone.
• Flaccid paralysis is characterized by a decrease in deep tendon reflexes and muscle tone.
• Myasthenia gravis is characterized by rapid fatigue of muscles, with progressive worsening over the day or with repetitive activity.

Muscular Dystrophy

• Duchenne muscular dystrophy is an X-linked disorder characterized by a waddling gait and progressive muscle weakness.
• On EMG, myotonic phenomena (myotonic discharges with specific sounds) and a decrease in motor action amplitude can be seen.

Epilepsy

• Absence seizures are characterized by a sudden loss of awareness, accompanied by a blank stare and cessation of any ongoing activity.
• Generalized 3-Hz spike-and-wave activity is typically seen on EEG during absence seizures.
• Valproic acid is the first-choice therapy for childhood absence epilepsy.

Benign Rolandic Epilepsy

• Affected children usually have focal motor seizures involving the face and arm, with abnormal movement or sensation around the face and mouth, drooling, and rhythmic guttural sound.

Infantile Spasms

• Infantile spasms are characterized by brief contractions of the neck, trunk, and arm muscles, followed by a phase of sustained muscle contraction lasting less than 2 seconds.
• Hypsarrhythmia is a characteristic EEG pattern seen in infantile spasms, consisting of chaotic high-voltage slow waves, spikes, and multifocal spikes.

Idiopathic Intracranial Hypertension

• Patients with idiopathic intracranial hypertension (pseudotumor cerebri) exhibit daily debilitating headaches, diplopia, abducens palsy, transient visual obscurations, and papilledema.

Muscle Disorders

• Juvenile myoclonic epilepsy is a type of epilepsy characterized by muscle jerks, especially in the early morning or with sleep deprivation.

Neurocutaneous Syndromes

• Neurofibromatosis type 1 is characterized by six or more café-au-lait spots larger than 5 mm, Lisch nodules, neurofibromas, and optic gliomas.
• Neurofibromatosis type 2 predisposes patients to multiple intracranial and spinal tumors, including bilateral acoustic schwannomas, schwannomas of other cranial and spinal nerves, meningiomas, and gliomas.

Tuberous Sclerosis

• Tuberous sclerosis skin lesions include hypomelanotic macules (ash leaf spots), facial angiofibromas (adenoma sebaceum), and shagreen patches (elevated, rough plaques).

Sturge-Weber Syndrome

• Sturge-Weber syndrome is associated with an ipsilateral facial port-wine stain.

Leukodystrophy

• Metachromatic leukodystrophy (MLD) is characterized by periventricular deep white matter signal change, leading to a "butterfly pattern" on MRI.

Meningocele and Dandy-Walker Malformation

• In a meningocele, the skin of the back is apparently intact, but defects of the underlying bone or spinal canal are present.
• Dandy-Walker malformation is diagnosed on the basis of complete or partial agenesis of the cerebellar vermis, cystic dilation of the fourth ventricle, and an enlarged posterior fossa.

Macrocrania and Lissencephaly

• Macrocrania is characterized by enlargement of the brain, and may be associated with an increase in skull thickness.
• Lissencephaly represents a severe defect in cortical migration, resulting in a smooth brain without sulcation (agyria).